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1. Zhang ZL, Liang CH, Liu YB, Xie SF, Yu YX, Wang QS, Liu ZY, Li JL: [Computed tomography and magnetic resonance imaging features of desmoid-type fibromatosis: comparison with the pathological findings]. Nan Fang Yi Ke Da Xue Xue Bao; 2010 Nov;30(11):2495-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Computed tomography and magnetic resonance imaging features of desmoid-type fibromatosis: comparison with the pathological findings].
  • OBJECTIVE: To explore the computed tomography (CT) and magnetic resonance imaging (MRI) features of desmoid-type fibromatosis, and improve the diagnostic accuracy and understanding of the disease.
  • METHODS: The CT and MRI features of 18 cases of surgically and pathologically confirmed desmoid-type fibromatosis were reviewed retrospectively.
  • RESULTS: In the extra abdominal cases, the tumors occurred in the head and neck in 3, in the dorsal part of the chest in 2, in the abdominal wall and groin area in 9, and in the peritoneal cavity in 4; concomitant Gardner syndrome was found in 1 case.
  • In 4 cases the tumor occurred within 1 to 3 years after abdominal surgeries.
  • On CT and MRI, the lesion appeared benign with malignant growth pattern, and caused compression of the adjacent organs and vessels or encasement of the vessels; the border was unclear without encapsulation, and necrosis and calcification was scarce.
  • The density and signal of the tumor were well distributed.
  • CONCLUSION: The CT and MRI features of desmoid-type fibromatosis are characteristic, and CT and MRI are valuable modalities for the diagnosis and differential diagnosis of the tumor.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Fibromatosis, Aggressive / radiography

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  • (PMID = 21097415.001).
  • [ISSN] 1673-4254
  • [Journal-full-title] Nan fang yi ke da xue xue bao = Journal of Southern Medical University
  • [ISO-abbreviation] Nan Fang Yi Ke Da Xue Xue Bao
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] China
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2. Ioannou M, Demertzis N, Iakovidou I, Kottakis S: The role of imatinib mesylate in adjuvant therapy of extra-abdominal desmoid tumors. Anticancer Res; 2007 Mar-Apr;27(2):1143-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The role of imatinib mesylate in adjuvant therapy of extra-abdominal desmoid tumors.
  • BACKGROUND: Extra-abdominal desmoid tumors are rare neoplasms with variable biological behavior.
  • The purpose of this study was to determine the possible presence of tyrosine-kinase receptors in extra-abdominal desmoid tumors as a marker for imatinib mesylate therapy.
  • PATIENTS AND METHODS: From 1999 to 2004, immunohistochemical methods were carried-out in 14 patients with histologically confirmed extra-abdominal desmoid tumors to determine c-KIT positivity (existence of tyrosine-kinase receptors and PDGFRA and PDGFRB).
  • RESULTS: All desmoid tumors were c-KIT negative, which demonstrates absence of tyrosine-kinase receptors.
  • CONCLUSION: The histological c-KIT markup is an easy and reliable method that can detect whether a desmoid tumor is sensitive to additional treatment with a tyrosine-kinase receptor inhibitor.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Fibromatosis, Aggressive / drug therapy. Piperazines / therapeutic use. Pyrimidines / therapeutic use

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  • (PMID = 17465254.001).
  • [ISSN] 0250-7005
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzamides; 0 / Piperazines; 0 / Pyrimidines; 8A1O1M485B / Imatinib Mesylate; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit; EC 2.7.10.1 / Receptor, Platelet-Derived Growth Factor alpha; EC 2.7.10.1 / Receptor, Platelet-Derived Growth Factor beta
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3. de Camargo VP, Keohan ML, D'Adamo DR, Antonescu CR, Brennan MF, Singer S, Ahn LS, Maki RG: Clinical outcomes of systemic therapy for patients with deep fibromatosis (desmoid tumor). Cancer; 2010 May 1;116(9):2258-65
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Clinical outcomes of systemic therapy for patients with deep fibromatosis (desmoid tumor).
  • BACKGROUND: In the current study, the authors examined the outcomes of patients with desmoid tumors who received systemic therapy at a single institution to provide a basis for the examination of newer agents.
  • METHODS: Records of patients with desmoid tumors who were treated with chemotherapy at the study institution were reviewed.
  • Patients without measurable disease and those receiving therapy could not be documented, and those receiving prophylactic therapy were excluded.
  • At the time of last follow-up, 9 patients had died, 7 of progressive disease.
  • An intra-abdominal primary tumor location was the most common (44%).
  • The greatest Response Evaluation Criteria in Solid Tumors (RECIST) response rate was observed with anthracyclines and hormonal therapy and the lowest response was noted with single-agent dacarbazine/temozolomide or tyrosine kinase inhibitors, principally imatinib.
  • On multivariate analysis, macroscopic nodular morphology and the presence of Gardner syndrome were the only tumor factors found to be associated with a greater time to disease progression.
  • CONCLUSIONS: Compared with other agents, antiestrogens and anthracycline-containing regimens appear to be associated with a higher radiological response rate against desmoid tumors.
  • Systemic therapy can be successful in patients with desmoid tumors, and is a viable option in lieu of morbid or disabling surgery.

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  • [Copyright] (c) 2010 American Cancer Society.
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  • (PMID = 20187095.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / RC2 CA148260-01; United States / NCI NIH HHS / CA / P01 CA47179; United States / NCI NIH HHS / CA / P01 CA047179-15A2; United States / CCR NIH HHS / RC / CA148260-01; United States / NCI NIH HHS / CA / P01 CA047179
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Other-IDs] NLM/ NIHMS219553; NLM/ PMC2925106
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4. Papagelopoulos PJ, Mavrogenis AF, Mitsiokapa EA, Papaparaskeva KT, Galanis EC, Soucacos PN: Current trends in the management of extra-abdominal desmoid tumours. World J Surg Oncol; 2006;4:21

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Current trends in the management of extra-abdominal desmoid tumours.
  • Extra-abdominal desmoid tumours are slow-growing, histologically benign tumours of fibroblastic origin with variable biologic behaviour.
  • They are locally aggressive and invasive to surrounding anatomic structures.
  • Magnetic resonance imaging is the modality of choice for the diagnosis and the evaluation of the tumours.
  • Current management of desmoids involves a multidisciplinary approach.
  • Wide margin surgical resection remains the main treatment modality for local control of the tumour.
  • Chemotherapy may be used for recurrent or unresectable disease.
  • Overall local recurrence rates vary and depend on patient's age, tumour location and margins at resection.

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  • (PMID = 16584569.001).
  • [ISSN] 1477-7819
  • [Journal-full-title] World journal of surgical oncology
  • [ISO-abbreviation] World J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC1456964
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5. Yang JL, Wang J, Zhou XY, Li XQ, Hou YY, Zhu XZ: [Clinicopathologic and genetic studies of desmoid-type fibromatosis]. Zhonghua Bing Li Xue Za Zhi; 2006 Mar;35(3):145-50
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Clinicopathologic and genetic studies of desmoid-type fibromatosis].
  • OBJECTIVE: To study the clinicopathological and genetic features of desmoid-type fibromatosis, and to investigate the feasibility of detecting trisomy 8 in formalin fixed, paraffin embedded (FFPE) tissue by fluorescence in-situ hybridization (FISH).
  • Histopathologic and immunohistochemical evaluations were available in 69 cases, and ultrastructural evaluation was done in 2 cases of desmoid-type fibromatosis.
  • FFPE tissue sections were available in 20 tumors for the trisomy 8 detection by FISH.
  • Clinically, there were 44 extra-abdominal tumors, 28 abdominal wall tumors and 23 intra-abdominal lesions mostly involving the mesentery.
  • Histologically, desmoid-type fibromatoses showed longitudinal fascicles of spindle fibroblasts and myofibroblasts in a predominantly collagenous background.
  • The tumor cells stained positive for vimentin, alpha-smooth muscle actin, desmin, and beta-catenin (47.8%, 33/69).
  • Ultrastructurally, most tumor cells had features of fibroblasts, including rich endoplasmic reticulum and Golgi apparatus.
  • Some tumor cells were myofibroblast-like cells exhibiting intercellular junctions, fibronexous junctions and stress fibers.
  • Trisomy 8 was detected in 6 of 20 cases of desmoid-type fibromatosis including 5 of the 8 recurrent tumors but only one of the 12 primary tumors.
  • The latter tumor also recurred three years later.
  • CONCLUSIONS: Desmoid-type fibromatosis is an intermediate (locally aggressive) tumor that occurs predominantly in young females.
  • Trisomy 8 can be detected in FFPE tissue by FISH, and its presence serves as a useful predictor of tumor recurrence and may define a subtype of desmoid-type fibromatosis with high recurrence rate.
  • [MeSH-major] Fibromatosis, Abdominal / pathology. Fibromatosis, Aggressive / pathology. Peritoneal Neoplasms / pathology. Trisomy
  • [MeSH-minor] Actins / analysis. Adult. Aged. Aged, 80 and over. Child. Chromosomes, Human, Pair 8 / genetics. Desmin / analysis. Feasibility Studies. Female. Humans. Immunohistochemistry. In Situ Hybridization, Fluorescence. Male. Mesentery. Middle Aged. Muscle, Smooth / chemistry. Neoplasm Recurrence, Local. Vimentin / metabolism. beta Catenin / analysis

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  • (PMID = 16630502.001).
  • [ISSN] 0529-5807
  • [Journal-full-title] Zhonghua bing li xue za zhi = Chinese journal of pathology
  • [ISO-abbreviation] Zhonghua Bing Li Xue Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Actins; 0 / Desmin; 0 / Vimentin; 0 / beta Catenin
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6. Agrawal PS, Jagtap SM, Mitra SR: Extra-abdominal desmoid tumour of the leg. Singapore Med J; 2008 Jan;49(1):e6-7

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Extra-abdominal desmoid tumour of the leg.
  • Extra-abdominal desmoid tumour is a rare tumour and only a few cases occurring in the limbs have been reported.
  • She had a mild, dull, aching pain in the tumour.
  • Wide local excision was done and the tumour was found mainly in the subcutaneous tissue, which histopathologically proved to be an extra-abdominal desmoid tumour.
  • This case had an abnormal radiological appearance of peripheral calcification of tumour and saucer-shaped lesion in the underlying tibial cortex.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Leg / pathology. Soft Tissue Neoplasms / diagnosis
  • [MeSH-minor] Adult. Biopsy. Disease-Free Survival. Female. Humans. Treatment Outcome

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  • (PMID = 18204758.001).
  • [ISSN] 0037-5675
  • [Journal-full-title] Singapore medical journal
  • [ISO-abbreviation] Singapore Med J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Singapore
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7. Matsuo T, Hiyama E, Sugita T, Shimose S, Kubo T, Mochizuki Y, Adachi N, Ochi M: Telomere length and telomerase activity in extra-abdominal desmoid tumors. Anticancer Res; 2007 Jan-Feb;27(1A):411-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Telomere length and telomerase activity in extra-abdominal desmoid tumors.
  • The telomere biology of extra-abdominal desmoids was investigated.
  • There was a significant correlation between telomere length and tumor size, with telomeres being shorter with increasing tumor size (p = 0.049), and between telomere length and PCNA-positive cell rate, with telomere shortening with increased positive cell rate (p = 0.017).
  • Telomere length increased with recurrence, but telomerase activity decreased, and rate of PCNA-positive cells became lower, whenever the tumors were recurrent.
  • Decreasing telomere length correlated with tumor size, probably due to increased duration of proliferation in the tumor, and tumor aggressiveness.
  • [MeSH-major] Fibromatosis, Aggressive / enzymology. Fibromatosis, Aggressive / genetics. Telomerase / metabolism. Telomere / metabolism
  • [MeSH-minor] Adolescent. Adult. Aged. Female. Humans. Immunohistochemistry. Male. Middle Aged. Neoplasm Recurrence, Local / enzymology. Neoplasm Recurrence, Local / genetics. Neoplasm Recurrence, Local / pathology. Proliferating Cell Nuclear Antigen / metabolism

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  • (PMID = 17352261.001).
  • [ISSN] 0250-7005
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Proliferating Cell Nuclear Antigen; EC 2.7.7.49 / Telomerase
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8. Shimoyama T, Hiraoka K, Shoda T, Hamada T, Fukushima N, Nagata K: Multicentric extra-abdominal desmoid tumors arising in bilateral lower limbs. Rare Tumors; 2010;2(1):e12

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Multicentric extra-abdominal desmoid tumors arising in bilateral lower limbs.
  • Extra-abdominal desmoid tumors preferentially affect the shoulders, arms, backs, buttocks, and thighs of young adults.
  • Multicentric occurrence is rather rare but seems to be another distinctive feature of extra-abdominal desmoid tumors.
  • In this article we report a rare case of multicentric extra-abdominal desmoid tumors arising in bilateral lower limbs.

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  • (PMID = 21139941.001).
  • [ISSN] 2036-3613
  • [Journal-full-title] Rare tumors
  • [ISO-abbreviation] Rare Tumors
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Italy
  • [Other-IDs] NLM/ PMC2994500
  • [Keywords] NOTNLM ; bilateral limbs / desmoid / radiation
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9. Nakayama T, Tsuboyama T, Toguchida J, Hosaka T, Nakamura T: Natural course of desmoid-type fibromatosis. J Orthop Sci; 2008 Jan;13(1):51-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Natural course of desmoid-type fibromatosis.
  • BACKGROUND: Desmoid-type fibromatosis is a locally aggressive tumor known to have high potential for local recurrence after resection, while it exhibits self-limiting behavior and shows growth arrest or spontaneous regression in many patients.
  • METHODS: We retrospectively reviewed clinical outcome and changes in tumor size in 11 consecutive patients with extremity and trunk desmoid-type fibromatoses, who were basically observed without any treatment after diagnosis.
  • RESULTS: For two patients in whom the tumors were initially incorrectly diagnosed as other tumors, surgical resection was performed.
  • For another two patients, surgical resections were performed in the follow-up periods due to tumor enlargement or joint contracture.
  • In all four patients who underwent surgery, tumors recurred shortly after resection and re-resection was not performed.
  • During the follow-up periods with a median length of 56 months, ten tumors eventually stopped growing, and three of them regressed spontaneously.
  • At the time of final follow-up, ten patients were alive with residual disease without severe morbidity.
  • In one patient, the tumor enlarged to over 30 cm in diameter with a substantial functional deficit.
  • CONCLUSIONS: Simple observation is a noninvasive and function-preserving treatment for desmoid-type fibromatosis.
  • [MeSH-major] Fibromatosis, Aggressive / pathology
  • [MeSH-minor] Adolescent. Adult. Cohort Studies. Female. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Neoplasm Regression, Spontaneous. Outcome and Process Assessment (Health Care). Retrospective Studies. Tomography, X-Ray Computed. Tumor Burden

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  • (PMID = 18274856.001).
  • [ISSN] 0949-2658
  • [Journal-full-title] Journal of orthopaedic science : official journal of the Japanese Orthopaedic Association
  • [ISO-abbreviation] J Orthop Sci
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Japan
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10. Zhang HY, Ke Q, Zhang Z, Zhang R, Fu J, Chen HJ, Wei B, Bu H: [Expression of beta-catenin and estrogen receptor in desmoid-type fibromatosis]. Sichuan Da Xue Xue Bao Yi Xue Ban; 2010 Jan;41(1):101-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Expression of beta-catenin and estrogen receptor in desmoid-type fibromatosis].
  • OBJECTIVE: To detect the expression of beta-catenin and Estrogen Receptor in desmoid-type fibromatosis.
  • METHODS: Nuclear beta-catenin expression was detected by immunohistochemistry in 77 lesions with desmoid-type fibromatosis and 171 other spindle cell lesions, including superficial fibromatosis (n = 18), nodular fasciitis (n = 36), keloid (n = 16), scar (n = 10), granulation tissue (n = 9), synovial sarcoma (n = 38), neufibroma (n = 13), solitary fibrous tumor (n =12), gastrointestinal stromal tumor (n = 10), low-grade myxofibrosarcoma (n = 3), low-grade fibromyxoid sarcoma (n = 3), and smooth muscle tumor (n = 10).
  • In addition, the immunohistochemical expressions of ER-alpha, ER-beta and Ki-67 were examined in all of the lesions with desmoid-type fibromatosis.
  • RESULTS: High-level nuclear beta-catenin staining was detected in a very limited subset of tissue types, which included 70.1% of lesions with desmoid-type fibromatosis (54/77) and 6.3% of lesions with keloid (1/16).
  • None of the lesions with desmoid-type fibromatosis expressed ER-alpha.
  • However, 62 (80.5%) of the lesions with desmoids-type fibromatosis were positive in ER-beta, which included 52 (67.5%) with high-level expression, and 10 (13%) with low-level expression.
  • The lesions with desmoid-type fibromatosis had very low Ki-67 positive rate.
  • The recurrence of desmoids-type fibromatosis was not correlated independently with beta-catenin, ER-beta or Ki-67.
  • CONCLUSION: High-level nuclear beta-catenin staining serves as a useful diagnostic tool for desmoid-type fibromatosis.
  • The high expression of ER-beta in desmoid-type fibromatosis provides a biological mechanism for the antiestrogenic compounds to treat fibromatosis.
  • Beta-catenin, ER-beta or Ki-67 can not predict the prognosis of desmoid-type fibromatosis.
  • [MeSH-major] Fibroma / metabolism. Receptors, Estrogen / metabolism. Soft Tissue Neoplasms / metabolism. beta Catenin / metabolism

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  • (PMID = 20369480.001).
  • [ISSN] 1672-173X
  • [Journal-full-title] Sichuan da xue xue bao. Yi xue ban = Journal of Sichuan University. Medical science edition
  • [ISO-abbreviation] Sichuan Da Xue Xue Bao Yi Xue Ban
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Receptors, Estrogen; 0 / beta Catenin
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11. Zampieri N, Cecchetto M, Zorzi MG, Pietrobelli A, Ottolenghi A, Camoglio F: An unusual case of extra-abdominal desmoid tumour. Eur J Cancer Care (Engl); 2010 May;19(3):410-2

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] An unusual case of extra-abdominal desmoid tumour.
  • Desmoid tumour is relatively rare and generally non-metastatisizing lesion of mesenchymal origin composed of fibrous tissue and fitting in the group of aggressive fibromatosis; it is a locally aggressive proliferative soft-tissue lesion with controversial nature.
  • This tumour accounts for 0.03% of all tumours and 3% of soft-tissue tumours with annual incidence of two to four cases per million.
  • Although desmoid tumours are more common in persons aged 10-40 years than in others, they do occur in young children and older adults; in children the sex incidence is equal.
  • This is a rare case of extra-abdominal desmoid tumour in a 14-year-old girl affected by spastic tetraparesis.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Soft Tissue Neoplasms / diagnosis

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  • (PMID = 19709174.001).
  • [ISSN] 1365-2354
  • [Journal-full-title] European journal of cancer care
  • [ISO-abbreviation] Eur J Cancer Care (Engl)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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12. Huang PW, Tzen CY: Prognostic factors in desmoid-type fibromatosis: a clinicopathological and immunohistochemical analysis of 46 cases. Pathology; 2010 Feb;42(2):147-50
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Prognostic factors in desmoid-type fibromatosis: a clinicopathological and immunohistochemical analysis of 46 cases.
  • AIMS: To determine risk factors for recurrence of desmoid-type fibromatosis (aggressive fibromatosis).
  • METHODS: Forty-six cases of desmoid-type fibromatosis in Taiwanese patients were analysed for an association between tumour recurrence and clinical features, pathology, and the presence of p53 protein and beta-catenin on immunohistochemical staining.
  • The only factor significantly associated with tumour recurrence was positive surgical margin (p = 0.035).
  • CONCLUSIONS: A positive surgical margin is a risk factor for recurrence of desmoid-type fibromatosis.
  • [MeSH-major] Fibromatosis, Abdominal / pathology. Fibromatosis, Aggressive / pathology
  • [MeSH-minor] Adult. Biomarkers, Tumor / metabolism. Female. Humans. Immunohistochemistry. Male. Neoplasm Recurrence, Local. Prognosis. Risk Factors. Tumor Suppressor Protein p53 / metabolism. beta Catenin / metabolism

