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1. Kohler EM, Chandra SH, Behrens J, Schneikert J: Beta-catenin degradation mediated by the CID domain of APC provides a model for the selection of APC mutations in colorectal, desmoid and duodenal tumours. Hum Mol Genet; 2009 Jan 15;18(2):213-26
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Beta-catenin degradation mediated by the CID domain of APC provides a model for the selection of APC mutations in colorectal, desmoid and duodenal tumours.
  • Biallelic mutation of the ADENOMATOUS POLYPOSIS COLI (APC) gene is a hallmark of sporadic colorectal cancer and colorectal, duodenal and desmoid tumours that develop in familial adenomatous polyposis (FAP) patients.
  • We describe here a new APC domain, the beta-catenin inhibitory domain (CID) of APC located between the second and third 20 amino acid repeats and therefore present in many truncated APC products found in human tumours.
  • It explains the interdependence of both APC mutations, not only in colorectal but also in duodenal and desmoid tumours.
  • [MeSH-minor] Cell Line, Tumor. Colorectal Neoplasms / genetics. Colorectal Neoplasms / metabolism. Duodenal Neoplasms / genetics. Duodenal Neoplasms / metabolism. Fibromatosis, Aggressive / genetics. Fibromatosis, Aggressive / metabolism. Gene Expression Regulation, Neoplastic. Humans. Models, Genetic. Protein Binding. Protein Structure, Tertiary

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  • (PMID = 18854359.001).
  • [ISSN] 1460-2083
  • [Journal-full-title] Human molecular genetics
  • [ISO-abbreviation] Hum. Mol. Genet.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Adenomatous Polyposis Coli Protein; 0 / beta Catenin
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2. Sinha A, Tekkis PP, Neale KF, Phillips RK, Clark SK: Risk factors predicting intra-abdominal desmoids in familial adenomatous polyposis: a single centre experience. Tech Coloproctol; 2010 Jun;14(2):141-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Risk factors predicting intra-abdominal desmoids in familial adenomatous polyposis: a single centre experience.
  • BACKGROUND: Desmoids are myofibroblastic proliferations occurring in 15% of patients with familial adenomatous polyposis (FAP), 70% being intra-abdominal desmoids (IAD).
  • Since the morbidity and mortality due to desmoids is almost entirely attributable to IAD, we aimed to identify specifically risk factors predicting IAD development in FAP.
  • Multivariate analysis was performed, and hazard ratios (HR) calculated for variables including female gender, 3' APC mutation, surgical intervention for FAP (colectomy with ileo-rectal anastomosis or restorative proctocolectomy), age at surgery and family history (FH) of desmoids.
  • No significant difference in IAD risk was detected between the type of surgical intervention (P = 0.37) or age at surgery (P = 0.29).
  • [MeSH-major] Adenomatous Polyposis Coli / pathology. Fibromatosis, Abdominal / epidemiology

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  • (PMID = 20352275.001).
  • [ISSN] 1128-045X
  • [Journal-full-title] Techniques in coloproctology
  • [ISO-abbreviation] Tech Coloproctol
  • [Language] eng
  • [Grant] United Kingdom / Cancer Research UK / /
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Italy
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3. Wiegmann B, Zardo P, Dickgreber N, Länger F, Fegbeutel C, Haverich A, Fischer S: Biological materials in chest wall reconstruction: initial experience with the Peri-Guard Repair Patch. Eur J Cardiothorac Surg; 2010 Mar;37(3):602-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • METHODS: Nine consecutive patients (seven males, median age: 61 years) underwent chest wall repair due to either secondary incisional herniation development after lung transplantation (LTX, n=3 patients) or malignant disease with chest wall infiltration.
  • Oncologic patients with a diagnosis of desmoid tumour, primary histiocytosis of ribs, sarcoma or lung cancers were accessed through posterolateral thoracotomy and received a resection of two to four ribs.
  • CONCLUSIONS: To achieve satisfactory results after chest wall reconstruction, a material with high-tensile strength, preferably soft structure, availability, ease of use and high biocompatibility is required.

