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1. Signoroni S, Frattini M, Negri T, Pastore E, Tamborini E, Casieri P, Orsenigo M, Da Riva L, Radice P, Sala P, Gronchi A, Bertario L, Pierotti MA, Pilotti S: Cyclooxygenase-2 and platelet-derived growth factor receptors as potential targets in treating aggressive fibromatosis. Clin Cancer Res; 2007 Sep 1;13(17):5034-40
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Cyclooxygenase-2 and platelet-derived growth factor receptors as potential targets in treating aggressive fibromatosis.
  • PURPOSE: To explore the molecular bases of potential new pharmacologic targets in aggressive fibromatosis (desmoid tumor).
  • EXPERIMENTAL DESIGN: Tumor specimens from 14 patients surgically treated for aggressive fibromatosis (6 familial adenomatous polyposis and 8 sporadic cases), analyzed for adenomatous polyposis coli (APC) and CTNNB1 (beta-catenin) mutations, were further investigated for beta-catenin, cyclooxygenase-2 (COX-2), platelet-derived growth factor (PDGF) receptor alpha (PDGFRA)/PDGF receptor beta (PDGFRB), their cognate ligands (PDGFA and PDGFB), and KIT using a comprehensive immunohistochemical, biochemical, molecular, and cytogenetic approach.
  • CONCLUSIONS: Aggressive fibromatosis is characterized by WNT/oncogene pathway alterations triggering COX-2-mediated constitutive coactivation of PDGFRA and PDGFRB, and may therefore benefit from combined nonsteroidal anti-inflammatory drug + tyrosine kinase inhibitor treatment.
  • [MeSH-major] Cyclooxygenase 2 Inhibitors / therapeutic use. Fibromatosis, Aggressive / drug therapy. Receptors, Platelet-Derived Growth Factor / antagonists & inhibitors

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  • [ErratumIn] Clin Cancer Res. 2008 Jul 1;14(13):4354
  • (PMID = 17785554.001).
  • [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; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Cyclooxygenase 2 Inhibitors; 0 / RNA, Messenger; 0 / beta Catenin; EC 1.14.99.1 / Cyclooxygenase 2; EC 2.7.10.1 / Receptors, Platelet-Derived Growth Factor
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2. Ewald C, Kuhn SA, Brodhun M, Kalff R: Nuchal extra-abdominal aggressive fibromatosis of desmoid type in a 77-year-old female. Neurol India; 2007 Oct-Dec;55(4):419-20
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  • [Title] Nuchal extra-abdominal aggressive fibromatosis of desmoid type in a 77-year-old female.
  • [MeSH-major] Fibromatosis, Abdominal / pathology. Head and Neck Neoplasms / pathology

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  • (PMID = 18040127.001).
  • [ISSN] 0028-3886
  • [Journal-full-title] Neurology India
  • [ISO-abbreviation] Neurol India
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] India
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3. Buitendijk S, van de Ven CP, Dumans TG, den Hollander JC, Nowak PJ, Tissing WJ, Pieters R, van den Heuvel-Eibrink MM: Pediatric aggressive fibromatosis: a retrospective analysis of 13 patients and review of literature. Cancer; 2005 Sep 1;104(5):1090-9
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  • [Title] Pediatric aggressive fibromatosis: a retrospective analysis of 13 patients and review of literature.
  • BACKGROUND: Aggressive fibromatosis (AF) is a soft tissue tumor and is rare in childhood, with high potential for local invasiveness and recurrence.
  • Incomplete resection was the most important determinant for disease recurrence; in the authors' opinion, the role of adjuvant therapy needs to be studied further.
  • Positive margins after surgery indicated a high risk for disease recurrence.
  • [MeSH-major] Fibromatosis, Aggressive / surgery. Soft Tissue Neoplasms / surgery

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  • (PMID = 16015632.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 37
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4. Kurtz JE, Asmane I, Voegeli AC, Neuville A, Dufresne A, Litique V, Chevreau C, Bergerat JP: A V530I Mutation in c-KIT Exon 10 Is Associated to Imatinib Response in Extraabdominal Aggressive Fibromatosis. Sarcoma; 2010;2010:458156
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  • [Title] A V530I Mutation in c-KIT Exon 10 Is Associated to Imatinib Response in Extraabdominal Aggressive Fibromatosis.
  • Aggressive fibromatosis (AF) or desmoid tumor is a rare condition, characterized by deep tissue invasion by a monoclonal fibroblastic neoplasm, developed from musculoaponeurotic structures.
  • Surgery is the treatment of choice, but negative margins can hardly been achieved in large tumors, and can lead to major functional disability.
  • Here, we describe for the first time a V530I KIT exon 10 mutant that was associated to a dramatic imatinib response in an extraabdominal aggressive fibromatosis.
  • In this paper, we discuss the KIT exon 10 mutations or polymorphisms that have been described in a variety of KIT-related conditions, including acute myelogenous leukemia, mastocytosis, and aggressive fibromatosis.