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  • (PMID = 20085516.001).
  • [ISSN] 1465-3931
  • [Journal-full-title] Pathology
  • [ISO-abbreviation] Pathology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / CTNNB1 protein, human; 0 / Tumor Suppressor Protein p53; 0 / beta Catenin
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13. Ogunsalu C, Barclay S: Aggressive infantile (desmoid-type) fibromatosis of the maxilla: a case report and new classification. West Indian Med J; 2005 Oct;54(5):337-40
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Aggressive infantile (desmoid-type) fibromatosis of the maxilla: a case report and new classification.
  • This paper describes the clinical, radiographic and histologic findings of an aggressive infantile (desmoid-type) fibromatosis of the face in a seven-year-old black Jamaican male.
  • The differential diagnosis, management and long term follow-up of this case are also mentioned The need for a less aggressive surgical management in this child and long-term follow-up is stressed.
  • This paper discusses the differential diagnosis and treatment of aggressive infantile fibromatosis and suggests a classification of the condition.
  • [MeSH-major] Fibroma / pathology. Fibroma / surgery. Maxillary Neoplasms / pathology. Maxillary Neoplasms / surgery. Neoplasm Invasiveness / pathology. Surgery, Oral / methods
  • [MeSH-minor] Biopsy, Needle. Child. Follow-Up Studies. Humans. Immunohistochemistry. Male. Neoplasm Staging. Risk Assessment. Treatment Outcome

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  • (PMID = 16459519.001).
  • [ISSN] 0043-3144
  • [Journal-full-title] The West Indian medical journal
  • [ISO-abbreviation] West Indian Med J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Jamaica
  • [Number-of-references] 15
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14. Kujak JL, Liu PT, Johnson GB, Callstrom MR: Early experience with percutaneous cryoablation of extra-abdominal desmoid tumors. Skeletal Radiol; 2010 Feb;39(2):175-82
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Early experience with percutaneous cryoablation of extra-abdominal desmoid tumors.
  • OBJECTIVE: Surgical resection, radiation therapy and chemotherapy are all accepted as standard treatments for extra-abdominal desmoid (EAD) tumors, but their effectiveness has been limited by frequent local recurrence.
  • The purpose of this article is to describe our early experiences with using percutaneous cryoablation for local control of extra-abdominal desmoid tumors in five patients whose tumors had failed to respond to standard therapy.
  • MATERIAL AND METHODS: In a retrospective search of our institution's radiology database for patients who had undergone percutaneous cryoablation for treatment of EAD tumors between June 2004 and July 2007, we identified five patients (three female and two male).
  • Three of these patients had been referred for cryoablation for local tumor control, and two had been referred for palliation of inoperable tumors.
  • The treated EAD tumors were located in the neck, shoulders and trunk and ranged in size from 3.0 cm to 10.0 cm.
  • Radiology records were reviewed to follow the size of the EAD tumors before and after cryotherapy.
  • RESULTS: For the three patients referred for local control of EAD tumors, complete tumor coverage with the ablation zones was achieved.
  • Their tumors had completely resolved on long-term imaging follow-up at 19 months and 43 months.
  • The third patient, with a 6.1 cm mass, reported improved mild pain at 6 months, and imaging showed a moderate decrease of tumor size.
  • For the two patients referred for palliative therapy, initial partial pain relief was felt 2 weeks after the procedure, At long-term (58 months) follow-up of one patient with a 9.1 cm mass, the tumor was still present although reduced in size, and local pain had returned to its former moderate level.
  • CONCLUSION: Cryoablation appears to be an effective alternative treatment for the achievement of local control of small and moderately sized EAD tumors, but it is likely of limited use in patients with larger tumors that have untreatable regions due to involvement of vital structures.
  • Continued research evaluating cryoablation for the treatment of EAD tumors is needed.
  • [MeSH-major] Cryosurgery / methods. Fibromatosis, Aggressive / diagnosis. Fibromatosis, Aggressive / surgery
  • [MeSH-minor] Abdominal Neoplasms / diagnosis. Abdominal Neoplasms / surgery. Adolescent. Adult. Child. Female. Humans. Male. Pilot Projects. Treatment Outcome. Young Adult

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  • (PMID = 19768644.001).
  • [ISSN] 1432-2161
  • [Journal-full-title] Skeletal radiology
  • [ISO-abbreviation] Skeletal Radiol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] Germany
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15. Jilong Y, Jian W, Xiaoyan Z, Xiaoqiu L, Xiongzeng Z: Analysis of APC/beta-catenin genes mutations and Wnt signalling pathway in desmoid-type fibromatosis. Pathology; 2007 Jun;39(3):319-25
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Analysis of APC/beta-catenin genes mutations and Wnt signalling pathway in desmoid-type fibromatosis.
  • OBJECTIVE: The abnormalities of the Wnt signalling pathway in desmoid-type fibromatosis were analysed, with the purpose of exploring the mechanism of tumorigenesis and progression.
  • Using the same materials, apoptosis of the tumour cells was investigated by terminal deoxynucleotidyl transferase mediated dUTP nick end-labelling (TUNEL) testing.
  • CONCLUSIONS: There are somatic mutations of the APC and beta-catenin gene in desmoid-type fibromatosis, and there are abnormalities in the Wnt signalling pathway.
  • [MeSH-major] Fibromatosis, Aggressive / genetics. Genes, APC. Signal Transduction / physiology. Wnt Proteins / metabolism. beta Catenin / genetics

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  • (PMID = 17558858.001).
  • [ISSN] 0031-3025
  • [Journal-full-title] Pathology
  • [ISO-abbreviation] Pathology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Ki-67 Antigen; 0 / Proto-Oncogene Proteins c-myc; 0 / Wnt Proteins; 0 / beta Catenin; 136601-57-5 / Cyclin D1
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16. Al-Otaibi ML, Turcotte RE, Hings I, Beaudet J, Isler M, Nahal A, Wong C: Low-dose chemotherapy for extra-abdominal desmoid tumor. Saudi Med J; 2008 Dec;29(12):1730-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Low-dose chemotherapy for extra-abdominal desmoid tumor.
  • OBJECTIVE: To assess the outcome of patients with extra-abdominal desmoid tumor treated with low dose chemotherapy (methotrexate and vinblastine) both for tumor response and treatment related toxicity.
  • METHODS: We retrospectively reviewed the outcome of 12 patients who underwent low dose chemotherapy for extra abdominal desmoid of different locations.
  • We evaluated the patients for their compliance, tumor response, complications of treatment, and impact of treatment on symptoms.
  • RESULTS: Disease related morbidity included pain in 7 patients, functional limitation in 7 and cosmetic defects in 3.
  • The mean tumor size was 11 cm (3-20 cm).
  • According to Response Evaluation Criteria in Solid Tumors, 6 tumors showed a partial response and 6 remained stable.
  • Of the 7 patients who had painful tumors, 6 achieved significant symptom relief.
  • At latest follow-up, tumors remained stable in 8, one has markedly regressed and 3 exhibited progression at an average of 54 months.
  • [MeSH-major] Antineoplastic Agents / administration & dosage. Fibroma / drug therapy

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  • (PMID = 19082222.001).
  • [ISSN] 0379-5284
  • [Journal-full-title] Saudi medical journal
  • [ISO-abbreviation] Saudi Med J
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Saudi Arabia
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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17. Constantinidou A, Scurr M, Jones R, Al-Muderis O, Judson I: Treatment of aggressive fibromatosis with pegylated liposomal doxorubicin: The Royal Marsden Hospital experience. J Clin Oncol; 2009 May 20;27(15_suppl):10519

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Treatment of aggressive fibromatosis with pegylated liposomal doxorubicin: The Royal Marsden Hospital experience.
  • : 10519 Background: Aggressive fibromatosis (AF) or desmoid tumors are monoclonal proliferations which are locally invasive but do not metastasise.
  • Sporadic tumors are usually associated with mutations in the beta-catenin gene CTNNB1whereas those occurring in the context of familial adenomatous polyposis usually have inactivating mutations in APC.
  • When surgery and radiotherapy are not applicable or fail to control the disease, systemic treatment with anti-oestrogens, non steroidal anti-inflammatory drugs (NSAIDs) and chemotherapy can be used.
  • All patients had progressive fibromatosis.
  • Objective response according to RECIST was achieved in 4/10 patients and in 5 patients the best response was stable disease.

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  • (PMID = 27963658.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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18. Dufresne A, Penel N, Salas S, Le Cesne A, Perol D, Bui B, Brain E, Ray-Coquard I, Jimenez M, Blay J: Updated outcome with long-term follow-up of imatinib for the treatment of progressive or recurrent aggressive fibromatosis (desmoid tumor): A FNCLCC/ French Sarcoma Group phase II trial. J Clin Oncol; 2009 May 20;27(15_suppl):10518

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Updated outcome with long-term follow-up of imatinib for the treatment of progressive or recurrent aggressive fibromatosis (desmoid tumor): A FNCLCC/ French Sarcoma Group phase II trial.
  • : 10518 Background: We present updated results from a previously reported phase II trial assessing clinical efficacy of imatinib in progressive or recurrent aggressive fibromatosis (Fayette et al.
  • ASCO 2007) Methods: Patients with aggressive fibromatosis not amenable to radiotherapy or non-mutilating surgery were eligible and received imatinib 400mg daily (increased to 800mg daily in case of progression) up to 1 year then stopped.
  • The primary end point was non-progressive disease rate at 3 months.
  • Two patients with mesenteric aggressive fibromatosis died from progressive disease.
  • CONCLUSIONS: With a 2.8 years median follow up, this is the largest study which confirms the high efficacy of imatinib (400 mg daily) in patients presenting with aggressive fibromatosis failing local treatment and with documented evidence of progressive disease before imatinib treatment.

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  • (PMID = 27963656.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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19. Camargo VP, Maki RG: Clinical outcomes of systemic therapy for patients with desmoids. J Clin Oncol; 2009 May 20;27(15_suppl):10585

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Clinical outcomes of systemic therapy for patients with desmoids.
  • : 10585 Background: Desmoids are rare, slow growing, histologically benign tumors without metastatic potential.
  • METHODS: We examined outcomes of patients with desmoid tumors receiving systemic therapy at a single institution, to provide a basis for examination of newer agents.
  • Retrospective chart review of 682 patients with desmoid tumors (1982-2006) from a prospectively collected sarcoma database.
  • Patients without measurable disease, those receiving therapy we could not document, and those receiving prophylactic therapy were excluded.
  • Nine patients died, 7 of progressive disease/surgical complications, and two with Gardner syndrome-related malignancies.
  • Intra-abdominal primary location was most common (29/70=41%).
  • CONCLUSIONS: Anthracycline-containing regimens, hormonal therapy, and tyrosine kinase inhibitors have modest activity against desmoid tumors.
  • The choice of therapy for these morbid and potentially fatal tumors should balance the efficacy of treatments with their short- and long-term side effects.

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  • (PMID = 27963883.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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20. Comandone A, Boglione A, Pochettino P, Berno E, Inguì M, Papotti M, Borasio P, Maggi G, Brach Del Prever E, Gino G: Primary sarcomas of the lungs and mediastinum: Clinicopathological study and therapy results of Piedmontese Group for Sarcomas. J Clin Oncol; 2009 May 20;27(15_suppl):e21509

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Pts characteristics: median age 41 (19-80 y), male/female 19/12; symptoms at diagnosis: dyspnoea (42%), chest and shoulder pain (39%), cough (35%), hemophtoae (13%), discomfort (10%).
  • 5 mediastinal tumours were located as follows: 2 in anterior part, 1 in posterior and 2 in the middle (sarcomas of the heart).
  • 26 lung sarcomas presented as a singular mass in 23 cases and as a metastatic disease in 3.
  • RESULTS: In 20/31 cases the tumour was immediately resected (3 mediastinal masses and 17 lung sarcomas).
  • The histology were: peripheral nerve tumour 7, leiomyosarcoma 4, MFH 2, fibrosarcoma 2, liposarcoma 1, angiosarcoma 2, undifferentiated sarcoma 1, solitary fibrous tumour 2, rhabdomyosarcoma 2, synovialsarcoma 2, pulmonary artery sarcoma 1, pleuropolmonary blastoma 1, malignant hemangiopericytoma 1, mixoid chondrosarcoma 1, ectopic osteosarcoma 1, aggressive fibromatosis 1.
  • Only 4 pts received neoadjuvant chemotherapy, 11 adjuvant CT, 5 exclusive CT + RT for inoperable disease.
  • Of these only 8 are alive (2 with disease).
  • Volume of disease, complete resection and grading are the dominant prognostic factors.

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  • (PMID = 27963441.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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21. Indelicato DJ, Keole SR, Shahlaee AH, Morris CG, Gibbs CP, Scarborough MT, Islam S, Marcus RB: Ewing tumors of the chest wall: Local control and long-term outcomes. J Clin Oncol; 2009 May 20;27(15_suppl):e21501

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Ewing tumors of the chest wall: Local control and long-term outcomes.
  • Pts characteristics: median age 41 (19-80 y), male/female 19/12; symptoms at diagnosis: dyspnoea (42%), chest and shoulder pain (39%), cough (35%), hemophtoae (13%), discomfort (10%).
  • 5 mediastinal tumours were located as follows: 2 in anterior part, 1 in posterior and 2 in the middle (sarcomas of the heart).
  • 26 lung sarcomas presented as a singular mass in 23 cases and as a metastatic disease in 3.
  • RESULTS: In 20/31 cases the tumour was immediately resected (3 mediastinal masses and 17 lung sarcomas).
  • The histology were: peripheral nerve tumour 7, leiomyosarcoma 4, MFH 2, fibrosarcoma 2, liposarcoma 1, angiosarcoma 2, undifferentiated sarcoma 1, solitary fibrous tumour 2, rhabdomyosarcoma 2, synovialsarcoma 2, pulmonary artery sarcoma 1, pleuropolmonary blastoma 1, malignant hemangiopericytoma 1, mixoid chondrosarcoma 1, ectopic osteosarcoma 1, aggressive fibromatosis 1.
  • Only 4 pts received neoadjuvant chemotherapy, 11 adjuvant CT, 5 exclusive CT + RT for inoperable disease.
  • Of these only 8 are alive (2 with disease).
  • Volume of disease, complete resection and grading are the dominant prognostic factors.

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  • (PMID = 27963390.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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22. Dumont AG, Lev D, Lazar A, Joensuu H, Trent J: Simultaneous gastrointestinal stromal tumor (GIST) and desmoid fibromatosis (DF). J Clin Oncol; 2009 May 20;27(15_suppl):10568

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Simultaneous gastrointestinal stromal tumor (GIST) and desmoid fibromatosis (DF).
  • : 10568 Background: Desmoid Fibromatosis (DF) is one of a group of rare fibrous tissue proliferations (2-4 cases per year per million) which tend to be locally aggressive but have no propensity for metastasis.
  • Gastrointestinal Stromal Tumor (GIST) is the most common mesenchymal tumors of the gastrointestinal tract and also rare (15-20 cases per year per million).
  • In 5 cases, the GIST was diagnosed prior to the DF while one case had both tumors synchronous at presentation.
  • The 3 others were intra-abdominal DF.

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  • (PMID = 27963790.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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23. Ozger H, Eralp L, Toker B, Ağaoğlu F, Dizdar Y: [Evaluation of prognostic factors affecting recurrences and disease-free survival in extra-abdominal desmoid tumors]. Acta Orthop Traumatol Turc; 2007 Aug-Oct;41(4):291-4

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Evaluation of prognostic factors affecting recurrences and disease-free survival in extra-abdominal desmoid tumors].
  • [Transliterated title] Ekstra-abdominal desmoid tümörlerde nüks ve hastaliksiz sağkalimi etkileyen prognostik faktörlerin değerlendirilmesi.
  • OBJECTIVES: We investigated treatment results and the role of potential prognostic factors in patients treated by surgery with or without adjuvant radiotherapy for primary or recurrent extra-abdominal desmoid tumors.
  • METHODS: The study included 38 patients (23 females, 15 males; mean age 24 years; range 5 to 61 years) who underwent surgical treatment for extra-abdominal desmoid tumors.
  • The mean disease-free survival was 38+/-8 months, and eight-year disease-free survival was 35.7+/-8.5%.
  • Disease-free survival did not differ significantly between patients receiving adjuvant radiotherapy (47.9+/-7.9 months) and those treated with surgery alone (37.9+/-12.4 months), and between patients who developed a recurrence at the resection site (12.1+/-4.7 months) or at a different site (24.3+/-1.0 months) (p>0.05).
  • None of the potential prognostic factors including gender, age, localization, surgical margin, or adjuvant irradiation were found to affect disease-free survival.
  • [MeSH-major] Fibromatosis, Aggressive / surgery. Neoplasm Recurrence, Local / surgery. Soft Tissue Neoplasms / surgery
  • [MeSH-minor] Adolescent. Adult. Axilla. Child. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Extremities. Female. Humans. Male. Middle Aged. Prognosis. Turkey / epidemiology

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  • (PMID = 18180559.001).
  • [ISSN] 1017-995X
  • [Journal-full-title] Acta orthopaedica et traumatologica turcica
  • [ISO-abbreviation] Acta Orthop Traumatol Turc
  • [Language] tur
  • [Publication-type] English Abstract; Evaluation Studies; Journal Article
  • [Publication-country] Turkey
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24. Amary MF, Pauwels P, Meulemans E, Roemen GM, Islam L, Idowu B, Bousdras K, Diss TC, O'Donnell P, Flanagan AM: Detection of beta-catenin mutations in paraffin-embedded sporadic desmoid-type fibromatosis by mutation-specific restriction enzyme digestion (MSRED): an ancillary diagnostic tool. Am J Surg Pathol; 2007 Sep;31(9):1299-309
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Detection of beta-catenin mutations in paraffin-embedded sporadic desmoid-type fibromatosis by mutation-specific restriction enzyme digestion (MSRED): an ancillary diagnostic tool.
  • Desmoid-type fibromatosis is a locally aggressive deep soft tissue tumor.
  • The aim of this study was to determine the specificity and sensitivity of these 3 most common beta-catenin mutations in the diagnosis of desmoid-type fibromatosis using paraffin-embedded material.
  • One hundred and thirty-three cases were analyzed, including 76 desmoid-type, and 18 superficial fibromatosis, in addition to a further 39 fibromatosis mimics.
  • Mutations were detected in 66 cases (87%) of 76 desmoid-type fibromatosis (71 extra-abdominal).
  • All desmoid-type fibromatosis cases and 72% of the mimics tested showed nuclear positivity for beta-catenin indicating immunohistochemistry is a sensitive but not a specific test for desmoid-type fibromatosis.
  • In contrast, to date, beta-catenin mutations have not been detected in any lesions which mimic desmoid-type fibromatosis.
  • Mutation-specific restriction enzyme digestion, a simple and efficient means of detecting the common beta-catenin mutations in desmoid-type fibromatosis, complements light microscopy in reaching a diagnosis.
  • [MeSH-major] DNA Mutational Analysis / methods. Fibromatosis, Aggressive / diagnosis. Gene Expression Regulation, Neoplastic. Mutation. Restriction Mapping. beta Catenin / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Base Sequence. Cell Nucleus / chemistry. Child. Codon. Diagnosis, Differential. Female. Humans. Immunohistochemistry / methods. Magnetic Resonance Imaging. Male. Middle Aged. Molecular Sequence Data. Paraffin Embedding. Predictive Value of Tests. Sensitivity and Specificity. Tissue Array Analysis

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  • (PMID = 17721184.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Comparative Study; Evaluation Studies; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / CTNNB1 protein, human; 0 / Codon; 0 / beta Catenin
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25. Janitzky A, Porsch M, Daher M, Küster D, Liehr UB: [Aggressive fibromatosis (desmoid tumor) : A rare differential diagnosis of metastasis of renal cell carcinoma]. Urologe A; 2010 Jan;49(1):81-3
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  • [Title] [Aggressive fibromatosis (desmoid tumor) : A rare differential diagnosis of metastasis of renal cell carcinoma].
  • [Transliterated title] Aggressive Fibromatose (Desmoidfibromatose) : Seltene Differentialdiagnose einer Nierenzellkarzinommetastase.
  • We report the case of a 65-year-old woman with an aggressive fibromatosis of the rectus abdominis muscle suspicious for a metastasis of renal cell carcinoma after tumor nephrectomy 3 years previously.
  • Aggressive fibromatoses (desmoid tumors) are rare semimalignant tumors of the connective tissue with local infiltration and destruction of tissue.
  • Complete resection is essential to avoid tumor relapse.
  • Aggressive fibromatosis must be considered in the differential diagnosis of renal cell carcinoma metastasis.
  • [MeSH-major] Abdominal Muscles / pathology. Carcinoma, Renal Cell / diagnosis. Carcinoma, Renal Cell / secondary. Fibromatosis, Abdominal / diagnosis. Kidney Neoplasms / diagnosis. Muscle Neoplasms / pathology
  • [MeSH-minor] Aged. Diagnosis, Differential. Female. Humans