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  • [Copyright] Copyright (c) 2009 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.
  • (PMID = 19733092.001).
  • [ISSN] 1873-734X
  • [Journal-full-title] European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
  • [ISO-abbreviation] Eur J Cardiothorac Surg
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biocompatible Materials
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4. O'Sullivan P, O'Dwyer H, Flint J, Munk PL, Muller N: Soft tissue tumours and mass-like lesions of the chest wall: a pictorial review of CT and MR findings. Br J Radiol; 2007 Jul;80(955):574-80
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Soft tissue tumours and mass-like lesions of the chest wall: a pictorial review of CT and MR findings.
  • Soft tissue tumours and tumour-like lesions of the chest wall are uncommon.
  • The purpose of this pictorial essay is to describe the imaging findings of chest wall soft tissue tumours and tumour-like lesions.
  • We searched the radiological and pathological archive at our institution retrospectively and reviewed the literature on soft tissue tumours of the chest wall.
  • Common chest wall soft tissue tumours and mass-like lesions include peripheral nerve tumours, lipomas, liposarcomas, haemangiomas, elastofibromas, metastases, lymphoma and abscesses.
  • Other lesions encountered include desmoid tumours and malignant fibrous histiocytoma.
  • Many have distinctive radiological findings or occur in specific locations, allowing a specific radiological diagnosis to be suggested.
  • [MeSH-major] Magnetic Resonance Imaging. Soft Tissue Neoplasms / diagnosis. Thoracic Neoplasms / diagnosis. Thoracic Wall / radiography. Tomography, X-Ray Computed

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  • (PMID = 16728417.001).
  • [ISSN] 1748-880X
  • [Journal-full-title] The British journal of radiology
  • [ISO-abbreviation] Br J Radiol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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5. Colović R, Radak V, Micev M, Colović N: [Desmoid tumor within lesser sac]. Srp Arh Celok Lek; 2006 Sep-Oct;134(9-10):438-40
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  • [Title] [Desmoid tumor within lesser sac].
  • Desmoid tumors or fibromatoses comprise a number of benign fibrous proliferative lesions that have local infiltrative growth and tendency to recur after incomplete excision.
  • Compressing the splenic vein, the tumor caused left-sided portal hypertension which subsided after the mass was removed.
  • The histological examination verified typical desmoid tumor.
  • [MeSH-major] Fibromatosis, Aggressive. Peritoneal Neoplasms

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  • (PMID = 17252913.001).
  • [ISSN] 0370-8179
  • [Journal-full-title] Srpski arhiv za celokupno lekarstvo
  • [ISO-abbreviation] Srp Arh Celok Lek
  • [Language] srp
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Serbia and Montenegro
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6. Bhama PK, Chugh R, Baker LH, Doherty GM: Gardner's syndrome in a 40-year-old woman: successful treatment of locally aggressive desmoid tumors with cytotoxic chemotherapy. World J Surg Oncol; 2006;4:96
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  • [Title] Gardner's syndrome in a 40-year-old woman: successful treatment of locally aggressive desmoid tumors with cytotoxic chemotherapy.
  • BACKGROUND: Desmoid tumors that present as a part of Gardener's syndrome can present very difficult management problems.
  • CASE PRESENTATION: We report a case of intra-abdominal desmoid tumor causing distal small bowel obstruction that complicated the management of a more proximal enterocutaneous fistula from the jejunum.
  • After failure of more conventional management options including imatinib, the patient's disease responded to doxorubicin and ifosfamide.
  • CONCLUSION: Systemic cytotoxic therapy with doxorubicin and ifosfamide can be useful for patients with complications from intra-abdominal desmoid tumor.

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  • (PMID = 17173703.001).
  • [ISSN] 1477-7819
  • [Journal-full-title] World journal of surgical oncology
  • [ISO-abbreviation] World J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC1716167
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7. Neuman HB, Brogi E, Ebrahim A, Brennan MF, Van Zee KJ: Desmoid tumors (fibromatoses) of the breast: a 25-year experience. Ann Surg Oncol; 2008 Jan;15(1):274-80
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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 (fibromatoses) of the breast: a 25-year experience.
  • BACKGROUND: Breast desmoid tumors are rare and often clinically mistaken for carcinoma.
  • We reviewed our 25-year institutional experience with breast desmoid tumors.
  • METHODS: A search of pathology and sarcoma databases (1982-2006) identified 32 patients with pathologically confirmed breast desmoids.
  • Eight patients (25%) had prior history of breast cancer and 14 (44%) of breast surgery, with desmoids diagnosed a median of 24 months postoperatively.
  • Treatment was surgical, with median tumor size of 2.5 cm (range, 0.3-15).
  • Eight patients (29%) had recurring tumors at a median 15 months.
  • Patients with recurring tumors were younger (median age: 28 vs. 46 years, p = 0.03).
  • A trend toward more frequent recurrences in patients with positive (5/9 patients) versus negative (3/19 patients) margins (p = 0.07) and larger tumors (p = 0.12) was observed.
  • CONCLUSIONS: In our series, breast desmoids presented as palpable masses suspicious for carcinoma clinically and radiographically.
  • Recurrences are common, with younger age and possibly positive margin status and larger tumor size associated with increased risk of recurrence.
  • [MeSH-major] Breast Neoplasms / pathology. Fibromatosis, Aggressive / pathology
  • [MeSH-minor] Adult. Aged. Female. Humans. Mammography. Mastectomy. Middle Aged. Neoplasm Recurrence, Local / surgery