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  • (PMID = 20339585.001).
  • [ISSN] 1369-1643
  • [Journal-full-title] Sarcoma
  • [ISO-abbreviation] Sarcoma
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Egypt
  • [Other-IDs] NLM/ PMC2841250
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5. Gursoy A, Cesur M, Aktaş B, Utkan G, Gedik VT, Erdogan M, Kamel N: Intracranial aggressive fibromatosis presenting as panhypopituitarism and diabetes insipidus. Pituitary; 2005;8(2):123-6
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  • [Title] Intracranial aggressive fibromatosis presenting as panhypopituitarism and diabetes insipidus.
  • Aggressive fibromatosis (AF) is a rare, locally aggressive, proliferative fibroblastic lesion affecting musculoaponeurotic structures, most often, of the limbs and trunk.
  • Intracranial AF is extremely rare and requires aggressive treatment to prevent recurrence.
  • The tumor was markedly isointense on both T2- and T1-weighted images relative to gray matter, and enhanced strongly after administration of gadolinium.
  • No other sites were found to be involved by thorax and abdominal tomography.
  • On the basis of reports that radiotherapy is an effective treatment for this kind of tumor, we administered radiation to the affected area, since chemotherapy and hormonal treatment of non-resectable tumors are not satisfactory.
  • [MeSH-major] Diabetes Insipidus / diagnosis. Fibromatosis, Aggressive / diagnosis. Hypopituitarism / diagnosis. Skull Neoplasms / diagnosis
  • [MeSH-minor] Adult. Diagnosis, Differential. Humans. Magnetic Resonance Imaging. Male


6. Folli F, Galimberti G, Pastore M, Davalli AM, Bosi E: Paraneoplastic insulin resistance syndrome in advanced aggressive fibromatosis (desmoid tumor) treated by imatinib mesylate. Diabetes Care; 2006 Sep;29(9):2178-80
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  • [Title] Paraneoplastic insulin resistance syndrome in advanced aggressive fibromatosis (desmoid tumor) treated by imatinib mesylate.
  • [MeSH-major] Fibromatosis, Aggressive / drug therapy. Insulin Resistance. Piperazines / therapeutic use. Pyrimidines / therapeutic use

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  • (PMID = 16936177.001).
  • [ISSN] 0149-5992
  • [Journal-full-title] Diabetes care
  • [ISO-abbreviation] Diabetes Care
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Benzamides; 0 / Hypoglycemic Agents; 0 / Insulin; 0 / Piperazines; 0 / Protein Kinase Inhibitors; 0 / Pyrimidines; 8A1O1M485B / Imatinib Mesylate
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7. Bauter N, D'Innocenzo R, Kahn M: A clinico-pathologic correlation. Aggressive fibromatosis and peripheral ossifying fibroma. J Mass Dent Soc; 2006;55(2):36-8
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  • [Title] A clinico-pathologic correlation. Aggressive fibromatosis and peripheral ossifying fibroma.
  • [MeSH-major] Fibroma, Ossifying / pathology. Fibromatosis, Aggressive / pathology. Maxillary Neoplasms / pathology. Neoplasms, Multiple Primary / pathology
  • [MeSH-minor] Diagnosis, Differential. Female. Humans. Middle Aged

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  • (PMID = 16937906.001).
  • [ISSN] 0025-4800
  • [Journal-full-title] Journal of the Massachusetts Dental Society
  • [ISO-abbreviation] J Mass Dent Soc
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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8. 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

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  • [Copyright] © 2010 AACR.
  • [ErratumIn] Cancer Res. 2011 Sep 15;71(18):6084. Nik-Amini, Saied [corrected to Amini-Nik, Saeid]
  • (PMID = 20841474.001).
  • [ISSN] 1538-7445
  • [Journal-full-title] Cancer research
  • [ISO-abbreviation] Cancer Res.
  • [Language] eng
  • [Grant] Canada / Canadian Institutes of Health Research / /
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / beta Catenin
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9. Sharma A, Ngan BY, Sándor GK, Campisi P, Forte V: Pediatric aggressive fibromatosis of the head and neck: a 20-year retrospective review. J Pediatr Surg; 2008 Sep;43(9):1596-604
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  • [Title] Pediatric aggressive fibromatosis of the head and neck: a 20-year retrospective review.
  • Aggressive fibromatosis in children is a rare, benign condition that is locally infiltrative and destructive.
  • Recently, nuclear beta-catenin expression has been suggested as a tumor-specific marker for aggressive fibromatosis (desmoid).
  • AIM: The aims of the study were to review our experience of the presentation, management, and treatment outcome of pediatric aggressive fibromatosis in the head and neck and to identify the presence of the desmoid tumor marker beta-catenin within this population.
  • METHOD: The study was conducted as a retrospective case review of children diagnosed with aggressive fibromatosis in the head and neck for a period of 20 years and a review of the literature.
  • Pathologic review of the original tumor specimens was undertaken for evidence of positive tumor margins and presence of nuclear beta-catenin expression.
  • This included 7 patients with extension of the tumor to the resection margin; all had good long-term outcomes with no disease progression.
  • Within our series of pediatric fibromatosis, only 4 cases (40%) had positive results for any nuclear beta-catenin expression, and 6 (60%) of 10 patients had negative results for beta-catenin.
  • Pediatric fibromatosis though aggressive is still a benign condition, and careful thought should be taken before considering adjuvant chemoradiotherapy.
  • Nuclear beta-catenin expression should not be considered a specific tumor marker for pediatric aggressive fibromatosis of the head and neck.
  • Pediatric aggressive fibromatosis in this region may be a distinct subtype of desmoid tumor from its adult form.
  • [MeSH-major] Fibromatosis, Aggressive. Head and Neck Neoplasms