26. Ridders J, Ernst A, Todt I, Seidl RO: [Extra-abdominal desmoid tumors. Case report and literature review]. HNO; 2005 Jul;53(7):639-44
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  • [Title] [Extra-abdominal desmoid tumors. Case report and literature review].
  • [Transliterated title] Extraabdominelle Fibromatose. Fallbericht und Literaturübersicht.
  • Musculoaponeurotic fibromatosis or desmoid tumors are rare.
  • We report the case of a 57 year old woman with a slowly growing tumor behind the sternocleidomastoid muscle, which was completely removed.
  • Histological examination confirmed the clinical suspicion of a desmoid tumor.
  • Desmoid tumors are aggressive, locally infiltrating, non-metastasizing tumors with a high local recurrence.
  • The diagnosis is made histologically, reactive fibromatosis and fibrosarcoma must be eliminated in differential diagnosis.
  • By the combination of different radiographic techniques, it is possible to describe the tumors and differentiate between vessels, nerves and bones.
  • Adjuvant therapy, such as x-ray treatment, chemo- and hormone therapy, are indicated when the tumor is inoperable or too extensive for surgery.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Head and Neck Neoplasms / diagnosis
  • [MeSH-minor] Abdominal Neoplasms / diagnosis. Abdominal Neoplasms / pathology. Abdominal Neoplasms / surgery. Adult. Calcinosis / diagnosis. Calcinosis / pathology. Calcinosis / surgery. Diagnosis, Differential. Disease Progression. Female. Humans. Magnetic Resonance Imaging. Neck Muscles / pathology. Neck Muscles / surgery. Tomography, X-Ray Computed. Ultrasonography

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  • (PMID = 15257395.001).
  • [ISSN] 0017-6192
  • [Journal-full-title] HNO
  • [ISO-abbreviation] HNO
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Germany
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27. Chung KH, Charlton A, Arbuckle S, Chaseling R, Owler BK: Metachronous multifocal desmoid-type fibromatoses along the neuraxis with adenomatous polyposis syndrome. J Neurosurg Pediatr; 2010 Oct;6(4):372-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Metachronous multifocal desmoid-type fibromatoses along the neuraxis with adenomatous polyposis syndrome.
  • Desmoid-type fibromatosis, aggressive fibromatosis, or desmoid tumor is an uncommon benign but locally aggressive fibroblastic lesion.
  • Although intraabdominal desmoid-type fibromatoses are well described in association with adenomatous polyposis syndrome, their occurrence along the neuraxis is extremely rare.
  • The authors report the case of a 14-year-old boy with metachronous intracranial and spinal desmoid-type fibromatoses with preceding medulloblastoma.
  • This is the first reported case of spinal desmoid-type fibromatosis in association with adenomatous polyposis syndrome.
  • The identification of an underlying genetic instability allows for screening to detect lesions and institute measures to avoid preventable mortality from nonneurological tumors.
  • [MeSH-major] Adenomatous Polyposis Coli / pathology. Cerebral Ventricle Neoplasms / pathology. Fibromatosis, Aggressive / pathology. Genes, APC. Neoplasms, Multiple Primary / pathology

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  • (PMID = 20887112.001).
  • [ISSN] 1933-0715
  • [Journal-full-title] Journal of neurosurgery. Pediatrics
  • [ISO-abbreviation] J Neurosurg Pediatr
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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28. de Bree E, Keus R, Melissas J, Tsiftsis D, van Coevorden F: Desmoid tumors: need for an individualized approach. Expert Rev Anticancer Ther; 2009 Apr;9(4):525-35
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  • [Title] Desmoid tumors: need for an individualized approach.
  • Desmoid tumor, also known as aggressive fibromatosis or desmoid-type fibromatosis, is a rare monoclonal, fibroblastic proliferation arising in musculoaponeurotic structures.
  • Although histologically benign, desmoids are often locally invasive and associated with a high local recurrence rate after resection.
  • Since it is a heterogeneous disease, in particular regarding clinical presentation, anatomic location and biological behavior, treatment should be individualized to reduce local tumor control failure with concurrently acceptable morbidity and preservation of quality of life.
  • Many issues regarding optimal treatment of desmoids remain controversial.
  • Radiotherapy for gross disease is considerably effective, but is associated with a relatively high rate of complications, which are usually mild or moderate and radiation dose dependent.
  • Risk factors for local tumor control failure include young age, large size, presentation as recurrent disease, limb/girdle or intra-abdominal location, involved surgical margins, omission of radiotherapy, radiation dose less than 50 Gy and insufficient radiation field size.
  • Increased comprehension of the pathogenesis and biological behavior of desmoids resulted in the emerging applicability of systemic therapies and a wait-and-see policy.
  • Considering the significant morbidity of surgery and/or radiotherapy for certain locations, especially mutilation and loss of function, and the tumor's natural history, which is often characterized by prolonged periods of stability or even regression, a period of watchful waiting may compose the most appropriate management in selected asymptomatic patients.
  • Attempts to complete eradication of the disease may be worse than the disease itself.
  • [MeSH-major] Fibromatosis, Aggressive / therapy
  • [MeSH-minor] Adult. Age Factors. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Case Management. Child. Combined Modality Therapy. Cyclooxygenase 2 Inhibitors / therapeutic use. Female. Humans. Male. Neoplasm Recurrence, Local. Pregnancy. Pregnancy Complications, Neoplastic / surgery. Radiotherapy / adverse effects. Radiotherapy, Adjuvant. Referral and Consultation. Risk Factors

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  • (PMID = 19374605.001).
  • [ISSN] 1744-8328
  • [Journal-full-title] Expert review of anticancer therapy
  • [ISO-abbreviation] Expert Rev Anticancer Ther
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Cyclooxygenase 2 Inhibitors
  • [Number-of-references] 89
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29. Mátrai Z, Papp J, Polgár C, Hitre E, Köves I, Oláh E, Andi J, Kiss A, Vámosi Nagy I, Tóth L, Orosz Z: [Long-term experience with therapy of a female patient with Gardner's syndrome, first presenting with extra-abdominal desmoid tumor, and review of the literature]. Magy Seb; 2009 Apr;62(2):75-82
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Long-term experience with therapy of a female patient with Gardner's syndrome, first presenting with extra-abdominal desmoid tumor, and review of the literature].
  • [Transliterated title] Extraabdominalis desmoid tumorral megjeleno Gardner-syndromás beteg kezelésével szerzett hosszú távú tapasztalataink és irodalmi áttekintés.
  • Gardner's syndrome is a clinical subgroup of familial adenomatous polyposis, an autosomal dominant disease.
  • It is characterized by gastrointestinal polyps and extra-intestinal manifestations including multiple osteomas, skin and soft tissue tumours.
  • Aggressive desmoid tumours can be very difficult to manage in patients with Gardner's syndrome.
  • We present a case of a 17-year-old female who presented with an aggressive desmoid tumor arising of the lumbar area as part of her Gardner's syndrome.
  • We conclude that desmoid tumors can precede gastrointestinal manifestations of familial adenomatous polyposis or Gardner's syndrome.
  • Desmoid tumours should be managed in a multidisciplinary setting, as well.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Fibromatosis, Aggressive / diagnosis. Fibromatosis, Aggressive / genetics. Gardner Syndrome / diagnosis. Gardner Syndrome / genetics. Genes, APC
  • [MeSH-minor] Adolescent. Antineoplastic Agents, Hormonal / therapeutic use. Base Sequence. Female. Germ-Line Mutation. Humans. Molecular Sequence Data. Neoplasm Staging. Polymorphism, Genetic

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  • (PMID = 19386568.001).
  • [ISSN] 0025-0295
  • [Journal-full-title] Magyar sebészet
  • [ISO-abbreviation] Magy Seb
  • [Language] hun
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] Hungary
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Antineoplastic Agents, Hormonal
  • [Number-of-references] 30
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30. Matono H, Oda Y, Nakamori M, Tamiya S, Yamamoto H, Yokoyama R, Saito T, Iwamoto Y, Tsuneyoshi M: Correlation between beta-catenin widespread nuclear expression and matrix metalloproteinase-7 overexpression in sporadic desmoid tumors. Hum Pathol; 2008 Dec;39(12):1802-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Correlation between beta-catenin widespread nuclear expression and matrix metalloproteinase-7 overexpression in sporadic desmoid tumors.
  • Desmoid tumors (desmoid-type fibromatoses) are locally aggressive soft tissue tumors associated with the Wnt/beta-catenin signaling pathway (APC-beta-catenin-Tcf pathway).
  • Matrix metalloproteinase-7, which is one of the target genes of the Wnt/beta-catenin signaling pathway, has been reported to play an important role in tumor progression.
  • We examined the immunohistochemical expression of beta-catenin and matrix metalloproteinase-7 in 72 samples (63 primary and 9 recurrent samples, 63 patients) of sporadic desmoid tumors without familial adenomatous polyposis, and the genetic alteration of the beta-catenin gene in 33 frozen materials (22 primary and 11 recurrent samples, 22 patients).
  • Immunohistochemically, there was a statistically significant correlation between widespread nuclear expression of beta-catenin and overexpression of matrix metalloproteinase-7 (P < .01 in extra-abdominal desmoid, Fisher test).
  • In the beta-catenin mutated group, matrix metalloproteinase-7 messenger RNA expression was significantly higher than that of the beta-catenin wild-type group (P = .0018, Mann-Whitney U test).
  • Our results suggest that the matrix metalloproteinase-7 gene may be up-regulated by mutated or continuously elevated beta-catenin protein and that the matrix metalloproteinase-7 gene may also be targeted in the Wnt/beta-catenin signaling pathway in sporadic desmoid tumors.
  • [MeSH-major] Fibromatosis, Aggressive / metabolism. Gene Expression Regulation, Neoplastic. Matrix Metalloproteinase 7 / metabolism. Soft Tissue Neoplasms / metabolism. beta Catenin / metabolism
  • [MeSH-minor] Adolescent. Adult. Aged. Biomarkers, Tumor / genetics. Biomarkers, Tumor / metabolism. Cell Nucleus / metabolism. Child. Child, Preschool. Female. Humans. Infant. Male. Middle Aged. Mutation, Missense. RNA, Messenger / metabolism. RNA, Neoplasm / analysis. Reverse Transcriptase Polymerase Chain Reaction. Young Adult

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  • (PMID = 18715618.001).
  • [ISSN] 1532-8392
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / CTNNB1 protein, human; 0 / RNA, Messenger; 0 / RNA, Neoplasm; 0 / beta Catenin; EC 3.4.24.23 / MMP7 protein, human; EC 3.4.24.23 / Matrix Metalloproteinase 7
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31. Seper L, Hoppe P, Kruse-Lösler B, Büchter A, Joos U, Kleinheinz J: [Aggressive fibromatosis in the jaw and facial region with bone involvement. A review]. Mund Kiefer Gesichtschir; 2005 Nov;9(6):349-62
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  • [Title] [Aggressive fibromatosis in the jaw and facial region with bone involvement. A review].
  • [Transliterated title] Aggressive Fibromatose im Kiefer-Gesichts-Bereich mit ossärer Beteiligung. Eine Ubersicht.
  • BACKGROUND: Aggressive fibromatosis (AF) involving bones of the head is rare and surgery is often complicated by a high recurrence rate.
  • [MeSH-major] Eyelid Neoplasms / diagnosis. Facial Neoplasms / diagnosis. Fibromatosis, Aggressive / diagnosis. Fibromatosis, Gingival / diagnosis. Jaw Neoplasms / diagnosis. Skull Neoplasms / diagnosis
  • [MeSH-minor] Aged. Female. Humans. Neoplasm Recurrence, Local / diagnosis. Neoplasm Recurrence, Local / surgery. Patient Care Team. Radiography, Panoramic. Reoperation. Surgery, Plastic

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  • (PMID = 16142459.001).
  • [ISSN] 1432-9417
  • [Journal-full-title] Mund-, Kiefer- und Gesichtschirurgie : MKG
  • [ISO-abbreviation] Mund Kiefer Gesichtschir
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article; Meta-Analysis
  • [Publication-country] Germany
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32. Paradol PO, Toussoun G, Delbaere M, Delaporte T, Delay E: [Extra-abdominal desmoid tumor in a scar of donor-site of a latissimus dorsi flap: case report]. Ann Chir Plast Esthet; 2008 Feb;53(1):63-9
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  • [Title] [Extra-abdominal desmoid tumor in a scar of donor-site of a latissimus dorsi flap: case report].
  • [Transliterated title] Tumeur desmoïde extra-abdominale survenue sur cicatrice de prélèvement de lambeau de grand dorsal: à propos d'un cas.
  • We will discuss one case of desmoid tumor arising from a latissimus dorsi flap donor-site scar.
  • A dorsal tumefaction, with a benign aspect, was observed during the follow-up period.
  • The biopsy showed an extra-abdominal desmoid tumor.
  • [MeSH-major] Cicatrix / complications. Fibromatosis, Aggressive / etiology. Mammaplasty / adverse effects. Skin Neoplasms / etiology. Surgical Flaps / adverse effects

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  • (PMID = 17418929.001).
  • [ISSN] 0294-1260
  • [Journal-full-title] Annales de chirurgie plastique et esthétique
  • [ISO-abbreviation] Ann Chir Plast Esthet
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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33. Rosato L, Mondini G, Serbelloni M, Bertone P, Orlassino R, Cossavella D: [Intra-abdominal desmoid tumors: rare but important disease]. G Chir; 2007 Jan-Feb;28(1-2):20-4

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Intra-abdominal desmoid tumors: rare but important disease].
  • Desmoid tumors are rare benign neoplasms with high tendency to local recurrence, and they can be divided into extra- and intra-abdominal types (mesenteric fibromatosis).
  • Six patients (3 men and 3 women) affected by extra-abdominal desmoid tumors have been treated with radical excision.
  • In two patients desmoid was intra-abdominal:.
  • 2) 52 years old man, submitted to an elective excision of a capsulated neoplasm of the little omentum, which had caused an oppressive abdominal pain.
  • In both cases the hystological diagnosis has been desmoid tumor.
  • Surgical treatment of desmoid tumors must aim at radical excision to avoid frequent recurrences (25-65%); these have stimulated the research of other kinds of treatments, since a new surgical operation itself can lead to a further recurrence.
  • Radiotherapy has been investigated with results in 79-96% of cases, antiestrogenic therapy has been used with success in 51% of patients, and high dose tamoxifen seemed to obtain a stable disease in non operable cases.
  • Conclusive results on the efficacy of these treatments have not been obtained yet, because of the rarity of the desmoid tumors even in greater Centres.
  • [MeSH-major] Fibromatosis, Abdominal / surgery. Mesentery. Peritoneal Neoplasms / surgery
  • [MeSH-minor] Abdominal Neoplasms / pathology. Abdominal Neoplasms / surgery. Aged. Female. Fibromatosis, Aggressive / surgery. Humans. Male. Middle Aged. Retrospective Studies. Treatment Outcome

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  • (PMID = 17313728.001).
  • [ISSN] 0391-9005
  • [Journal-full-title] Il Giornale di chirurgia
  • [ISO-abbreviation] G Chir
  • [Language] ita
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Italy
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34. Desai SR, Dombale VD, Janugade HB: Infantile fibromatosis (desmoid type)--a case report. Indian J Pathol Microbiol; 2005 Jul;48(3):379-80
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  • [Title] Infantile fibromatosis (desmoid type)--a case report.
  • Infantile fibromatosis represents the childhood counter part of musculoaponeurotic fibromatosis & arises as a solitary mass in skeletal muscle, adjacent fascia, aponeurosis or periosteum.
  • Microscopically it exists in two forms diffuse (mesenchymal) & desmoid.
  • The less common desmoid form rarely occurs in infancy.
  • The differential diagnosis of this type is infantile fibrosarcoma.
  • The tumor may locally recur if inadequately excised.
  • We report a case of infantile fibromatosis of desmoid type occurring in 10 months male child for its extreme rarity.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Neck Muscles / pathology. Soft Tissue Neoplasms / pathology

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  • (PMID = 16761760.001).
  • [ISSN] 0377-4929
  • [Journal-full-title] Indian journal of pathology & microbiology
  • [ISO-abbreviation] Indian J Pathol Microbiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] India
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35. Kuhnen C, Helwing M, Rabstein S, Homann HH, Müller KM: [Desmoid-type fibromatosis (aggressive fibromatosis)]. Pathologe; 2005 Mar;26(2):117-26
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Desmoid-type fibromatosis (aggressive fibromatosis)].
  • [Transliterated title] Desmoidfibromatosen (aggressive Fibromatosen) Klinisch-pathologische Korrelationen und Differenzialdiagnose spindelzelliger fibroblastisch/myofibroblastischer Tumoren des Weichgewebes.
  • Desmoid-type fibromatoses (aggressive fibromatoses) represent infiltrative, locally destructively growing soft tissue tumors with a high potential for recurrence.
  • Desmoid tumors of 33 adult patients were analysed regarding clinical and morphological aspects (sex, age distribution, site, size, mitotic rate, tumor microvessel density, surgical margins, additional radiotherapy).
  • No prognostic significance of tumor microvessel density was evident.
  • The presented results may indicate an increased risk for local relapse in those desmoid-type fibromatoses which are mitotically active.
  • Postoperative radiotherapy seems to be effective in the treatment of aggressive fibromatosis to avoid tumor recurrence.
  • Differential diagnosis of desmoid-type fibromatosis/aggressive fibromatosis in adulthood include various fibroblastic/myofibroblastic soft tissue tumors such as nodular fasciitis, fibrosarcoma, low-grade fibromyxoid sarcoma, myofibroblastic sarcoma as well as leiomyosarcoma and soft tissue leiomyoma.
  • [MeSH-major] Fibromatosis, Aggressive / pathology

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  • (PMID = 15657684.001).
  • [ISSN] 0172-8113
  • [Journal-full-title] Der Pathologe
  • [ISO-abbreviation] Pathologe
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Germany
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36. Owens CL, Sharma R, Ali SZ: Deep fibromatosis (desmoid tumor): cytopathologic characteristics, clinicoradiologic features, and immunohistochemical findings on fine-needle aspiration. Cancer; 2007 Jun 25;111(3):166-72
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Deep fibromatosis (desmoid tumor): cytopathologic characteristics, clinicoradiologic features, and immunohistochemical findings on fine-needle aspiration.
  • BACKGROUND: Deep fibromatosis or desmoid tumor (DF/DT) is a low-grade, soft tissue lesion that is notable for its infiltration and local recurrence and its inability to metastasize.
  • METHODS: The surgical pathology files of The Johns Hopkins Hospital revealed 17 patients with a diagnosis of DF/DT with prior cytology in a 16-year period (1989-2005).
  • In patients with archived tissue, an immunohistochemical panel was performed that included beta-catenin, desmin, CD-34, and c-kit.
  • Predominantly bland spindled cells with long, fusiform nuclei and metachromatic matrix material were present in most tumors.
  • The tumor cells were present both singly and as fragments embedded in the matrix.
  • Nine patients had paraffin-embedded tissue samples available for immunohistochemical staining.
  • [MeSH-major] Fibromatosis, Aggressive / metabolism. Fibromatosis, Aggressive / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Antigens, CD34 / analysis. Biopsy, Fine-Needle. Child. Desmin / analysis. Diagnosis, Differential. Female. Humans. Immunohistochemistry. Male. Middle Aged. Proto-Oncogene Proteins c-kit / analysis. beta Catenin / analysis