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  • (PMID = 17896146.001).
  • [ISSN] 1534-4681
  • [Journal-full-title] Annals of surgical oncology
  • [ISO-abbreviation] Ann. Surg. Oncol.
  • [Language] eng
  • [Grant] United States / AHRQ HHS / HS / 5 T32 HS000066
  • [Publication-type] Journal Article; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
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8. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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9. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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10. Melis M, Zager JS, Sondak VK: Multimodality management of desmoid tumors: how important is a negative surgical margin? J Surg Oncol; 2008 Dec 15;98(8):594-602
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Multimodality management of desmoid tumors: how important is a negative surgical margin?
  • Desmoid tumors are rare, locally invasive mesenchymal tumors without metastatic potential.
  • We reviewed the current evidence for multimodality therapy of desmoids, with a focus on the importance of resection margins, and present our own algorithm for treatment.
  • [MeSH-major] Fibromatosis, Abdominal / pathology. Fibromatosis, Abdominal / therapy. Fibromatosis, Aggressive / pathology. Fibromatosis, Aggressive / therapy. Neoplasm Recurrence, Local / prevention & control
  • [MeSH-minor] Adult. Algorithms. Child. Combined Modality Therapy. Disease-Free Survival. Humans. Neoplasm, Residual. Treatment Outcome

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  • (PMID = 19072851.001).
  • [ISSN] 1096-9098
  • [Journal-full-title] Journal of surgical oncology
  • [ISO-abbreviation] J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 61
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11. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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12. 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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13. Urresola A, Sáez F, Canteli B, Elorriaga R, López-Duque JC: [Desmoplastic fibroma of bone: a report of two cases]. Radiologia; 2007 May-Jun;49(3):205-10
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Desmoplastic fibroma of bone: a report of two cases].
  • [Transliterated title] Fibroma desmoplásico óseo: a propósito de dos casos.
  • Desmoplastic fibroma is a rare bone tumor with benign, locally aggressive, lytic behavior.
  • Histologically, it is very similar to the more common and better known desmoid tumor.
  • We present two cases; the tumor was located in the proximal tip of the fibula in one case and in the proximal tip of the femur in the other.
  • At MRI, one of the tumors was very heterogeneous, with two differentiated areas on T2-weighted sequences: the upper zone was hypointense and the lower zone showed intermediate signal intensity.
  • We also describe the behavior of the tumor after the administration of intravenous Gadolinium: the upper zone showed no significant enhancement and the lower showed significant enhancement in the early phase.
  • [MeSH-major] Bone Neoplasms / diagnosis. Fibroma, Desmoplastic / diagnosis

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  • (PMID = 17524342.001).
  • [ISSN] 0033-8338
  • [Journal-full-title] Radiología
  • [ISO-abbreviation] Radiologia
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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14. Sinno H, Zadeh T: Desmoid tumors of the pediatric mandible: case report and review. Ann Plast Surg; 2009 Feb;62(2):213-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Desmoid tumors of the pediatric mandible: case report and review.
  • Desmoid tumors are benign fibrous neoplasms originating from the musculoaponeurotic structures throughout the body.
  • Twenty-five percent of all desmoid tumors occur in children under 15 years of age.
  • The infrequency of these tumors has limited studies to case reports and retrospective reviews dictating the authors recommended treatments and management.
  • We present a case report of desmoid tumor involving the left mandible in a 14-month-old infant.
  • His treatment course included 2 excisions, removal of the free rib graft secondary to persistence of the tumor, and wound dehiscence, and later a free fibular osteomyocutaneous flap for reconstruction.
  • We then retrospectively reviewed all published data of desmoid tumor involving the pediatric mandible since 1950 to 2007 in the PubMed database.
  • There appeared to be left-sided predominance of desmoid tumors in the pediatric mandible with a ratio of 3:1.
  • The mean size of the tumors was 4.6 cm (SD +/- 2.1) at the largest diameter.
  • It is found that when compared with conservative management, radiation therapy, chemotherapy, and curettage or surgical local excisions as treatment options the most efficient treatment was partial mandiblectomy, which resulted in complete tumor dissipation with no tumor recurrence.
  • In summary, this is the largest review of the pediatric desmoid tumor of the mandible to date where we provide for the first time an algorithm for the management and treatment of the pediatric desmoid tumor of the mandible.
  • [MeSH-major] Fibromatosis, Aggressive. Mandibular Neoplasms

