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1. Ilaslan H, Schils J, Joyce M, Marks K, Sundaram M: Radiofrequency ablation: another treatment option for local control of desmoid tumors. Skeletal Radiol; 2010 Feb;39(2):169-73
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  • [Title] Radiofrequency ablation: another treatment option for local control of desmoid tumors.
  • Desmoid tumors are fibrous neoplasms that are infiltrative and locally aggressive.
  • Although they are histologically benign with negligible metastatic potential, recurrence after surgical resection is common.
  • Since April 2003, we have used radiofrequency ablation to treat five desmoid tumors in four patients.
  • Complications were seen in two patients; one patient had cellulitis and another had soft tissue necrosis.
  • [MeSH-major] Catheter Ablation / methods. Fibromatosis, Aggressive / diagnosis. Fibromatosis, Aggressive / surgery

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  • (PMID = 19816682.001).
  • [ISSN] 1432-2161
  • [Journal-full-title] Skeletal radiology
  • [ISO-abbreviation] Skeletal Radiol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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2. Körner M, Waser B, Reubi JC: High expression of neuropeptide Y1 receptors in ewing sarcoma tumors. Clin Cancer Res; 2008 Aug 15;14(16):5043-9
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  • [Title] High expression of neuropeptide Y1 receptors in ewing sarcoma tumors.
  • PURPOSE: Peptide receptors are frequently overexpressed in human tumors, allowing receptor-targeted scintigraphic imaging and therapy with radiolabeled peptide analogues.
  • EXPERIMENTAL DESIGN: Tumor tissues of 88 cases, including Ewing sarcoma family of tumors (ESFT), synovial sarcomas, osteosarcomas, chondrosarcomas, liposarcomas, angiosarcomas, rhabdomyosarcomas, leiomyosarcomas, and desmoid tumors, were investigated for NPY receptor protein with in vitro receptor autoradiography using (125)I-labeled NPY receptor ligands and for NPY receptor mRNA expression with in situ hybridization.
  • RESULTS: ESFT expressed the NPY receptor subtype Y1 on tumor cells in remarkably high incidence (84%) and density (mean, 5,314 dpm/mg tissue).
  • Likewise, synovial sarcomas expressed Y1 on tumor cells in high density (mean, 7,497 dpm/mg; incidence, 40%).
  • The remaining tumors expressed NPY receptor subtypes Y1 or Y2 at lower levels.
  • The high Y1 expression on tumor cells of ESFT and synovial sarcomas and on blood vessels in many other sarcomas represents an attractive basis for an in vivo tumor targeting.
  • [MeSH-major] Biomarkers, Tumor / analysis. Bone Neoplasms / metabolism. Receptors, Neuropeptide Y / biosynthesis. Sarcoma, Ewing / metabolism
  • [MeSH-minor] Arteries / metabolism. Autoradiography. Gene Expression. Humans. In Situ Hybridization. RNA, Messenger / analysis. Sarcoma / blood supply. Sarcoma / metabolism. Soft Tissue Neoplasms / blood supply. Soft Tissue Neoplasms / metabolism

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  • (PMID = 18698022.001).
  • [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
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / RNA, Messenger; 0 / Receptors, Neuropeptide Y; 0 / neuropeptide Y-Y1 receptor
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3. Wilhelm A, Jolles HI, Krishna M: Anterior mediastinal desmoid tumor with CT and MR imaging. J Thorac Imaging; 2007 Aug;22(3):252-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Anterior mediastinal desmoid tumor with CT and MR imaging.
  • Desmoid tumors, or aggressive fibromatoses, are rare, histologically benign tumors with biologic behavior that is intermediate between benign fibrous proliferations and low-grade fibrosarcoma.
  • These tumors can cause significant morbidity and mortality by their propensity for local invasion and recurrence following surgical excision.
  • A case of an anterior mediastinal desmoid tumor in a 67-year-old woman is reported with computed tomography and magnetic resonance imaging findings.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Mediastinal Neoplasms / diagnosis
  • [MeSH-minor] Aged. Contrast Media. Diagnosis, Differential. Female. Humans. Magnetic Resonance Imaging. Tomography, X-Ray Computed

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  • (PMID = 17721335.001).
  • [ISSN] 0883-5993
  • [Journal-full-title] Journal of thoracic imaging
  • [ISO-abbreviation] J Thorac Imaging
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Contrast Media
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4. Lazar AJ, Hajibashi S, Lev D: Desmoid tumor: from surgical extirpation to molecular dissection. Curr Opin Oncol; 2009 Jul;21(4):352-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Desmoid tumor: from surgical extirpation to molecular dissection.
  • PURPOSE OF REVIEW: Desmoid tumors are associated with a variable and unpredictable clinical course.
  • Little is known regarding the molecular determinates of desmoid tumor behavior.
  • Some recent work has focused on the role of beta-catenin in desmoid tumor biology.
  • RECENT FINDINGS: Given the variable clinical course of desmoid tumors, the interpretation of factors classically associated with recurrence such as microscopic status of margins appears more nuanced that previously thought.
  • The application of multidisciplinary assessment with multimodality treatment, including surgery, radiation and systemic therapies may underlie these changes and now form the basis of care for this tumor.
  • SUMMARY: Establishing the population benefiting most from various treatment modalities and combinations is critical for progress in this disease.
  • Additional study of the molecular determinates of desmoid behavior is needed to guide therapeutic selection.
  • [MeSH-major] Fibromatosis, Aggressive / therapy

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  • (PMID = 19436199.001).
  • [ISSN] 1531-703X
  • [Journal-full-title] Current opinion in oncology
  • [ISO-abbreviation] Curr Opin Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / CTNNB1 protein, human; 0 / Wnt Proteins; 0 / beta Catenin
  • [Number-of-references] 52
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5. 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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  • [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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6. Welle MM, Sutter E, Malik Y, Konar M, Rüfenacht S, Howard JE: Fibromatosis in a young Bernese Mountain Dog: clinical, imaging, and histopathological findings. J Vet Diagn Invest; 2009 Nov;21(6):895-900
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  • [Title] Fibromatosis in a young Bernese Mountain Dog: clinical, imaging, and histopathological findings.
  • A young, intact, male Bernese Mountain Dog was presented to the animal hospital for lameness and diffuse thickening of the soft tissue in the right hind limb.
  • Biopsies taken from the lesions revealed an infiltrative mass composed mainly of collagen fibers and a low density of benign-appearing fibroblasts.
  • These findings were compatible with a diagnosis of a fibromatosis.
  • Taking the age of onset into account, infantile fibromatosis was most likely.
  • A deep fibromatosis, similar to that seen in adults, could not be excluded based on histology.
  • [MeSH-major] Dog Diseases / pathology. Fibroma / veterinary. Hindlimb / pathology

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  • (PMID = 19901299.001).
  • [ISSN] 1040-6387
  • [Journal-full-title] Journal of veterinary diagnostic investigation : official publication of the American Association of Veterinary Laboratory Diagnosticians, Inc
  • [ISO-abbreviation] J. Vet. Diagn. Invest.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Vimentin
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7. de Campos FG, Perez RO, Imperiale AR, Seid VE, Nahas SC, Cecconello I: Evaluating causes of death in familial adenomatous polyposis. J Gastrointest Surg; 2010 Dec;14(12):1943-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • At diagnosis, 57 patients (58.7%) already had CRC-associated polyposis.
  • CRC, other tumors (desmoid tumors, lymphoma, and gastric cancer), and other causes (complication of duodenal cancer surgery, complication after ileorectal anastomosis (IRA), and coronary disease) were responsible for 12 (63.1%), four (21.1%), and three (15.8%) of all deaths, respectively.
  • Desmoid disease was the second cause of death (10.5% of all causes), leading to a fatal outcome 22% of all patients who developed DT during the study period.
  • (3) long-term survival was also strongly related to the development of extracolonic neoplasia, especially desmoid tumors and gastroduodenal carcinoma;.

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  • (PMID = 20676788.001).
  • [ISSN] 1873-4626
  • [Journal-full-title] Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract
  • [ISO-abbreviation] J. Gastrointest. Surg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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8. Lips DJ, Barker N, Clevers H, Hennipman A: The role of APC and beta-catenin in the aetiology of aggressive fibromatosis (desmoid tumors). Eur J Surg Oncol; 2009 Jan;35(1):3-10
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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 APC and beta-catenin in the aetiology of aggressive fibromatosis (desmoid tumors).
  • BACKGROUND: Aggressive fibromatosis (syn. desmoid tumor) is a sporadically occurring neoplastic proliferation of fibroblasts originating from musculoaponeurotic planes, forming invasively growing masses without the capability to metastasize.
  • Better understanding of the aetiology of aggressive fibromatosis is needed to be able to develop new treatment strategies to cope with the high recurrence rates.
  • The following search terms were used: 'aggressive fibromatosis', 'desmoid tumor', 'adenomatous polyposis coli', 'APC', 'beta-catenin', 'Wnt', 'Wingless' and 'Wnt/Wingless'.
  • RESULTS: The neoplastic nature of aggressive fibromatosis and the role of the adenomatous polyposis coli (APC) and beta-catenin signaling cascade in driving the onset and progression of this disease are discussed.
  • CONCLUSION: Mutations in either the APC or beta-catenin genes are likely to be a major driving force in the formation of these desmoid tumors.
  • [MeSH-major] Adenomatous Polyposis Coli Protein / genetics. Fibromatosis, Aggressive / genetics. Fibromatosis, Aggressive / metabolism. Signal Transduction / genetics. beta Catenin / genetics


9. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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10. Tulchinsky H, Keidar A, Goldman G, Klausner JM, Rabau M: Surgical treatment and long-term outcome of patients with familial adenomatous polyposis: 16 years experience at the Tel Aviv Sourasky Medical Center. Isr Med Assoc J; 2005 Feb;7(2):82-5
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  • Complications and extra-intestinal manifestations are inherent to the procedure.
  • Extracolonic manifestations developed in 38 patients, including desmoid tumors (in 12), duodenal adenomas (in 9), pouch adenomas (in 5), and rectal stump adenomas (in 3).
  • Two patients died (4%) because of desmoid tumor and malignant fibrous histiocytoma.


11. Chen CB, Chiou YY, Chen CH, Chou YH, Chiang JH, Chang CY: Sonographic and computed tomography findings of intra-abdominal desmoid tumor. J Chin Med Assoc; 2010 Jul;73(7):393-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Sonographic and computed tomography findings of intra-abdominal desmoid tumor.
  • Intra-abdominal desmoid tumor is rare and seldom reported in the literature.
  • Here, we present a 29-year-old male who suffered from intra-abdominal desmoid tumor, and describe its imaging findings on ultrasound and abdominal computed tomography.
  • This tumor usually presents as a large homogeneous hypodense solid mass on computed tomography, and demonstrates mild enhancement after contrast medium administration.
  • Although rare, it should be included in the differential diagnosis when a patient presents with a large abdominal mass.
  • [MeSH-major] Abdominal Neoplasms / diagnosis. Fibromatosis, Aggressive / diagnosis. Tomography, X-Ray Computed / methods
  • [MeSH-minor] Adult. Diagnosis, Differential. Humans. Magnetic Resonance Imaging. Male

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  • [Copyright] 2010 Elsevier. Published by Elsevier B.V. All rights reserved.
  • (PMID = 20688307.001).
  • [ISSN] 1728-7731
  • [Journal-full-title] Journal of the Chinese Medical Association : JCMA
  • [ISO-abbreviation] J Chin Med Assoc
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] China (Republic : 1949- )
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12. 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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13. Lahat G, Nachmany I, Itzkowitz E, Abu-Abeid S, Barazovsky E, Merimsky O, Klauzner J: Surgery for sporadic abdominal desmoid tumor: is low/no recurrence an achievable goal? Isr Med Assoc J; 2009 Jul;11(7):398-402
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  • [Title] Surgery for sporadic abdominal desmoid tumor: is low/no recurrence an achievable goal?
  • BACKGROUND: Sporadic abdominal desmoid tumors are rare and data on these tumors as a distinct disease entity are lacking.
  • Previous abdominal surgery, trauma, pregnancy and estrogen intake are considered risk factors.
  • Although desmoids are benign, invasion and a high recurrence rate are common.
  • OBJECTIVES: To evaluate outcomes of surgery for this rare disease.
  • METHODS: Since 1995, 16 patients with pathologically confirmed desmoid tumor were operated on in our center.
  • All patients presented with an isolated mass; 7 (50%) originated in the abdominal wall, 6 (37.5%) were retroperitoneal and 3 were (18.8%) mesenteric.
  • All tumors except one were completely excised.
  • CONCLUSIONS: The perception of sporadic abdominal desmoids as tumors with a high recurrence rate (20-70%) is probably incorrect.
  • [MeSH-major] Abdominal Neoplasms / surgery. Fibromatosis, Aggressive / surgery

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  • (PMID = 19911489.001).
  • [ISSN] 1565-1088
  • [Journal-full-title] The Israel Medical Association journal : IMAJ
  • [ISO-abbreviation] Isr. Med. Assoc. J.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Israel
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14. 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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  • [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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15. Vaidya JS, Kocabayoglu P, McLean D, Jordan L: Eyes do not see what the mind does not know--a desmoid tumour in the breast. Int J Surg; 2008 Dec;6(6):469-70
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  • [Title] Eyes do not see what the mind does not know--a desmoid tumour in the breast.
  • [MeSH-major] Breast / pathology. Breast Neoplasms / diagnosis. Fibromatosis, Aggressive / diagnosis

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  • (PMID = 19059147.001).
  • [ISSN] 1743-9159
  • [Journal-full-title] International journal of surgery (London, England)
  • [ISO-abbreviation] Int J Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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16. 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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  • [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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17. Zhu Z, Li F, Zhuang H, Yan J, Wu C, Cheng W: FDG PET/CT detection of intussusception caused by aggressive fibromatosis. Clin Nucl Med; 2010 May;35(5):370-3
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  • [Title] FDG PET/CT detection of intussusception caused by aggressive fibromatosis.
  • [MeSH-major] Fibromatosis, Aggressive / complications. Fluorodeoxyglucose F18. Intussusception / etiology. Intussusception / radionuclide imaging. Positron-Emission Tomography. Tomography, X-Ray Computed

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  • (PMID = 20395719.001).
  • [ISSN] 1536-0229
  • [Journal-full-title] Clinical nuclear medicine
  • [ISO-abbreviation] Clin Nucl Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0Z5B2CJX4D / Fluorodeoxyglucose F18
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18. Dar'ialova SL, Boĭko AV, Novikova OV, Bychkova NM: [The enigma of desmoid fibroma]. Vopr Onkol; 2006;52(4):472-8
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  • [Title] [The enigma of desmoid fibroma].
  • [MeSH-major] Antineoplastic Agents, Hormonal / therapeutic use. Fibroma, Desmoplastic. Fibromatosis, Aggressive
  • [MeSH-minor] Abdominal Neoplasms / drug therapy. Adolescent. Adult. Buserelin / therapeutic use. Child. Child, Preschool. Female. Follow-Up Studies. Goserelin / therapeutic use. Humans. Infant. Male. Middle Aged. Tamoxifen / therapeutic use. Treatment Outcome