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  • (PMID = 17477374.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD34; 0 / Desmin; 0 / beta Catenin; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit
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37. Liu QY, Chen JY, Liang BL, Li HG, Gao M, Lin XF: [Imaging manifestations and pathologic features of soft tissue desmoid-type fibromatosis]. Ai Zheng; 2008 Dec;27(12):1287-92
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  • [Title] [Imaging manifestations and pathologic features of soft tissue desmoid-type fibromatosis].
  • BACKGROUND & OBJECTIVE: Soft tissue desmoid-type fibromatosis is a type of benign, infiltrative tumors which are rarely seen.
  • This study was to analyze CT and MR manifestations and pathologic features of soft tissue desmoid-type fibromatosis to improve its diagnostic accuracy.
  • METHODS: A total of 34 soft tissue desmoid-type fibromatosis from 29 patients, including 20 primary and 14 recurrent tumors were analyzed.
  • Features of the growth pattern, CT or MRI findings and pathologic appearances of the tumors were studied.
  • RESULTS: The mean size of the 34 tumors was 6.5 cm.
  • Of the 34 tumors, 31(91.2%) had an ill-defined or partially ill-defined margin, 31(91.2%) had a lobulated or irregular contour, 17(50.0%) had neurovascular involvement, 15(44.1%) had bone involvement, 23(67.6%) had extra-compartmental extension.
  • Among the eight tumors scanned by CT, six showed slight hypodensity on the non-enhanced CT scan, and seven were inhomogeneously enhanced after contrast injection.
  • Twenty-six tumors scanned by MRI appeared either iso-or slightly hyperintense on T1W images, hyperintense on T2W images, and moderate or intense enhancement after gadolinium administration.
  • Heterogeneous signal intensity was detected in 88.5%(23/26) tumors.
  • Linear and curvilinear areas of signal void interspersed throughout the tumors were found in 22 tumors(84.6%) on both T1W and T2W images.
  • Histologic analysis revealed that the tumor was composed of spindle cells and collagen bundles, with a variable amount of intermingled collagen surrounding the spindle cells.
  • Nuclear atypia was not seen,and occasional mitoses were present in the tumor cells.
  • CONCLUSION: The characteristic manifestations of CT or MR images of desmoid-type fibromatosis provide important evidence to discriminate benign and malignant soft tissue tumors.
  • [MeSH-major] Extremities / radiography. Fibromatosis, Aggressive / diagnosis. Magnetic Resonance Imaging / methods. Soft Tissue Neoplasms / diagnosis. Tomography, X-Ray Computed / methods
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Child, Preschool. Female. Humans. Image Enhancement. Infant. Male. Middle Aged. Neoplasm Recurrence, Local. Retrospective Studies. Thoracic Wall / pathology. Thoracic Wall / radiography. Young Adult

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  • (PMID = 19079995.001).
  • [Journal-full-title] Ai zheng = Aizheng = Chinese journal of cancer
  • [ISO-abbreviation] Ai Zheng
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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38. Mankin HJ, Hornicek FJ, Springfield DS: Extra-abdominal desmoid tumors: a report of 234 cases. J Surg Oncol; 2010 Oct 1;102(5):380-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Extra-abdominal desmoid tumors: a report of 234 cases.
  • BACKGROUND/OBJECTIVES: To report on the clinical presentation and outcome for 234 patients with extra-abdominal desmoids tumors.
  • METHODS: Since 1977, the authors have treated 234 patients with extra-abdominal desmoid tumors.
  • The tumors arose adjacent to muscles or bones and the largest number were in the foot, shoulder thigh and calf.
  • None of the patients died of disease but 5 required amputations.
  • CONCLUSIONS: The authors concluded that despite the benign nature of the disease, these patients are difficult to treat and the results are sometimes considerably less than optimal.
  • [MeSH-major] Fibromatosis, Abdominal / surgery. Fibromatosis, Aggressive / surgery. Neoplasm Recurrence, Local / surgery. Neoplasms, Multiple Primary / surgery

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  • [Copyright] J. Surg. Oncol. 2010;102:380-384. © 2009 Wiley-Liss, Inc.
  • (PMID = 19877160.001).
  • [ISSN] 1096-9098
  • [Journal-full-title] Journal of surgical oncology
  • [ISO-abbreviation] J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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39. Montagliani L, Duverger V: [Desmoid tumors]. J Chir (Paris); 2008 Jan-Feb;145(1):20-6

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Desmoid tumors].
  • Desmoid tumors are a rare form of malignancy with a great propensity for local extension and recurrence.
  • They typically occur in the abdominal wall or within the abdomen but also may occur extra-abdominally.
  • [MeSH-major] Fibromatosis, Abdominal / surgery. Fibromatosis, Aggressive / surgery. Mesentery. Peritoneal Neoplasms / surgery
  • [MeSH-minor] Algorithms. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Chemotherapy, Adjuvant. Evidence-Based Medicine. Humans. Neoplasm Recurrence, Local / surgery. Prognosis. Radiotherapy, Adjuvant. Treatment Outcome

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  • (PMID = 18438278.001).
  • [ISSN] 0021-7697
  • [Journal-full-title] Journal de chirurgie
  • [ISO-abbreviation] J Chir (Paris)
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Number-of-references] 29
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40. Dafford K, Kim D, Nelson A, Kline D: Extraabdominal desmoid tumors. Neurosurg Focus; 2007;22(6):E21

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Extraabdominal desmoid tumors.
  • OBJECT: Desmoid tumors are fibrous, slow-growing, nonmalignant tumors with a low potential for metastasis.
  • METHODS: The authors undertook a retrospective study of 15 desmoid tumors in 11 women and four men (ranging in age from 32 to 67 years; median 48 years) treated at their institution.
  • This study included further resection for recurrent tumors in nine of 15 patients (60%).
  • There was tumor recurrence in two patients (13%) leading to further surgical intervention.
  • CONCLUSIONS: This case series included many recurrent desmoid tumors of the brachial plexus.
  • Most of these lesions were relatively large tumors, predominantly involved with the plexal elements adding to the challenge of the resection.
  • Currently, function-sparing excision is considered the optimal treatment for desmoid tumors arising in extraabdominal sites.
  • Adjunctive radiation or brachytherapy is reserved for a patient with further recurrence in whom resection would be disfiguring or in whom the disease is more refractory.
  • [MeSH-major] Fibromatosis, Abdominal / diagnosis. Fibromatosis, Abdominal / surgery
  • [MeSH-minor] Adult. Aged. Female. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Neoplasm Recurrence, Local / diagnosis. Neoplasm Recurrence, Local / pathology. Neoplasm Recurrence, Local / surgery. Postoperative Complications / diagnosis. Postoperative Complications / pathology. Postoperative Complications / surgery. Retrospective Studies

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  • (PMID = 17613213.001).
  • [ISSN] 1092-0684
  • [Journal-full-title] Neurosurgical focus
  • [ISO-abbreviation] Neurosurg Focus
  • [Language] eng
  • [Publication-type] Case Reports; Comparative Study; Journal Article
  • [Publication-country] United States
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41. Suresh CS, Ali AA: Desmoid tumor of the tongue. Med Oral Patol Oral Cir Bucal; 2008 Dec;13(12):E761-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Desmoid tumor of the tongue.
  • Desmoid tumors are rare neoplasms accounting for 0.03% of all neoplasms and have an estimated incidence of 2 to 4 per million per year.
  • World Health Organization currently refers to all of the deep types of fibromatosis as desmoid-type of fibromatoses.
  • The term "desmoid" refers to the hard, tendon-like appearance of the tumor.
  • About fifty percent of desmoid tumors arise in the abdominal region.
  • The extra-abdominal desmoid tumors present a difficult problem in recognition and management especially because of the striking discrepancy between its deceptively harmless microscopic appearance and its potential to attain a large size, to recur, and to infiltrate neighboring tissues in the manner of a fibrosarcoma.
  • Desmoid tumors are very rare in the oral cavity with less than 5% of cases constituting oral soft tissue fibromatosis.
  • A 22-year old mentally retarded female patient with desmoid tumor occurring in the tongue is presented here.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Tongue Neoplasms / pathology

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  • (PMID = 19047962.001).
  • [ISSN] 1698-6946
  • [Journal-full-title] Medicina oral, patología oral y cirugía bucal
  • [ISO-abbreviation] Med Oral Patol Oral Cir Bucal
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Spain
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42. Lanckohr C, Debiec-Rychter M, Müller O, Homann HH, Lehnhardt M, Herter P, Kuhnen C: [Gardner fibroma: case report and discussion of a new soft tissue tumor entity]. Pathologe; 2010 Mar;31(2):97-105

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Gardner fibroma: case report and discussion of a new soft tissue tumor entity].
  • Gardner fibroma represents a rare and recently described soft tissue tumor entity in children and young adults.
  • The case of a 13-year-old male patient with Gardner fibroma and osteoma and multicentric desmoid type fibromatosis in his mother is presented with detection of a (heterozygotic) germline mutation of the APC gene leading to a de novo stop codon (deletion of base pairs 5033-5036).
  • FISH analysis revealed a structural loss of heterozygosity (LOH) in the APC gene on chromosomal locus 5q21 in one out of five analysed desmoids of the mother, no LOH of APC gene in the Gardner fibroma.
  • Gardner fibroma in children and young adults may serve as an indicator lesion for familial adenomatous polyposis (FAP), Gardner syndrome, a familial desmoid type fibromatosis without other manifestations of APC or a new APC gene mutation.
  • For the clinician, this diagnosis should be commented upon accordingly by the surgical pathologist.
  • As the result of a detected APC gene mutation, continuous follow-up for the development of colorectal tumors and desmoid type fibromatosis as well as a familial screening for FAP is recommended.
  • [MeSH-major] Fibromatosis, Aggressive / genetics. Fibromatosis, Aggressive / pathology. Gardner Syndrome / genetics. Gardner Syndrome / pathology. Genes, APC. Germ-Line Mutation / genetics. Loss of Heterozygosity. Osteoma / genetics. Osteoma / pathology. Soft Tissue Neoplasms / genetics. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Adolescent. Adult. Biomarkers, Tumor / genetics. Breast Neoplasms / genetics. Breast Neoplasms / pathology. Chromosome Deletion. Chromosomes, Human, Pair 5 / genetics. Codon, Terminator / genetics. DNA Mutational Analysis. Female. Follow-Up Studies. Heterozygote Detection. Humans. In Situ Hybridization, Fluorescence. Male. Neoplasm Recurrence, Local / genetics. Neoplasm Recurrence, Local / pathology. Young Adult

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  • (PMID = 20063100.001).
  • [ISSN] 1432-1963
  • [Journal-full-title] Der Pathologe
  • [ISO-abbreviation] Pathologe
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Codon, Terminator
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43. Kitazawa S, Kitazawa R, Obayashi C, Yamamoto T: Desmoid tumor with ossification in chest wall: possible involvement of BAMBI promoter hypermethylation in metaplastic bone formation. J Bone Miner Res; 2005 Aug;20(8):1472-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Desmoid tumor with ossification in chest wall: possible involvement of BAMBI promoter hypermethylation in metaplastic bone formation.
  • A rare case of desmoid-type fibromatosis with focal metaplastic bone in the chest wall suggested that enhanced responsiveness to BMP signaling by decreasing BAMBI expression through promoter hypermethylation plays a crucial role in the formation of metaplastic bone.
  • INTRODUCTION: Desmoid-type fibromatosis, originating from mesenchymal cells with myofibroblastic features, is a locally aggressive and frequently recurring infiltrative lesion.
  • RESULTS: Accumulation of altered beta-catenin associated with a somatic heterozygous activating mutation in codon 41 was detected in the typical desmoid-type fibromatosis and at the ossifying focus.
  • Hypermethylation of the BAMBI promoter was observed in microdissected tissue from the ossifying focus but not in that from the typical desmoid-type fibromatosis.
  • [MeSH-major] DNA Methylation. Fibromatosis, Aggressive / pathology. Membrane Proteins / genetics. Ossification, Heterotopic / genetics. Thoracic Wall

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  • (PMID = 16007344.001).
  • [ISSN] 0884-0431
  • [Journal-full-title] Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research
  • [ISO-abbreviation] J. Bone Miner. Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / BAMBI protein, human; 0 / BMP2 protein, human; 0 / Bone Morphogenetic Protein 2; 0 / Bone Morphogenetic Proteins; 0 / Membrane Proteins; 0 / Transforming Growth Factor beta
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44. Schlemmer M: Desmoid tumors and deep fibromatoses. Hematol Oncol Clin North Am; 2005 Jun;19(3):565-71, vii-viii

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Desmoid tumors and deep fibromatoses.
  • Desmoid tumors (also called deep fibromatoses) are rare benign tumors associated with pregnancy and Gardner syndrome.
  • These tumors are characterized by bland-appearing fibroblasts, indistinct margins, and an ability to cause pathology by local invasion and recurrence.
  • They arise in the abdominal cavity, in the abdominal wall, or in the extremities/trunk, each with a slightly different biologic behavior.
  • Though they are not cancer and do not metastasize, desmoids can cause significant morbidity and occasionally death through local/regional invasion of critical structures.
  • This article highlights the biology and clinical features of desmoid tumors.
  • [MeSH-major] Fibromatosis, Aggressive. Gardner Syndrome

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  • (PMID = 15939197.001).
  • [ISSN] 0889-8588
  • [Journal-full-title] Hematology/oncology clinics of North America
  • [ISO-abbreviation] Hematol. Oncol. Clin. North Am.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 44
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45. Hauben EI, Jundt G, Cleton-Jansen AM, Yavas A, Kroon HM, Van Marck E, Hogendoorn PC: Desmoplastic fibroma of bone: an immunohistochemical study including beta-catenin expression and mutational analysis for beta-catenin. Hum Pathol; 2005 Sep;36(9):1025-30
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Desmoplastic fibroma of bone: an immunohistochemical study including beta-catenin expression and mutational analysis for beta-catenin.
  • Desmoplastic fibroma of bone is a very rare primary bone tumor morphologically resembling desmoid-type fibromatosis, its much more common counterpart of soft tissue.
  • The aim of this study is to investigate the immunohistochemical profile and the involvement of the beta-catenin pathway in desmoplastic fibroma as it is known in desmoid-type fibromatosis.
  • Immunohistochemistry was performed on 13 cases of desmoplastic fibroma for muscle-specific markers, estrogen and progesterone receptors, CD117, beta-catenin, and the potential downstream target of beta-catenin, namely, cyclin D1.
  • The epidemiological, histological, and immunohistochemical findings in desmoplastic fibroma are suggestive of desmoplastic fibroma being the bony counterpart of the more common desmoid-type fibromatosis of soft tissue.
  • However, the beta-catenin pathway does not seem to have the same essential role in the tumorigenesis of desmoplastic fibroma, as it has in desmoid-type fibromatosis.
  • [MeSH-major] Bone Neoplasms / metabolism. Cytoskeletal Proteins / genetics. Cytoskeletal Proteins / metabolism. Fibroma, Desmoplastic / metabolism. Trans-Activators / genetics. Trans-Activators / metabolism

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  • (PMID = 16153468.001).
  • [ISSN] 0046-8177
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / CTNNB1 protein, human; 0 / Cytoskeletal Proteins; 0 / Trans-Activators; 0 / beta Catenin
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46. Sakorafas GH, Nissotakis C, Peros G: Abdominal desmoid tumors. Surg Oncol; 2007 Aug;16(2):131-42

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Abdominal desmoid tumors.
  • Desmoid tumors are rare, benign, fibromatous lesions that are the result of abnormal proliferation of myofibroblasts.
  • Desmoid tumors can be classified as extra-abdominal and abdominal.
  • Abdominal desmoid tumors are either superficial or intraabdominal.
  • These tumors are associated with a high recurrence rates, even if their microscopic characters indicate a benign disease; their biologic behavior often indicates rather a "malignant" disease, which can cause even the death.
  • Intraabdominal desmoid tumors can engulf surrounding viscera and vessels, thereby greatly complicating their surgical treatment.
  • Simple observation is a reasonable management option for asymptomatic patients; spontaneous regression of these tumors may be observed.
  • Complete excision is the treatment of choice for tumors causing symptoms or complications.
  • [MeSH-major] Abdominal Neoplasms / pathology. Abdominal Neoplasms / therapy. Fibromatosis, Aggressive / pathology. Fibromatosis, Aggressive / therapy
  • [MeSH-minor] Algorithms. Diagnosis, Differential. Diagnostic Imaging. Genetic Testing. Humans. Medical History Taking

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  • (PMID = 17719772.001).
  • [ISSN] 0960-7404
  • [Journal-full-title] Surgical oncology
  • [ISO-abbreviation] Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Netherlands
  • [Number-of-references] 86
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47. Fiore M, Rimareix F, Mariani L, Domont J, Collini P, Le Péchoux C, Casali PG, Le Cesne A, Gronchi A, Bonvalot S: Desmoid-type fibromatosis: a front-line conservative approach to select patients for surgical treatment. Ann Surg Oncol; 2009 Sep;16(9):2587-93
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Desmoid-type fibromatosis: a front-line conservative approach to select patients for surgical treatment.
  • PURPOSE: Surgery is still the standard treatment for desmoid-type fibromatosis (DF).
  • The disease remained stable in more than half of patients.
  • This study was designed to evaluate this approach on the natural history of the disease in a larger series of patients.
  • RESULTS: Seventy-four patients presented with primary tumor, 68 with recurrence.
  • Half of patients had medium-term stable disease after W&S or MT.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Fibromatosis, Aggressive / surgery. Neoplasm Recurrence, Local / diagnosis

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  • (PMID = 19568815.001).
  • [ISSN] 1534-4681
  • [Journal-full-title] Annals of surgical oncology
  • [ISO-abbreviation] Ann. Surg. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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48. Shi B, Zhu Y, Xu Z, Liu Y, Zheng B, Qi T: Aggressive fibromatosis in the urological system. Report of two adult patients and review of the literature. Urol Int; 2007;78(1):93-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Aggressive fibromatosis in the urological system. Report of two adult patients and review of the literature.
  • Aggressive fibromatoses (AF) are locally aggressive neoplasms that do not metastasize but are frequently associated with one or more recurrences and subsequent associated morbidity.
  • AF in the urological system is quite rare and has mainly been described in single case reports or as isolated cases in a large series of extra-abdominal desmoid tumors.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Genital Neoplasms, Male / diagnosis. Scrotum. Urinary Bladder Neoplasms / diagnosis
  • [MeSH-minor] Adult. Cystoscopy. Diagnosis, Differential. Follow-Up Studies. Humans. Male. Tomography, X-Ray Computed. Urography. Urologic Surgical Procedures, Male / methods


49. Okuno S: The enigma of desmoid tumors. Curr Treat Options Oncol; 2006 Nov;7(6):438-43
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The enigma of desmoid tumors.
  • Desmoid tumors (aggressive fibromatosis) are rare neoplastic tumors that may occur sporadically or in association with familial adenomatous polyposis (FAP).
  • The etiology of these tumors is unknown, but hormonal, genetic, and physical factors play a role in their development and growth.
  • A distinction is often made between desmoids in patients with FAP and those in patients without FAP, but clinically these tumors are treated the same; the only difference is the preferential intra-abdominal location of FAP desmoids.
  • The goal of desmoid treatment is local control.
  • In addition, because desmoids spontaneously regress, any claim of successful intervention must be viewed skeptically.
  • Patients with symptomatic, progressive disease who can tolerate chemotherapy should be presented with the option of low-dose or standard antisarcoma chemotherapy.
  • The treatment of desmoid tumors remains an enigma.
  • Further clinical trials are needed to help the clinician navigate his or her way through the morass of desmoid tumor therapies.
  • [MeSH-major] Fibromatosis, Abdominal / diagnosis. Fibromatosis, Abdominal / drug therapy

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  • (PMID = 17032556.001).
  • [ISSN] 1527-2729
  • [Journal-full-title] Current treatment options in oncology
  • [ISO-abbreviation] Curr Treat Options Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Anti-Inflammatory Agents, Non-Steroidal; 094ZI81Y45 / Tamoxifen; 9008-11-1 / Interferons
  • [Number-of-references] 22
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50. Tamura K, Tani M, Kinoshita H, Yamaue H: Mesenteric desmoid tumor of the interposed jejunal pouch after total gastrectomy. World J Surg Oncol; 2006;4:27

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Mesenteric desmoid tumor of the interposed jejunal pouch after total gastrectomy.
  • BACKGROUND: Desmoid tumor is a rare entity, and most desmoid tumors are located in abdominal wall or extra-abdominal tissues.
  • Occurrence of desmoid tumor in mesentry is extremely rare.
  • CASE PRESENTATION: we report a mesenteric desmoid tumor in a 73-years-old woman who had undergone total gastrectomy reconstructed with jejunal pouch interposition for gastric carcinoma.
  • After 1 year, a tumor was originating from mesentery of the interposed jejunal pouch was identified, and the patient underwent resection of the large mass which was found to invade pancreas.
  • Histological examination revealed desmoid tumor.
  • CONCLUSION: Desmoid tumor is rare, and it was difficult for the differential diagnosis of desmoid tumor or recurrent tumor.