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  • (PMID = 19158537.001).
  • [ISSN] 1536-3708
  • [Journal-full-title] Annals of plastic surgery
  • [ISO-abbreviation] Ann Plast Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 56
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15. Summa A, Cerasti D, Crisi G, Ormitti F, Ventura E, Sabato M: Desmoplastic Fibroma of the Mandible: Usefulness of CT and MR Imaging in Diagnosis and Treatment. A Case Report. Neuroradiol J; 2010 Mar;23(1):109-13
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  • [Title] Desmoplastic Fibroma of the Mandible: Usefulness of CT and MR Imaging in Diagnosis and Treatment. A Case Report.
  • Desmoplastic fibroma (DF) is a rare non-metastasizing benign neoplasm of the bone characterized by aggressive local infiltration, also known as desmoid tumour.
  • Desmoplastic fibroma of the mandible may recur locally when incompletely excised.
  • This case report describes the imaging findings of a histopathologically proven desmoplastic fibroma involving the right mandibular region in a three-year-old Italian girl.
  • We also discuss the usefulness of different imaging modalities (CT and MRI) in DF diagnosis and treatment.

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  • (PMID = 24148342.001).
  • [ISSN] 1971-4009
  • [Journal-full-title] The neuroradiology journal
  • [ISO-abbreviation] Neuroradiol J
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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16. Barrera E, García A, Ferrufino J: [Desmoid tumor: report of a case]. Rev Gastroenterol Peru; 2005 Jul-Sep;25(3):288-90
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  • [Title] [Desmoid tumor: report of a case].
  • [Transliterated title] Tumor desmoide: reporte de un caso.
  • The desmoid tumor is a solid tumor with soft parts and with various possible locations.
  • The diagnosis was made by pathological anatomy.
  • [MeSH-major] Fibromatosis, Abdominal
  • [MeSH-minor] Diagnosis, Differential. Duodenal Obstruction / diagnosis. Duodenal Obstruction / etiology. Duodenal Obstruction / pathology. Duodenal Obstruction / radiography. Duodenal Obstruction / surgery. Duodenum / pathology. Female. Humans. Middle Aged

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  • (PMID = 16237475.001).
  • [ISSN] 1022-5129
  • [Journal-full-title] Revista de gastroenterología del Perú : órgano oficial de la Sociedad de Gastroenterología del Perú
  • [ISO-abbreviation] Rev Gastroenterol Peru
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Peru
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17. Lee YC, Lee JW: Innovative treatment for huge nuchal desmoid tumour: a case report with a 2-year follow-up. J Plast Reconstr Aesthet Surg; 2010 Aug;63(8):e622-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Innovative treatment for huge nuchal desmoid tumour: a case report with a 2-year follow-up.
  • BACKGROUND: The desmoid tumour is a monoclonal neoplasm originating from musculoaponeurotic tissues.
  • It is benign in histological presentations and yet its locally invasive behaviour could lead to dire consequences such as disfigurement, functional impairment or even mortality.
  • We experienced an extremely difficult case who had a huge nuchal desmoid tumour measuring 45x35x20 cm in dimension with extension to the anterior neck and thoracic paraspinal area.
  • Moreover, the tumour burden was so immense that the patient was plunged into profound hypoproteinaemic, septic and anaemic status, with severe pain, bleeding and odour that mandated prompt and daring management.
  • METHODS: In an effort to prevent uncontrollable tumour bleeding, we embarked on a series of strategic measures, including pre-surgical embolisation, innovative tourniquet technique, a novel method of ligature deployment, staged tumour excision and adjunct methods, such as ethanol injection and irradiation therapy.
  • RESULTS: The huge nuchal desmoid tumour was successfully excised under the planned strategies.
  • Fortunately, she eventually survived and exhibited no evidences of tumour relapse at 2 years' follow-up.
  • [MeSH-major] Embolization, Therapeutic / methods. Fibromatosis, Aggressive / therapy. Head and Neck Neoplasms / therapy. Suture Techniques / instrumentation. Tourniquets

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  • [Copyright] Copyright 2010 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.
  • (PMID = 20304713.001).
  • [ISSN] 1878-0539
  • [Journal-full-title] Journal of plastic, reconstructive & aesthetic surgery : JPRAS
  • [ISO-abbreviation] J Plast Reconstr Aesthet Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
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18. Tkachev SI, Aliev MD, Glebovskaia VV, Ivanov SM, Trofimova OP, Karapetian RM, Gutnik RA, Bokhian AIu: [Thermoradiotherapy as a component of desmoid tumor management: 10-year experience]. Vopr Onkol; 2005;51(3):347-9
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  • [Title] [Thermoradiotherapy as a component of desmoid tumor management: 10-year experience].
  • Data on radio- and thermoradiotherapy of 83 patients with extra-abdominal desmoid tumors are discussed.
  • Monitoring tumor temperature during local hyperthermia is a factor of relapse-free survival of vital importance.
  • [MeSH-major] Fibromatosis, Aggressive / radiotherapy. Hyperthermia, Induced
  • [MeSH-minor] Disease-Free Survival. Follow-Up Studies. Humans. Radiotherapy / methods. Treatment Outcome