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  • (PMID = 17024827.001).
  • [ISSN] 0507-3758
  • [Journal-full-title] Voprosy onkologii
  • [ISO-abbreviation] Vopr Onkol
  • [Language] rus
  • [Publication-type] Journal Article
  • [Publication-country] Russia (Federation)
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 094ZI81Y45 / Tamoxifen; 0F65R8P09N / Goserelin; PXW8U3YXDV / Buserelin
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19. Meazza C, Casanova M, Trecate G, Ferrari A: Objective response to hydroxyurea in a patient with heavily pre-treated aggressive fibromatosis. Pediatr Blood Cancer; 2010 Sep;55(3):587-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Objective response to hydroxyurea in a patient with heavily pre-treated aggressive fibromatosis.
  • [MeSH-major] Fibromatosis, Aggressive / drug therapy. Hydroxyurea / therapeutic use

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  • (PMID = 20658640.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] United States
  • [Chemical-registry-number] X6Q56QN5QC / Hydroxyurea
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20. Pérez García FJ, Pinto Blazquez J, Rodríguez Martínez JJ, Gutiérrez García R, Jorge Barreiro JI, Velasco Alonso J: [Mesenteric desmoid tumor mimicking a testicular cancer recurrence]. Arch Esp Urol; 2007 Jul-Aug;60(6):703-6
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  • [Title] [Mesenteric desmoid tumor mimicking a testicular cancer recurrence].
  • [Transliterated title] Tumor desmoide mesentérico simulando una recidiva de cáncer testicular.
  • OBJECTIVE: Report of one case of desmoid tumor in a patient who had been treated of a testicular seminoma 26 months before, with excision of a retroperitoneal mass and chemotherapy.
  • On followup he presented with a mesenteric abdominal mass which was clinically labeleled as a recurrence of the seminoma.
  • RESULTS: Histologically it was reported as a mesenteric desmoid tumor.
  • Differential diagnosis with gastrointestinal stromal tumor was performed with immunohistochemical studies.
  • CONCLUSIONS: Desmoid tumor is rare.
  • There are few cases reported in patients with history of previous testicular tumor.
  • It should be included in the differential diagnosis of testicular tumor recurrences.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Mesentery. Peritoneal Neoplasms / diagnosis. Seminoma / diagnosis. Testicular Neoplasms / diagnosis
  • [MeSH-minor] Adult. Diagnosis, Differential. Humans. Male. Neoplasm Recurrence, Local / diagnosis

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  • (PMID = 17847750.001).
  • [ISSN] 0004-0614
  • [Journal-full-title] Archivos españoles de urología
  • [ISO-abbreviation] Arch. Esp. Urol.
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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21. Arena S, Salamone S, Cianci R, Scollo C, Masucci R, Giannone G, Manusia M, Vigneri R, La Rosa GL: Aggressive fibromatosis of the neck initiated after thyroidectomy. J Endocrinol Invest; 2006 Jan;29(1):78-81
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  • [Title] Aggressive fibromatosis of the neck initiated after thyroidectomy.
  • Fine needle aspiration biopsy revealed the presence of fibroblast-like cells, partially with atypical features and no colloid: the cytological diagnosis was suspicious for an indeterminate (mesenchymal) neoplasm.
  • Histological diagnosis, after extensive surgery, indicated aggressive fibromatosis.
  • [MeSH-major] Fibromatosis, Aggressive / etiology. Goiter, Nodular / surgery

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  • [ISSN] 0391-4097
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22. Kasper B, Dimitrakopoulou-Strauss A, Strauss LG, Hohenberger P: Positron emission tomography in patients with aggressive fibromatosis/desmoid tumours undergoing therapy with imatinib. Eur J Nucl Med Mol Imaging; 2010 Oct;37(10):1876-82
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Positron emission tomography in patients with aggressive fibromatosis/desmoid tumours undergoing therapy with imatinib.
  • PURPOSE: We used (18)F-FDG PET to evaluate the FDG uptake in patients with aggressive fibromatosis (AF, also known as desmoid tumours) undergoing therapy with imatinib (imatinib mesylate, Glivec).
  • Restaging according to the Response Evaluation Criteria in Solid Tumors (RECIST) was performed in parallel using CT and/or MRI and served as reference.
  • RESULTS: The clinical outcomes in nine evaluable patients were as follows: seven patients with stable disease, and two patients with progressive disease.
  • [MeSH-major] Fibromatosis, Aggressive / diagnostic imaging. Fibromatosis, Aggressive / drug therapy. Piperazines / therapeutic use. Positron-Emission Tomography. Pyrimidines / therapeutic use

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  • (PMID = 20559633.001).
  • [ISSN] 1619-7089
  • [Journal-full-title] European journal of nuclear medicine and molecular imaging
  • [ISO-abbreviation] Eur. J. Nucl. Med. Mol. Imaging
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Benzamides; 0 / Piperazines; 0 / Pyrimidines; 0Z5B2CJX4D / Fluorodeoxyglucose F18; 8A1O1M485B / Imatinib Mesylate
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23. Church J, Lynch C, Neary P, LaGuardia L, Elayi E: A desmoid tumor-staging system separates patients with intra-abdominal, familial adenomatous polyposis-associated desmoid disease by behavior and prognosis. Dis Colon Rectum; 2008 Jun;51(6):897-901
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  • [Title] A desmoid tumor-staging system separates patients with intra-abdominal, familial adenomatous polyposis-associated desmoid disease by behavior and prognosis.
  • PURPOSE: Intra-abdominal desmoid tumors associated with familial adenomatous polyposis are heterogeneous.
  • A recent staging system categorizes desmoids according to size, symptoms, and complications.
  • RESULTS: There were 21 patients with Stage I tumors, 36 with Stage II, 26 with Stage III, and 18 with Stage IV.
  • Twelve patients with Stage I and five with Stage II tumors needed no treatment.
  • Eight patients with Stage I disease received medical treatment, as did 26 with Stage II, 16 with Stage III, and 15 with Stage IV.
  • Six Stage I tumors had surgery, as did 20 Stage II, 12 Stage III, and 13 Stage IV.
  • Chemotherapy was given to 1 Stage II tumor, 7 Stage III, and 5 Stage IV.
  • No patient with Stages I or II disease died.
  • Four Stage III patients (15 percent) and 8 Stage IV patients (44 percent) died from desmoids.
  • Finally 89 percent with Stage I, 65 percent with Stage II, 59 percent with Stage III, and 50 percent with Stage IV disease were asymptomatic; 81 percent of Stage I desmoids, 78 percent of Stage II, 42 percent of Stage III, and 28 percent of Stage IV were stable or disappeared.
  • CONCLUSION: Desmoid staging identifies tumors by prognosis and its use for designing prospective treatment studies is reasonable.
  • [MeSH-major] Adenomatous Polyposis Coli / complications. Fibromatosis, Abdominal / pathology. Fibromatosis, Aggressive / pathology. Neoplasm Staging / methods


24. Ekwueme KC, McCarthy E, Healey C, Ellis A, Rooney PS: A novel frameshift mutation in the APC gene at exon 15 in familial adenomatous polyposis (FAP) with desmoid tumour. Scand J Gastroenterol; 2007 Jun;42(6):788-90
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A novel frameshift mutation in the APC gene at exon 15 in familial adenomatous polyposis (FAP) with desmoid tumour.
  • [MeSH-major] Adenomatous Polyposis Coli / genetics. Exons. Fibromatosis, Aggressive / complications. Frameshift Mutation. Genes, APC

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  • (PMID = 17506007.001).
  • [ISSN] 0036-5521
  • [Journal-full-title] Scandinavian journal of gastroenterology
  • [ISO-abbreviation] Scand. J. Gastroenterol.
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] Norway
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25. Ristić D, Tomasević Z: Therapy of aggressive fibromatosis is still an open question: a series of patients treated at a single institution. J BUON; 2005 Jul-Sep;10(3):381-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Therapy of aggressive fibromatosis is still an open question: a series of patients treated at a single institution.
  • PURPOSE: This study was an attempt to evaluate the possible role of chemo-hormonotherapy as a possible approach in managing inoperable, deep extra-abdominal aggressive fibromatosis.
  • PATIENTS AND METHODS: A series of patients with inoperable, deep extra-abdominal aggressive fibromatosis, were treated with combination chemo-hormotherapy.
  • Extremities were the most frequent localization (5/9), followed by chest wall in 3 and abdominal wall in one patient.
  • Tumor size in most patients was 5-10 cm, and 3 patients had bulky disease (over 10 cm).
  • Complete remission (CR) was observed in one patient, partial remission (PR) in 4 and stabilization of disease (SD) in 4 patients.
  • No relapse of disease was observed up until now.
  • CONCLUSION: Systemic treatment should be considered in patients with aggressive fibromatosis for whom local treatment approaches are not possible or have failed.

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  • (PMID = 17357193.001).
  • [ISSN] 1107-0625
  • [Journal-full-title] Journal of B.U.ON. : official journal of the Balkan Union of Oncology
  • [ISO-abbreviation] J BUON
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Greece
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26. Zippel DB, Temple WJ: When is a neoplasm not a neoplasm? When it is a desmoid. J Surg Oncol; 2007 Mar 1;95(3):190-1
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] When is a neoplasm not a neoplasm? When it is a desmoid.
  • [MeSH-major] Fibromatosis, Aggressive / therapy
  • [MeSH-minor] Anti-Inflammatory Agents, Non-Steroidal / therapeutic use. Antineoplastic Agents, Hormonal / therapeutic use. Celecoxib. Humans. Neoadjuvant Therapy. Neoplasm Recurrence, Local / prevention & control. Pyrazoles / therapeutic use. Sulfonamides / therapeutic use. Tamoxifen / therapeutic use

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  • (PMID = 17323331.001).
  • [ISSN] 0022-4790
  • [Journal-full-title] Journal of surgical oncology
  • [ISO-abbreviation] J Surg Oncol
  • [Language] eng
  • [Publication-type] Editorial
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Anti-Inflammatory Agents, Non-Steroidal; 0 / Antineoplastic Agents, Hormonal; 0 / Pyrazoles; 0 / Sulfonamides; 094ZI81Y45 / Tamoxifen; JCX84Q7J1L / Celecoxib
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27. Ferchichi L, Kourda N, Zermani R, Aouem J, Zaouche A, Abdjellil Z, Najah N, Baltagi Ben Jilani S: [Extragastrointestinal stromal tumors: a report of 4 cases]. Ann Chir; 2006 Apr;131(4):271-5
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  • [Title] [Extragastrointestinal stromal tumors: a report of 4 cases].
  • [Transliterated title] Les tumeurs stromales extradigestives: à propos de quatre observations.
  • Gastrointestinal stromal tumors (GIST) are mesenchymal tumors that arise from the wall of the gastrointestinal tract expressing CD117 and/or the CD34.
  • Similar tumors were described in the soft tissue of the abdomen and are so-called extragastrointestinal stromal tumors (EGIST).
  • The tumors occurred in 2 women and 2 men, who ranged in age from 42 to 71 years.
  • Three tumors arose from the soft tissue of the abdominal cavity, and the remainder arose from the retroperitoneum.
  • Three cases were composed purely of short fusiform cells tumors, the last case showed an epithelioid pattern.
  • All the tumors expressed CD117.
  • Immunohistochemistry is useful in distinguishing EGIST from other mesenchymal tumors.
  • Intra-abdominal aggressive fibromatosis may express actin and CD117 but catenin is also positive, leiomyosarcoma expresses the actin and/or desmin but CD117 is usually negative, retroperitoneal dedifferentiated liposarcoma is CD117 negative and PS100 positive, inflammatory myofibroblastic tumor is negative for CD117 and CD34.
  • The solitary fibrous tumor expresses CD34 and is negative to CD117.
  • Some tumors expressing CD117 such desmoplastic round cell tumor should not be confused with EGIST.
  • On the occasion of these four observations, we will discuss the clinical aspects and the main differential diagnoses of this tumor.
  • [MeSH-major] Abdominal Neoplasms. Soft Tissue Neoplasms
  • [MeSH-minor] Adult. Aged. Female. Gastrointestinal Stromal Tumors. Humans. Male. Middle Aged. Retrospective Studies

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  • (PMID = 16472759.001).
  • [ISSN] 0003-3944
  • [Journal-full-title] Annales de chirurgie
  • [ISO-abbreviation] Ann Chir
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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28. Meneu Díaz JC, Moreno González E, García García JI, Moreno A, Montejo JC, Colina F, Pérez B, Rodríguez S, Abradelos de Usera M, Garfia C, Fundora Y, Jiménez Galanes S, Lumbreras C, León M, Pérez Cerdá F, Solís-Herruzo JA: First Spanish series of intestinal transplantation in adult recipients. Rev Esp Enferm Dig; 2006 Oct;98(10):723-39
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  • RESULTS: to this date 5 transplants have been carried out in 4 patients (2 retransplants, 2 desmoid tumors, 1 short bowel syndrome after excision as a result of mesenteric ischemia).