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  • (PMID = 16740152.001).
  • [ISSN] 1477-7819
  • [Journal-full-title] World journal of surgical oncology
  • [ISO-abbreviation] World J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC1481628
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51. González MA, Menéndez R, Ayala JM, Herrero M, Cuesta J, Domínguez A, Martínez M, Graña JL, Pozo F: [Intra-abdominal desmoid tumor]. Cir Esp; 2005 Jun;77(6):362-4
Genetic Alliance. consumer health - Desmoid Tumor.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Intra-abdominal desmoid tumor].
  • [Transliterated title] Tumor desmoide intraabdominal.
  • Aggressive fibromatosis (desmoid tumor) are rare connective tissue tumors that occur sporadically or in association with familial adenomatous polyposis.
  • Biopsy is required to establish the diagnosis.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Fibromatosis, Aggressive / surgery. Retroperitoneal Neoplasms / pathology. Retroperitoneal Neoplasms / surgery

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  • (PMID = 16420952.001).
  • [ISSN] 0009-739X
  • [Journal-full-title] Cirugía española
  • [ISO-abbreviation] Cir Esp
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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52. Hosalkar HS, Torbert JT, Fox EJ, Delaney TF, Aboulafia AJ, Lackman RD: Musculoskeletal desmoid tumors. J Am Acad Orthop Surg; 2008 Apr;16(4):188-98

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Musculoskeletal desmoid tumors.
  • Desmoid tumors are benign tumors that exhibit varying degrees of local aggressiveness and diverse growth patterns.
  • Magnetic resonance imaging remains the modality of choice for assessment of the nature and size of the soft-tissue lesion and involvement of surrounding structures.
  • Despite the benign nature of these tumors, multidisciplinary care is needed to provide combined treatment options.
  • Chemotherapy in low doses is an excellent first-round treatment in any patient in whom contemplated local treatment may produce local morbidity and adjacent tissue injury.
  • [MeSH-major] Fibromatosis, Aggressive. Muscle Neoplasms
  • [MeSH-minor] Humans. Soft Tissue Neoplasms / diagnosis. Soft Tissue Neoplasms / pathology. Soft Tissue Neoplasms / therapy

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  • (PMID = 18390481.001).
  • [ISSN] 1067-151X
  • [Journal-full-title] The Journal of the American Academy of Orthopaedic Surgeons
  • [ISO-abbreviation] J Am Acad Orthop Surg
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 65
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53. Tkachev SI, Aliev MD, Glebovskaia VV, Ivanov SM, Trofimova OP, Karapetian RM, Gutnik RA, Bokhian AIu: [Thermoradiotherapy as a component of desmoid tumor management: 10-year experience]. Vopr Onkol; 2005;51(3):347-9
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  • [Title] [Thermoradiotherapy as a component of desmoid tumor management: 10-year experience].
  • Data on radio- and thermoradiotherapy of 83 patients with extra-abdominal desmoid tumors are discussed.
  • Monitoring tumor temperature during local hyperthermia is a factor of relapse-free survival of vital importance.
  • [MeSH-major] Fibromatosis, Aggressive / radiotherapy. Hyperthermia, Induced
  • [MeSH-minor] Disease-Free Survival. Follow-Up Studies. Humans. Radiotherapy / methods. Treatment Outcome

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  • (PMID = 16279100.001).
  • [ISSN] 0507-3758
  • [Journal-full-title] Voprosy onkologii
  • [ISO-abbreviation] Vopr Onkol
  • [Language] rus
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Russia (Federation)
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54. Leal RF, Silva PV, Ayrizono Mde L, Fagundes JJ, Amstalden EM, Coy CS: Desmoid tumor in patients with familial adenomatous polyposis. Arq Gastroenterol; 2010 Oct-Dec;47(4):373-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Desmoid tumor in patients with familial adenomatous polyposis.
  • CONTEXT: Desmoid tumors constitute one of the most important extraintestinal manifestations of familial adenomatous polyposis.
  • The development of desmoids is responsible for increasing morbidity and mortality rates in cases of familial adenomatous polyposis.
  • OBJECTIVES: To evaluate the occurrence of desmoid tumors in familial adenomatous polyposis cases following prophylactic colectomy and to present patient outcome.
  • Desmoid tumors were found in nine (13.2%) of these patients, who were studied retrospectively by consulting their medical charts with respect to clinical and surgical data.
  • RESULTS: Of nine patients, seven (77.8%) were submitted to laparotomy for tumor resection.
  • Desmoid tumors were found in the abdominal wall in 3/9 cases (33.3%) and in an intra-abdominal site in the remaining six cases (66.7%).
  • Median time elapsed between ileal pouch-anal anastomosis and diagnosis of desmoid tumor was 37.5 months (range 14-60 months), while the median time between colectomy with ileorectal anastomosis and diagnosis was 63.7 months (range 25-116 months).
  • In 6/9 (66.7%) patients with desmoid tumors, the disease was either under control or there was no evidence of tumor recurrence at a follow-up visit made a mean of 63.1 months later (range 12-240 months).
  • CONCLUSIONS: Desmoid tumors were found in 13.2% of cases of familial adenomatous polyposis following colectomy; therefore, familial adenomatous polyposis patients should be followed-up and surveillance should include abdominal examination to detect signs and symptoms.
  • [MeSH-major] Abdominal Neoplasms / surgery. Colectomy. Fibromatosis, Abdominal / surgery. Fibromatosis, Aggressive / surgery
  • [MeSH-minor] Abdominal Wall / surgery. Adult. Anastomosis, Surgical. Female. Follow-Up Studies. Humans. Male. Middle Aged. Treatment Outcome. Young Adult


55. Chummun S, McLean NR, Abraham S, Youseff M: Desmoid tumour of the breast. J Plast Reconstr Aesthet Surg; 2010 Feb;63(2):339-45
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  • [Title] Desmoid tumour of the breast.
  • Desmoid tumour of the breast is a rare fibroblastic tumour whose spectrum ranges from being locally inert to aggressive and destructive, and represents 0.2% of all breast tumours.
  • Ultrasound showed a well-defined, hypoechoic mass arising within the muscles of the anterior chest, deep beneath the implant and not involving the underlying rib.
  • A provisional diagnosis of scarring and fibroblastic proliferation was made.
  • A final diagnosis of aggressive fibromatosis was made.
  • Although the association of desmoid tumour and breast implants has been described, this case is unique as the FNA was highly suggestive of a silicone granuloma and the diagnosis of desmoid tumour was made on definitive pathology.
  • The aetiology of desmoid tumours is reviewed and current treatment modalities discussed.
  • [MeSH-major] Breast Neoplasms / diagnosis. Breast Neoplasms / surgery. Fibromatosis, Aggressive / diagnosis. Fibromatosis, Aggressive / surgery

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  • [Copyright] 2008 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.
  • (PMID = 19059821.001).
  • [ISSN] 1878-0539
  • [Journal-full-title] Journal of plastic, reconstructive & aesthetic surgery : JPRAS
  • [ISO-abbreviation] J Plast Reconstr Aesthet Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
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56. Nieuwenhuis MH, Hartgrink HH, Meijer S, Menko FH, Vasen HF: [Desmoid tumour as indication of familial adenomatous polyposis]. Ned Tijdschr Geneeskd; 2010;154:A2235
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  • [Title] [Desmoid tumour as indication of familial adenomatous polyposis].
  • In two patients, a man aged 43 and a woman aged 40 years, who presented with a desmoid tumour, familial adenomatous polyposis (FAP) was diagnosed three and six years later, respectively.
  • Desmoid-type fibromatoses usually develop sporadically, but may also be an extracolonic manifestation of FAP.
  • All patients with desmoids diagnosed who are under age 60, or with desmoids located intra-abdominally or in the abdominal wall, should be referred for colonic and genetic evaluation.
  • In all further patients with a desmoid tumour, the possibility of FAP should be considered and patient data and the family history should be evaluated thoroughly.
  • [MeSH-major] Adenomatous Polyposis Coli / diagnosis. Adenomatous Polyposis Coli / pathology. Fibromatosis, Aggressive / diagnosis. Fibromatosis, Aggressive / pathology
  • [MeSH-minor] Adult. Colonic Neoplasms / diagnosis. Colonic Neoplasms / secondary. Colonic Neoplasms / surgery. Female. Genes, APC. Genetic Predisposition to Disease. Humans. Male. Mutation. Neoplasm Metastasis. Rectal Neoplasms / diagnosis. Rectal Neoplasms / secondary. Rectal Neoplasms / surgery

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  • (PMID = 20977806.001).
  • [ISSN] 1876-8784
  • [Journal-full-title] Nederlands tijdschrift voor geneeskunde
  • [ISO-abbreviation] Ned Tijdschr Geneeskd
  • [Language] dut
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Netherlands
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57. Oddsson SJ, Kristvinsson H, Jónsson JG, Torfason B, Gudbjartsson T: [Desmoid tumor of chest wall--an important differential diagnosis to malignancies]. Laeknabladid; 2006 Nov;92(11):777-80
Genetic Alliance. consumer health - Desmoid Tumor.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Desmoid tumor of chest wall--an important differential diagnosis to malignancies].
  • [Transliterated title] Desmoid-aexli í brjóstvegg--mikilvaeg mismunagreining vieth illkynja mein.
  • Desmoid tumors are rare solid tumors that arise from musculoaponeurotic tissues.
  • They are classified as benign as they do not metastasize.
  • Desmoid tumors can, however, exhibit rapid local growth and clinically they can mimic sarcomas.
  • Their histological appearance can also resemble some malignant neoplasms such as low grade sarcomas, rendering the differential diagnosis difficult.
  • The tumor increased in size over several weeks and caused local radiating chest pain.
  • Open biopsy revealed a desmoid tumor.
  • The tumor was resected together with a part of the anterior hemithorax, and the defect in the chest wall covered with a Goretex-patch.
  • Six months postoperatively, the patient is doing well with no signs of locally recurrent disease.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Thoracic Neoplasms / diagnosis. Thoracic Wall / pathology
  • [MeSH-minor] Diagnosis, Differential. Female. Humans. Middle Aged. Tomography, X-Ray Computed

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  • (PMID = 17093329.001).
  • [ISSN] 0023-7213
  • [Journal-full-title] Læknablađiđ
  • [ISO-abbreviation] Laeknabladid
  • [Language] ice
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Iceland
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58. Sturt NJ, Clark SK: Current ideas in desmoid tumours. Fam Cancer; 2006;5(3):275-85; discussion 287-8

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Current ideas in desmoid tumours.
  • Desmoid tumours are rare neoplasms of fibroblastic origin which arise with disproportionate frequency in patients with familial adenomatous polyposis (FAP).
  • Risk factors include trauma, having a distal germline APC mutation, having a family history of desmoids, and probably oestrogens.
  • Desmoids are now known to be true neoplasms but with normal telomere length and telomerase activity.
  • FAP-associated tumours seem to carry biallelic APC mutations, one of which lies in the distal part of the gene.
  • Such loss of wild-type APC seems to occur relatively late in tumour development.
  • FAP-associated desmoids tend to arise in the abdomen or abdominal wall.
  • CT scanning gives the best information on tumour anatomy whilst T2-weighted MRI indicates likely behaviour.
  • Cytotoxic chemotherapy is an option in unresectable tumours.
  • Surgery is a reasonable first-line treatment in abdominal wall tumours but is risky for intra-abdominal tumours and may necessitate massive small bowel resection.
  • Desmoids are the greatest remaining challenge in the management of FAP and further research into their aetiology needs to be combined with multicentre clinical trials of new treatments in order to improve management of the disease.
  • [MeSH-major] Adenomatous Polyposis Coli / complications. Fibromatosis, Abdominal / etiology. Fibromatosis, Aggressive / etiology

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  • (PMID = 16998673.001).
  • [ISSN] 1389-9600
  • [Journal-full-title] Familial cancer
  • [ISO-abbreviation] Fam. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Netherlands
  • [Number-of-references] 68
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59. Leithner A, Gapp M, Radl R, Pascher A, Krippl P, Leithner K, Windhager R, Beham A: Immunohistochemical analysis of desmoid tumours. J Clin Pathol; 2005 Nov;58(11):1152-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Immunohistochemical analysis of desmoid tumours.
  • BACKGROUND/AIMS: Although the standard treatment for desmoid tumours is complete surgical resection with wide margins, the optimal adjuvant treatment for recurrent or inoperable disease is unclear, often being based on sporadic immunohistochemical reports with a low number of cases.
  • METHODS: One hundred and sixteen tissue samples from 80 patients (49 female, 31 male; mean age, 34 years; range, 0-83) with desmoid tumours (46 extra-abdominal, 21 abdominal, 13 intra-abdominal) were tested for oestrogen receptors alpha and beta, progesterone and androgen receptors, and somatostatin, in addition to HER2, cathepsin D, Ki-67, and c-KIT by immunohistochemistry.
  • Positive staining for the androgen receptor was found in six extra-abdominal cases.
  • Staining for oestrogen receptor beta was positive in four extra-abdominal, two abdominal, and one intra-abdominal case.
  • Staining for somatostatin was positive in six extra-abdominal, two abdominal, and one intra-abdominal case, and staining for cathepsin D was positive in all cases.
  • Positive staining for Ki-67 was found in 14 extra-abdominal, three abdominal, and three intra-abdominal cases.
  • C-KIT was detectable in one abdominal case only.
  • CONCLUSIONS: The data from this immunohistochemical study show that the published effects of antioestrogens and imatinib mesylate in the treatment of aggressive fibromatoses may not be attributable to oestrogen receptor alpha or c-KIT expression.
  • [MeSH-major] Biomarkers, Tumor / analysis. Fibromatosis, Aggressive / metabolism. Soft Tissue Neoplasms / chemistry
  • [MeSH-minor] Abdominal Neoplasms / chemistry. Abdominal Neoplasms / drug therapy. Abdominal Neoplasms / pathology. Adolescent. Adult. Aged. Aged, 80 and over. Cathepsin D / analysis. Chemotherapy, Adjuvant. Child. Child, Preschool. Female. Humans. Infant. Infant, Newborn. Ki-67 Antigen / analysis. Male. Middle Aged. Neoplasm Proteins / analysis. Proto-Oncogene Proteins c-kit / analysis. Receptors, Androgen / analysis. Receptors, Estrogen / analysis. Receptors, Progesterone / analysis. Somatostatin / analysis

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  • (PMID = 16254103.001).
  • [ISSN] 0021-9746
  • [Journal-full-title] Journal of clinical pathology
  • [ISO-abbreviation] J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Ki-67 Antigen; 0 / Neoplasm Proteins; 0 / Receptors, Androgen; 0 / Receptors, Estrogen; 0 / Receptors, Progesterone; 51110-01-1 / Somatostatin; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit; EC 3.4.23.5 / Cathepsin D
  • [Other-IDs] NLM/ PMC1770757
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60. Jalini L, Hemming D, Bhattacharya V: Intraabdominal desmoid tumour presenting with perforation. Surgeon; 2006 Apr;4(2):114-6

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Intraabdominal desmoid tumour presenting with perforation.
  • We present a rare case of a desmoid tumour presenting with perforation of the small bowel.
  • Although desmoid is classified pathologically as a benign tumour, its infiltrative nature leads to a locally aggressive mass, which can invade surrounding structures and organs making surgical resection difficult.
  • Some unresectable tumours show oestrogen receptor positive cells and can be managed with tamoxifen.
  • [MeSH-major] Fibromatosis, Abdominal / pathology. Intestinal Perforation / etiology. Intestine, Small. Mesentery. Peritoneal Neoplasms / pathology

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  • (PMID = 16623170.001).
  • [ISSN] 1479-666X
  • [Journal-full-title] The surgeon : journal of the Royal Colleges of Surgeons of Edinburgh and Ireland
  • [ISO-abbreviation] Surgeon
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Scotland
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61. Bonvalot S, Rimareix F, Causeret S, Le Péchoux C, Boulet B, Terrier P, Le Cesne A, Muret J: Hyperthermic isolated limb perfusion in locally advanced soft tissue sarcoma and progressive desmoid-type fibromatosis with TNF 1 mg and melphalan (T1-M HILP) is safe and efficient. Ann Surg Oncol; 2009 Dec;16(12):3350-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Hyperthermic isolated limb perfusion in locally advanced soft tissue sarcoma and progressive desmoid-type fibromatosis with TNF 1 mg and melphalan (T1-M HILP) is safe and efficient.
  • BACKGROUND: In a prior randomized phase II trial comparing hyperthermic isolated limb perfusion (HILP) with four different doses of tumor necrosis factor alpha (TNF-alpha), no dose effect was detected for response, but systemic toxicity was far lower with low-dose TNF-alpha.
  • The objective of the present study was to confirm these data on a larger sample size of locally advanced or recurrent extremity soft tissue sarcomas with low-dose TNF-alpha.
  • The remnant tumor was resected 2 months later.
  • Tumor grades (FNCLCC) were 1 (23 patients), 2 (34 patients), and 3 (43 patients).
  • Age, sex, tumor size, recurrence, uni- or multifocality, grade, preoperative chemotherapy, and a previously irradiated field were not predictive of response or local toxicity.
  • [MeSH-major] Antineoplastic Agents, Alkylating / administration & dosage. Chemotherapy, Cancer, Regional Perfusion. Extremities / pathology. Fibromatosis, Aggressive / therapy. Hyperthermia, Induced. Melphalan / administration & dosage. Sarcoma / therapy. Tumor Necrosis Factor-alpha / administration & dosage
  • [MeSH-minor] Adolescent. Adult. Aged. Drug Therapy, Combination. Female. Follow-Up Studies. Humans. Male. Middle Aged. Neoplasm Staging. Prognosis. Prospective Studies. Survival Rate. Young Adult

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  • (PMID = 19830495.001).
  • [ISSN] 1534-4681
  • [Journal-full-title] Annals of surgical oncology
  • [ISO-abbreviation] Ann. Surg. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 0 / Tumor Necrosis Factor-alpha; Q41OR9510P / Melphalan
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62. Ferenc T, Wroński JW, Kopczyński J, Kulig A, Sidor M, Stalińska L, Dziki A, Sygut J: Analysis of APC, alpha-, beta-catenins, and N-cadherin protein expression in aggressive fibromatosis (desmoid tumor). Pathol Res Pract; 2009;205(5):311-24
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Analysis of APC, alpha-, beta-catenins, and N-cadherin protein expression in aggressive fibromatosis (desmoid tumor).
  • The aims of this study were to analyze the cadherin/catenin adhesion complex in cells from abdominal and extra-abdominal aggressive fibromatosis tumors, and to estimate the correlation between the expression of the tested proteins and the clinical data of the desmoid patients.
  • Immunohistochemistry was used to examine the expression of the cadherin/catenin adhesion complex: APC protein, alpha-, beta-catenin, and N-cadherin in archival material derived from 15 cases of extra-abdominal desmoid tumor (E-AD) and 20 cases of abdominal (AD) desmoid tumor.
  • In the E-AD group, in both cases of recurrent tumors, no alpha-catenin expression was observed but the expression of this protein was detected in primary tumors.
  • In the groups investigated, no statistically significant correlation was found between alpha-catenin, beta-catenin (c), (n) and (c+n) expression, and tumor size (p>0.1).
  • The results regarding beta-catenin expression obtained in our study confirm the previous findings that nuclear accumulation of this protein plays a crucial role in the pathogenesis of aggressive fibromatosis.
  • [MeSH-major] Adenomatous Polyposis Coli Protein / biosynthesis. Cadherins / biosynthesis. Fibromatosis, Aggressive / metabolism. alpha Catenin / biosynthesis. beta Catenin / biosynthesis
  • [MeSH-minor] Adult. Age Factors. Aged. Biomarkers, Tumor / analysis. Female. Gene Expression. Humans. Immunohistochemistry. Male. Young Adult