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  • (PMID = 16279100.001).
  • [ISSN] 0507-3758
  • [Journal-full-title] Voprosy onkologii
  • [ISO-abbreviation] Vopr Onkol
  • [Language] rus
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Russia (Federation)
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19. 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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  • [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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20. Bonvalot S, Rimareix F, Bouzaiene H: [Surgical news of soft tissue sarcomas, fibromatosis and GIST]. Bull Cancer; 2010 Jun;97(6):645-56
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Surgical news of soft tissue sarcomas, fibromatosis and GIST].
  • [Transliterated title] Actualités chirurgicales des sarcomes, des fibromatoses et des tumeurs stromales gastro-intestinales.
  • Concerning desmoids, authors address the question whether surgery and other aggressive treatments should systematically be part of first-line treatment, as growth arrest occurred in 2/3 of non-operated patients.
  • In the same way, surgical indications for gastrointestinal stromal tumors evolved: with the development of investigations, more micro-GIST are discovered, rising the question of wait and see policy for some of them.
  • Secondary excision of residual disease has been shown to be related to a good prognosis in responding patients to imatinib, but it is still not demonstrated whether this is due to surgery itself or to a selection bias.
  • [MeSH-major] Fibromatosis, Aggressive / surgery. Gastrointestinal Stromal Tumors / surgery. Retroperitoneal Neoplasms / surgery. Sarcoma / surgery

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  • (PMID = 20547479.001).
  • [ISSN] 1769-6917
  • [Journal-full-title] Bulletin du cancer
  • [ISO-abbreviation] Bull Cancer
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzamides; 0 / Piperazines; 0 / Pyrimidines; 8A1O1M485B / Imatinib Mesylate
  • [Number-of-references] 54
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21. 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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22. McCall T, Rao G, Jensen R: Development and rapid growth of a desmoid tumor in the surgical corridor after suboccipital craniotomy for recurrent low-grade astrocytoma. J Neurooncol; 2006 Nov;80(2):167-70
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  • [Title] Development and rapid growth of a desmoid tumor in the surgical corridor after suboccipital craniotomy for recurrent low-grade astrocytoma.
  • Desmoid tumors are histologically benign but locally invasive tumors that can occur in the head and neck.
  • We present the rare case of a desmoid tumor that occurred in the surgical corridor after suboccipital craniotomy for recurrent low-grade astrocytoma.
  • Histology demonstrated clear surgical margins and a tumor of low cellularity consistent with a desmoid tumor.
  • Desmoid tumors should be considered in the differential diagnosis for superficial masses occurring in the surgical bed after posterior cervical surgery.
  • [MeSH-major] Astrocytoma / surgery. Brain Neoplasms / pathology. Fibromatosis, Aggressive / pathology. Postoperative Complications / pathology

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  • (PMID = 16645711.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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23. Fiore M, Rimareix F, Mariani L, Domont J, Collini P, Le Péchoux C, Casali PG, Le Cesne A, Gronchi A, Bonvalot S: Desmoid-type fibromatosis: a front-line conservative approach to select patients for surgical treatment. Ann Surg Oncol; 2009 Sep;16(9):2587-93
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Desmoid-type fibromatosis: a front-line conservative approach to select patients for surgical treatment.
  • PURPOSE: Surgery is still the standard treatment for desmoid-type fibromatosis (DF).
  • The disease remained stable in more than half of patients.
  • This study was designed to evaluate this approach on the natural history of the disease in a larger series of patients.
  • RESULTS: Seventy-four patients presented with primary tumor, 68 with recurrence.
  • Half of patients had medium-term stable disease after W&S or MT.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Fibromatosis, Aggressive / surgery. Neoplasm Recurrence, Local / diagnosis

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  • (PMID = 19568815.001).
  • [ISSN] 1534-4681
  • [Journal-full-title] Annals of surgical oncology
  • [ISO-abbreviation] Ann. Surg. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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24. Carretto E, Dall'Igna P, Alaggio R, Siracusa F, Granata C, Ferrari A, Cecchetto G: Fibrous hamartoma of infancy: an Italian multi-institutional experience. J Am Acad Dermatol; 2006 May;54(5):800-3
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • BACKGROUND: Fibrous hamartoma (FH) of infancy is a benign mesenchymal tumor, occurring as a superficial mass.
  • Surgery was radical in 10 patients, with microscopic residual disease in 6; all of them are alive with no evidence of disease 2 to 49 months after diagnosis.
  • One patient, treated with a local reexcision for macroscopic residual disease (and chemotherapy for a synchronous desmoid fibromatosis) is well 83 months after diagnosis; the last patient, with a lesion of the labia majora, only underwent biopsy and is doing well, awaiting plastic surgery.
  • An aggressive approach should be avoided, as the overall prognosis is excellent.