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  • (PMID = 18778992.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 55
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10. De Riu G, Meloni SM, Raho MT, Tullio A: Complications of mandibular reconstruction in childhood: Report of a case of Juvenile Aggressive Fibromatosis. J Craniomaxillofac Surg; 2006 Apr;34(3):168-72
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  • [Title] Complications of mandibular reconstruction in childhood: Report of a case of Juvenile Aggressive Fibromatosis.
  • Juvenile aggressive fibromatosis is an acquired disease affecting young children.
  • There are two types: superficial and deep; the first is not aggressive whilst the second invades other tissues deeply.
  • This is a case report of the deep variant of juvenile aggressive fibromatosis of the lateral mandible affecting a 24-month-old young female patient.
  • The tumour has been treated surgically by resection of the mandible and reconstruction with a rib-graft.
  • [MeSH-major] Bone Resorption / surgery. Fibromatosis, Aggressive / surgery. Mandibular Neoplasms / surgery. Osteogenesis, Distraction / methods. Postoperative Complications / surgery

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  • (PMID = 16549363.001).
  • [ISSN] 1010-5182
  • [Journal-full-title] Journal of cranio-maxillo-facial surgery : official publication of the European Association for Cranio-Maxillo-Facial Surgery
  • [ISO-abbreviation] J Craniomaxillofac Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Scotland
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11. Ravaioli A, Nicoletti S, Tamburini E, Papi M: Control of aggressive fibromatosis by treatment with imatinib mesylate: a step forward? J Cancer Res Clin Oncol; 2009 Feb;135(2):325-6
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  • [Title] Control of aggressive fibromatosis by treatment with imatinib mesylate: a step forward?
  • Numerous studies referring to conventional chemotherapy for aggressive fibromatosis with the use of doxorubicin, cyclophosphamide, vincristin, vinblastine and other drugs have been published.
  • Imatinib mesylate is a recently developed oral anticancer agent designed to selectively inhibit tyrosine kinases implicated in oncogenesis and it seems to represent a promising opportunity (also in first line) in the treatment of patients with advanced disease not candidate to prior surgery.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Fibromatosis, Aggressive / drug therapy. Piperazines / therapeutic use. Pyrimidines / therapeutic use

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  • [CommentOn] J Cancer Res Clin Oncol. 2007 Aug;133(8):533-8 [17453242.001]
  • (PMID = 18825412.001).
  • [ISSN] 1432-1335
  • [Journal-full-title] Journal of cancer research and clinical oncology
  • [ISO-abbreviation] J. Cancer Res. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Comment; Letter
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzamides; 0 / Piperazines; 0 / Pyrimidines; 5V9KLZ54CY / Vinblastine; 8A1O1M485B / Imatinib Mesylate; YL5FZ2Y5U1 / Methotrexate
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12. El-Haddad M, El-Sebaie M, Ahmad R, Khalil E, Shahin M, Pant R, Memon M, Al-Hebshi A, Khafaga Y, Al-Shabanah M, Allam A: Treatment of aggressive fibromatosis: the experience of a single institution. Clin Oncol (R Coll Radiol); 2009 Dec;21(10):775-80
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  • [Title] Treatment of aggressive fibromatosis: the experience of a single institution.
  • AIMS: Aggressive fibromatosis is a locally aggressive infiltrative low-grade tumour, although pathologically benign, and it does not metastasise, yet it can cause serious local distressing symptoms by virtue of local destruction and impairment of local function.
  • The aim of this study was to emphasise the role of radiotherapy and adequate surgery in the treatment of fibromatosis in patients presenting with newly diagnosed or recurrent disease and to analyse our treatment results over 15 years for this rare tumour type.
  • MATERIALS AND METHODS: Fifty-four patients with confirmed diagnosis of aggressive fibromatosis treated at King Faisal Specialist Hospital between 1990 and 2006 were identified from our local cancer registry.
  • RESULTS: Fifty-two per cent (28/54 patients) of our patient population had tumour recurrence when first presented to King Faisal Specialist Hospital.
  • CONCLUSION: Aggressive fibromatosis is effectively treated with surgery and postoperative radiotherapy.
  • Patients first presenting with tumour recurrence may still have local tumour control comparable with newly diagnosed patients.
  • [MeSH-major] Fibroma / radiotherapy. Fibroma / surgery