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  • (PMID = 17094721.001).
  • [ISSN] 1130-0108
  • [Journal-full-title] Revista española de enfermedades digestivas : organo oficial de la Sociedad Española de Patología Digestiva
  • [ISO-abbreviation] Rev Esp Enferm Dig
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Spain
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29. Roa JA, Martínez GR, González LM, Cedillo EA, Pineda NF: [Muscle-aponeurotic aggressive fibromatosis management. 10 years of experience]. Acta Ortop Mex; 2008 Mar-Apr;22(2):85-9
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  • [Title] [Muscle-aponeurotic aggressive fibromatosis management. 10 years of experience].
  • [Transliterated title] Manejo de la fibromatosis musculoaponeurótica agresiva. 10 años de experiencia.
  • INTRODUCTION: This work presents clinical evolution and functional outcome of patients with histologic diagnosis of Muscle-aponeurotic aggressive fibromatosis in 10 years of follow up in the National Rehabilitation Institute.
  • MATERIAL AND METHODS: We performed a descriptive, retrospective, cross sectioned clinical trial in the Bone Tumour Department.
  • INCLUSION CRITERIA: both genres, any age, clinical and histological diagnosis.
  • EXCLUSION CRITERIA: incomplete file, lost to follow up, other diagnosis.
  • CONCLUSIONS: If clinical suspicion of Muscle-aponeurotic fibromatosis appears, one should start protocol work up: laboratory, radiographic assessment, nuclear medicine, biopsy and histologic and immune-histochemistry.
  • One should perform broad resections aiming to preserve the most possible extremity tissue disease free.
  • [MeSH-major] Fibromatosis, Aggressive. Muscle Neoplasms

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  • (PMID = 18669308.001).
  • [ISSN] 2306-4102
  • [Journal-full-title] Acta ortopédica mexicana
  • [ISO-abbreviation] Acta Ortop Mex
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Mexico
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30. Coco DP, Hirsch MS, Hornick JL: Smoothelin is a specific marker for smooth muscle neoplasms of the gastrointestinal tract. Am J Surg Pathol; 2009 Dec;33(12):1795-801
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  • Smoothelin expression in mesenchymal tumors of the gastrointestinal (GI) tract has not been evaluated earlier.
  • The purpose of this study was to determine whether immunostaining for smoothelin could help distinguish smooth muscle neoplasms from their morphologic mimics, particularly KIT-negative gastrointestinal stromal tumors (GISTs), desmin-positive GISTs, and desmoid fibromatosis.
  • A total of 150 mesenchymal neoplasms of the GI tract, abdominal cavity, and retroperitoneum were retrieved from consult and surgical pathology archives, including 54 GISTs (8 KIT-negative; 13 desmin-positive), 17 GI leiomyosarcomas (LMS), 11 GI mural leiomyomas, 13 leiomyomas of the muscularis mucosae, 12 gastric schwannomas, 15 inflammatory myofibroblastic tumors, 9 cases of mesenteric desmoid fibromatosis, 10 dedifferentiated liposarcomas, and 9 malignant peripheral nerve sheath tumors.
  • Cytoplasmic expression of smoothelin was present in all 24 (100%) benign smooth muscle tumors (mural leiomyomas and leiomyomas of the muscularis mucosae).
  • None of the GISTs, desmoid tumors, inflammatory myofibroblastic tumors, schwannomas, dedifferentiated liposarcomas, or malignant peripheral nerve sheath tumors showed cytoplasmic reactivity for smoothelin.
  • Nuclear expression of smoothelin was not detected in any of the other tumor types examined.
  • In summary, diffuse cytoplasmic staining for smoothelin is highly sensitive and specific for benign leiomyomas of the GI tract.
  • Aberrant nuclear expression is common in GI LMS and may also be seen in GISTs, especially epithelioid and mixed-type tumors.
  • These findings suggest that the extent and pattern of smoothelin expression may help differentiate between benign and malignant mesenchymal tumors of the GI tract, and may be useful in distinguishing leiomyomas from KIT-negative and/or desmin-positive GISTs.
  • [MeSH-major] Biomarkers, Tumor / analysis. Cytoskeletal Proteins / analysis. Gastrointestinal Neoplasms / chemistry. Muscle Proteins / analysis. Smooth Muscle Tumor / chemistry
  • [MeSH-minor] Cell Differentiation. Cell Nucleus / chemistry. Cytoplasm / chemistry. Desmin / analysis. Diagnosis, Differential. Humans. Immunohistochemistry. Neoplasm Staging. Predictive Value of Tests. Proto-Oncogene Proteins c-kit / analysis. Sensitivity and Specificity

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  • (PMID = 19950405.001).
  • [ISSN] 1532-0979
  • [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 / Cytoskeletal Proteins; 0 / Desmin; 0 / Muscle Proteins; 0 / SMTN protein, human; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit
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31. Skubitz KM, Manivel JC, Clohisy DR, Frolich JW: Response of imatinib-resistant extra-abdominal aggressive fibromatosis to sunitinib: case report and review of the literature on response to tyrosine kinase inhibitors. Cancer Chemother Pharmacol; 2009 Aug;64(3):635-40
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Response of imatinib-resistant extra-abdominal aggressive fibromatosis to sunitinib: case report and review of the literature on response to tyrosine kinase inhibitors.
  • PURPOSE: Aggressive fibromatosis (AF) is usually a slowly growing locally invasive tumor, but may exhibit a much more aggressive phenotype.
  • METHODS: We report a case of an aggressive multicentric extra-abdominal AF that was responsive to sunitinib, but resistant to imatinib.
  • After 13 months, tumors recurred.
  • [MeSH-major] Fibromatosis, Aggressive / drug therapy. Indoles / therapeutic use. Protein Kinase Inhibitors / therapeutic use. Pyrroles / therapeutic use
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Benzamides. Drug Resistance, Neoplasm. Female. Follow-Up Studies. Humans. Imatinib Mesylate. Neoplasm Recurrence, Local. Piperazines / therapeutic use. Pyrimidines / therapeutic use. Treatment Outcome. Young Adult

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  • (PMID = 19404642.001).
  • [ISSN] 1432-0843
  • [Journal-full-title] Cancer chemotherapy and pharmacology
  • [ISO-abbreviation] Cancer Chemother. Pharmacol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Benzamides; 0 / Indoles; 0 / Piperazines; 0 / Protein Kinase Inhibitors; 0 / Pyrimidines; 0 / Pyrroles; 0 / sunitinib; 8A1O1M485B / Imatinib Mesylate
  • [Number-of-references] 40
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32. 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
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  • [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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33. Teo HE, Peh WC, Shek TW: Case 84: desmoid tumor of the abdominal wall. Radiology; 2005 Jul;236(1):81-4
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  • [Title] Case 84: desmoid tumor of the abdominal wall.
  • [MeSH-major] Fibromatosis, Abdominal / diagnosis
  • [MeSH-minor] Adult. Diagnosis, Differential. Female. Humans. Magnetic Resonance Imaging. Tomography, X-Ray Computed

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  • (PMID = 15987965.001).
  • [ISSN] 0033-8419
  • [Journal-full-title] Radiology
  • [ISO-abbreviation] Radiology
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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34. Güzey FK, Emel E, Bas NS, Ozkan N, Turgut H, Sel B: Aggressive postoperative lumbar fibromatosis after the placement of instrumentation for treatment of spondylolisthesis. Case report. J Neurosurg Spine; 2006 Apr;4(4):338-41
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  • [Title] Aggressive postoperative lumbar fibromatosis after the placement of instrumentation for treatment of spondylolisthesis. Case report.
  • This 50-year-old woman presented with a paravertebral lumbar fibromatosis (desmoid tumor) after undergoing the placement of instrumentation for lumbar spondylolisthesis.
  • The tumor developed just cranial to the previous skin incision.
  • Fibromatoses, or desmoid tumors, are uncommon infiltrative lesions that affect musculoaponeurotic structures, most often of the trunk and limbs.
  • In cases involving well-defined tumors in which radical resection is possible, surgery is the treatment of choice; however, the recurrence rate is high.
  • Although rare, this distinctive tumor should be considered in the differential diagnosis of postoperative paravertebral bulgings after neurosurgical and spinal operations.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Soft Tissue Neoplasms / pathology. Spondylolisthesis / surgery

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  • (PMID = 16619683.001).
  • [ISSN] 1547-5654
  • [Journal-full-title] Journal of neurosurgery. Spine
  • [ISO-abbreviation] J Neurosurg Spine
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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35. Zisis C, Dountsis A, Nikolaides A, Dahabreh J: Desmoid tumors of the chest wall. Asian Cardiovasc Thorac Ann; 2006 Oct;14(5):359-62
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  • [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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36. Eralp L, Toker B, Akgül T, Ozger H, Kocaoğlu M, Hayat S: [Applications of external fixation for management of complications associated with musculoskeletal tumors and related surgery]. Acta Orthop Traumatol Turc; 2009 May-Jul;43(3):219-28
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  • [Title] [Applications of external fixation for management of complications associated with musculoskeletal tumors and related surgery].
  • OBJECTIVES: We evaluated the results of, and the course of treatment with, external fixation (EF) in treating complications associated with bone tumors and related surgery.
  • Histologic diagnoses were osteosarcoma (n=3), Ewing's sarcoma (n=3), hereditary multiple exostosis (n=3), chondrosarcoma (n=2), synovial sarcoma (n=2), Ollier's disease, giant cell tumor of bone, desmoid fibroma, chondromyxoid fibroma, and enchondroma.
  • Complications secondary to bone tumors (n=4) and occurring following limb salvage surgery (n=14) were treated with Ilizarov circular EF in nine patients, unilateral EF in six patients, and both in three patients.
  • The second group consisted of six patients who had shortening secondary to tumor surgery.
  • The third group included four patients with deformity and shortening secondary to multiple exostosis (n=3) and Ollier's disease.
  • CONCLUSION: The use of EF in the management of complications associated with bone tumors and related surgery yields successful results especially in young patients.

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  • (PMID = 19717939.001).
  • [ISSN] 1017-995X
  • [Journal-full-title] Acta orthopaedica et traumatologica turcica
  • [ISO-abbreviation] Acta Orthop Traumatol Turc
  • [Language] tur
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Turkey
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37. Brueckl WM, Ballhausen WG, Förtsch T, Günther K, Fiedler W, Gentner B, Croner R, Boxberger F, Kirchner T, Hahn EG, Hohenberger W, Wein A: Genetic testing for germline mutations of the APC gene in patients with apparently sporadic desmoid tumors but a family history of colorectal carcinoma. Dis Colon Rectum; 2005 Jun;48(6):1275-81
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Genetic testing for germline mutations of the APC gene in patients with apparently sporadic desmoid tumors but a family history of colorectal carcinoma.
  • PURPOSE: Desmoid tumors, also known as aggressive fibromatosis, occur with an incidence of 10 to 15 percent in patients affected by familial adenomatous polyposis, an autosomal inherited disease caused by germline mutations in the APC gene.
  • The aim of this study was to find out whether there are APC germline mutations in apparently sporadic desmoid tumor patients without clinical or familial signs of familial adenomatous polyposis but with a family history of colorectal carcinoma in at least one family member.
  • Additionally, genomic DNA from five desmoid tumors was analyzed for loss of heterozygosity at D5S346 close to the APC locus.
  • RESULTS: No translational stop mutations typical for familial adenomatous polyposis could be found in the APC gene in any of the analyzed blood samples from the desmoid tumor patients.
  • Additionally, no loss of heterozygosity at D5S346 was found in four of five desmoids; one tumor was not informative.
  • CONCLUSIONS: These results may suggest that patients with sporadic desmoids and no clinical signs of familial adenomatous polyposis detected on careful examination, esophagogastroduodenoscopy, and complete colonoscopy do not need to be tested routinely for germline mutations of the APC gene.
  • [MeSH-major] Carcinoma / genetics. Colorectal Neoplasms / genetics. Fibromatosis, Aggressive / genetics. Genes, APC. Germ-Line Mutation

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  • (PMID = 15793634.001).
  • [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
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38. Guney Y, Hiçsönmez A, Andrieu MN, Kurtman C: Outcome of aggressive fibromatosis treated with radiation therapy. Scott Med J; 2007 Nov;52(4):11-4
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  • [Title] Outcome of aggressive fibromatosis treated with radiation therapy.
  • INTRODUCTION: The purpose of this study is to report the clinical course and outcome in 7 patients with aggressive fibromatosis.
  • Patients' demographic information, including age and gender, tumour characteristics, surgical resection, and the use of radiotherapy were recorded and evaluated.
  • At follow-up, three patients had no evidence of disease, three patients were alive with disease, and one patient died 15 days after radiotherapy.
  • CONCLUSION: Local control is the primary problem in aggressive fibromatosis.
  • There is no appropriate treatment for aggressive fibromatosis and the type of treatment depends on tumour characteristics and location as well as patient characteristics.
  • [MeSH-major] Fibromatosis, Abdominal / radiotherapy. Fibromatosis, Aggressive / radiotherapy

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  • (PMID = 18092630.001).
  • [ISSN] 0036-9330
  • [Journal-full-title] Scottish medical journal
  • [ISO-abbreviation] Scott Med J
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Scotland
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39. Dalal AK, Singal R, Dalal U, Attri AK, Sahu P, Gupta A: An unusual case of chest wall desmoid tumor. Indian J Surg; 2010 Jul;72(Suppl 1):336-8
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  • [Title] An unusual case of chest wall desmoid tumor.
  • Desmoid tumor of the chest wall are uncommon fibromatous tumors characterized by their local invasion and frequent recurrences.
  • Extra-abdominal sites are mainly the shoulder girdle, the pelvic girdle and distal ends of the lower limbs.
  • The chest wall represents 8-10% of cases and the tumor is exceptionally intrathoracic.
  • We present here a rare case of a large desmoid tumor of left antero-lateral chest wall.
  • In the presented case, wide excision of the tumor and chest wall reconstruction was done.

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  • [ISSN] 0972-2068
  • [Journal-full-title] The Indian journal of surgery
  • [ISO-abbreviation] Indian J Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] India
  • [Other-IDs] NLM/ PMC3451859
  • [Keywords] NOTNLM ; Chest wall / Extra abdominal tumor / Surgery
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40. Ibrahim M, Sandogji H, Allam A: Huge intrathoracic desmoid tumor. Ann Thorac Med; 2009 Jul;4(3):146-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Huge intrathoracic desmoid tumor.
  • Desmoid tumors are soft-tissue neoplasms arising from fascial or musculo-aponeurotic structures.
  • Most reported thoracic desmoid tumors originate from the chest wall.
  • However, intrathoracic desmoid tumors are rare.
  • The patient was diagnosed to have a huge intrathoracic desmoid tumor, which was successfully resected.