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  • (PMID = 16635660.001).
  • [ISSN] 1097-6787
  • [Journal-full-title] Journal of the American Academy of Dermatology
  • [ISO-abbreviation] J. Am. Acad. Dermatol.
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] United States
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25. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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26. 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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27. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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28. 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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29. 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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30. 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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31. 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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  • 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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32. 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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  • [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


33. 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.


34. 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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  • [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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35. 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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  • [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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36. 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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37. 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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38. 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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39. 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
  • [Journal-full-title] Journal of endocrinological investigation
  • [ISO-abbreviation] J. Endocrinol. Invest.
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40. 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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41. 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


42. 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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  • [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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43. 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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44. 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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45. 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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46. 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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  • [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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47. 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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48. 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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49. 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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50. 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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51. 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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52. 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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53. 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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54. Bhattacharya B, Dilworth HP, Iacobuzio-Donahue C, Ricci F, Weber K, Furlong MA, Fisher C, Montgomery E: Nuclear beta-catenin expression distinguishes deep fibromatosis from other benign and malignant fibroblastic and myofibroblastic lesions. Am J Surg Pathol; 2005 May;29(5):653-9
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  • [Title] Nuclear beta-catenin expression distinguishes deep fibromatosis from other benign and malignant fibroblastic and myofibroblastic lesions.
  • Deep fibromatoses (desmoid tumors) are clonal myofibroblastic proliferations that are prone to aggressive local recurrences but that do not metastasize.
  • Virtually all deep fibromatoses have somatic beta-catenin or adenomatous polyposis coli (APC) gene mutations leading to intranuclear accumulation of beta-catenin.
  • Since low-grade sarcomas in general lack beta-catenin and since reactive proliferations would not be expected to have it, we predicted that nuclear beta-catenin expression would be detected in deep fibromatoses but absent in other entities in the differential diagnosis.
  • We evaluated the role of beta-catenin to help differentiate distinguish deep fibromatoses from congeners.
  • Formalin-fixed, paraffin-embedded sections from 21 lesions from 20 patients with deep fibromatoses were stained with monoclonal beta-catenin antibody (Transduction Laboratories) and compared with low-grade fibromyxoid sarcoma (n=12), leiomyosarcoma (n=10), various other fibrosarcoma variants (n=13, including 3 myofibrosarcomas, 3 sclerosing epithelioid fibrosarcomas, 5 low-grade fibrosarcomas, 1 classic fibrosarcoma arising in dermatofibrosarcoma protuberans, 1 inflammatory myxohyaline tumor/myxoinflammatory fibroblastic sarcoma), myofibroma/myofibromatosis (n=12), nodular fasciitis (n=11), and scars (n=9).
  • All 21 examples of deep fibromatosis displayed nuclear beta-catenin (focal nuclear staining in one case to 90% staining).
  • All other lesions tested (n=67) lacked nuclear labeling for beta-catenin, showing only cytoplasmic accumulation. beta-Catenin immunohistochemistry separates deep fibromatosis from entities in the differential diagnosis, a finding that can be exploited for diagnosis.
  • Most fibromatoses have diffuse nuclear staining although occasional examples only focally label.
  • [MeSH-major] Cell Nucleus / pathology. Cytoskeletal Proteins / metabolism. Fibroblasts / pathology. Fibromatosis, Abdominal / pathology. Sarcoma / pathology. Trans-Activators / metabolism
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Biomarkers, Tumor / metabolism. Child. Cicatrix / metabolism. Cicatrix / pathology. Cytoplasm / metabolism. Cytoplasm / pathology. Diagnosis, Differential. Fasciitis / metabolism. Fasciitis / pathology. Female. Humans. Male. Middle Aged. beta Catenin

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  • (PMID = 15832090.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / CTNNB1 protein, human; 0 / Cytoskeletal Proteins; 0 / Trans-Activators; 0 / beta Catenin
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55. 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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56. 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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  • (PMID = 23133290.001).
  • [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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57. Ibrahim M, Sandogji H, Allam A: Huge intrathoracic desmoid tumor. Ann Thorac Med; 2009 Jul;4(3):146-8
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  • [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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58. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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59. 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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60. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • 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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61. 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