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  • (PMID = 19875275.001).
  • [ISSN] 1433-2981
  • [Journal-full-title] Clinical oncology (Royal College of Radiologists (Great Britain))
  • [ISO-abbreviation] Clin Oncol (R Coll Radiol)
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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13. Yang JL, Wang J, Zhou XY, Zhu XZ: [Abnormalities of chromosome 8, APC and beta-catenin genes in aggressive fibromatosis]. Zhonghua Zhong Liu Za Zhi; 2008 Feb;30(2):116-20
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  • [Title] [Abnormalities of chromosome 8, APC and beta-catenin genes in aggressive fibromatosis].
  • OBJECTIVE: To explore the role of abnormalities of chromosome 8, APC and beta-catenin genes in tumorigenesis of aggressive fibromatosis.
  • RESULTS: The rate of trisomy 8 in recurrent tumors (62.5%, 5/8) was significantly higher than that in the primary tumors (8.3%, 1/12).
  • Somatic substitution of APC gene was found in 18 of 69 (26.1%) aggressive fibrometases.
  • CONCLUSION: Trisomy 8 may serve as a useful predictor of recurrence in aggressive fibromatosis.
  • There are somatic mutations of the APC and beta-catenin genes in the aggressive fibromatosis, and there are abnormalities in the Wnt signaling pathway.
  • [MeSH-major] Adenomatous Polyposis Coli Protein / genetics. Chromosomes, Human, Pair 8. Fibromatosis, Aggressive / genetics. Trisomy. beta Catenin / genetics
  • [MeSH-minor] Apoptosis. Cyclin D1 / metabolism. Genes, APC. Humans. Ki-67 Antigen / metabolism. Neoplasm Recurrence, Local. Point Mutation. Proto-Oncogene Proteins c-myc / metabolism. Signal Transduction. Wnt Proteins / metabolism

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  • (PMID = 18646694.001).
  • [ISSN] 0253-3766
  • [Journal-full-title] Zhonghua zhong liu za zhi [Chinese journal of oncology]
  • [ISO-abbreviation] Zhonghua Zhong Liu Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Adenomatous Polyposis Coli Protein; 0 / Ki-67 Antigen; 0 / Proto-Oncogene Proteins c-myc; 0 / Wnt Proteins; 0 / beta Catenin; 136601-57-5 / Cyclin D1
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14. Ferenc T, Wroński JW, Kopczyński J, Kulig A, Sidor M, Stalińska L, Dziki A, Sygut J: Analysis of APC, alpha-, beta-catenins, and N-cadherin protein expression in aggressive fibromatosis (desmoid tumor). Pathol Res Pract; 2009;205(5):311-24
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  • [Title] Analysis of APC, alpha-, beta-catenins, and N-cadherin protein expression in aggressive fibromatosis (desmoid tumor).
  • The aims of this study were to analyze the cadherin/catenin adhesion complex in cells from abdominal and extra-abdominal aggressive fibromatosis tumors, and to estimate the correlation between the expression of the tested proteins and the clinical data of the desmoid patients.
  • Immunohistochemistry was used to examine the expression of the cadherin/catenin adhesion complex: APC protein, alpha-, beta-catenin, and N-cadherin in archival material derived from 15 cases of extra-abdominal desmoid tumor (E-AD) and 20 cases of abdominal (AD) desmoid tumor.
  • In the E-AD group, in both cases of recurrent tumors, no alpha-catenin expression was observed but the expression of this protein was detected in primary tumors.
  • In the groups investigated, no statistically significant correlation was found between alpha-catenin, beta-catenin (c), (n) and (c+n) expression, and tumor size (p>0.1).
  • The results regarding beta-catenin expression obtained in our study confirm the previous findings that nuclear accumulation of this protein plays a crucial role in the pathogenesis of aggressive fibromatosis.
  • [MeSH-major] Adenomatous Polyposis Coli Protein / biosynthesis. Cadherins / biosynthesis. Fibromatosis, Aggressive / metabolism. alpha Catenin / biosynthesis. beta Catenin / biosynthesis
  • [MeSH-minor] Adult. Age Factors. Aged. Biomarkers, Tumor / analysis. Female. Gene Expression. Humans. Immunohistochemistry. Male. Young Adult

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  • (PMID = 19124205.001).
  • [ISSN] 1618-0631
  • [Journal-full-title] Pathology, research and practice
  • [ISO-abbreviation] Pathol. Res. Pract.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Adenomatous Polyposis Coli Protein; 0 / Biomarkers, Tumor; 0 / Cadherins; 0 / alpha Catenin; 0 / beta Catenin
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15. Camargo VP, Maki RG: Clinical outcomes of systemic therapy for patients with desmoids. J Clin Oncol; 2009 May 20;27(15_suppl):10585

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

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  • (PMID = 27963883.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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16. 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

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Therapy of aggressive fibromatosis is still an open question: a series of patients treated at a single institution.
  • PURPOSE: This study was an attempt to evaluate the possible role of chemo-hormonotherapy as a possible approach in managing inoperable, deep extra-abdominal aggressive fibromatosis.
  • PATIENTS AND METHODS: A series of patients with inoperable, deep extra-abdominal aggressive fibromatosis, were treated with combination chemo-hormotherapy.
  • Extremities were the most frequent localization (5/9), followed by chest wall in 3 and abdominal wall in one patient.
  • Tumor size in most patients was 5-10 cm, and 3 patients had bulky disease (over 10 cm).
  • Complete remission (CR) was observed in one patient, partial remission (PR) in 4 and stabilization of disease (SD) in 4 patients.
  • No relapse of disease was observed up until now.
  • CONCLUSION: Systemic treatment should be considered in patients with aggressive fibromatosis for whom local treatment approaches are not possible or have failed.