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  • (PMID = 19641648.001).
  • [ISSN] 1817-1737
  • [Journal-full-title] Annals of thoracic medicine
  • [ISO-abbreviation] Ann Thorac Med
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] India
  • [Other-IDs] NLM/ PMC2714571
  • [Keywords] NOTNLM ; Chest wall / desmoid / fibromatosis / intrathoracic / tumor
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41. Shimizu T, Uehara T, Akahane T, Isobe K, Arai H: Recurrence potential of diffuse-type giant cell tumor in the foot: radiologic and pathologic features. Foot Ankle Int; 2005 Jun;26(6):474-8
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  • [Title] Recurrence potential of diffuse-type giant cell tumor in the foot: radiologic and pathologic features.
  • BACKGROUND: Aggressive musculoskeletal tumors in the foot, such as diffuse-type giant cell tumors or extra-abdominal desmoid tumors, are difficult to treat because the foot does not have enough soft tissue to allow wide tumor resection.
  • We reviewed the clinical behavior of diffuse-type giant cell tumor in the foot and evaluated the recurrence potential of these tumors from radiologic and pathologic perspectives.
  • Radiologic studies, including sonography, computed tomography (CT), magnetic resonance imaging (MRI), and bone and gallium citrate scintigraphy, were obtained followed by surgical treatment and histologic evaluation of the tumor.
  • Although CT and MRI findings were similar in the recurrent and nonrecurrent tumors, marked differences were found between the two by scintigraphy; positive radiotracer uptake to the affected foot with gallium citrate scintigraphy was noted only in recurrent tumors, although positive accumulation was seen in all patients with bone scintigraphy.
  • Histologically, the necrotic area and mitotic activity were more apparent in recurrent than in the nonrecurrent tumors, and tumor cell dyscohesion was noted in the former, (the intercellular space was increased).
  • CONCLUSIONS: Repeated recurrence with tumor invasion into tarsal bone resulted in breakage of the tarsal arch that supports the body's weight.
  • Amputation would be necessary for patients in whom the disease had progressed to obtain local cure and relief of pain.
  • In the present study, we found two features of the recurrence potential of diffuse-type giant cell tumors: sparse cell to cell contact on pathologic examination and positive accumulation in the tumor on gallium citrate scintigraphy.
  • We concluded that giant cell tumors with these two features have a strong potential for local recurrence, and thus require intensive followup.
  • [MeSH-major] Foot Bones / pathology. Foot Diseases / pathology. Giant Cell Tumor of Bone / pathology. Neoplasm Recurrence, Local

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  • (PMID = 15960914.001).
  • [ISSN] 1071-1007
  • [Journal-full-title] Foot & ankle international
  • [ISO-abbreviation] Foot Ankle Int
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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42. 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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  • [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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43. Rozen P, Macrae F: Familial adenomatous polyposis: The practical applications of clinical and molecular screening. Fam Cancer; 2006;5(3):227-35
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  • Correlations between the location of the family-specific mutation on the APC gene and clinical manifestations of the disease are of some assistance in clinical management, though there is heterogeneity in clinical course even between family members with the same mutation.
  • FAP is important to recognize, as there are disease-specific management implications with respect to offering mutational analysis of the APC (and perhaps other) genes for predictive testing of other family members, endoscopic diagnostic procedures, surveillance planning, and surgical management.
  • Extra-colonic manifestations, including duodenal polyposis, desmoid disease and other tumours, can dominate clinical care after colectomy.
  • The inheritable and lethal nature of the disease, together with the availability of effective treatment strategies, makes a sensitive clinical and psychosocial approach important to maximize compliance and good outcomes for all members of affected families.
  • [MeSH-major] Adenomatous Polyposis Coli / diagnosis

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  • (PMID = 16998668.001).
  • [ISSN] 1389-9600
  • [Journal-full-title] Familial cancer
  • [ISO-abbreviation] Fam. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Adenomatous Polyposis Coli Protein
  • [Number-of-references] 64
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44. Iwasa T, Sadamoto Y, Itaba S, Nasu T, Ihara Y, Misawa T, Nakamura K: [A case of mesenteric desmoid tumor]. Nihon Shokakibyo Gakkai Zasshi; 2007 Jun;104(6):804-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [A case of mesenteric desmoid tumor].
  • Abdominal computed tomography showed a tumor, about 5 cm in diameter at the duodeno-jejunal junction.
  • The preoperative diagnosis was submucosal or mesenteric tumor.
  • The tumor derived from the mesenterium and involved the anal side of the small intestine.
  • The tumor was removed with partial excision of the upper jejunum.
  • The diagnosis of mesenteric desmoid tumor was confirmed, histologically.
  • [MeSH-major] Fibromatosis, Abdominal / diagnosis. Ileus / etiology. Mesentery. Peritoneal Neoplasms / diagnosis

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  • (PMID = 17548947.001).
  • [ISSN] 0446-6586
  • [Journal-full-title] Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterology
  • [ISO-abbreviation] Nihon Shokakibyo Gakkai Zasshi
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
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45. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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46. Koh PK, Loi C, Cao X, Cheah PY, Ho KS, Ooi BS, Tang CL, Eu KW: Mesenteric desmoid tumors in Singapore familial adenomatous polyposis patients: clinical course and genetic profile in a predominantly Chinese population. Dis Colon Rectum; 2007 Jan;50(1):75-82
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  • [Title] Mesenteric desmoid tumors in Singapore familial adenomatous polyposis patients: clinical course and genetic profile in a predominantly Chinese population.
  • PURPOSE: This study examined the mutational profile of the adenomatous polyposis coli gene in relation to the development of desmoid tumors in familial adenomatous polyposis patients from a predominantly Chinese population.
  • Identification of specific adenomatous polyposis coli gene mutation was performed and clinical course of associated desmoid disease obtained from case records and a computerized database.
  • Of these, 23 (11.2 percent) developed desmoids.
  • Of the 92 patients with mutations 5' to codon 1444, 11 patients (12 percent) developed desmoids compared with 6 of 15 (40 percent) patients with adenomatous polyposis coli gene mutations 3' to codon 1444 (P<0.01).
  • The clinical course of desmoid tumors can be divided into stable (n=11), variable (n=3), progressive (n=6), and aggressive growth (n=3).
  • Only 3 (13 percent) patients with aggressive tumor growth required chemotherapy.
  • There was no correlation between the site of mutation and the clinical progression of the desmoids.
  • Seventy-four percent of these desmoids (17/23) developed at a mean interval of 2.98 years after restorative proctocolectomy, while only 30 percent (7/23) were diagnosed preoperatively or discovered during the initial surgery.
  • The most common complications related to the mesenteric desmoids were intestinal obstruction (21.7 percent), ureteric obstruction (17.4 percent), and encasement of superior mesenteric vessels (13 percent).
  • CONCLUSION: The clinical course of desmoids in an individual familial adenomatous polyposis patient remains unpredictable and no reliable genetic marker is available for prognostication in desmoid disease.
  • [MeSH-major] Adenomatous Polyposis Coli / genetics. Fibromatosis, Abdominal / genetics

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  • (PMID = 17082890.001).
  • [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 / Codon
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47. Wu C, Amini-Nik S, Nadesan P, Stanford WL, Alman BA: Aggressive fibromatosis (desmoid tumor) is derived from mesenchymal progenitor cells. Cancer Res; 2010 Oct 1;70(19):7690-8
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  • [Title] Aggressive fibromatosis (desmoid tumor) is derived from mesenchymal progenitor cells.
  • The cellular origins from which most tumors arise are poorly defined, especially in mesenchymal neoplasms.
  • Aggressive fibromatosis, also known as desmoid tumor, is a locally invasive soft tissue tumor that has mesenchymal characteristics.
  • We found that aggressive fibromatosis tumors express genes and cell surface markers characteristic of mesenchymal stem cells (MSC).
  • In mice that are genetically predisposed to develop aggressive fibromatosis tumors (Apc(wt/1638N)), we found that the number of tumors formed was proportional to the number of MSCs present.
  • Doubly mutant mice deficient in Sca-1 developed substantially fewer aggressive fibromatosis tumors than wild-type (WT) littermates, but Sca-1 deficiency had no effect on the formation of epithelial-derived intestinal polyps.
  • MSCs isolated from Apc(wt/1638N) mice (or mice expressing a stabilized form of β-catenin) induced aberrant cellular growth reminiscent of aggressive fibromatosis tumors after engraftment to immunocompromised mice, but WT cells and mature fibroblasts from the same animals did not.
  • Taken together, our findings indicate that aggressive fibromatosis is derived from MSCs, and that β-catenin supports tumorigenesis by maintaining mesenchymal progenitor cells in a less differentiated state.
  • Protecting this progenitor cell population might prevent tumor formation in patients harboring a germline APC mutation, where fibromatosis is currently the leading cause of mortality.
  • [MeSH-major] Fibroma / pathology. Mesenchymal Stromal Cells / pathology


48. Pourebrahim R, Van Dam K, Bauters M, De Wever I, Sciot R, Cassiman JJ, Tejpar S: ZIC1 gene expression is controlled by DNA and histone methylation in mesenchymal proliferations. FEBS Lett; 2007 Oct 30;581(26):5122-6
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  • RNA and protein analysis revealed the consistent upregulation of the neural transcription factors ZIC1 and ZIC4 in desmoid tumors and other fibroproliferative disorders.
  • The 5' flanking region of the ZIC1 promoter was unmethylated in desmoid tumor fibroblasts, while a hypermethylated ZIC1 promoter was found in human and mouse cell lines not expressing the gene.
  • [MeSH-major] DNA Methylation. Fibromatosis, Aggressive / genetics. Gene Expression Regulation. Histones / metabolism. Transcription Factors / genetics

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  • (PMID = 17936758.001).
  • [ISSN] 0014-5793
  • [Journal-full-title] FEBS letters
  • [ISO-abbreviation] FEBS Lett.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Histones; 0 / Transcription Factors; 0 / ZIC1 protein, human; 776B62CQ27 / decitabine; EC 2.1.1.- / DNA Modification Methylases; M801H13NRU / Azacitidine
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49. Altmann S, Lenz-Scharf O, Schneider W: [Therapeutic options for aggressive fibromatosis]. Handchir Mikrochir Plast Chir; 2008 Apr;40(2):88-93
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  • [Title] [Therapeutic options for aggressive fibromatosis].
  • [Transliterated title] Therapieoptionen bei der aggressiven fibromatose.
  • INTRODUCTION: Aggressive fibromatosis, e.g., desmoid tumour, is a rare neoplasm of the connective tissue with local infiltrative growth.
  • Because of the high recurrence rates and destruction of the surrounding tissue, these tumours are classified as semi-malignant.
  • However, desmoid tumors tend not to metastasise.
  • Arising from deep musculoaponeurotic structures, a monoclonal proliferation of fibroblasts occurs.
  • Radical surgical treatment with tumour excision accompanied by radiotherapy is the current standard therapy that can be supplemented by pharmacological treatment in a few cases.
  • PATIENTS AND METHOD: We report on 9 patients (5 males and 4 females) with surgical therapy for aggressive fibromatosis.
  • The neoplasm was located on the extremities in 7 cases (4 x upper extremity, 3 x lower extremity), one tumour was situated in the chin and one in the rectus abdominis muscle.
  • All patients were treated with radical tumour resection.
  • RESULTS: In 6 cases complete tumour resection (R0) was achieved.
  • In 3 cases a total tumour resection was impossible (R1 resection).
  • The aggressive fibromatosis had infiltrated the pelvis in 2 cases implying a hemipelvectomy for R0 resection which was not practicable.
  • DISCUSSION: Aggressive fibromatosis is a semimalignant neoplasm of the connective tissue with an extremely high recurrence rate.
  • Radiotherapy is indicated for patients with non-resectable tumours.
  • [MeSH-major] Fibromatosis, Aggressive / therapy. Soft Tissue Neoplasms / therapy

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  • (PMID = 18437666.001).
  • [ISSN] 0722-1819
  • [Journal-full-title] Handchirurgie, Mikrochirurgie, plastische Chirurgie : Organ der Deutschsprachigen Arbeitsgemeinschaft für Handchirurgie : Organ der Deutschsprachigen Arbeitsgemeinschaft für Mikrochirurgie der Peripheren Nerven und Gefässe : Organ der Vereinigung der Deutschen Plastischen Chirurgen
  • [ISO-abbreviation] Handchir Mikrochir Plast Chir
  • [Language] ger
  • [Publication-type] Comparative Study; English Abstract; Evaluation Studies; Journal Article
  • [Publication-country] Germany
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50. Teoh KH, Reddy S, Beggs I, Al-Nafussi A, Mander BJ, Porter DE: Malignant peripheral nerve sheath tumour in the ischio-rectal fossa. Colorectal Dis; 2009 Jun;11(5):533-4
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  • [Title] Malignant peripheral nerve sheath tumour in the ischio-rectal fossa.
  • We report a case in which the diagnosis changed between biopsy (desmoid tumour) and resection (malignant peripheral nerve sheath tumour), requiring a multidisciplinary surgical approach involving different sub-specialties.
  • [MeSH-minor] Aged. Buttocks / pathology. Diagnosis, Differential. Fibromatosis, Aggressive / pathology. Humans. Ischium. Magnetic Resonance Imaging. Male. Patient Care Team / organization & administration. Rectum

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  • (PMID = 18681893.001).
  • [ISSN] 1463-1318
  • [Journal-full-title] Colorectal disease : the official journal of the Association of Coloproctology of Great Britain and Ireland
  • [ISO-abbreviation] Colorectal Dis
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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51. Lakranbi M, Smahi M, Maidi M, Bouchikh M, Msougar Y, Ouadnouni Y, Fenan H, Achir A, Caidi M, Alaziz A, Benosman A: [Desmoid tumors of the chest wall: report of 12 cases]. Pan Afr Med J; 2009;3:13
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  • [Title] [Desmoid tumors of the chest wall: report of 12 cases].
  • [Transliterated title] Les tumeurs desmoides de la paroi thoracique : à propos de 12 cas.

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  • (PMID = 21532722.001).
  • [ISSN] 1937-8688
  • [Journal-full-title] The Pan African medical journal
  • [ISO-abbreviation] Pan Afr Med J
  • [Language] fre
  • [Publication-type] Journal Article
  • [Publication-country] Uganda
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52. 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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  • [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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53. Picariello L, Carbonell Sala S, Martineti V, Gozzini A, Aragona P, Tognarini I, Paglierani M, Nesi G, Brandi ML, Tonelli F: A comparison of methods for the analysis of low abundance proteins in desmoid tumor cells. Anal Biochem; 2006 Jul 15;354(2):205-12
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  • [Title] A comparison of methods for the analysis of low abundance proteins in desmoid tumor cells.
  • The desmoids are a group of rare clinically diverse, deep-seated fibrous neoplasms.
  • These tumors may be sporadic or associated with a genetic disease such as familial adenomatous polyposis (FAP).
  • Although surgery is widely accepted as the first-line treatment for extra-abdominal and abdominal wall desmoids, a proportion of cases are successfully palliated with either estrogen antagonists (tamoxifen, toremifene, and raloxifene) or nonsteroidal anti-inflammatory drugs.
  • We describe and compare four methods for evaluating the expression of estrogen receptors alpha/beta and COX-1 and COX-2 in desmoid tumor-derived cells and tissues: immunocytochemistry, immunohistochemistry, RT-PCR, and two-color Western blot detection with the Odyssey infrared imaging system.
  • Through this comparative analysis, Western blot with Odyssey was recognized as the best method to analyze the expression particularly of low expressed proteins in desmoid-derived cells.
  • The use of a specific and reliable assessment method becomes fundamental in the evaluation of the presence and modulation of proteins which are important but weakly expressed in these rare tumors.
  • [MeSH-major] Fibromatosis, Aggressive / metabolism. Neoplasm Proteins / analysis. Neoplasm Proteins / metabolism
  • [MeSH-minor] Base Sequence. Blotting, Western. Cyclooxygenase 1 / analysis. Cyclooxygenase 1 / genetics. Cyclooxygenase 1 / metabolism. Cyclooxygenase 2 / analysis. Cyclooxygenase 2 / genetics. Cyclooxygenase 2 / metabolism. Estrogen Receptor alpha / analysis. Estrogen Receptor alpha / genetics. Estrogen Receptor alpha / metabolism. Estrogen Receptor beta / analysis. Estrogen Receptor beta / genetics. Estrogen Receptor beta / metabolism. Gene Expression. Humans. Immunohistochemistry. Membrane Proteins / analysis. Membrane Proteins / genetics. Membrane Proteins / metabolism. RNA, Messenger / genetics. RNA, Messenger / metabolism. RNA, Neoplasm / genetics. RNA, Neoplasm / metabolism. Reverse Transcriptase Polymerase Chain Reaction. Tumor Cells, Cultured