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  • (PMID = 19124205.001).
  • [ISSN] 1618-0631
  • [Journal-full-title] Pathology, research and practice
  • [ISO-abbreviation] Pathol. Res. Pract.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Adenomatous Polyposis Coli Protein; 0 / Biomarkers, Tumor; 0 / Cadherins; 0 / alpha Catenin; 0 / beta Catenin
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63. Kourda N, Ben Slama S, Mrabet N, Sayari S, Zouache A, Ben Jilani SB, Zermani R: [Abdominal desmoid tumor: pathologic and therapeutic concepts]. Tunis Med; 2008 Oct;86(10):916-20
Genetic Alliance. consumer health - Desmoid Tumor.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Abdominal desmoid tumor: pathologic and therapeutic concepts].
  • [Transliterated title] Tumeur desmoide abdominale: concepts histopathologiques et therapeutiques.
  • BACKGROUND: Abdominal desmoid tumors arise in young adults between 20 and 40 years.
  • METHODS: From 1990 to 2004, three cases of abdominal desmoid tumors were diagnosed in the Pathologic laboratory of the Charles Nicole Hospital of Tunis.
  • All the patients were operated and the size of tumours varied to 5 to 7cm.
  • CONCLUSION: Abdominal desmoid tumors are locally aggressive but never give metastasis.
  • [MeSH-major] Abdominal Neoplasms / pathology. Abdominal Neoplasms / therapy. Fibromatosis, Aggressive / pathology. Fibromatosis, Aggressive / therapy

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  • (PMID = 19472812.001).
  • [ISSN] 0041-4131
  • [Journal-full-title] La Tunisie médicale
  • [ISO-abbreviation] Tunis Med
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Tunisia
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64. Rampone B, Pedrazzani C, Marrelli D, Pinto E, Roviello F: Updates on abdominal desmoid tumors. World J Gastroenterol; 2007 Dec 7;13(45):5985-8

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Updates on abdominal desmoid tumors.
  • Desmoid tumor is a monoclonal, fibroblastic proliferation arising in musculoaponeurotic structures.
  • This connective tissue hyperplasia infiltrates locally, recurs frequently after resection but does not metastasize.
  • Abdominal desmoid occurs sporadically, in association with some familial syndromes and often represents a clinical dilemma for surgeons.
  • The enigmatic biology and anatomical location of abdominal desmoids make treatment recommendations difficult.
  • [MeSH-major] Fibromatosis, Abdominal / therapy. Neoplasm Recurrence, Local

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  • (PMID = 18023087.001).
  • [ISSN] 1007-9327
  • [Journal-full-title] World journal of gastroenterology
  • [ISO-abbreviation] World J. Gastroenterol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] China
  • [Number-of-references] 50
  • [Other-IDs] NLM/ PMC4250878
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65. Skapek SX, Ferguson WS, Granowetter L, Devidas M, Perez-Atayde AR, Dehner LP, Hoffer FA, Speights R, Gebhardt MC, Dahl GV, Grier HE, Pediatric Oncology Group: Vinblastine and methotrexate for desmoid fibromatosis in children: results of a Pediatric Oncology Group Phase II Trial. J Clin Oncol; 2007 Feb 10;25(5):501-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Vinblastine and methotrexate for desmoid fibromatosis in children: results of a Pediatric Oncology Group Phase II Trial.
  • PURPOSE: To determine the efficacy and safety of using vinblastine (Vbl) and methotrexate (Mtx) in children with desmoid-type fibromatosis that is recurrent or not amenable to treatment with radiation or surgery.
  • Response was assessed by bidimensional measurements of tumor on axial imaging (magnetic resonance imaging or computed tomography).
  • A measurable response was documented in eight patients (31%), and 10 patients had stable disease documented as the best response to treatment.
  • Eighteen patients had disease progression at a median time of 9.1 months.
  • Eight patients remain free of disease progression at a median of 43.4 months from study entry.
  • CONCLUSION: Vbl and Mtx are well tolerated in children with desmoid-type fibromatosis.
  • Furthermore, this combination can promote tumor regression or block tumor growth in most children.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Fibromatosis, Aggressive / drug therapy
  • [MeSH-minor] Adolescent. Antimetabolites, Antineoplastic / administration & dosage. Antineoplastic Agents, Phytogenic / administration & dosage. Child. Child, Preschool. Disease-Free Survival. Drug Administration Schedule. Female. Humans. Infant. Injections, Intravenous. Kaplan-Meier Estimate. Male. Methotrexate / administration & dosage. Prospective Studies. Time Factors. Treatment Outcome. Vinblastine / administration & dosage

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  • (PMID = 17290057.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA098543-02; United States / NCI NIH HHS / CA / CA29139
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Multicenter Study; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antimetabolites, Antineoplastic; 0 / Antineoplastic Agents, Phytogenic; 5V9KLZ54CY / Vinblastine; YL5FZ2Y5U1 / Methotrexate
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66. Motsch C, Schmitt J, Roessner A, Mittler U, Freigang B: [Desmoid tumors of the head and neck]. Laryngorhinootologie; 2007 Jul;86(7):524-7
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  • [Title] [Desmoid tumors of the head and neck].
  • Desmoid tumors of the head and neck, also known as aggressive fibromatoses, are rare.
  • They are soft tissue neoplasms arising from musculoaponeurotic structures and characterized of locally aggressive infiltration and recurrences.
  • Complete surgical excision of desmoid tumors is considered to be the only effective method of cure.
  • MRI is the first choice in the preoperative evaluation of neck desmoids.
  • We describe the successful treatment of desmoid tumors in two cases (M. sternocleidomastoideus, M. levator scapulae).
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Head and Neck Neoplasms / diagnosis. Muscle Neoplasms / diagnosis. Neck Muscles
  • [MeSH-minor] Biopsy. Chemotherapy, Adjuvant. Child. Combined Modality Therapy. Cranial Nerve Injuries / diagnosis. Cranial Nerve Injuries / prevention & control. Female. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Monitoring, Intraoperative. Neck Dissection. Neoplasm, Residual / diagnosis. Neoplasm, Residual / drug therapy. Neoplasm, Residual / radiotherapy. Neoplasm, Residual / surgery. Postoperative Complications / diagnosis. Postoperative Complications / prevention & control. Radiotherapy, Adjuvant. Reoperation

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  • (PMID = 17219337.001).
  • [ISSN] 0935-8943
  • [Journal-full-title] Laryngo- rhino- otologie
  • [ISO-abbreviation] Laryngorhinootologie
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Germany
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67. Oudidi A, Hachimi H, El Alami MN: [Desmoid tumor of the parotid gland]. Rev Stomatol Chir Maxillofac; 2006 Dec;107(6):470-3
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  • [Title] [Desmoid tumor of the parotid gland].
  • [Transliterated title] Tumeur desmoïde de la glande parotide.
  • INTRODUCTION: Desmoid tumor is a benign microscopic tumor that belongs to the group of the deep fibromatosis.
  • It usually arises from facial or musculoaponeurotic structures in the abdomen but rarely is located in the head or neck.
  • Physical examination revealed a tumor in the parotid gland, which was hard and adherent deeply, measuring 4 cm/3 cm and sensitive to palpation; without satellite nodes nor facial paralysis.
  • Pathology was in favor of a desmoid tumor.
  • DISCUSSION: Desmoid tumors are deep fibromatosis characterized by their slow growth and especially by considerable infiltration of the adjacent structures but without potential for metastasis.
  • Although very rare, the cervical localizations are especially aggressive.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Parotid Neoplasms / pathology

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  • (PMID = 17195002.001).
  • [ISSN] 0035-1768
  • [Journal-full-title] Revue de stomatologie et de chirurgie maxillo-faciale
  • [ISO-abbreviation] Rev Stomatol Chir Maxillofac
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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68. Okamoto K, Kurihara Y, Imamura K, Kanemaki Y, Nakajima Y, Fukuda M, Maeda I: Desmoid tumor of the breast: the role of proton magnetic resonance spectroscopy for a benign breast lesion mimicking a malignancy. Breast J; 2008 Jul-Aug;14(4):376-8
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  • [Title] Desmoid tumor of the breast: the role of proton magnetic resonance spectroscopy for a benign breast lesion mimicking a malignancy.
  • Desmoid tumor of the breast is an extremely rare condition.
  • It is difficult to provide a correct preoperative diagnosis of desmoid tumor of the breast because of its tendency to mimic breast carcinoma on physical examination and conventional imaging such as mammography and sonography.
  • We present a case of desmoid tumor of the breast that mimicked breast carcinoma, in which proton magnetic resonance spectroscopy assisted the result of biopsy, thus enabling a correct preoperative diagnosis.
  • [MeSH-major] Breast Neoplasms / diagnosis. Fibromatosis, Aggressive / diagnosis. Magnetic Resonance Spectroscopy
  • [MeSH-minor] Adult. Diagnosis, Differential. Female. Humans. Protons

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  • (PMID = 18687071.001).
  • [ISSN] 1524-4741
  • [Journal-full-title] The breast journal
  • [ISO-abbreviation] Breast J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Protons
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69. Tejpar S, Michils G, Denys H, Van Dam K, Nik SA, Jadidizadeh A, Cassiman JJ: Analysis of Wnt/Beta catenin signalling in desmoid tumors. Acta Gastroenterol Belg; 2005 Jan-Mar;68(1):5-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Analysis of Wnt/Beta catenin signalling in desmoid tumors.
  • Desmoid tumors are fibromatous lesions occurring both sporadically and in patients with familial adenomatous polyposis (FAP).
  • Because of the association of these tumors with the hereditary colorectal cancer syndrome FAP we set out to define the molecular events driving desmoid tumorigenesis, hypothezising these might be identical to events driving colorectal tumorigenesis.
  • We found that whereas FAP-associated desmoid tumors are caused by germline APC mutations followed by somatic inactivation of the wild-type APC allele, sporadic desmoids are usually characterized by oncogenic mutations in the b-catenin gene, both identical molecular alterations to those found in the vast majority of colorectal cancers.
  • Next we set out to investigate the cellular pathways activated by these mutations, and identified activation of the Wnt signaling pathway in desmoid tumors.
  • Currently we are investigating tissue-specific downstream effectors of the Wnt pathway that might be responsible for the behaviour of these invasive fibrous tumors.
  • Our findings also point to a role for this pathway in the regulation of normal myofibroblast proliferation and suggest novel treatments in desmoid tumors and other fibrous proliferative disorders.
  • [MeSH-major] Adenomatous Polyposis Coli / genetics. Cytoskeletal Proteins / genetics. Fibromatosis, Aggressive / genetics. Genetic Predisposition to Disease. Polymorphism, Genetic. Trans-Activators / genetics

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  • (PMID = 15832580.001).
  • [ISSN] 1784-3227
  • [Journal-full-title] Acta gastro-enterologica Belgica
  • [ISO-abbreviation] Acta Gastroenterol. Belg.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Review
  • [Publication-country] Belgium
  • [Chemical-registry-number] 0 / CTNNB1 protein, human; 0 / Cytoskeletal Proteins; 0 / Trans-Activators; 0 / beta Catenin
  • [Number-of-references] 20
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70. Soufi M, Lahlou MK, Bensaid M, Messrouri R, Benamer S, Essadel A, Mdaghri J, Mohammadine E, Taghy A, Settaf A, Chad B: [Desmoid tumors of the abdominal wall: three cases]. Rev Med Liege; 2009 Dec;64(12):633-8

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Desmoid tumors of the abdominal wall: three cases].
  • [Transliterated title] Les tumeurs desmoïdes de la paroi abdominale. A propos de trois cas.
  • Desmoids tumors are rare.
  • They often develop from the fascia and muscles of the abdominal wall.
  • They are considered as benign, but endowed with local aggressiveness.
  • WE report three cases of histology proven desmoids tumors of the abdominal wall treated between 2000 and 2007.
  • Etiologic factors, diagnosis, surgical management and adjuvant therapy in case of incomplete resection or reccurrence are discussed.
  • [MeSH-major] Abdominal Wall / surgery. Fibromatosis, Aggressive / pathology. Fibromatosis, Aggressive / surgery

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  • (PMID = 20143748.001).
  • [ISSN] 0370-629X
  • [Journal-full-title] Revue médicale de Liège
  • [ISO-abbreviation] Rev Med Liege
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Belgium
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71. Hosalkar HS, Fox EJ, Delaney T, Torbert JT, Ogilvie CM, Lackman RD: Desmoid tumors and current status of management. Orthop Clin North Am; 2006 Jan;37(1):53-63

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Desmoid tumors and current status of management.
  • Desmoid tumors, also known as aggressive fibromatosis, are rare fibroblastic tumors that exhibit a wide range of local aggressiveness, from largely indolent to locally destructive.
  • Understanding of the pathogenesis and the great heterogeneity in the natural history of desmoid tumors is invaluable to the development of therapeutic strategies.
  • The rarity of cases in even major tumor centers has traditionally limited the ability to study this disease.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Fibromatosis, Aggressive / therapy. Soft Tissue Neoplasms / pathology. Soft Tissue Neoplasms / therapy
  • [MeSH-minor] Adolescent. Adult. Biopsy, Needle. Chemotherapy, Adjuvant. Combined Modality Therapy / methods. Female. Humans. Immunohistochemistry. Magnetic Resonance Imaging / methods. Male. Middle Aged. Neoplasm Staging. Prognosis. Radiotherapy, Adjuvant. Risk Assessment. Survival Analysis. Treatment Outcome

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  • (PMID = 16311111.001).
  • [ISSN] 0030-5898
  • [Journal-full-title] The Orthopedic clinics of North America
  • [ISO-abbreviation] Orthop. Clin. North Am.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 51
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72. Zisis C, Dountsis A, Nikolaides A, Dahabreh J: Desmoid tumors of the chest wall. Asian Cardiovasc Thorac Ann; 2006 Oct;14(5):359-62

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Desmoid tumors of the chest wall.
  • Chest wall desmoid tumors (DT) are rare pathologic entities with microscopic features similar to, or undistinguishable from, fibromas or fibrosarcomas.
  • A resection of the lesion was performed with negative margins of 4 cm around the tumor (wide resection).
  • One patient had a recurrence 15 months later, and was admitted for complementary resection, and remains disease-free for 5 years.
  • The rest 3 patients are disease-free for 6 months to 5 years.
  • Resection must include all adjacent, overlying and underlying musculature as well as soft tissues and any spare skin from the procedure should be used.
  • [MeSH-major] Fibromatosis, Aggressive / surgery. Neoplasm Recurrence, Local / surgery. Soft Tissue Neoplasms / surgery

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  • (PMID = 17005879.001).
  • [ISSN] 1816-5370
  • [Journal-full-title] Asian cardiovascular & thoracic annals
  • [ISO-abbreviation] Asian Cardiovasc Thorac Ann
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biocompatible Materials
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73. Carneiro C, Hurtubis C, Singh M, Robinson W: Desmoid tumors of the right rectus abdominus muscle in postpartum women. Arch Gynecol Obstet; 2009 Jun;279(6):869-73
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Desmoid tumors of the right rectus abdominus muscle in postpartum women.
  • BACKGROUND: Desmoid tumors are benign neoplasms that most often arise from muscle aponeurosis and have been associated with both trauma and pregnancy.
  • The etiology of desmoids has not been determined.
  • CLINICAL CHARACTERISTICS: We present here four almost identical cases with desmoids occurring in the same location, the right rectus abdominus muscle in young post partum females.
  • All were over the age of 30 at the time of diagnosis.
  • DISCUSSION: All four patients are disease free at a median follow-up of 2.5 years.
  • The possible etiology of desmoids tumors in this location in postpartum females is discussed.
  • [MeSH-major] Fibromatosis, Abdominal / pathology. Muscle Neoplasms / pathology. Rectus Abdominis / pathology

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  • (PMID = 19020890.001).
  • [ISSN] 1432-0711
  • [Journal-full-title] Archives of gynecology and obstetrics
  • [ISO-abbreviation] Arch. Gynecol. Obstet.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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74. Souza FF, Fennessy FM, Yang Q, van den Abbeele AD: Case report. PET/CT appearance of desmoid tumour of the chest wall. Br J Radiol; 2010 Feb;83(986):e39-42

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Case report. PET/CT appearance of desmoid tumour of the chest wall.
  • Desmoid tumours are rare, poorly circumscribed tumours that have a firm consistency and, although benign, have a remarkable tendency to infiltrate into surrounding structures.
  • Extra-abdominal desmoid tumours involve mainly the extremities or the chest wall and are usually managed by wide radical resection.
  • Moreover, desmoid tumours involving the chest wall are locally aggressive tumours with a high recurrence rate.
  • We report a case of a pathologically proven desmoid tumour of the chest wall in a patient with a history of bilateral breast cancer and oesophageal cancer.
  • We discuss the imaging appearances of this tumour on positron emission tomography combined with computed tomography (PET/CT) and magnetic resonance imaging.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Neoplasms, Second Primary / diagnosis
  • [MeSH-minor] Aged. Biopsy. Diagnosis, Differential. Female. Fluorodeoxyglucose F18. Humans. Magnetic Resonance Imaging. Positron-Emission Tomography. Radiopharmaceuticals. Thoracic Wall

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  • (PMID = 20139256.001).
  • [ISSN] 1748-880X
  • [Journal-full-title] The British journal of radiology
  • [ISO-abbreviation] Br J Radiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
  • [Other-IDs] NLM/ PMC3473531
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75. Sciallis GF, Sciallis AP: Becker nevus with an underlying desmoid tumor: a case report and review including Mayo Clinic's experience. Arch Dermatol; 2010 Dec;146(12):1408-12
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  • [Title] Becker nevus with an underlying desmoid tumor: a case report and review including Mayo Clinic's experience.
  • Our objectives were to report the occurrence of a Becker nevus with an underlying desmoid soft-tissue tumor; to review Mayo Clinic's experience with Becker nevi, concentrating on Becker nevi associated with bone, vascular, neural, and other soft-tissue abnormalities; to inform physicians of the Becker nevus syndrome; and finally to alert clinicians to evaluate a Becker nevus with its associations in mind.
  • OBSERVATIONS: A 46-year-old woman had a Becker nevus with an underlying desmoid-type fibromatosis (desmoid tumor) presenting clinically as a "painful dimple" within the nevus.
  • Review of medical records for 1997 through 2006 at Mayo Clinic, Rochester, Minnesota, yielded 52 patients with Becker nevi, 12 of whom had an associated bone, vascular, neural, congenital, or other soft-tissue abnormality, ranging from liposarcoma to an accessory areola.
  • CONCLUSIONS: We add to the literature a unique case of desmoid-type fibromatosis immediately beneath a Becker melanosis, which presented as a painful dimple.
  • We hope to raise awareness that a Becker nevus may be associated with other abnormalities, including an infiltrative soft-tissue tumor.
  • We also emphasize the importance of follow-up, including inspection of not only the surface but also the deep tissues underlying the Becker nevus.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Neoplasms, Multiple Primary / diagnosis. Nevus, Pigmented / diagnosis. Skin Neoplasms / diagnosis. Soft Tissue Neoplasms / diagnosis
  • [MeSH-minor] Arm. Back. Diagnosis, Differential. Female. Humans. Magnetic Resonance Imaging. Middle Aged. Syndrome

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  • (PMID = 21173323.001).
  • [ISSN] 1538-3652
  • [Journal-full-title] Archives of dermatology
  • [ISO-abbreviation] Arch Dermatol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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76. Wong SL: Diagnosis and management of desmoid tumors and fibrosarcoma. J Surg Oncol; 2008 May 1;97(6):554-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Diagnosis and management of desmoid tumors and fibrosarcoma.
  • Fibrous tumors represent a diverse subtype of soft tissue tumors and can represent benign conditions as well as frankly malignant sarcomas.
  • Desmoid tumors and dermatofibrosarcoma protuberans are more difficult to classify and tend to be considered in the intermediate risk category.
  • They are distinct entities, but both are locally aggressive processes which are plagued with attendant morbidity and high recurrence rates.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Fibromatosis, Aggressive / therapy. Fibrosarcoma / diagnosis. Fibrosarcoma / therapy

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  • [Copyright] (c) 2008 Wiley-Liss, Inc.
  • (PMID = 18425778.001).
  • [ISSN] 0022-4790
  • [Journal-full-title] Journal of surgical oncology
  • [ISO-abbreviation] J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 37
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77. Soon JL, Lau WK, Seow-Choen F, Cheng CW: Unresectable desmoid tumours causing obstructive uropathy in familial adenomatous polyposis. Asian J Surg; 2005 Jul;28(3):233-7
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  • [Title] Unresectable desmoid tumours causing obstructive uropathy in familial adenomatous polyposis.
  • Desmoid tumour-related ureteral obstruction in familial adenomatous polyposis presents difficult management problems.
  • Both our patients developed intra-abdominal desmoid tumours following proctocolectomies with pouch reconstruction (performed 0.7 and 2.5 years earlier).