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  • (PMID = 17357193.001).
  • [ISSN] 1107-0625
  • [Journal-full-title] Journal of B.U.ON. : official journal of the Balkan Union of Oncology
  • [ISO-abbreviation] J BUON
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Greece
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17. Dumont AG, Lev D, Lazar A, Joensuu H, Trent J: Simultaneous gastrointestinal stromal tumor (GIST) and desmoid fibromatosis (DF). J Clin Oncol; 2009 May 20;27(15_suppl):10568

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

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  • (PMID = 27963790.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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18. Seper L, Hoppe P, Kruse-Lösler B, Büchter A, Joos U, Kleinheinz J: [Aggressive fibromatosis in the jaw and facial region with bone involvement. A review]. Mund Kiefer Gesichtschir; 2005 Nov;9(6):349-62
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Aggressive fibromatosis in the jaw and facial region with bone involvement. A review].
  • [Transliterated title] Aggressive Fibromatose im Kiefer-Gesichts-Bereich mit ossärer Beteiligung. Eine Ubersicht.
  • BACKGROUND: Aggressive fibromatosis (AF) involving bones of the head is rare and surgery is often complicated by a high recurrence rate.
  • [MeSH-major] Eyelid Neoplasms / diagnosis. Facial Neoplasms / diagnosis. Fibromatosis, Aggressive / diagnosis. Fibromatosis, Gingival / diagnosis. Jaw Neoplasms / diagnosis. Skull Neoplasms / diagnosis
  • [MeSH-minor] Aged. Female. Humans. Neoplasm Recurrence, Local / diagnosis. Neoplasm Recurrence, Local / surgery. Patient Care Team. Radiography, Panoramic. Reoperation. Surgery, Plastic

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  • (PMID = 16142459.001).
  • [ISSN] 1432-9417
  • [Journal-full-title] Mund-, Kiefer- und Gesichtschirurgie : MKG
  • [ISO-abbreviation] Mund Kiefer Gesichtschir
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article; Meta-Analysis
  • [Publication-country] Germany
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19. Constantinidou A, Jones RL, Scurr M, Al-Muderis O, Judson I: Pegylated liposomal doxorubicin, an effective, well-tolerated treatment for refractory aggressive fibromatosis. Eur J Cancer; 2009 Nov;45(17):2930-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pegylated liposomal doxorubicin, an effective, well-tolerated treatment for refractory aggressive fibromatosis.
  • BACKGROUND: Aggressive fibromatosis (AF) or desmoid tumour is a monoclonal proliferation which is locally invasive but does not metastasize.
  • If local treatment fails to control the disease, systemic treatment with anti-oestrogens, non-steroidal anti-inflammatory drugs (NSAIDs) or chemotherapy can be used.
  • In one case ongoing shrinkage of the tumour was observed for over 12 months and partial remission was achieved at 14 months after the completion of treatment.
  • Seven patients achieved stable disease.
  • Nine patients (75%) had no evidence of progression at the end of this follow-up period and disease control has ranged from 7 to 39 months with a median of 14 months.
  • [MeSH-major] Antibiotics, Antineoplastic / therapeutic use. Doxorubicin / analogs & derivatives. Fibromatosis, Aggressive / drug therapy. Polyethylene Glycols / therapeutic use
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Disease Progression. Drug Administration Schedule. Female. Follow-Up Studies. Humans. Male. Middle Aged. Treatment Outcome. Young Adult

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  • [CommentIn] Eur J Cancer. 2009 Nov;45(17):2928-9 [19804964.001]
  • (PMID = 19767198.001).
  • [ISSN] 1879-0852
  • [Journal-full-title] European journal of cancer (Oxford, England : 1990)
  • [ISO-abbreviation] Eur. J. Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 0 / liposomal doxorubicin; 30IQX730WE / Polyethylene Glycols; 80168379AG / Doxorubicin
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20. Skubitz KM, Manivel JC, Clohisy DR, Frolich JW: Response of imatinib-resistant extra-abdominal aggressive fibromatosis to sunitinib: case report and review of the literature on response to tyrosine kinase inhibitors. Cancer Chemother Pharmacol; 2009 Aug;64(3):635-40
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Response of imatinib-resistant extra-abdominal aggressive fibromatosis to sunitinib: case report and review of the literature on response to tyrosine kinase inhibitors.
  • PURPOSE: Aggressive fibromatosis (AF) is usually a slowly growing locally invasive tumor, but may exhibit a much more aggressive phenotype.
  • METHODS: We report a case of an aggressive multicentric extra-abdominal AF that was responsive to sunitinib, but resistant to imatinib.
  • After 13 months, tumors recurred.
  • [MeSH-major] Fibromatosis, Aggressive / drug therapy. Indoles / therapeutic use. Protein Kinase Inhibitors / therapeutic use. Pyrroles / therapeutic use
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Benzamides. Drug Resistance, Neoplasm. Female. Follow-Up Studies. Humans. Imatinib Mesylate. Neoplasm Recurrence, Local. Piperazines / therapeutic use. Pyrimidines / therapeutic use. Treatment Outcome. Young Adult