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  • (PMID = 16729958.001).
  • [ISSN] 0003-2697
  • [Journal-full-title] Analytical biochemistry
  • [ISO-abbreviation] Anal. Biochem.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Estrogen Receptor alpha; 0 / Estrogen Receptor beta; 0 / Membrane Proteins; 0 / Neoplasm Proteins; 0 / RNA, Messenger; 0 / RNA, Neoplasm; EC 1.14.99.1 / Cyclooxygenase 1; EC 1.14.99.1 / Cyclooxygenase 2; EC 1.14.99.1 / PTGS1 protein, human; EC 1.14.99.1 / PTGS2 protein, human
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54. 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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  • [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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55. Drouet A, Le Moigne F, Have L, Blondet R, Jacquin O, Chauvin F: Common peroneal nerve palsy following TNF-based isolated limb perfusion for irresectable extremity desmoid tumor. Orthop Traumatol Surg Res; 2009 Dec;95(8):639-44
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  • [Title] Common peroneal nerve palsy following TNF-based isolated limb perfusion for irresectable extremity desmoid tumor.
  • Hyperthermic isolated limb perfusion (ILP) (2 mg, TNF-alpha and 100mg, melphalan) was performed for an irresectable right thigh desmoid tumor with calf extension in a 49-year-old man.
  • [MeSH-major] Chemotherapy, Cancer, Regional Perfusion / adverse effects. Fibromatosis, Aggressive / drug therapy. Melphalan / adverse effects. Paralysis / chemically induced. Soft Tissue Neoplasms / drug therapy. Tumor Necrosis Factor-alpha / adverse effects
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / adverse effects. Electromyography. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Neoplasm Staging. Peroneal Nerve / drug effects. Peroneal Nerve / physiopathology. Risk Assessment. Severity of Illness Index. Thigh. Treatment Outcome

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  • (PMID = 19944662.001).
  • [ISSN] 1877-0568
  • [Journal-full-title] Orthopaedics & traumatology, surgery & research : OTSR
  • [ISO-abbreviation] Orthop Traumatol Surg Res
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Tumor Necrosis Factor-alpha; Q41OR9510P / Melphalan
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56. 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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57. Singh N, Sharma R, Dorman SA, Dy VC: An unusual presentation of desmoid tumor in the ileum. Am Surg; 2006 Sep;72(9):821-4
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  • [Title] An unusual presentation of desmoid tumor in the ileum.
  • Desmoid tumors are nonencapsulated, locally invasive tumors of fibrous origin.
  • A wide variety of locations have been documented in the literature with respect to the origin of these tumors.
  • Intra-abdominal desmoids are commonly found in the mesentery.
  • We present the report of a case in which a sporadic desmoid tumor originated from the wall of the ileum.
  • Of all the options described, surgery with tumor-free margins has been defined as the best treatment modality.
  • [MeSH-major] Fibromatosis, Abdominal / diagnosis. Ileal Neoplasms / diagnosis

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  • (PMID = 16986393.001).
  • [ISSN] 0003-1348
  • [Journal-full-title] The American surgeon
  • [ISO-abbreviation] Am Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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58. Bell DM, Dekmezian RH, Husain SA, Luna MA: Oral calcifying fibrous pseudotumor: case analysis and review. Head Neck Pathol; 2008 Dec;2(4):343-7
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  • Calcifying fibrous pseudotumor (CFT) is a rare distinct soft-tissue lesion characterized histologically by lymphoplasmacytic aggregates in a rich collagenized background with abundant psammomatous and dystrophic calcifications.
  • Occurring most often in children and young adults, CFTs are clinically benign lesions that can form over a broad anatomic distribution, including in subcutaneous and deep soft tissues, as well as in serosal and visceral locations.
  • Simple excision with a margin of normal tissue is the treatment of choice.
  • In this article, we describe a case of CFT in a 29-year-old woman with a 7-cm mass on the right upper gingiva and hard palate, discuss the differential diagnosis with other oral spindle cell lesions, such as, desmoid fibromatosis, nodular fasciitis, inflammatory myofibroblastic tumors, solitary fibrous tumor and also review the recent literature on this rare benign entity.
  • [MeSH-major] Calcinosis / pathology. Granuloma, Plasma Cell / diagnosis. Mouth Diseases / diagnosis
  • [MeSH-minor] Adult. Diagnosis, Differential. Fasciitis / diagnosis. Female. Fibromatosis, Aggressive / diagnosis. Fibrosis. Gingiva / pathology. Humans. Palate, Hard / pathology. Solitary Fibrous Tumors / diagnosis. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 20614307.001).
  • [ISSN] 1936-0568
  • [Journal-full-title] Head and neck pathology
  • [ISO-abbreviation] Head Neck Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC2807574
  • [Keywords] NOTNLM ; Calcifying / Fibrous / Oral / Pseudotumor / Tumor
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59. Bhosale PR, Patnana M, Viswanathan C, Szklaruk J: The inguinal canal: anatomy and imaging features of common and uncommon masses. Radiographics; 2008 May-Jun;28(3):819-35; quiz 913
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  • A variety of benign and malignant masses can be found in the inguinal canal (IC).
  • Benign causes of masses in the IC include spermatic cord lipoma, hematoma, abscess, neurofibroma, varicocele, desmoid tumor, air, bowel contrast material, hydrocele, and prostheses.
  • Metastases to the IC can occur from alveolar rhabdomyosarcoma, monophasic sarcoma, prostate cancer, Wilms tumor, carcinoid tumor, melanoma, or pancreatic cancer.
  • In patients with a known malignancy and peritoneal carcinomatosis, the diagnosis of metastases can be suggested when a mass is detected in the IC.
  • When peritoneal disease is not evident, a mass in the IC is indicative of stage IV disease and may significantly alter clinical and surgical treatment of the patient.
  • [MeSH-major] Abdominal Neoplasms / diagnosis. Inguinal Canal / pathology. Inguinal Canal / radiography. Peritoneal Diseases / diagnosis
  • [MeSH-minor] Adult. Aged. Female. Humans. Male. Middle Aged. Rare Diseases / diagnosis

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  • [Copyright] Copyright RSNA, 2008.
  • (PMID = 18480486.001).
  • [ISSN] 1527-1323
  • [Journal-full-title] Radiographics : a review publication of the Radiological Society of North America, Inc
  • [ISO-abbreviation] Radiographics
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 88
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60. 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
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  • [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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61. Cohen S, Ad-El D, Benjaminov O, Gutman H: Post-traumatic soft tissue tumors: case report and review of the literature a propos a post-traumatic paraspinal desmoid tumor. World J Surg Oncol; 2008;6:28
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  • [Title] Post-traumatic soft tissue tumors: case report and review of the literature a propos a post-traumatic paraspinal desmoid tumor.
  • BACKGROUND: Antecedent trauma has been implicated in the causation of soft tissue tumors.
  • We postulate possible mechanisms in the genesis of soft tissue tumors following antecedent traumatic injury.
  • CASE PRESENTATION: We present a 27-year-old woman with a paraspinal desmoid tumor, diagnosed 3-years following a motor vehicle accident.
  • CONCLUSION: Soft tissue tumors arising at the site of previous trauma may be desmoids, pseudolipomas or rarely, other soft tissue growths.
  • The cause-and-effect issue of desmoid or other soft tissue tumors goes beyond their diagnosis and treatment.
  • [MeSH-major] Fibromatosis, Aggressive / etiology. Neoplasms, Post-Traumatic / etiology. Soft Tissue Injuries / complications. Soft Tissue Neoplasms / etiology. Spinal Neoplasms / etiology

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  • (PMID = 18312655.001).
  • [ISSN] 1477-7819
  • [Journal-full-title] World journal of surgical oncology
  • [ISO-abbreviation] World J Surg Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2270823
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62. Goto T, Nemoto T, Ogura K, Hozumi T, Funata N: Successful treatment of desmoid tumor of the chest wall with tranilast: a case report. J Med Case Rep; 2010;4:384
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  • [Title] Successful treatment of desmoid tumor of the chest wall with tranilast: a case report.
  • INTRODUCTION: Desmoid tumor is characterized by infiltrative growth and local recurrence often occurs after surgery.
  • In addition, these non-surgical treatments are also performed in patients with unresectable desmoid tumors.
  • We successfully treated a patient with a desmoid tumor with tranilast; an anti-allergic agent.
  • CASE PRESENTATION: A 48-year-old Japanese man with a slow-growing desmoid tumor on his chest wall was treated with an oral administration of tranilast (300 mg per day, three times a day).
  • Two years and two months after the commencement of his therapy, the tumor became impalpable.
  • Two years after discontinuation of the treatment, a physical examination showed no recurrence of the tumor and he continued in a state of remission.
  • We were successfully able to reduce the size of the tumor and thereafter maintain the reduced size.
  • Because tranilast has substantially fewer adverse effects than cytotoxic agents, it could be a very useful therapeutic agent for desmoid tumor.

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  • (PMID = 21114809.001).
  • [ISSN] 1752-1947
  • [Journal-full-title] Journal of medical case reports
  • [ISO-abbreviation] J Med Case Rep
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2999613
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63. Solazzo M, Marcianò P, Benzi F, Puccio F: Ileal resection and small bowel transplantation for large mesenteric desmoid tumor. Tech Coloproctol; 2005 Apr;9(1):72-3
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  • [Title] Ileal resection and small bowel transplantation for large mesenteric desmoid tumor.
  • [MeSH-major] Fibromatosis, Aggressive / surgery. Ileum / surgery. Intestine, Small / transplantation. Mesentery. Peritoneal Neoplasms / surgery

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  • (PMID = 15884154.001).
  • [ISSN] 1123-6337
  • [Journal-full-title] Techniques in coloproctology
  • [ISO-abbreviation] Tech Coloproctol
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] Italy
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64. Rajesh A, Sandrasegaran K: Mesenteric desmoid mimicking recurrent testicular cancer. Abdom Imaging; 2005 Nov-Dec;30(6):777-9
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  • [Title] Mesenteric desmoid mimicking recurrent testicular cancer.
  • The detection of a solid abdominal mass after surgery for testicular cancer most frequently represents metastatic disease.
  • We report an unusual case of mesenteric desmoid tumor presenting after retroperitoneal lymph node dissection for metastatic testicular cancer.
  • [MeSH-major] Fibromatosis, Aggressive / radiography. Mesentery. Neoplasm Recurrence, Local / radiography. Peritoneal Neoplasms / radiography. Testicular Neoplasms / radiography

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  • (PMID = 16096867.001).
  • [ISSN] 0942-8925
  • [Journal-full-title] Abdominal imaging
  • [ISO-abbreviation] Abdom Imaging
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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65. Yu YH, Son BK, Jun DW, Kim SH, Jo YJ, Park YS, Hong BW, Joo JE: [A case of desmoid tumor presenting as intra-abdominal abscess]. Korean J Gastroenterol; 2009 May;53(5):315-9
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  • [Title] [A case of desmoid tumor presenting as intra-abdominal abscess].
  • Desmoid tumor is a rare benign tumor derived from fibrous sheath or musculoaponeurotic structure.
  • The tumor is benign histologically but considered as malignant clinically because it has high propensity on infiltrative growth with local invasion and tendency to recurrence after local excision.
  • Especially, when this tumor happens to be in the intra-abdomen, the prognosis is worse because it can cause intestinal obstruction, ureter obstruction and, fistula formation.
  • This tumor occurs more frequently in patients with familial adenomatous polyposis (FAP), in post-partume women, and at old surgical incision site.
  • We report a rare case of the young male patient who presented with an acute abdomen and underwent laparotomy and was found to have an intra-abdominal desmoid tumor with abscess formation.
  • [MeSH-major] Fibromatosis, Abdominal / diagnosis. Peritoneal Neoplasms / diagnosis
  • [MeSH-minor] Abdominal Abscess / diagnosis. Adult. Diagnosis, Differential. Humans. Male. Tomography, X-Ray Computed

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  • (PMID = 19458469.001).
  • [ISSN] 1598-9992
  • [Journal-full-title] The Korean journal of gastroenterology = Taehan Sohwagi Hakhoe chi
  • [ISO-abbreviation] Korean J Gastroenterol
  • [Language] kor
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Korea (South)
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66. Michopoulou A, Germanos S, Kanakopoulos D, Milonas A, Orfanos N, Spyratou C, Markidis P: Management of a large abdominal wall desmoid tumor during pregnancy. Case report. Ann Ital Chir; 2010 Mar-Apr;81(2):153-6
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  • [Title] Management of a large abdominal wall desmoid tumor during pregnancy. Case report.
  • Desmoid tumors, characterized by aggressive local infiltration of surrounding tissues, are uncommon benign neoplasms with no metastatic potential, that occasionally may attain large size.
  • We report a case of a 37-year-old woman with an abdominal wall desmoid tumor that appeared and grew rapidly during her pregnancy, diagnosed by trucut core biopsy.
  • Complete surgical excision of a 20 x 16 cm in size tumor and immediate reconstruction with mesh was performed in the postpartum period.
  • Optimal management of large abdominal wall desmoids during pregnancy has to be individualized, with wide surgical excision remaining the treatment of choice.
  • [MeSH-major] Abdominal Wall. Fibromatosis, Aggressive / surgery. Pregnancy Complications, Neoplastic / surgery