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  • (PMID = 16024324.001).
  • [ISSN] 1015-9584
  • [Journal-full-title] Asian journal of surgery
  • [ISO-abbreviation] Asian J Surg
  • [Language] ENG
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] China
  • [Number-of-references] 12
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78. Ishizuka M, Hatori M, Dohi O, Suzuki T, Miki Y, Tazawa C, Sasano H, Kokubun S: Expression profiles of sex steroid receptors in desmoid tumors. Tohoku J Exp Med; 2006 Nov;210(3):189-98
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  • [Title] Expression profiles of sex steroid receptors in desmoid tumors.
  • Desmoid tumors are benign fibrous neoplasms which arise from the fibrous tissue of intra- and extra- abdominal sites, but their clinical management is sometimes difficult because of extensive infiltration into the surrounding tissues.
  • Desmoid tumors commonly occur in women, especially after childbirth.
  • Recently, both clinical and experimental findings indicate the possible roles of sex steroids in the development and progression of desmoid tumors but detailed information is still ambiguous.
  • In this study, we first examined immunoreactivity of sex steroid receptors in desmoid tumors (27 cases) by immunohistochemistry and compared the findings with those in reactive self-limiting lesions associated with fibrosis (8 cases).
  • Estrogen receptor (ER) alpha and ERbeta immunoreactivities were detected in 7.4% (2/27) and 7.4% (2/27) of desmoid tumors, respectively.
  • One desmoid tumor expressed both ERalpha and ERbeta.
  • Sex steroid receptor mRNAs was further examined by reverse transcription and polymerase chain reaction (RT-PCR) analysis using fresh frozen tissues, demonstrating the expression of PR (PR-A and/or PR-B) and AR mRNAs in eight desmoid tumors examined and all cases of reactive fibrosis.
  • These results indicate that sex steroid hormones might play an important role in the pathogenesis of desmoid tumors and could lead to the introduction of novel hormone therapeutic approaches in managing patients with recurrent desmoid tumors.
  • [MeSH-major] Fibromatosis, Aggressive / metabolism. Gene Expression Regulation, Neoplastic. Neoplasms / metabolism. Receptors, Steroid / metabolism

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  • (PMID = 17077595.001).
  • [ISSN] 0040-8727
  • [Journal-full-title] The Tohoku journal of experimental medicine
  • [ISO-abbreviation] Tohoku J. Exp. Med.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Estrogen Receptor alpha; 0 / Estrogen Receptor beta; 0 / Receptors, Progesterone; 0 / Receptors, Steroid
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79. Seinfeld J, Kleinschmidt-DeMasters BK, Tayal S, Lillehei KO: Desmoid-type fibromatosis involving the brachial plexus. Neurosurg Focus; 2007;22(6):E22
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Desmoid-type fibromatosis involving the brachial plexus.
  • Desmoid-type fibromatosis involving the brachial plexus is a rare and challenging disease.
  • The authors describe their experience in four surgically treated patients with desmoid-type fibromatosis involving the brachial plexus and review the relevant neurosurgical literature.
  • All tumors were assessed for c-KIT oncogene mutations in hopes of establishing a biological basis for using the tyrosine kinase inhibitor imatimib mesylate as an adjuvant therapy.
  • Three patients experienced tumor recurrence requiring reoperation.
  • Fractionated radiotherapy achieved local control in three patients, and the disease in one patient progressed beyond the treatment field.
  • Single base pair changes at exon 10 of the c-KIT oncogene were identified in three tumors.
  • One tumor with this mutation did not respond to treatment with imatimib mesylate.
  • Analysis of these cases emphasizes the need for careful resection in patients with desmoid-type fibromatosis and supports the conclusion that without adjuvant radiotherapy a high local recurrence rate can be anticipated.
  • For optimal local disease control, the authors recommend postsurgical radiation therapy regardless of the extent of resection achieved.
  • [MeSH-major] Brachial Plexus / pathology. Brachial Plexus / surgery. Fibromatosis, Abdominal / radiotherapy. Fibromatosis, Abdominal / surgery
  • [MeSH-minor] Adult. Aged. Female. Fibromatosis, Aggressive / diagnosis. Fibromatosis, Aggressive / radiotherapy. Fibromatosis, Aggressive / surgery. Humans. Male. Middle Aged. Retrospective Studies. Treatment Outcome

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  • (PMID = 17613214.001).
  • [ISSN] 1092-0684
  • [Journal-full-title] Neurosurgical focus
  • [ISO-abbreviation] Neurosurg Focus
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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80. Mayer M, Kulig A, Sygut J, Dziki A, Simon D, Latos-Bieleńska A, Ferenc T: Molecular cytogenetic analysis of chromosome aberrations in desmoid tumors. Pol J Pathol; 2007;58(3):167-71

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Molecular cytogenetic analysis of chromosome aberrations in desmoid tumors.
  • To date there are only few reports concerning chromosomal changes in desmoid tumors.
  • To extend the knowledge in this field we examined 19 samples from the patients diagnosed with desmoid tumors.
  • In the present study formalin-fixed and paraffin-embedded desmoid tumors were analyzed using fluorescence in situ hybridization (FISH) with a-satellite probes for chromosomes X, Y, 8 and 20.
  • Chromosomal abnormalities were found in 6 cases, both abdominal and extra-abdominal tumors.
  • Our findings confirm earlier reports concerning the diversity of chromosomal changes in desmoid tumors and might suggest that both groups of abdominal and extra-abdominal tumors are genuine neoplasms.
  • [MeSH-major] Chromosome Aberrations. Cytogenetic Analysis. Fibromatosis, Aggressive / genetics

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  • (PMID = 18074861.001).
  • [ISSN] 1233-9687
  • [Journal-full-title] Polish journal of pathology : official journal of the Polish Society of Pathologists
  • [ISO-abbreviation] Pol J Pathol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Poland
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81. Lev D, Kotilingam D, Wei C, Ballo MT, Zagars GK, Pisters PW, Lazar AA, Patel SR, Benjamin RS, Pollock RE: Optimizing treatment of desmoid tumors. J Clin Oncol; 2007 May 1;25(13):1785-91
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Optimizing treatment of desmoid tumors.
  • PURPOSE: This study compared a large series of desmoid patients treated at a single institution to a previously published series from the same institution to determine if patient population characteristics, treatment approaches, and clinical outcomes had undergone change over the two study periods.
  • MATERIALS AND METHODS: Data from a prospective soft tissue tumor database was used to analyze clinical courses of 189 desmoid patients treated at The University of Texas M.D.
  • Anderson Cancer Center (UTMDACC) from 1995 to 2005 as compared with 189 UTMDACC desmoid patients treated between 1965 and 1994.
  • RESULTS: A nearly three-fold increase in annualized UTMDACC desmoid referral volume with significantly higher percentages and numbers of primary desmoid tumor referrals to UTMDACC was observed in the most recent study period.
  • While the recent series patients had higher rates of macroscopic residual disease and equivalent rates of positive microscopic margins after definitive surgery, the estimated 5-year local recurrence rate of 20% was improved compared with the 30% rate observed in the earlier series.
  • CONCLUSION: Increased awareness of the complex multidisciplinary management needed for desmoid tumor control may underlie significantly increased numbers of referrals to UTMDACC, especially primary untreated desmoids.
  • Increased neoadjuvant treatments may be associated with improved desmoid patient outcomes.
  • These trends should be supported, particularly if personalized molecular-based therapies are to be rapidly and effectively deployed for the benefit of those afflicted by this rare and potentially debilitating disease.
  • [MeSH-major] Fibromatosis, Aggressive / therapy

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  • (PMID = 17470870.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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82. Bonvalot S, Rimareix F, Paumier A, Roberti E, Bouzaiene H, Le Péchoux C: [What is new in the local approach of limb sarcomas and desmoid tumours?]. Cancer Radiother; 2010 Oct;14(6-7):455-9
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  • [Title] [What is new in the local approach of limb sarcomas and desmoid tumours?].
  • [Transliterated title] Actualisation de la stratégie thérapeutique locorégionale dans les sarcomes des tissus mous et les tumeurs desmoïdes des membres.
  • The treatment of soft tissue sarcomas of limbs should be discussed within an experienced multimodality team.
  • Surgical resection remains the cornerstone of therapy for localized disease and achieves a five years overall survival around 75% and a local recurrence rate as low as 10% in the best series.
  • Molecular genetics of sarcomas and quality of margins are essential to guide diagnosis and therapeutic selection.
  • Many retrospective studies and two randomized studies (one of adjuvant brachytherapy and one of external beam radiotherapy) have shown that adjuvant radiotherapy after complete surgery significantly reduces the risk of local recurrence in extremity soft tissue sarcomas.
  • Presently, the role of systematic first-line invasive treatment (including surgery and/or radiotherapy) of desmoids is debated.
  • It is becoming evident that up to 50% of patients with desmoids benefit from a front-line non-aggressive policy, because growth arrest is a common feature of this disease.
  • Additional study of the molecular determinants of desmoid behaviour is needed to guide treatment.
  • [MeSH-major] Extremities / surgery. Fibromatosis, Aggressive / surgery. Radiotherapy, Adjuvant. Sarcoma / surgery. Soft Tissue Neoplasms / surgery
  • [MeSH-minor] Amputation. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Chemotherapy, Cancer, Regional Perfusion. Combined Modality Therapy. Diagnostic Imaging / methods. Humans. Neoadjuvant Therapy. Neoplasm Recurrence, Local / epidemiology. Neoplasm Recurrence, Local / prevention & control. Patient Care Team. Practice Guidelines as Topic. Randomized Controlled Trials as Topic. Reconstructive Surgical Procedures. Surgical Flaps

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  • [Copyright] Copyright © 2010. Published by Elsevier SAS.
  • (PMID = 20797892.001).
  • [ISSN] 1769-6658
  • [Journal-full-title] Cancer radiothérapie : journal de la Société française de radiothérapie oncologique
  • [ISO-abbreviation] Cancer Radiother
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] France
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83. Lazar AJ, Tuvin D, Hajibashi S, Habeeb S, Bolshakov S, Mayordomo-Aranda E, Warneke CL, Lopez-Terrada D, Pollock RE, Lev D: Specific mutations in the beta-catenin gene (CTNNB1) correlate with local recurrence in sporadic desmoid tumors. Am J Pathol; 2008 Nov;173(5):1518-27
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Specific mutations in the beta-catenin gene (CTNNB1) correlate with local recurrence in sporadic desmoid tumors.
  • Desmoid fibromatosis is a rare, nonmetastatic neoplasm marked by local invasiveness and relentless recurrence.
  • Molecular determinants of desmoid recurrence remain obscure. beta-Catenin deregulation has been commonly identified in sporadic desmoids although the incidence of CTNNB1 (the gene encoding beta-catenin) mutations is uncertain.
  • Consequently, we evaluated the prevalence of CTNNB1 mutations in a large cohort of sporadic desmoids and examined whether mutation type was relevant to desmoid outcome.
  • Desmoid specimens (195 tumors from 160 patients, 1985 to 2005) and control dermal scars were assembled into a clinical data-linked tissue microarray.
  • CTNNB1 genotyping was performed on a 138-sporadic desmoid subset.
  • CTNNB1 mutations were observed in 117 of 138 (85%) of desmoids.
  • Five-year recurrence-free survival was significantly poorer in 45F-mutated desmoids (23%, P < 0.0001) versus either 41A (57%) or nonmutated tumors (65%).
  • Nuclear beta-catenin expression was observed in 98% of specimens and intensity was inversely correlated with incidence of desmoid recurrence (P < 0.01).
  • In conclusion, CTNNB1 mutations are highly common in desmoid tumors.
  • [MeSH-major] Fibromatosis, Aggressive / genetics. Mutation / genetics. beta Catenin / genetics
  • [MeSH-minor] Adult. Base Sequence. Cell Nucleus / metabolism. Cell Nucleus / pathology. Cohort Studies. DNA Mutational Analysis. Exons / genetics. Female. Humans. Kaplan-Meier Estimate. Male. Middle Aged. Molecular Sequence Data. Proportional Hazards Models. Recurrence. Regression Analysis. Tissue Array Analysis

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  • (PMID = 18832571.001).
  • [ISSN] 1525-2191
  • [Journal-full-title] The American journal of pathology
  • [ISO-abbreviation] Am. J. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / beta Catenin
  • [Other-IDs] NLM/ PMC2570141
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84. Perera ND, Vithana VH: Giant retroperitoneal desmoid tumour. Ceylon Med J; 2006 Sep;51(3):124-5

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Giant retroperitoneal desmoid tumour.
  • A giant retroperitoneal desmoid (30 x 15 cm), in a 16-year old girl arising from psoas fascia is reported.
  • Despite debulking surgery, adjuvant radiotherapy, anti-oestrogen agents and non-steroidal anti-inflammatory agents, 3 years later she died from tumour invasion of major blood vessels and bowel, leading to massive gastrointestinal bleeding.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Retroperitoneal Neoplasms / diagnosis

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  • (PMID = 17315594.001).
  • [ISSN] 0009-0875
  • [Journal-full-title] The Ceylon medical journal
  • [ISO-abbreviation] Ceylon Med J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Sri Lanka
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85. Bertagnolli MM, Morgan JA, Fletcher CD, Raut CP, Dileo P, Gill RR, Demetri GD, George S: Multimodality treatment of mesenteric desmoid tumours. Eur J Cancer; 2008 Nov;44(16):2404-10
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Multimodality treatment of mesenteric desmoid tumours.
  • BACKGROUND: Desmoid tumours are rare neoplasms characterised by clonal proliferation of myofibroblasts that do not metastasise, but often exhibit an infiltrative pattern and functional impairment.
  • When desmoids arise in the intestinal mesentery, surgical resection is seldom possible without life-altering loss of intestinal function.
  • METHODS: Retrospective review of the clinical management of 52 consecutive patients treated for desmoids of the intestinal mesentery from January 2001 to August 2006.
  • A multidisciplinary treatment plan was developed based on primary disease extent, tumour behaviour and resectability.
  • Patients with stable but unresectable disease were observed without treatment.
  • Patients with resectable disease underwent surgery, and patients with unresectable progressing disease received chemotherapy, most commonly liposomal doxorubicin, followed by surgery if chemotherapy rendered the disease resectable.
  • RESULTS: At a median follow-up of 50.0 months (range 4.6-212), 50 patients (96%) have either no recurrence or radiographically stable disease.
  • CONCLUSION: These data indicate that the extent of disease; tumour behaviour and resectability are the important factors when defining a treatment plan for mesenteric desmoid tumours.
  • A multidisciplinary approach of surgery combined with chemotherapy is an effective and function-sparing strategy for managing this disease.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Fibromatosis, Aggressive / drug therapy. Fibromatosis, Aggressive / surgery. Mesentery. Peritoneal Neoplasms / drug therapy. Peritoneal Neoplasms / surgery
  • [MeSH-minor] Adult. Aged. Combined Modality Therapy. Doxorubicin / administration & dosage. Female. Fibromatosis, Abdominal / drug therapy. Fibromatosis, Abdominal / surgery. Humans. Male. Middle Aged. Retrospective Studies. Tomography, X-Ray Computed. Treatment Outcome. Vinblastine / administration & dosage. Vinblastine / analogs & derivatives. Young Adult

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  • (PMID = 18706807.001).
  • [ISSN] 1879-0852
  • [Journal-full-title] European journal of cancer (Oxford, England : 1990)
  • [ISO-abbreviation] Eur. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 5V9KLZ54CY / Vinblastine; 80168379AG / Doxorubicin; Q6C979R91Y / vinorelbine
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86. Church J, Berk T, Boman BM, Guillem J, Lynch C, Lynch P, Rodriguez-Bigas M, Rusin L, Weber T, Collaborative Group of the Americas on Inherited Colorectal Cancer: Staging intra-abdominal desmoid tumors in familial adenomatous polyposis: a search for a uniform approach to a troubling disease. Dis Colon Rectum; 2005 Aug;48(8):1528-34
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Staging intra-abdominal desmoid tumors in familial adenomatous polyposis: a search for a uniform approach to a troubling disease.
  • INTRODUCTION: Desmoid tumors are a clinical problem in 12 to 15 percent of patients with familial adenomatous polyposis.
  • There is no predictably effective treatment for intra-abdominal desmoid tumors, which sometimes cause significant complications by their effects on the ureters or bowel.
  • The relative rarity and the clinical heterogeneity of intra-abdominal desmoid tumors make randomized studies difficult to do.
  • METHODS: Intra-abdominal desmoid tumors can be staged according to their size, clinical presentation and growth pattern.
  • CONCLUSION: A way of staging intra-abdominal desmoid tumors is proposed to facilitate stratification by disease severity during collaborative studies of various treatments.
  • [MeSH-major] Abdominal Neoplasms / pathology. Adenomatous Polyposis Coli / pathology. Fibromatosis, Aggressive / pathology. Neoplasms, Multiple Primary / pathology
  • [MeSH-minor] Abdominal Wall / pathology. Clinical Protocols. Genes, APC. Genotype. Humans. Mesentery / pathology. Mutation / genetics. Neoplasm Staging. Patient Care Planning. Peritoneal Neoplasms / pathology. Receptors, Estrogen / analysis. Receptors, Progesterone / analysis

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  • (PMID = 15906134.001).
  • [ISSN] 0012-3706
  • [Journal-full-title] Diseases of the colon and rectum
  • [ISO-abbreviation] Dis. Colon Rectum
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Receptors, Estrogen; 0 / Receptors, Progesterone
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87. Kumar V, Khanna S, Khanna AK, Khanna R: Desmoid tumors: experience of 32 cases and review of the literature. Indian J Cancer; 2009 Jan-Mar;46(1):34-9

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Desmoid tumors: experience of 32 cases and review of the literature.
  • BACKGROUND: Desmoids are infiltrative, locally destructive, soft tissue tumors.
  • AIM: Present study aimed at reporting the 10-year experience of 32 desmoid cases and reviewing some facts with symptoms, investigation, and treatment of the disease.
  • MATERIALS AND METHODS: Thirty two cases of desmoid tumors were reviewed over a 10-year span.
  • The commonest site of presentation was the abdominal wall.
  • The tumors were found in the rectus sheath in 14 patients (64%) and were laterally situated in 8 patients (36%).
  • Locally recurrent desmoids were seen in eight patients (25%).
  • CONCLUSIONS: In our experience, 25% of the desmoid tumors (8/32) were recurrent and postoperative radiotherapy did not seem to influence the local recurrence rate.
  • The most important predictor for recurrence was tumors of > 5 cm.
  • [MeSH-major] Fibromatosis, Aggressive / radiotherapy. Fibromatosis, Aggressive / surgery. Neoplasm Recurrence, Local / diagnosis. Neoplasm, Residual / diagnosis

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  • (PMID = 19282564.001).
  • [ISSN] 0019-509X
  • [Journal-full-title] Indian journal of cancer
  • [ISO-abbreviation] Indian J Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] India
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88. Fallen T, Wilson M, Morlan B, Lindor NM: Desmoid tumors -- a characterization of patients seen at Mayo Clinic 1976-1999. Fam Cancer; 2006;5(2):191-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Desmoid tumors -- a characterization of patients seen at Mayo Clinic 1976-1999.
  • Desmoid tumors occur with high frequency in individuals with Familial Adenomatous Polyposis (FAP).
  • Because of this, individuals developing desmoid tumors may be referred for genetic risk assessment.
  • Determining whether a person has a FAP-related desmoid tumor or a sporadic desmoid can be challenging.
  • We sought to characterize the patients who were seen at our institution to determine if there were clinical differences in presentation between FAP-associated and sporadic desmoid tumors.
  • We searched the Mayo Clinic-modified H-ICDA (Hospital adaptation of the International Classification of Diseases) diagnostic codes for all diagnoses of desmoid tumors in patients seen between 1976 and 1999.
  • Charts were reviewed to determine accuracy of diagnosis, age when seen, gender, site of tumor, and presence of polyposis.
  • Location of development of desmoid tumors was correlated with but not specific for distinguishing FAP from non-FAP desmoids.
  • Abdominal desmoids comprised the majority of FAP desmoids and extra-abdominal desmoids comprised the majority of non-FAP desmoids (P<0.001) but age was not a discriminating factor.
  • Using Bayesian analysis, we demonstrate how these findings can assist genetic professionals in their evaluation of patients with desmoid tumors by providing prior probabilities of FAP based upon clinical presentation.
  • [MeSH-major] Adenomatous Polyposis Coli / complications. Fibromatosis, Aggressive / genetics