62. 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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63. 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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  • [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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64. de Bree E, Keus R, Melissas J, Tsiftsis D, van Coevorden F: Desmoid tumors: need for an individualized approach. Expert Rev Anticancer Ther; 2009 Apr;9(4):525-35
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  • [Title] Desmoid tumors: need for an individualized approach.
  • Desmoid tumor, also known as aggressive fibromatosis or desmoid-type fibromatosis, is a rare monoclonal, fibroblastic proliferation arising in musculoaponeurotic structures.
  • Although histologically benign, desmoids are often locally invasive and associated with a high local recurrence rate after resection.
  • Since it is a heterogeneous disease, in particular regarding clinical presentation, anatomic location and biological behavior, treatment should be individualized to reduce local tumor control failure with concurrently acceptable morbidity and preservation of quality of life.
  • Many issues regarding optimal treatment of desmoids remain controversial.
  • Radiotherapy for gross disease is considerably effective, but is associated with a relatively high rate of complications, which are usually mild or moderate and radiation dose dependent.
  • Risk factors for local tumor control failure include young age, large size, presentation as recurrent disease, limb/girdle or intra-abdominal location, involved surgical margins, omission of radiotherapy, radiation dose less than 50 Gy and insufficient radiation field size.
  • Increased comprehension of the pathogenesis and biological behavior of desmoids resulted in the emerging applicability of systemic therapies and a wait-and-see policy.
  • Considering the significant morbidity of surgery and/or radiotherapy for certain locations, especially mutilation and loss of function, and the tumor's natural history, which is often characterized by prolonged periods of stability or even regression, a period of watchful waiting may compose the most appropriate management in selected asymptomatic patients.
  • Attempts to complete eradication of the disease may be worse than the disease itself.
  • [MeSH-major] Fibromatosis, Aggressive / therapy
  • [MeSH-minor] Adult. Age Factors. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Case Management. Child. Combined Modality Therapy. Cyclooxygenase 2 Inhibitors / therapeutic use. Female. Humans. Male. Neoplasm Recurrence, Local. Pregnancy. Pregnancy Complications, Neoplastic / surgery. Radiotherapy / adverse effects. Radiotherapy, Adjuvant. Referral and Consultation. Risk Factors

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  • (PMID = 19374605.001).
  • [ISSN] 1744-8328
  • [Journal-full-title] Expert review of anticancer therapy
  • [ISO-abbreviation] Expert Rev Anticancer Ther
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Cyclooxygenase 2 Inhibitors
  • [Number-of-references] 89
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65. 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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66. Janitzky A, Porsch M, Daher M, Küster D, Liehr UB: [Aggressive fibromatosis (desmoid tumor) : A rare differential diagnosis of metastasis of renal cell carcinoma]. Urologe A; 2010 Jan;49(1):81-3
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  • [Title] [Aggressive fibromatosis (desmoid tumor) : A rare differential diagnosis of metastasis of renal cell carcinoma].
  • [Transliterated title] Aggressive Fibromatose (Desmoidfibromatose) : Seltene Differentialdiagnose einer Nierenzellkarzinommetastase.
  • We report the case of a 65-year-old woman with an aggressive fibromatosis of the rectus abdominis muscle suspicious for a metastasis of renal cell carcinoma after tumor nephrectomy 3 years previously.
  • Aggressive fibromatoses (desmoid tumors) are rare semimalignant tumors of the connective tissue with local infiltration and destruction of tissue.
  • Complete resection is essential to avoid tumor relapse.
  • Aggressive fibromatosis must be considered in the differential diagnosis of renal cell carcinoma metastasis.
  • [MeSH-major] Abdominal Muscles / pathology. Carcinoma, Renal Cell / diagnosis. Carcinoma, Renal Cell / secondary. Fibromatosis, Abdominal / diagnosis. Kidney Neoplasms / diagnosis. Muscle Neoplasms / pathology
  • [MeSH-minor] Aged. Diagnosis, Differential. Female. Humans


67. 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


68. 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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69. 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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70. 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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71. 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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72. 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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73. 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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74. 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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75. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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76. 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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77. 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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78. 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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79. 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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80. Palacios E: Aggressive fibromatosis of the neck. Ear Nose Throat J; 2005 Feb;84(2):76-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Aggressive fibromatosis of the neck.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Head and Neck Neoplasms / diagnosis
  • [MeSH-minor] Adult. Diagnosis, Differential. Female. Humans


81. 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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82. 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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83. 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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84. 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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85. 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