67. Bahrami A, Folpe AL: Adult-type fibrosarcoma: A reevaluation of 163 putative cases diagnosed at a single institution over a 48-year period. Am J Surg Pathol; 2010 Oct;34(10):1504-13
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  • [Title] Adult-type fibrosarcoma: A reevaluation of 163 putative cases diagnosed at a single institution over a 48-year period.
  • Adult-type fibrosarcoma (FS) was once considered the most common adult sarcoma, but is now considered a diagnosis of exclusion.
  • One hundred ninety-five cases diagnosed as adult FS in somatic soft tissue were retrieved from our institutional archives for the period 1960 to 2008.
  • Revised diagnoses were based on clinical, morphologic, IHC, and molecular findings.
  • Twelve patients (50%) died of locally aggressive and/or metastatic disease (median follow-up 1-year; range <1 to 8 y), 6 patients (25%) were alive without disease (median follow-up 11.5 y; range 2.5 to 35 y), and 6 patients (25%) died of other causes (median follow-up 10 y; range 9 to 18 y) ().
  • Non-FS (137 cases) were reclassified as: undifferentiated pleomorphic sarcoma (32 cases), SS (21 cases), solitary fibrous tumor (14 cases), myxofibrosarcoma (11 cases), malignant peripheral nerve sheath tumor (8 cases), FS dermatofibrosarcoma protuberans, and desmoplastic melanoma (4 cases each), low-grade fibromyxoid sarcoma, sarcomatoid carcinoma, desmoid-type fibromatosis, rhabdomyosarcoma, myofibroblastic sarcoma, spindle-cell liposarcoma (3 cases each), sclerosing epithelioid FS, fibroma-like epithelioid sarcoma, leiomyosarcoma, cellular fibrous histiocytoma (2 cases each), and others (17 cases).
  • Exclusive of undifferentiated pleomorphic sarcoma, the distinction of which from FS is subjective, 64% of putative FS were reclassified, most commonly as monophasic SS and solitary fibrous tumor.
  • We conclude that true FS is exceedingly rare, accounting for <1% of approximately 10,000 adult soft tissue sarcomas seen at our institution during this time period, and should be diagnosed with great caution.
  • [MeSH-major] Fibrosarcoma / diagnosis. Soft Tissue Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Adult. Age Factors. Aged. Aged, 80 and over. Biomarkers, Tumor / metabolism. Child. Child, Preschool. DNA, Neoplasm / analysis. Female. Gene Rearrangement. Humans. In Situ Hybridization, Fluorescence. Male. Middle Aged. Minnesota / epidemiology. Proto-Oncogene Proteins / genetics. Proto-Oncogene Proteins / metabolism. Repressor Proteins / genetics. Repressor Proteins / metabolism. Young Adult


68. 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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69. Gould CF, Ly JQ, Lattin GE Jr, Beall DP, Sutcliffe JB 3rd: Bone tumor mimics: avoiding misdiagnosis. Curr Probl Diagn Radiol; 2007 May-Jun;36(3):124-41
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  • [Title] Bone tumor mimics: avoiding misdiagnosis.
  • Whether discovered incidentally or as part of a focused diagnostic evaluation, the finding of a benign osseous lesion that has radiologic features resembling a bone tumor is not uncommon.
  • Some of the more common benign and nonneoplastic entities that can sometimes be confused with tumors are the following: cortical desmoid, Brodie abscess, synovial herniation pit, pseudocyst, enostosis, intraosseous ganglion cyst, fibrous dysplasia, stress fracture, avulsion fracture (healing stage), bone infarct, myositis ossificans, brown tumor, and subchondral cyst.
  • Accurate diagnosis and management of these lesions require a basic understanding of their epidemiology, clinical presentations, anatomic distributions, imaging features, differential considerations, and therapeutic options.
  • This in-depth review of 13 potential bone tumor mimics will assist the radiologist in correctly identifying these benign lesions and in avoiding misdiagnosis and related morbidity.
  • [MeSH-major] Bone Diseases / diagnosis. Bone Neoplasms / diagnosis. Magnetic Resonance Imaging
  • [MeSH-minor] Ankle Joint / radiography. Bone Cysts / diagnosis. Bone Cysts / pathology. Bone and Bones / blood supply. Diagnosis, Differential. Femur Neck / pathology. Fibromatosis, Aggressive / diagnosis. Fibrous Dysplasia of Bone / diagnosis. Fractures, Stress / diagnosis. Ganglion Cysts / diagnosis. Humans. Infarction / diagnosis. Myositis Ossificans / diagnosis. Tomography, X-Ray Computed

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  • (PMID = 17484955.001).
  • [ISSN] 0363-0188
  • [Journal-full-title] Current problems in diagnostic radiology
  • [ISO-abbreviation] Curr Probl Diagn Radiol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 48
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70. Kertész E, Túri J, Gyenes V, Vajda A: [Gardner-syndrome: case report]. Fogorv Sz; 2005 Oct;98(5):213-5
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  • The major symptoms of Gardner-syndrome are described by the authors (multiple osteomas, desmoid tumors, colon polyps with malignant tendency) with a case appearing in their department.
  • The importance of early diagnosis is also emphasized.
  • [MeSH-major] Gardner Syndrome / diagnosis. Gardner Syndrome / surgery
  • [MeSH-minor] Adenomatous Polyposis Coli / diagnosis. Adenomatous Polyposis Coli / surgery. Adult. Colon, Sigmoid / pathology. Early Diagnosis. Female. Fibromatosis, Aggressive / diagnosis. Fibromatosis, Aggressive / surgery. Humans. Mandibular Neoplasms / diagnosis. Mandibular Neoplasms / surgery. Osteoma / diagnosis. Osteoma / surgery. Rectum / pathology

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  • (PMID = 16315858.001).
  • [ISSN] 0015-5314
  • [Journal-full-title] Fogorvosi szemle
  • [ISO-abbreviation] Fogorv Sz
  • [Language] hun
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Hungary
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71. Mazeh H, Nissan A, Simanovsky N, Hiller N: Desmoid tumor causing duodenal obstruction. Isr Med Assoc J; 2006 Apr;8(4):288-9
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  • [Title] Desmoid tumor causing duodenal obstruction.
  • [MeSH-major] Duodenal Diseases / etiology. Duodenal Neoplasms / complications. Fibromatosis, Aggressive / complications. Intestinal Obstruction / etiology


72. Chang YN, Chuang YC, Lin YS: Fibromatosis of the submandibular region. J Chin Med Assoc; 2009 Sep;72(9):492-4
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  • [Title] Fibromatosis of the submandibular region.
  • Fibromatoses are benign tumors that can occur anywhere in the body.
  • They are locally aggressive and tend to recur; they also cause considerable morbidity, particularly when they arise in the head and neck.
  • Fibromatosis in the submandibular region is rare.
  • Here, we present a case of fibromatosis of the submandibular region in a 42-year-old female who presented with a right submandibular mass.
  • The patient underwent complete excision of the fibromatosis, showed negative margins and had no adjuvant therapy.
  • [MeSH-major] Fibroma / pathology. Submandibular Gland Neoplasms / pathology

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  • (PMID = 19762319.001).
  • [ISSN] 1728-7731
  • [Journal-full-title] Journal of the Chinese Medical Association : JCMA
  • [ISO-abbreviation] J Chin Med Assoc
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] China (Republic : 1949- )
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73. Solanki NS, Macfarlane PL, Marshall NJ: Images for surgeons. An extra-abdominal desmoid tumour in a young woman. ANZ J Surg; 2010 Oct;80(10):743-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Images for surgeons. An extra-abdominal desmoid tumour in a young woman.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Shoulder. Soft Tissue Neoplasms / pathology

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  • (PMID = 21061751.001).
  • [ISSN] 1445-2197
  • [Journal-full-title] ANZ journal of surgery
  • [ISO-abbreviation] ANZ J Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Australia
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74. Shido Y, Nishida Y, Nakashima H, Katagiri H, Sugiura H, Yamada Y, Ishiguro N: Surgical treatment for local control of extremity and trunk desmoid tumors. Arch Orthop Trauma Surg; 2009 Jul;129(7):929-33
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  • [Title] Surgical treatment for local control of extremity and trunk desmoid tumors.
  • INTRODUCTION: Surgical treatment has been the mainstay for extremity and trunk desmoid tumors with a negative surgical margin.
  • The purpose of this study was to analyze the clinical outcome of patients with extremity and trunk desmoid tumors after surgical treatment and to determine the factors influencing local recurrence.
  • MATERIALS AND METHODS: Between January 1991 and December 2003, 30 of 58 patients with desmoid tumors referred to our institutions and surgically treated and followed up for more than 2 years were selected for this study.
  • Patient age, gender, tumor size, location, status of disease (primary or recurrent), and surgical margins were analyzed as possible risk factors for recurrence.
  • Tumor size, surgical margin and previous surgical history were not associated with local recurrence.
  • Younger age and female gender had a propensity for local recurrence, suggesting that the biological behavior of desmoid tumor may depend on the status of the disease at presentation.
  • [MeSH-major] Fibromatosis, Aggressive / epidemiology. Fibromatosis, Aggressive / surgery. Neoplasm Recurrence, Local / epidemiology

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  • (PMID = 18810472.001).
  • [ISSN] 1434-3916
  • [Journal-full-title] Archives of orthopaedic and trauma surgery
  • [ISO-abbreviation] Arch Orthop Trauma Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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75. Bertani E, Chiappa A, Testori A, Mazzarol G, Biffi R, Martella S, Pace U, Soteldo J, Vigna PD, Lembo R, Andreoni B: Desmoid tumors of the anterior abdominal wall: results from a monocentric surgical experience and review of the literature. Ann Surg Oncol; 2009 Jun;16(6):1642-9
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  • [Title] Desmoid tumors of the anterior abdominal wall: results from a monocentric surgical experience and review of the literature.
  • BACKGROUND: Desmoid tumor, also known as aggressive fibromatosis, is a rare soft tissue tumor.
  • For those cases localized in the anterior abdominal wall, radical resection and reconstruction with a mesh is indicated.
  • Because the rarity of the disease, randomized trials are lacking, but in reported retrospective series, it is clear that although it is considered a benign lesion, local recurrence is not uncommon.
  • METHODS: We analyzed the records of 14 consecutive patients (3 men, 11 women, mean age 36 years, range 25-51 years) with desmoid tumor of the anterior abdominal wall treated at the European Institute of Oncology.
  • The surgical strategy was the same in all cases: wide surgical excision and immediate plastic reconstruction with mesh after intraoperative confirmation by frozen sections of disease-free margins of >1 cm.
  • CONCLUSIONS: Radical resection aided by intraoperative margin evaluation via frozen sections followed by immediate mesh reconstruction is a safe procedure and can provide definitive cure without functional limitations for patients with desmoid tumors of the anterior abdominal wall.
  • [MeSH-major] Fibromatosis, Aggressive / surgery. Soft Tissue Neoplasms / surgery
  • [MeSH-minor] Abdominal Wall. Adult. Female. Humans. Male. Middle Aged. Surgical Mesh

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  • (PMID = 19296178.001).
  • [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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76. 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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  • [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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77. Pojchamarnwiputh S, Muttarak M, Na-Chiangmai W, Chaiwun B: Benign breast lesions mimicking carcinoma at mammography. Singapore Med J; 2007 Oct;48(10):958-68
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  • [Title] Benign breast lesions mimicking carcinoma at mammography.
  • Many benign breast lesions pose diagnostic challenges.
  • These lesions include abscess, haematoma, radial scar, post surgical scar, diabetic mastopathy, focal fibrosis, sclerosing adenosis, granular cell tumour, extra-abdominal desmoid tumour, medial insertion of pectoralis muscle and sternalis muscle, and axillary lymphadenopathy (due to HIV infection, collagen vascular lesions, tuberculous and bacterial lymphadenitis).
  • Radiologists should be familiar with the characteristic imaging features of these benign lesions, and should include these benign lesions in the differential diagnosis whenever malignant-appearing findings are encountered.
  • Correlation of the patient's clinical features with the mammographical findings and additional use of ultrasonography, fine-needle aspiration biopsy or core biopsy are helpful in establishing the final diagnosis and obviating unnecessary surgical intervention.
  • [MeSH-minor] Adult. Biopsy, Needle. Breast Neoplasms / pathology. Breast Neoplasms / radiography. Breast Neoplasms / ultrasonography. Carcinoma / pathology. Carcinoma / radiography. Carcinoma / ultrasonography. Diagnosis, Differential. Female. Histocytochemistry. Humans. Middle Aged

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  • (PMID = 17909685.001).
  • [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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78. Mendenhall WM, Zlotecki RA, Morris CG, Hochwald SN, Scarborough MT: Aggressive fibromatosis. Am J Clin Oncol; 2005 Apr;28(2):211-5
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  • [Title] Aggressive fibromatosis.
  • The purpose of this article is to review the pertinent literature and to define the optimal treatment of patients with aggressive fibromatosis.
  • Moderate-dose radiotherapy alone for gross disease or after a microscopically incomplete resection yields local control rates of approximately 75% to 80%.
  • Thus, the optimal management for aggressive fibromatosis depends on tumor location and extent.
  • Radiotherapy is indicated for patients with unresectable tumors; those with positive margins after resection should be considered for adjuvant radiotherapy depending on the location and extent of the tumor.
  • Pharmacologic treatment should be considered for patients with progressive disease after unsuccessful local-regional therapy.
  • [MeSH-major] Fibromatosis, Aggressive / therapy

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  • (PMID = 15803019.001).
  • [ISSN] 1537-453X
  • [Journal-full-title] American journal of clinical oncology
  • [ISO-abbreviation] Am. J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 31
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79. Rakha EA, Kandil MA, El-Santawe MG: Gigantic recurrent abdominal desmoid tumour: a case report. Hernia; 2007 Apr;11(2):193-7
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  • [Title] Gigantic recurrent abdominal desmoid tumour: a case report.
  • Deeply seated fibromatosis or desmoid tumour (DT) is a rare entity characterized by benign proliferation of fibroblasts.
  • Although non-malignant, this tumour can be life-threatening due to its invasive property and high recurrence rate.
  • We report a unique case of a huge recurrent abdominal DT (36 cm in diameter, 25 kg in weight) that caused pressure necrosis and sloughing of the overlying anterior abdominal wall, and produced a large fungating mass protruding outside the abdomen.
  • Although preoperative neoadjuvant therapies were all ineffective, radical surgical removal of the tumour was successful.
  • The procedure was followed by an excellent clinical recovery and the patient is still alive with no evidence of recurrent disease after a 6-year follow-up.
  • [MeSH-major] Fibromatosis, Abdominal / pathology. Neoplasm Recurrence, Local / pathology