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  • (PMID = 19404642.001).
  • [ISSN] 1432-0843
  • [Journal-full-title] Cancer chemotherapy and pharmacology
  • [ISO-abbreviation] Cancer Chemother. Pharmacol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Benzamides; 0 / Indoles; 0 / Piperazines; 0 / Protein Kinase Inhibitors; 0 / Pyrimidines; 0 / Pyrroles; 0 / sunitinib; 8A1O1M485B / Imatinib Mesylate
  • [Number-of-references] 40
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21. Constantinidou A, Scurr M, Jones R, Al-Muderis O, Judson I: Treatment of aggressive fibromatosis with pegylated liposomal doxorubicin: The Royal Marsden Hospital experience. J Clin Oncol; 2009 May 20;27(15_suppl):10519

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

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  • (PMID = 27963658.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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22. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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23. 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


24. 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

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  • (PMID = 15794540.001).
  • [ISSN] 0145-5613
  • [Journal-full-title] Ear, nose, & throat journal
  • [ISO-abbreviation] Ear Nose Throat J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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25. 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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26. Ketelsen D, Hartmann J, Horger M: [MR-imaging of aggressive fibromatosis]. Rofo; 2008 Feb;180(2):83-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [MR-imaging of aggressive fibromatosis].
  • [Transliterated title] MR-Bildgebung der Aggressiven Fibromatose.
  • Aggressive fibromatosis is a rare mesenchymal benign tumor characterized histologically by proliferation of fibroblasts and myofibroblasts with marked production of intercellular collagen.
  • Predominant low signal intensity on all pulse sequences is said to be characteristic of, but not specific for, aggressive fibromatosis.
  • The principal role of imaging in the management of aggressive fibromatosis is preoperative planning an the detection of recurrence or disease progression in nonsurgically managed patients.
  • [MeSH-major] Abdominal Neoplasms / diagnosis. Fibromatosis, Aggressive / diagnosis. Image Enhancement / methods. Magnetic Resonance Imaging / methods

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  • (PMID = 18224580.001).
  • [ISSN] 1438-9029
  • [Journal-full-title] RöFo : Fortschritte auf dem Gebiete der Röntgenstrahlen und der Nuklearmedizin
  • [ISO-abbreviation] Rofo
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 5
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27. Micke O, Seegenschmiedt MH, German Cooperative Group on Radiotherapy for Benign Diseases: Radiation therapy for aggressive fibromatosis (desmoid tumors): results of a national Patterns of Care Study. Int J Radiat Oncol Biol Phys; 2005 Mar 1;61(3):882-91
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  • [Title] Radiation therapy for aggressive fibromatosis (desmoid tumors): results of a national Patterns of Care Study.
  • PURPOSE: After a general Patterns of Care Study (PCS) the German Cooperative Group on Radiotherapy for Benign Diseases (GCG-BD) initiated a multicenter cohort study to analyze the radiation therapy practice for aggressive fibromatosis.
  • METHODS AND MATERIALS: In 2002 a PCS was conducted in all German radiotherapy (RT) institutions by mailing a standardized structured questionnaire, to assess patients accrual, number, pretreatment, treatment indications, RT, and target volume concepts for irradiation in aggressive fibromatosis.
  • The local control rate was 81.4% in primary RT for unresectable tumors and 79.6% in postoperative RT.
  • No clear dose-response relationship could be established, but there was a tendency toward a lower local control rate in patients with a higher number of operative procedures before RT and patients treated for recurrent aggressive fibromatosis.
  • CONCLUSIONS: This study comprises the largest database of cases reported for RT in aggressive fibromatosis.
  • Radiotherapy provides a high local control rate in the postoperative setting and in unresectable tumors.
  • [MeSH-major] Fibromatosis, Aggressive / radiotherapy