86. Chugh R, Wathen JK, Patel SR, Maki RG, Meyers PA, Schuetze SM, Priebat DA, Thomas DG, Jacobson JA, Samuels BL, Benjamin RS, Baker LH, Sarcoma Alliance for Research through Collaboration (SARC): Efficacy of imatinib in aggressive fibromatosis: Results of a phase II multicenter Sarcoma Alliance for Research through Collaboration (SARC) trial. Clin Cancer Res; 2010 Oct 1;16(19):4884-91
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Efficacy of imatinib in aggressive fibromatosis: Results of a phase II multicenter Sarcoma Alliance for Research through Collaboration (SARC) trial.
  • PURPOSE: Aggressive fibromatoses (AF; desmoid tumors) are rare clonal neoplastic proliferations of connective tissues that can be locally aggressive despite wide surgical resection and/or radiation therapy.
  • EXPERIMENTAL DESIGN: Patients ≥10 years old with desmoid tumors that were not curable by surgical management or in whom curative surgery would lead to undesirable functional impairment were eligible.
  • Tissue specimens were analyzed by immunohistochemistry for expression of cKIT, platelet-derived growth factor receptor α (PDGFRα), PDGFRβ, AKT, PTEN, FKHR, and β-catenin.
  • Tumor DNA was analyzed for PDGFRα exon 18 and APC mutations by allelic discrimination PCR.
  • Expression and polymorphisms of target proteins were identified in tissue samples, but no significant correlation with outcome was observed using the samples available.
  • CONCLUSION: Imatinib may have a role in the management of unresectable or difficult to resect desmoid tumors.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Fibromatosis, Aggressive / drug therapy. Piperazines / therapeutic use. Pyrimidines / therapeutic use


87. 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


88. 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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  • [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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89. 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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90. 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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91. 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


92. 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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93. 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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94. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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95. Lev D, Kotilingam D, Wei C, Ballo MT, Zagars GK, Pisters PW, Lazar AA, Patel SR, Benjamin RS, Pollock RE: Optimizing treatment of desmoid tumors. J Clin Oncol; 2007 May 1;25(13):1785-91
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Optimizing treatment of desmoid tumors.
  • PURPOSE: This study compared a large series of desmoid patients treated at a single institution to a previously published series from the same institution to determine if patient population characteristics, treatment approaches, and clinical outcomes had undergone change over the two study periods.
  • MATERIALS AND METHODS: Data from a prospective soft tissue tumor database was used to analyze clinical courses of 189 desmoid patients treated at The University of Texas M.D.
  • Anderson Cancer Center (UTMDACC) from 1995 to 2005 as compared with 189 UTMDACC desmoid patients treated between 1965 and 1994.
  • RESULTS: A nearly three-fold increase in annualized UTMDACC desmoid referral volume with significantly higher percentages and numbers of primary desmoid tumor referrals to UTMDACC was observed in the most recent study period.
  • While the recent series patients had higher rates of macroscopic residual disease and equivalent rates of positive microscopic margins after definitive surgery, the estimated 5-year local recurrence rate of 20% was improved compared with the 30% rate observed in the earlier series.
  • CONCLUSION: Increased awareness of the complex multidisciplinary management needed for desmoid tumor control may underlie significantly increased numbers of referrals to UTMDACC, especially primary untreated desmoids.
  • Increased neoadjuvant treatments may be associated with improved desmoid patient outcomes.
  • These trends should be supported, particularly if personalized molecular-based therapies are to be rapidly and effectively deployed for the benefit of those afflicted by this rare and potentially debilitating disease.
  • [MeSH-major] Fibromatosis, Aggressive / therapy

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  • (PMID = 17470870.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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96. 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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97. 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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98. Yang JL, Wang J, Zhu XZ: [Progress in the research of pathology and genetics of desmoid tumor]. Zhonghua Bing Li Xue Za Zhi; 2005 Aug;34(8):537-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Progress in the research of pathology and genetics of desmoid tumor].
  • [MeSH-major] Abdominal Neoplasms / genetics. Abdominal Neoplasms / pathology. Fibromatosis, Aggressive / genetics. Fibromatosis, Aggressive / pathology

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  • (PMID = 16383304.001).
  • [ISSN] 0529-5807
  • [Journal-full-title] Zhonghua bing li xue za zhi = Chinese journal of pathology
  • [ISO-abbreviation] Zhonghua Bing Li Xue Za Zhi
  • [Language] chi
  • [Publication-type] Journal Article; Review
  • [Publication-country] China
  • [Chemical-registry-number] 0 / beta Catenin
  • [Number-of-references] 35
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99. 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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100. 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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