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  • (PMID = 17149531.001).
  • [ISSN] 1265-4906
  • [Journal-full-title] Hernia : the journal of hernias and abdominal wall surgery
  • [ISO-abbreviation] Hernia
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] France
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80. 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
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  • [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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81. Egberts JH, Scharrer ML, Hinz S, Schafmayer C, Klomp HJ, Faendrich F, Tepel J: Small bowel cancer: single-centre results over a period of 12 years. Hepatogastroenterology; 2007 Jan-Feb;54(73):129-34
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  • BACKGROUND/AIMS: Tumors of the small bowel are rare, accounting for about 3-6% of all gastrointestinal neoplasms.
  • However, diagnosis and treatment are difficult and an ongoing challenge.
  • RESULTS: Subgroups consisted of adenocarcinoma (n=16; 37.2%), neuroendocrine tumors (n=12; 27.9%), gastrointestinal stroma tumor (GIST) (n=10; 23.3%), lymphoma (n=3; 7%) and desmoid tumor (n=2; 4.6%).
  • Tumor localizations were within duodenum (46.5%), jejunum (16.3%) and ileum (37.2%).
  • Adenocarcinoma patients showed preponderance of advanced tumor stages: stage I/II in 5 pts, III/IV in 11 patients.
  • Stage distribution for patients with neuroendocrine tumors was 3 each for I and II and 6 for III.
  • Patients with neuroendocrine tumors showed best survival results (75/57%), GIST patients 60/35% and adenocarcinoma (27% each).
  • There was a strong trend towards better survival at early tumor stages in patients with adenocarcinoma and neuroendocrine tumors.
  • CONCLUSIONS: Early diagnosis is essential for prognosis of small bowel malignancies.
  • [MeSH-minor] Adenocarcinoma / diagnosis. Adenocarcinoma / epidemiology. Adenocarcinoma / pathology. Adult. Aged. Aged, 80 and over. Carcinoid Tumor / mortality. Carcinoma, Neuroendocrine / diagnosis. Carcinoma, Neuroendocrine / epidemiology. Duodenal Neoplasms / diagnosis. Duodenal Neoplasms / epidemiology. Female. Gastrointestinal Stromal Tumors / epidemiology. Germany / epidemiology. Humans. Ileal Neoplasms / diagnosis. Ileal Neoplasms / epidemiology. Jejunal Neoplasms / epidemiology. Male. Middle Aged. Neoplasm Staging

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  • (PMID = 17419246.001).
  • [ISSN] 0172-6390
  • [Journal-full-title] Hepato-gastroenterology
  • [ISO-abbreviation] Hepatogastroenterology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Greece
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82. 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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  • [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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83. Beck AH, Espinosa I, Gilks CB, van de Rijn M, West RB: The fibromatosis signature defines a robust stromal response in breast carcinoma. Lab Invest; 2008 Jun;88(6):591-601
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  • [Title] The fibromatosis signature defines a robust stromal response in breast carcinoma.
  • Breast cancer is a heterogeneous disease, and the influence of stromal gene and protein expression patterns on the biological and clinical heterogeneity of the disease is poorly understood.
  • We previously demonstrated that evaluation of the gene expression patterns of two soft-tissue tumors (desmoid-type fibromatosis (DTF) and solitary fibrous tumor) could be used to identify distinct stromal reaction patterns in breast carcinoma.
  • Using multiple tissue microarrays of archival breast cancer specimens obtained from a total of 745 patients, we demonstrated that a subset of breast cancers show coordinate expression of DTF core proteins by stromal cells in the tumor microenvironment.
  • We evaluated the protein expression of a single DTF core protein (SPARC) on a tissue microarray with clinical outcome data and demonstrated that breast cancers with strong stromal protein expression of SPARC show a trend for increased survival.
  • [MeSH-major] Breast Neoplasms / genetics. Breast Neoplasms / pathology. Carcinoma / genetics. Fibroma / genetics. Fibroma / pathology
  • [MeSH-minor] Data Interpretation, Statistical. Disease-Free Survival. Female. Gene Expression Profiling. Gene Expression Regulation, Neoplastic. Humans. Immunohistochemistry. Middle Aged. Oligonucleotide Array Sequence Analysis. Receptors, Estrogen / metabolism. Reproducibility of Results. Stromal Cells / metabolism. Stromal Cells / pathology. Versicans / analysis

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  • (PMID = 18414401.001).
  • [ISSN] 1530-0307
  • [Journal-full-title] Laboratory investigation; a journal of technical methods and pathology
  • [ISO-abbreviation] Lab. Invest.
  • [Language] eng
  • [Grant] United States / NLM NIH HHS / LM / T15 LM007033
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Receptors, Estrogen; 0 / VCAN protein, human; 126968-45-4 / Versicans
  • [Other-IDs] NLM/ NIHMS775562; NLM/ PMC4847137
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84. Palacios E: Aggressive fibromatosis of the neck. Ear Nose Throat J; 2005 Feb;84(2):76-7
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  • [Title] Aggressive fibromatosis of the neck.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Head and Neck Neoplasms / diagnosis
  • [MeSH-minor] Adult. Diagnosis, Differential. Female. Humans


85. Saito M, Sakurai S, Motegi A, Saito K, Sano T, Nakajima T: Comparative study using rabbit-derived polyclonal, mouse-derived monoclonal, and rabbit-derived monoclonal antibodies for KIT immunostaining in GIST and other tumors. Pathol Int; 2007 Apr;57(4):200-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Comparative study using rabbit-derived polyclonal, mouse-derived monoclonal, and rabbit-derived monoclonal antibodies for KIT immunostaining in GIST and other tumors.
  • Twenty-three gastrointestinal stromal tumors (GIST) were stained, including five KIT-weak or -negative GIST with PDGFRA gene mutations from a previous report, six Ewing/malignant primitive peripheral neuroectodermal tumor, six malignant melanomas, two neuroblastomas, six seminomas, seven thymic carcinoma and seven small cell carcinomas of the lung as KIT-expressing tumors, and four leiomyomas, six leiomyosarcomas, five gastric schwannomas, four solitary fibrous tumors, one inflammatory fibroid polyp and six desmoid tumors as KIT-non-expressing tumors.
  • The positive rates of RabMA Y145 in KIT-expressing tumors were almost equal to pAb A4502, whereas those of three MMA had lower rates.
  • None of the KIT-non-expressing tumors was positive for Y145, whereas some leiomyosarcomas and desmoid tumors were positive for A4502.
  • At present, pAb A4502 or RabMA Y145 seems to be suitable for KIT immunostaining in formalin-fixed paraffin-embedded tumor specimens, especially in the differential diagnosis of GIST from other mesenchymal tumors.
  • [MeSH-major] Antibodies, Monoclonal. Gastrointestinal Stromal Tumors / diagnosis. Gastrointestinal Stromal Tumors / metabolism. Proto-Oncogene Proteins c-kit / metabolism
  • [MeSH-minor] Animals. Diagnosis, Differential. Humans. Melanoma / metabolism. Mice. Neuroblastoma / metabolism. Neuroectodermal Tumors / metabolism. Rabbits. Seminoma / metabolism. Sensitivity and Specificity

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  • (PMID = 17316415.001).
  • [ISSN] 1320-5463
  • [Journal-full-title] Pathology international
  • [ISO-abbreviation] Pathol. Int.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit
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86. 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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87. Chang TC, Wu SL, Hsiao YL: Medullary thyroid carcinoma: pitfalls in diagnosis by fine needle aspiration cytology and relationship of cytomorphology to RET proto-oncogene mutations. Acta Cytol; 2005 Sep-Oct;49(5):477-82
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  • [Title] Medullary thyroid carcinoma: pitfalls in diagnosis by fine needle aspiration cytology and relationship of cytomorphology to RET proto-oncogene mutations.
  • OBJECTIVE: To elucidate the pitfalls in the diagnosis of medullary thyroid carcinoma (MTC) by fine needle aspiration cytology (FNAC) and the relationship of cytomorphology to RET proto-oncogene mutations.
  • RESULTS: Twenty-eight (82.4%) of 34 cases were diagnosed correctly as MTC by FNAC, 3 cases were misdiagnosed as follicular neoplasm and 1 as desmoid, and 2 cases were suspicious for MTC.
  • [MeSH-minor] Adolescent. Adult. Aged. Biopsy, Fine-Needle / standards. Cell Nucleus / pathology. Cell Shape. Codon / genetics. DNA / analysis. DNA / genetics. DNA Mutational Analysis. Diagnosis, Differential. Epithelial Cells / pathology. Female. Germ-Line Mutation / genetics. Humans. Male. Middle Aged. Polymerase Chain Reaction. Predictive Value of Tests. Reproducibility of Results. Thyroid Gland / pathology

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  • (PMID = 16334022.001).
  • [ISSN] 0001-5547
  • [Journal-full-title] Acta cytologica
  • [ISO-abbreviation] Acta Cytol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Codon; 9007-49-2 / DNA; EC 2.7.10.1 / Proto-Oncogene Proteins c-ret
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88. Giuliani A, Demoro M, Ciardi A, Scimó M, Galati F, Lonardo MT, Galati G: Mesenteric fibromatosis. Case report. J Exp Clin Cancer Res; 2007 Sep;26(3):425-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Mesenteric fibromatosis. Case report.
  • Deep fibromatosis is a rare locally aggressive but not metastasizing proliferation.
  • Intra-abdominal fibromatosis (IAF) occurs either in association with Gardner's syndrome or as a sporadic event and presents in most cases differential diagnostic problems with myofibroblastic or fibroblastic tumors, characterized by a more aggressive biological behaviour such as gastrointestinal stromal tumors (GISTs).
  • In absence of loco-regional and/or distant metastasis differential diagnosis may be difficult and represents a topical issue, since it influences treatment choice.
  • We describe the case of a patient with sporadic IAF in which the tumor locally involved the mesentery and presented no loco-regional and distant spread.
  • On histology, some morphological features of the neoplasm were in common with a GIST.
  • Definitive diagnosis was made, postoperatively, on the basis of immunohistochemical findings.
  • [MeSH-major] Abdominal Neoplasms / diagnosis. Fibroma / diagnosis. Mesentery
  • [MeSH-minor] Diagnosis, Differential. Gardner Syndrome / pathology. Humans. Male. Middle Aged

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  • (PMID = 17987807.001).
  • [ISSN] 0392-9078
  • [Journal-full-title] Journal of experimental & clinical cancer research : CR
  • [ISO-abbreviation] J. Exp. Clin. Cancer Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
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89. Andino L, Cagle PT, Murer B, Lu L, Popper HH, Galateau-Salle F, Sienko AE, Barrios R, Zander DS: Pleuropulmonary desmoid tumors: immunohistochemical comparison with solitary fibrous tumors and assessment of beta-catenin and cyclin D1 expression. Arch Pathol Lab Med; 2006 Oct;130(10):1503-9
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  • [Title] Pleuropulmonary desmoid tumors: immunohistochemical comparison with solitary fibrous tumors and assessment of beta-catenin and cyclin D1 expression.
  • CONTEXT: Desmoid tumors arising in the lung and pleura are extremely rare and can resemble other, more common neoplasms native to these sites.
  • Alterations of the adenomatous polyposis coli/beta-catenin pathway have been detected in sporadic desmoid tumors and have been associated with nuclear accumulation of beta-catenin and overexpression of cyclin D1.
  • OBJECTIVE: To analyze the expression of beta-catenin and cyclin D1 in desmoid tumors and solitary fibrous tumors (SFTs), and to compare the utilities of these substances for distinguishing between these entities with those of other, more commonly used stains.
  • DESIGN: Formalin-fixed, paraffin-embedded sections of 4 desmoid tumors (1 pulmonary, 1 pleural, 2 pleural/chest wall), and 5 benign and 6 malignant SFTs of the pleura were immunostained for beta-catenin, cyclin D1, ALK1, CD34, vimentin, desmin, smooth muscle actin, muscle-specific actin, S100, and pancytokeratin.
  • Staining intensity and the percentage of stained tumor cells were assessed semiquantitatively.
  • RESULTS: Diffuse moderate or strong nuclear staining for beta-catenin was found in all desmoid tumors, 4 of 5 benign SFTs, and 2 of 6 malignant SFTs.
  • All cases except 1 benign SFT showed concurrent cytoplasmic staining.
  • The best distinction between desmoid tumors and SFTs was provided by CD34 (desmoid tumors, 0/4; SFTs, 8/11) and smooth muscle actin (desmoid tumors, 4/4; SFTs, 0/11).
  • CONCLUSIONS: Our findings suggest that alterations in the adenomatous polyposis coli/beta-catenin pathway and cyclin D1 dysregulation may contribute to the pathogenesis of pleuropulmonary desmoid tumors and SFTs.
  • CD34 and smooth muscle actin stains are particularly useful for differentiating between pleuropulmonary desmoid tumors and SFTs.
  • [MeSH-minor] Actins / metabolism. Adult. Aged. Antigens, CD34 / metabolism. Cell Nucleus / metabolism. Child, Preschool. Cytoplasm / metabolism. Diagnosis, Differential. Female. Fibromatosis, Aggressive. Humans. Immunohistochemistry. Male. Middle Aged. Muscle, Smooth / metabolism. Staining and Labeling

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  • (PMID = 17090192.001).
  • [ISSN] 1543-2165
  • [Journal-full-title] Archives of pathology & laboratory medicine
  • [ISO-abbreviation] Arch. Pathol. Lab. Med.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Actins; 0 / Antigens, CD34; 0 / beta Catenin; 136601-57-5 / Cyclin D1
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90. Schlager A, Altchek A, Kalir T, Deligdisch L, Weber KJ: Mesenteric fibromatosis masquerading as an ovarian neoplasm twenty years after Chernobyl radiation exposure. Gynecol Oncol; 2006 Sep;102(3):587-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Mesenteric fibromatosis masquerading as an ovarian neoplasm twenty years after Chernobyl radiation exposure.
  • BACKGROUND: Since their initial description in 1832, desmoid tumors have been reported to occur in virtually every part of the body.
  • Intra-abdominal desmoid tumors, or mesenteric fibromatosis, are often associated with Familial Adenomatous Polyposis mutation in a syndrome known as Gardner's Syndrome.
  • Although sporadic cases of desmoid tumors do occur, unlike Gardner's Syndrome, they predominantly occur extra-abdominally.
  • CASE: Case report of a 61-year-old female who presented with two months of abdominal pain, progressive lower abdominal distension and a 10-15 pound weight gain accompanied by one week of urinary hesitancy and frequency.
  • Patient underwent a diagnostic workup for an ovarian neoplasm, but was found at surgery to have mesentric fibromatosis.
  • CONCLUSION: Although uncommon, mesentric fibromatosis must be considered in the differential diagnosis when evaluating a patient with an abdominal mass of unknown origin.
  • [MeSH-major] Fibromatosis, Abdominal / diagnosis. Fibromatosis, Aggressive / diagnosis. Ovarian Neoplasms / diagnosis
  • [MeSH-minor] Diagnosis, Differential. Female. Humans. Middle Aged. Power Plants. Radioactive Hazard Release. Ukraine