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  • (PMID = 15708271.001).
  • [ISSN] 0360-3016
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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28. Tjandra SS, Hsu C, Goh YI, Gurung A, Poon R, Nadesan P, Alman BA: IFN-{beta} signaling positively regulates tumorigenesis in aggressive fibromatosis, potentially by modulating mesenchymal progenitors. Cancer Res; 2007 Aug 1;67(15):7124-31
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] IFN-{beta} signaling positively regulates tumorigenesis in aggressive fibromatosis, potentially by modulating mesenchymal progenitors.
  • Aggressive fibromatosis (also called desmoid tumor) is a benign, locally invasive, soft tissue tumor composed of cells with mesenchymal characteristics.
  • These tumors are characterized by increased levels of beta-catenin-mediated T-cell factor (TCF)-dependent transcriptional activation.
  • We found that type 1 IFN signaling is activated in human and murine aggressive fibromatosis tumors and that the expression of associated response genes is regulated by beta-catenin.
  • When mice deficient for the type 1 IFN receptor (Ifnar1-/-) were crossed with mice predisposed to developing aggressive fibromatosis tumors (Apc/Apc1638N), a significant decrease in aggressive fibromatosis tumor formation was observed compared with littermate controls, showing a novel role for type 1 IFN signaling in promoting tumor formation.
  • Type 1 IFN activation inhibits cell proliferation but does not alter cell apoptosis or the level of beta-catenin-mediated TCF-dependent transcriptional activation in aggressive fibromatosis cell cultures.
  • Intriguingly, Ifnar1-/- mice have smaller numbers of mesenchymal progenitor cells compared with littermate controls, and treatment of aggressive fibromatosis cell cultures with IFN increases the proportion of cells that exclude Hoechst dye and sort to the side population, raising the possibility that type 1 IFN signaling regulates the number of precursor cells present that drive aggressive fibromatosis tumor formation and maintenance.
  • This study identified a novel role for IFN type 1 signaling as a positive regulator of neoplasia and suggests that IFN treatment is a less than optimal therapy for this tumor type.
  • [MeSH-major] Fibromatosis, Aggressive / metabolism. Genes, APC / physiology. Interferon-beta / physiology. Receptor, Interferon alpha-beta / metabolism. Receptor, Interferon alpha-beta / physiology. Signal Transduction / physiology
  • [MeSH-minor] Animals. Blotting, Western. Cell Proliferation. Cell Transformation, Neoplastic. Colony-Forming Units Assay. Female. Fibroblasts / metabolism. Flow Cytometry. Humans. Male. Mesenchymal Stromal Cells. Mice. Neoplasm Invasiveness / pathology. T Cell Transcription Factor 1 / metabolism. Transcription, Genetic. Transgenes / physiology. Tumor Cells, Cultured. beta Catenin / metabolism

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  • [ErratumIn] Cancer Res. 2008 Feb 1;68(3):956. Goh, Ingrid [corrected to Goh, Y Ingrid]
  • (PMID = 17671179.001).
  • [ISSN] 0008-5472
  • [Journal-full-title] Cancer research
  • [ISO-abbreviation] Cancer Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / CTNNB1 protein, mouse; 0 / IFNAR1 protein, human; 0 / Ifnar1 protein, mouse; 0 / T Cell Transcription Factor 1; 0 / beta Catenin; 156986-95-7 / Receptor, Interferon alpha-beta; 77238-31-4 / Interferon-beta
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29. Kruse AL, Luebbers HT, Grätz KW, Obwegeser JA: Aggressive fibromatosis of the head and neck: a new classification based on a literature review over 40 years (1968-2008). Oral Maxillofac Surg; 2010 Dec;14(4):227-32
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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 head and neck: a new classification based on a literature review over 40 years (1968-2008).
  • BACKGROUND: Fibromatosis is an aggressive fibrous tumor of unknown etiology that is, in some cases, lethal.
  • METHODS: An evidence-based literature review was conducted from the last 40 years regarding aggressive fibromatosis in the head and neck.
  • RESULTS: Between 1968 and 2008, 179 cases with aggressive fibromatosis of the head and neck were published.
  • [MeSH-major] Fibromatosis, Aggressive / classification. Head and Neck Neoplasms / classification

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  • (PMID = 20407799.001).
  • [ISSN] 1865-1569
  • [Journal-full-title] Oral and maxillofacial surgery
  • [ISO-abbreviation] Oral Maxillofac Surg
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Germany
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30. Wcislo G, Szarlej-Wcislo K, Szczylik C: Control of aggressive fibromatosis by treatment with imatinib mesylate. A case report and review of the literature. J Cancer Res Clin Oncol; 2007 Aug;133(8):533-8
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  • [Title] Control of aggressive fibromatosis by treatment with imatinib mesylate. A case report and review of the literature.
  • OBJECTIVE: There has been only one report available that focuses on the treatment with imatinib mesylate of two individual persons with aggressive fibromatosis.
  • The novel therapy should be considered as salvage in patients with aggressive fibromatosis expressed platelet-derived growth factor receptor-alfa, beta (PDGFR-alfa, PDGFR-beta), and/or c-kit, whose tumors are uncontrollable by the standard management.
  • METHODS: The patient was suffering from aggressive fibromatosis after prior therapy including surgery (R2), radiotherapy, and systemic treatment with combination of tamoxifen and sulindac.
  • The tumor specimen was immunostained for PDGFR-beta and c-kit (CD117), and PDGFR-alfa and cytokines platelet-derived growth factor-alfa and beta were not assessed.
  • RESULTS: After three months of the therapy, radiographic (met criteria of SD but small decrease of the tumor was noted) and clinical responses were recorded for the first time.
  • The last MRI revealed readily a smaller tumor (35 x 20 mm) after such a therapy lasted more than 3 years.
  • CONCLUSIONS: Treatment with imatinib mesylate has been a well-accepted therapy for chronic myelogenous leukemia (CML) and gastrointestinal stromal tumors (GIST).
  • The salvage therapy for aggressive fibromatosis with imatinib mesylate seems to be an attractive opportunity for patients with the advanced disease, whose prior therapy failed.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Fibromatosis, Aggressive / drug therapy. Piperazines / therapeutic use. Pyrimidines / therapeutic use