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  • (PMID = 16736290.001).
  • [ISSN] 1389-9600
  • [Journal-full-title] Familial cancer
  • [ISO-abbreviation] Fam. Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Netherlands
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89. Nieuwenhuis MH, De Vos Tot Nederveen Cappel W, Botma A, Nagengast FM, Kleibeuker JH, Mathus-Vliegen EM, Dekker E, Dees J, Wijnen J, Vasen HF: Desmoid tumors in a dutch cohort of patients with familial adenomatous polyposis. Clin Gastroenterol Hepatol; 2008 Feb;6(2):215-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Desmoid tumors in a dutch cohort of patients with familial adenomatous polyposis.
  • BACKGROUND & AIMS: Desmoid tumors are a severe extracolonic manifestation in familial adenomatous polyposis (FAP).
  • Identification of risk factors might be helpful in the management of FAP patients with such tumors.
  • The aim of this study was to assess potential risk factors for the development of desmoids in a cohort of Dutch FAP patients.
  • METHODS: The medical records of 735 FAP patients were analyzed for the occurrence of desmoids.
  • Relative risks and survival times were calculated to assess the influence of potential risk factors (female sex, family history, mutation site, abdominal surgery, and pregnancy) on desmoid development.
  • RESULTS: Desmoid tumors were identified in 66 of the 735 patients (9%).
  • The cumulative risk of developing desmoids was 14%.
  • No correlation was found between specific adenomatous polyposis coli mutation sites and desmoid development.
  • Patients with a positive family history for desmoids had a significant increased risk to develop this tumor (30% vs 6.7%, P < .001).
  • No association was found between female sex or pregnancy and desmoid development.
  • Most desmoid patients (95%) had undergone previous abdominal surgery.
  • In a substantial proportion of patients with an ileorectal anastomosis, it was impossible to convert the ileorectal anastomosis to an ileal pouch-anal anastomosis as a result of desmoid development.
  • CONCLUSIONS: A positive family history of desmoids is an evident risk factor for developing desmoids.
  • Most desmoids develop after colectomy.
  • No correlation was found between desmoids and the adenomatous polyposis coli gene mutation site, female sex, and pregnancy.
  • Ileal pouch-anal anastomosis is the appropriate type of surgery in FAP patients with a positive family history for desmoids.
  • [MeSH-major] Adenomatous Polyposis Coli / complications. Fibromatosis, Aggressive / epidemiology

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  • (PMID = 18237870.001).
  • [ISSN] 1542-7714
  • [Journal-full-title] Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association
  • [ISO-abbreviation] Clin. Gastroenterol. Hepatol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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90. Bölke E, Krasniqi H, Lammering G, Engers R, Matuschek C, Gripp S, Gerber PA, Fischer G, Peiper M, Shaikh S, Budach W, Orth K: Chest wall and intrathoracic desmoid tumors: surgical experience and review of the literature. Eur J Med Res; 2009 Jun 18;14(6):240-3

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Chest wall and intrathoracic desmoid tumors: surgical experience and review of the literature.
  • Desmoid tumors are fibroblastic/myofibroblastic neoplasms, which originate from musculo-aponeurotic structures and are classified as deep fibromatoses.
  • Despite their benign histologic appearance and lack of metastatic potential, desmoid tumors may cause aggres?sive local infiltrations and compression of surrounding structures.
  • Molecular studies have demonstrated that these patients are associated with a bi-allelic APC mutation in the affected tissue.
  • Radical tumor resection with free margins remains the first therapy of choice.
  • [MeSH-major] Fibromatosis, Abdominal / pathology. Fibromatosis, Aggressive / pathology. Thoracic Neoplasms / pathology. Thoracic Wall / pathology

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  • [Cites] Cancer. 2000 Apr 1;88(7):1517-23 [10738207.001]
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  • (PMID = 19541583.001).
  • [ISSN] 0949-2321
  • [Journal-full-title] European journal of medical research
  • [ISO-abbreviation] Eur. J. Med. Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 31
  • [Other-IDs] NLM/ PMC3352015
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91. Speake D, Evans DG, Lalloo F, Scott NA, Hill J: Desmoid tumours in patients with familial adenomatous polyposis and desmoid region adenomatous polyposis coli mutations. Br J Surg; 2007 Aug;94(8):1009-13
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Desmoid tumours in patients with familial adenomatous polyposis and desmoid region adenomatous polyposis coli mutations.
  • BACKGROUND: :The aim of this study was to determine the proportion of patients with familial adenomatous polyposis (FAP) who had mutations in the desmoid region of the adenomatous polyposis coli (APC) gene that phenotypically expresses desmoid disease, and to determine the role for surgery in these patients.
  • RESULTS: Of 363 patients with FAP, 47 from ten families had APC mutations in the desmoid region 3' to codon 1399.
  • Of 22 patients undergoing surgery, 16 developed desmoids, and of these 12 had mesenteric desmoid disease.
  • Complications from mesenteric desmoids were death (two patients), enterectomy (three), local resection (three), fistula (one), cholangitis and local resection (one), bowel obstruction (one) and bowel and ureteric obstruction (one).
  • CONCLUSION: In individuals with 3' APC mutations, abdominal surgery is associated with a 65 per cent risk of developing mesenteric desmoids.
  • [MeSH-major] Adenomatous Polyposis Coli / genetics. Fibromatosis, Aggressive / genetics. Genes, APC. Mesentery. Mutation / genetics. Peritoneal Neoplasms / genetics

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  • [Copyright] Copyright (c) 2007 British Journal of Surgery Society Ltd.
  • (PMID = 17410559.001).
  • [ISSN] 0007-1323
  • [Journal-full-title] The British journal of surgery
  • [ISO-abbreviation] Br J Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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92. Jeblaoui Y, Bouguila J, Haddad S, Helali M, Zaïri I, Zitouni K, Mokhtar M, Adouani A: [Mandibular aggressive fibromatosis]. Rev Stomatol Chir Maxillofac; 2007 Apr;108(2):153-5
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  • [Title] [Mandibular aggressive fibromatosis].
  • [Transliterated title] Fibromatose agressive mandibulaire.
  • INTRODUCTION: Aggressive fibromatosis is a rare histologically benign fibrous tumor with a potential for locoregional aggression.
  • Surgical treatment is the reference, chemotherapy, radiotherapy and homonotherapy being proposed as complementary treatment or for inoperable tumors.
  • CASE REPORT: A three-year-old patient underwent surgery for removal of a mandibular tumor.
  • Pathology reported aggressive fibromatosis.
  • DISCUSSION: Conservative surgery for aggressive mandibular fibromatosis appears to be preferable to radical mutilating surgery which would have a major impact on facial growth in children.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Mandibular Neoplasms / pathology

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  • (PMID = 17350660.001).
  • [ISSN] 0035-1768
  • [Journal-full-title] Revue de stomatologie et de chirurgie maxillo-faciale
  • [ISO-abbreviation] Rev Stomatol Chir Maxillofac
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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93. Eren S: A sporadic abdominal desmoid tumour case presenting with intermittent intestinal obstruction. Eur J Pediatr Surg; 2007 Aug;17(4):285-8
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  • [Title] A sporadic abdominal desmoid tumour case presenting with intermittent intestinal obstruction.
  • Desmoid tumours, also known as aggressive fibromatosis, are rare lesions with an intermediate biological behaviour between benign fibrous lesions and fibrosarcomas.
  • Although abdominal desmoids have an increased incidence in Gardner's syndrome, they are rarely found in an isolated form.
  • We report the findings of a barium study, ultrasound, computed tomography and magnetic resonance imaging in a nine-year-old boy with intermittent nausea and vomiting, diagnosed as having a desmoid tumour.
  • Although intra-abdominal desmoids are usually detected as a solitary lesion in sporadic cases, the presented case had two mesenteric lesions in the left upper quadrant.
  • [MeSH-major] Abdominal Neoplasms / complications. Digestive System Surgical Procedures / methods. Fibromatosis, Aggressive / complications. Intestinal Obstruction / etiology
  • [MeSH-minor] Child. Diagnosis, Differential. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Male. Tomography, X-Ray Computed

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  • (PMID = 17806029.001).
  • [ISSN] 0939-7248
  • [Journal-full-title] European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift für Kinderchirurgie
  • [ISO-abbreviation] Eur J Pediatr Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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94. Arshad AR, Normala B: Surgical management of large desmoid tumour of the anterior abdominal wall. Asian J Surg; 2008 Apr;31(2):90-5

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Surgical management of large desmoid tumour of the anterior abdominal wall.
  • Desmoid tumours are uncommon.
  • They are locally invasive and incomplete excision leads to recurrence, which can pose a significant management challenge.
  • Patients therefore require effective treatment, which essentially entails tumour excision with a clear surgical margin.
  • The resulting wide defect may lead to difficulty in closure of the anterior abdominal wall.
  • We report our experience in treating large desmoid tumours of the anterior abdominal wall.
  • Between January 2000 and December 2001, three patients with large desmoid tumour of the anterior abdominal wall were treated with wide excision, which included a 3-cm margin of uninvolved tissues.
  • This led to a considerable abdominal wall defect.
  • The peritoneal defect was closed as a separate layer, though under considerable tension, while the abdominal wall musculature defect was closed with a polypropylene mesh.
  • Follow-up until December 2006 has not revealed any tumour recurrence or hernia development.
  • Wide excision of an anterior abdominal wall desmoid tumour with a clear margin of 3 cm including the peritoneum should be considered when managing such tumours.

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  • (PMID = 18490222.001).
  • [ISSN] 1015-9584
  • [Journal-full-title] Asian journal of surgery
  • [ISO-abbreviation] Asian J Surg
  • [Language] ENG
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] China
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95. Coffin CM, Hornick JL, Zhou H, Fletcher CD: Gardner fibroma: a clinicopathologic and immunohistochemical analysis of 45 patients with 57 fibromas. Am J Surg Pathol; 2007 Mar;31(3):410-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Gardner fibroma: a clinicopathologic and immunohistochemical analysis of 45 patients with 57 fibromas.
  • Gardner fibroma (GAF) is a benign soft tissue lesion with a predilection for childhood and adolescence and an association with familial adenomatous polyposis (FAP) and desmoid type fibromatosis (desmoid).
  • Information about family history, intestinal polyps, colon cancer, and soft tissue tumors was available in 23 patients.
  • Sixty-nine percent had known FAP or adenomatous polyposis coli (APC), 22% had no history of familial polyps or soft tissue tumors, and 13% had an individual or family history of soft tissue masses and/or desmoids, with follow-up periods of 6 months to 26 years (median 3 y, mean 5 y).
  • The age range at initial diagnosis was 2 months to 36 years.
  • Eight patients (18%) had documented desmoids concurrently or later; 4 of these had FAP and 1 had familial desmoids.
  • In conclusion, GAF has a predilection for childhood and early adulthood, a strong association with FAP/APC, an association with concurrent or subsequent development of desmoids, and overexpression of beta-catenin and other proteins in the APC and Wnt pathways.
  • [MeSH-major] Fibroma / pathology. Gardner Syndrome / pathology. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Adenomatous Polyposis Coli / complications. Adenomatous Polyposis Coli / metabolism. Adenomatous Polyposis Coli / pathology. Adolescent. Adult. Biomarkers, Tumor / metabolism. Cell Nucleus / metabolism. Cell Nucleus / pathology. Child. Child, Preschool. Cyclin D. Cyclins / metabolism. Female. Fibromatosis, Aggressive / complications. Fibromatosis, Aggressive / metabolism. Fibromatosis, Aggressive / pathology. Humans. Immunohistochemistry. Infant. Male. Proto-Oncogene Proteins c-myc / metabolism. beta Catenin / metabolism

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  • (PMID = 17325483.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Cyclin D; 0 / Cyclins; 0 / MYC protein, human; 0 / Proto-Oncogene Proteins c-myc; 0 / beta Catenin
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96. Fimmanò A, Coppola Bottazzi E, Cirillo C, Tammaro P, Casazza D: [Desmoid tumor of the chest wall foiling surgery]. Ann Ital Chir; 2006 Mar-Apr;77(2):169-72; discussion 172
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Desmoid tumor of the chest wall foiling surgery].
  • [Transliterated title] Desmoide della parete toracica, ad insorgenza postchirurgica.
  • CASE REPORT: The patient was subjected to laboratory tests, which showed nothing pathological, and to instrumental tests (RX and TAC of the chest, bony scintigraphy) which showed a roundish solid tumefaction, with no "secondary" interest of bony tissue.
  • The anatomo-pathological test showed a desmoid fibromatosis (desmoid tumor) extra-abdominal (12.5 x 9 x 5 cm).
  • [MeSH-major] Fibromatosis, Aggressive / surgery. Thoracic Wall
  • [MeSH-minor] Female. Follow-Up Studies. Humans. Middle Aged. Neoplasm Recurrence, Local / surgery. Thoracotomy. Time Factors

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  • (PMID = 17147093.001).
  • [ISSN] 0003-469X
  • [Journal-full-title] Annali italiani di chirurgia
  • [ISO-abbreviation] Ann Ital Chir
  • [Language] ita
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Italy
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97. Maeda R, Isowa N, Onuma H, Miura H, Tokuyasu H, Kawasaki Y: Desmoid tumor of the chest wall in an elderly woman. Gen Thorac Cardiovasc Surg; 2009 Oct;57(10):554-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Desmoid tumor of the chest wall in an elderly woman.
  • We report a case of desmoid tumor of the chest wall in a 79-year-old woman.
  • Magnetic resonance imaging of the chest revealed a soft tissue tumor in the right lateral chest wall with unclear margins that extended into the intercostal muscles.
  • Because open biopsy suggested a desmoid tumor, full-thickness chest wall resection with reconstruction was performed.
  • The final diagnosis was desmoid tumor of the chest wall.
  • Wide surgical resection during the initial operation is an essential element in the treatment of this tumor.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Intercostal Muscles / pathology. Ribs / pathology. Thoracic Neoplasms / diagnosis. Thoracic Wall / pathology
  • [MeSH-minor] Aged. Biopsy. Female. Fluorodeoxyglucose F18. Humans. Magnetic Resonance Imaging. Neoplasm Invasiveness. Positron-Emission Tomography. Radiopharmaceuticals. Thoracotomy. Tomography, X-Ray Computed

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  • (PMID = 19830521.001).
  • [ISSN] 1863-6713
  • [Journal-full-title] General thoracic and cardiovascular surgery
  • [ISO-abbreviation] Gen Thorac Cardiovasc Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
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98. Peerlinck I, Amini-Nik S, Phillips RK, Iggo R, Lemoine NR, Tejpar S, Vassaux G: Therapeutic potential of replication-selective oncolytic adenoviruses on cells from familial and sporadic desmoid tumors. Clin Cancer Res; 2008 Oct 1;14(19):6187-92
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Therapeutic potential of replication-selective oncolytic adenoviruses on cells from familial and sporadic desmoid tumors.
  • PURPOSE: Constitutive activation of the Wnt signaling pathway is a hallmark of many cancers and has been associated with familial and sporadic desmoid tumors.
  • The aim of the present study is to assess the therapeutic potential of oncolytic adenoviruses selectively replicating in cells in which the Wnt signaling pathway is active on primary cells from desmoid tumors.
  • EXPERIMENTAL DESIGN: Primary cells extracted from familial (n = 3) or sporadic (n = 3) desmoid tumors were cultured short term.
  • RESULTS: Although cells extracted from one sporadic desmoid tumor responded very well to the oncolytic action of the adenoviruses (<20% of viable cells upon infection at a multiplicity of infection of 10), cells from two tumor samples were totally resistant to the viral action.
  • CONCLUSIONS: Our experimental data suggest that the response of desmoid tumor cells to oncolytic adenovirus is neither correlated to the type of mutation activating the Wnt signaling pathway nor to the familial or sporadic nature of the tumor.
  • In addition, they highlight the variability of infectivity of individual tumors and predict a great variability in the response to oncolytic adenoviruses.

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  • (PMID = 18829497.001).
  • [ISSN] 1078-0432
  • [Journal-full-title] Clinical cancer research : an official journal of the American Association for Cancer Research
  • [ISO-abbreviation] Clin. Cancer Res.
  • [Language] ENG
  • [Grant] United Kingdom / Cancer Research UK / / A6251; United Kingdom / Medical Research Council / / G84/6703; United Kingdom / Cancer Research UK / / ; United Kingdom / Medical Research Council / /
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC2575844; NLM/ UKMS2611
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99. Salas S, Chibon F, Noguchi T, Terrier P, Ranchere-Vince D, Lagarde P, Benard J, Forget S, Blanchard C, Dômont J, Bonvalot S, Guillou L, Leroux A, Mechine-Neuville A, Schöffski P, Laë M, Collin F, Verola O, Carbonnelle A, Vescovo L, Bui B, Brouste V, Sobol H, Aurias A, Coindre JM: Molecular characterization by array comparative genomic hybridization and DNA sequencing of 194 desmoid tumors. Genes Chromosomes Cancer; 2010 Jun;49(6):560-8

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Molecular characterization by array comparative genomic hybridization and DNA sequencing of 194 desmoid tumors.
  • Desmoid tumors are fibroblastic/myofibroblastic proliferations.
  • Previous studies reported that CTNNB1 mutations were detected in 84% and that mutations of the APC gene were found in several cases of sporadic desmoid tumors lacking CTNNB1 mutations.
  • Forty tumors were analyzed by comparative genomic hybridization (CGH).
  • We performed array CGH on frozen samples of 194 tumors, and we screened for APC mutations in patients without CNNTB1 mutation.
  • A high frequency of genomically normal tumors was observed.
  • Four relevant and recurrent alterations (loss of 6q, loss of 5q, gain of 20q, and gain of Chromosome 8) were found in 40 out of 46 tumors with chromosomal changes.
  • Alterations of APC, including loss of the entire locus, and CTNNB1 mutation could explain the tumorigenesis in 89% of sporadic desmoids tumors and desmoids tumors occurring in the context of Gardner's syndrome.
  • A better understanding of the pathogenetic pathways in the initiation and progression of desmoid tumors requires studies of 8q and 20q gains, as well as of 6q and 5q losses, and study of the Wnt/beta-catenin pathway.
  • [MeSH-major] Abdominal Neoplasms / genetics. Fibromatosis, Aggressive / genetics

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  • [Copyright] (c) 2010 Wiley-Liss, Inc.
  • (PMID = 20232483.001).
  • [ISSN] 1098-2264
  • [Journal-full-title] Genes, chromosomes & cancer
  • [ISO-abbreviation] Genes Chromosomes Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / CTNNB1 protein, human; 0 / beta Catenin
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100. Eren S: A sporadic abdominal desmoid tumour case presenting with intermittent intestinal obstruction. Eur J Pediatr Surg; 2005 Jun;15(3):196-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A sporadic abdominal desmoid tumour case presenting with intermittent intestinal obstruction.
  • Desmoid tumours, also known as aggressive fibromatoses, are rare lesions having intermediate biological behaviour between benign fibrous lesions and fibrosarcomas.
  • Although abdominal desmoids have an increased incidence in Gardner's syndrome, they are rarely found in isolated form.
  • Although intraabdominal desmoids are usually detected as a solitary lesion in sporadic cases, the case presented here had two mesenteric lesions in the left upper quadrant.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Intestinal Obstruction / complications. Mesentery. Peritoneal Neoplasms / diagnosis
  • [MeSH-minor] Child. Colon / pathology. Humans. Male. Nausea / etiology. Neoplasm Invasiveness. Vomiting / etiology

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  • (PMID = 15999314.001).
  • [ISSN] 0939-7248
  • [Journal-full-title] European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift für Kinderchirurgie
  • [ISO-abbreviation] Eur J Pediatr Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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