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  • (PMID = 16678243.001).
  • [ISSN] 0090-8258
  • [Journal-full-title] Gynecologic oncology
  • [ISO-abbreviation] Gynecol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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91. Kaifi JT, Strelow A, Schurr PG, Reichelt U, Yekebas EF, Wachowiak R, Quaas A, Strate T, Schaefer H, Sauter G, Schachner M, Izbicki JR: L1 (CD171) is highly expressed in gastrointestinal stromal tumors. Mod Pathol; 2006 Mar;19(3):399-406
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  • [Title] L1 (CD171) is highly expressed in gastrointestinal stromal tumors.
  • The treatment strategy for mesenchymal tumors of the gastrointestinal tract is based upon typing of the tumor.
  • Especially differential diagnosis of gastrointestinal stromal tumors (GISTs) to leiomyomas is crucial for determining radicality of surgery.
  • L1 cell adhesion molecule (CD171) plays an essential role in tumor progression.
  • The aim of this study was to determine expression of L1 in GISTs, smooth muscle tumors, desmoid-type fibromatosis and peripheral nerve sheath tumors (PNSTs).
  • We retrospectively analyzed a total of 129 surgically resected primary tumors or metastases of 72 GISTs, 29 smooth muscle tumors, seven PNSTs and 21 desmoid-type fibromatosis by immunohistochemistry for c-kit, CD34, smooth muscle actin, desmin, vimentin, S-100 and L1 expression.
  • L1 expression was detected in 53 (74%) of 72 GISTs but in none of 29 smooth muscle tumors or 21 desmoid-type fibromatosis (P<0.01 by Fisher's test).
  • In all, four (57%) of seven peripheral nerve sheath tumors were L1-positive.
  • Survival analysis of 55 surgically completely resected GISTs presenting without metastasis at initial diagnosis revealed no tumor-specific death among L1-negative patients (P=0.13 by log-rank test; median follow-up time 41 months) and one recurrence was observed (P=0.12).
  • Interestingly high levels of L1 were seen in tumor vascular endothelial cells of smooth muscle tumors and PNSTs, but not in GISTs.
  • Our data show that L1 is highly expressed in GISTs but not in smooth muscle tumors and desmoid-type fibromatosis being important differential diagnoses.
  • [MeSH-major] Gastrointestinal Stromal Tumors / pathology. Neural Cell Adhesion Molecule L1 / biosynthesis
  • [MeSH-minor] Adult. Diagnosis, Differential. Fibromatosis, Aggressive / metabolism. Fibromatosis, Aggressive / pathology. Humans. Immunohistochemistry. Leiomyoma / metabolism. Leiomyoma / pathology. Middle Aged. Nerve Sheath Neoplasms / metabolism. Nerve Sheath Neoplasms / pathology. Survival Analysis

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  • (PMID = 16400320.001).
  • [ISSN] 0893-3952
  • [Journal-full-title] Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
  • [ISO-abbreviation] Mod. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Neural Cell Adhesion Molecule L1
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92. Odessey EA, Cohn AB, Casper F, Schechter LS: Hereditary gingival fibromatosis: aggressive 2-stage surgical resection in lieu of traditional therapy. Ann Plast Surg; 2006 Nov;57(5):557-60
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Hereditary gingival fibromatosis: aggressive 2-stage surgical resection in lieu of traditional therapy.
  • INTRODUCTION: Hereditary gingival fibromatosis (HGF) is a rare condition characterized by progressive enlargement of the gingiva.
  • CONCLUSIONS: In an effort to address the high recurrence rate, we describe our aggressive surgical approach, including resection of the hypertrophied gingiva and alveolar processes.
  • [MeSH-major] Fibromatosis, Gingival / genetics. Fibromatosis, Gingival / surgery. Reconstructive Surgical Procedures / methods


93. Jakowski JD, Mayerson J, Wakely PE Jr: Fine-needle aspiration biopsy of the distal extremities: a study of 141 cases. Am J Clin Pathol; 2010 Feb;133(2):224-31
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  • Cases were included only if there was a subsequent tissue biopsy or a minimum 1-year clinical follow-up.
  • Specific benign or malignant diagnoses were achievable in 71.6% of cases, whereas the remaining cases were given a descriptive diagnosis (26.2%) or, infrequently, a "suspicious for" diagnosis (2.1%).
  • Overall sensitivity and specificity for distinguishing a benign from malignant entity from all 4 sites were 100% and 96%, respectively, whereas positive and negative predictive values were 88% and 100%, respectively.
  • Of the benign neoplasms, giant cell tumor of tendon sheath (17) and desmoid-type fibromatosis (7) were most common.
  • An FNAB-procured cytopathologic diagnosis is clinically reliable in a high percentage of distal extremity mass lesions.
  • [MeSH-major] Biopsy, Fine-Needle. Extremities. Soft Tissue Neoplasms / diagnosis
  • [MeSH-minor] Ankle. Foot. Ganglion Cysts / diagnosis. Hand. Humans. Sarcoma / diagnosis. Sensitivity and Specificity. Wrist

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  • (PMID = 20093231.001).
  • [ISSN] 1943-7722
  • [Journal-full-title] American journal of clinical pathology
  • [ISO-abbreviation] Am. J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article
  • [Publication-country] United States
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94. Khanfir K, Zouhair A: Review: aggressive fibromatosis and breast implant. Clin Adv Hematol Oncol; 2008 Sep;6(9):693-4
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  • [Title] Review: aggressive fibromatosis and breast implant.
  • [MeSH-major] Breast Implants / adverse effects. Breast Neoplasms / pathology. Fibromatosis, Aggressive / pathology


95. Barbier O, Anract P, Pluot E, Larouserie F, Sailhan F, Babinet A, Tomeno B: Primary or recurring extra-abdominal desmoid fibromatosis: assessment of treatment by observation only. Orthop Traumatol Surg Res; 2010 Dec;96(8):884-9
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  • [Title] Primary or recurring extra-abdominal desmoid fibromatosis: assessment of treatment by observation only.
  • INTRODUCTION: Extra-abdominal desmoid fibromatosis (EADF) is a benign tumoral condition, classically managed by more or less radical and sometimes mutilating excision.
  • One primary tumor showed spontaneous regression, and one recurrence still showed evolution at end of follow-up (23 months).
  • [MeSH-major] Fibroma / therapy. Fibromatosis, Aggressive / therapy. Neoplasm Recurrence, Local / therapy. Soft Tissue Neoplasms / therapy
  • [MeSH-minor] Adolescent. Adult. Aged. Disease Progression. Female. Humans. Kaplan-Meier Estimate. Magnetic Resonance Imaging. Male. Middle Aged. Observation. Retrospective Studies. Young Adult

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  • [Copyright] Copyright © 2010 Elsevier Masson SAS. All rights reserved.
  • (PMID = 21075698.001).
  • [ISSN] 1877-0568
  • [Journal-full-title] Orthopaedics & traumatology, surgery & research : OTSR
  • [ISO-abbreviation] Orthop Traumatol Surg Res
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] France
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96. Amini Nik S, Ebrahim RP, Van Dam K, Cassiman JJ, Tejpar S: TGF-beta modulates beta-Catenin stability and signaling in mesenchymal proliferations. Exp Cell Res; 2007 Aug 1;313(13):2887-95
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  • The results show that the TGF-beta pathway is active in desmoids cells and in in situ tumors.
  • Taken together, we propose that TGF-beta is a modulator of beta-Catenin levels in tumoral fibroblasts and non-tumoral fibroblasts, despite the oncogenic mutations already present in this gene in tumoral fibroblasts of desmoid tumors.
  • [MeSH-major] Fibromatosis, Aggressive / metabolism. Transforming Growth Factor beta / metabolism. beta Catenin / metabolism
  • [MeSH-minor] Animals. Cell Line. Cell Proliferation. Fibroblasts / drug effects. Fibroblasts / metabolism. Fibroblasts / pathology. Glycogen Synthase Kinase 3 / metabolism. Humans. Insulin-Like Growth Factor Binding Protein 6 / genetics. Mesoderm / metabolism. Mesoderm / pathology. Mice. Phosphorylation. Promoter Regions, Genetic / drug effects. Serine / metabolism. Signal Transduction. Transcription, Genetic / drug effects. Tumor Cells, Cultured. Wnt Proteins / metabolism

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  • (PMID = 17601559.001).
  • [ISSN] 0014-4827
  • [Journal-full-title] Experimental cell research
  • [ISO-abbreviation] Exp. Cell Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Insulin-Like Growth Factor Binding Protein 6; 0 / Transforming Growth Factor beta; 0 / Wnt Proteins; 0 / beta Catenin; 452VLY9402 / Serine; EC 2.7.11.1 / glycogen synthase kinase 3 beta; EC 2.7.11.26 / Glycogen Synthase Kinase 3
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97. Altomare DF, Rotelli MT, Rinaldi M, Bocale D, Lippolis C, Lobascio P, Cavallini A: Potential role of the steroid receptor pattern in the response of inoperable intra-abdominal desmoid to toremifene after failure of tamoxifen therapy. Int J Colorectal Dis; 2010 Jun;25(6):787-9
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  • [Title] Potential role of the steroid receptor pattern in the response of inoperable intra-abdominal desmoid to toremifene after failure of tamoxifen therapy.
  • [MeSH-major] Abdomen / pathology. Estrogen Receptor Modulators / therapeutic use. Fibromatosis, Aggressive / drug therapy. Fibromatosis, Aggressive / surgery. Receptors, Steroid / metabolism. Tamoxifen / therapeutic use. Toremifene / therapeutic use

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  • (PMID = 20012438.001).
  • [ISSN] 1432-1262
  • [Journal-full-title] International journal of colorectal disease
  • [ISO-abbreviation] Int J Colorectal Dis
  • [Language] eng
  • [Publication-type] Case Reports; Letter; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Adenomatous Polyposis Coli Protein; 0 / Estrogen Receptor Modulators; 0 / Receptors, Steroid; 094ZI81Y45 / Tamoxifen; 7NFE54O27T / Toremifene
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98. Wong LL, Lurie F, Takanishi DM Jr: Other primary neoplasms in patients with hepatocellular cancer: prognostic implications? Hawaii Med J; 2007 Aug;66(8):204, 206-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • METHODS: A retrospective analysis from an established, prospective database of patients with HCC during 1991-2004 was used to determine demographic data, risk factors, characteristics of the associated second primary neoplasm, and survival.
  • MAIN FINDINGS: Of 306 patients with HCC, 23 patients (7.5%) were identified with a second neoplasm.
  • Two of these patients had a third neoplasm.
  • Second associated primaries included 7 genitourinary (prostate--2, bladder--2, testicular--1, renal--1, ovarian--1), 7 gastrointestinal (colon--6, gastric--1), 3 breast, 2 skin, 2 lung, 2 hematologic, 1 tongue, and 1 desmoid.
  • Mean, 1 and 2-year survival after diagnosis of HCC was 1014.8 days, 75%, and 30%, compared to 782.6 days, 54.6%, and 41.1%, respectively, in the group with HCC only (p > 0.05).
  • CONCLUSION: The incidence of other primary tumors in the setting of HCC is relatively common with a strong clustering of genitourinary and gastrointestinal malignancies.
  • There was no statistically significant difference in overall survival compared to patients with HCC only, suggesting that the association of other primary tumors with HCC does not confer a worse prognosis.

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  • (PMID = 17941372.001).
  • [ISSN] 0017-8594
  • [Journal-full-title] Hawaii medical journal
  • [ISO-abbreviation] Hawaii Med J
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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99. Limaïem F, Ayadi-Kaddour A, Aissa I, Marghli A, Ghedira H, El Mezni F: Desmoid tumour of the chest wall in a patient with a previous aortocoronary bypass: a complication or a coincidence? Pathologica; 2008 Oct;100(5):424-7
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  • [Title] Desmoid tumour of the chest wall in a patient with a previous aortocoronary bypass: a complication or a coincidence?
  • Desmoid tumour is an uncommon, locally invasive non-metastasizing neoplastic lesion.
  • The pathogenesis of this tumour is unclear and its treatment is controversial.
  • In this paper, the authors report a new case of chest wall desmoid tumour in a 67-year-old man previously operated by sternotomy for a triple aortocoronary bypass.
  • To the best of our knowledge only two cases of thoracic desmoid tumours following aortocoronary bypass have been reported in literature.
  • The correlation between the site of the surgical approach and the tumour, and the time interval between surgery and presentation, supports the hypothesis of a causal role of surgical trauma in the occurrence of this tumour.
  • The Authors discuss the pathogenesis of this tumour with review of the current literature.
  • [MeSH-major] Coronary Artery Bypass. Fibromatosis, Aggressive / pathology. Postoperative Complications / pathology. Thoracic Neoplasms / pathology. Thoracic Wall / pathology. Thoracotomy / adverse effects
  • [MeSH-minor] Aged. Disease Susceptibility. Humans. Male. Wound Healing

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  • (PMID = 19253606.001).
  • [ISSN] 0031-2983
  • [Journal-full-title] Pathologica
  • [ISO-abbreviation] Pathologica
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Italy
  • [Number-of-references] 30
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100. Durno C, Monga N, Bapat B, Berk T, Cohen Z, Gallinger S: Does early colectomy increase desmoid risk in familial adenomatous polyposis? Clin Gastroenterol Hepatol; 2007 Oct;5(10):1190-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Does early colectomy increase desmoid risk in familial adenomatous polyposis?
  • BACKGROUND & AIMS: Desmoid tumors are non-metastasizing fibromatoses that occur in 10%-20% of subjects with familial adenomatous polyposis (FAP).
  • Intra-abdominal desmoid tumors are a major cause of mortality in FAP.
  • FAP-associated desmoid tumors are linked to trauma, particularly abdominal surgery, family history of desmoids, hormonal factors, and the location of the APC mutation.
  • We hypothesized that prophylactic colectomy at an early age might increase the risk of developing desmoids.
  • The aim of this study was to determine whether colectomy earlier in life is a risk factor for the development of desmoid tumors.
  • METHODS: An analysis was made of the association between development of desmoid and age at colectomy, family history of desmoids, gender, and APC mutation in FAP patients in the Registry (1980-2005) at Mount Sinai Hospital, Toronto, Ontario, Canada.
  • Desmoid prevalence was 14% (n = 121).
  • Female patients were more likely to develop desmoids than male patients (17% vs 11%, P = .03).
  • Female patients who had an early colectomy were more than 2 times more likely to develop a desmoid, compared with women who had a colectomy at >18 years (P = .01).
  • Early colectomy did not increase risk of developing a desmoid in male patients (P = .42).
  • Female patients who had an early colectomy (</=18 years) were 2.5 times more likely to develop desmoids, compared with male patients who had a late colectomy (P = .05).
  • The prevalence of desmoids in the 5' and 3' groups was 13% and 38%, respectively (P = .0005).
  • Patients with a mutation after codon 1399 were found to have 4 times greater chance of developing a desmoid.
  • CONCLUSIONS: Female patients with FAP are more likely to develop desmoids than male patients.
  • Female patients who had an early colectomy are at significantly greater risk of developing a desmoid compared with female patients who had a colectomy in adulthood.
  • Patients with APC mutations beyond codon 1399 are more likely to develop desmoids.
  • These results suggest that delayed colectomy might be considered in young female patients with FAP to decrease the chances of developing desmoids.
  • [MeSH-major] Abdominal Neoplasms / etiology. Adenomatous Polyposis Coli / surgery. Colectomy / adverse effects. Fibromatosis, Aggressive / etiology

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  • (PMID = 17916546.001).
  • [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; Multicenter Study
  • [Publication-country] United States
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