31. Dufresne A, Penel N, Salas S, Le Cesne A, Perol D, Bui B, Brain E, Ray-Coquard I, Jimenez M, Blay J: Updated outcome with long-term follow-up of imatinib for the treatment of progressive or recurrent aggressive fibromatosis (desmoid tumor): A FNCLCC/ French Sarcoma Group phase II trial. J Clin Oncol; 2009 May 20;27(15_suppl):10518

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

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  • (PMID = 27963656.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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32. Caglar K, Ozyönüm H, Akalin M, Balci S: Effective treatment of multifocal aggressive fibromatosis with low-dose chemotherapy. Turk J Pediatr; 2006 Oct-Dec;48(4):365-8
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  • [Title] Effective treatment of multifocal aggressive fibromatosis with low-dose chemotherapy.
  • Desmoid tumor (aggressive fibromatosis), as a member of a group of borderline neoplasms, is a rare tumor of fibroblastic origin that remains difficult to treat.
  • In this report we describe a new patient with multifocal aggressive fibromatosis who was successfully treated with low-dose chemotherapy consisting of methotrexate and vinblastine.
  • [MeSH-major] Antimetabolites, Antineoplastic / therapeutic use. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Fibromatosis, Aggressive / drug therapy. Methotrexate / therapeutic use. Vinblastine / therapeutic use

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  • (PMID = 17290575.001).
  • [ISSN] 0041-4301
  • [Journal-full-title] The Turkish journal of pediatrics
  • [ISO-abbreviation] Turk. J. Pediatr.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Turkey
  • [Chemical-registry-number] 0 / Antimetabolites, Antineoplastic; 5V9KLZ54CY / Vinblastine; YL5FZ2Y5U1 / Methotrexate
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33. Li CF, MacDonald JR, Wei RY, Ray J, Lau K, Kandel C, Koffman R, Bell S, Scherer SW, Alman BA: Human sterile alpha motif domain 9, a novel gene identified as down-regulated in aggressive fibromatosis, is absent in the mouse. BMC Genomics; 2007;8:92
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  • [Title] Human sterile alpha motif domain 9, a novel gene identified as down-regulated in aggressive fibromatosis, is absent in the mouse.
  • In the mesenchymal neoplasm, aggressive fibromatosis, subtractive hybridization identified sterile alpha motif domain 9 (SAMD9) as a substantially down regulated gene in neoplasia.
  • SAMD9 is expressed at a lower level in a variety of neoplasms associated with beta-catenin stabilization, such as aggressive fibromatosis, breast, and colon cancers.
  • Over expression of SAMD9 in the colon cancer cell line, SW480, reduces the volume of tumors formed when transplanted into immune-deficient mice.
  • [MeSH-major] Down-Regulation / genetics. Fibromatosis, Aggressive / genetics. Gene Expression Regulation, Neoplastic. Proteins / genetics. Proteins / physiology
  • [MeSH-minor] Animals. Base Sequence. Breast Neoplasms / pathology. Cell Line. Cell Proliferation. Colonic Neoplasms / pathology. Cytoplasm / chemistry. Female. Humans. Male. Mice. Molecular Sequence Data. Rats. Species Specificity. Tumor Cells, Cultured. Tumor Suppressor Proteins / genetics

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  • (PMID = 17407603.001).
  • [ISSN] 1471-2164
  • [Journal-full-title] BMC genomics
  • [ISO-abbreviation] BMC Genomics
  • [Language] eng
  • [Databank-accession-numbers] GENBANK/ AF445355/ AF453311/ AF474973/ AY195582/ AY195583/ AY195584/ AY195585/ AY195586/ AY195587/ DQ068177
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Proteins; 0 / SAMD9 protein, human; 0 / Tumor Suppressor Proteins
  • [Other-IDs] NLM/ PMC1855325
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34. Yildiz F, Kars A, Cengiz M, Yildiz O, Akyürek S, Selek U, Ozyigit G, Atahan IL: 1,25-Dihydroxy vitamin D3: can it be an effective therapeutic option for aggressive fibromatosis. Med Hypotheses; 2005;64(2):333-6
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  • [Title] 1,25-Dihydroxy vitamin D3: can it be an effective therapeutic option for aggressive fibromatosis.
  • Aggressive fibromatosis (AF), also known as desmoid tumor is a monoclonal fibroblastic proliferation in a collagen matrix that arises in musculoaponeurotic structures.
  • Though considered as benign, they are locally invasive and their propensity for recurrence after conservative surgery is well documented.
  • Since vitamin D has an antineoplastic activity and negative effect on collagen synthesis and deposition, it is proposed that 1,25-dihydroxy vitamin D3 can be a right therapeutic option for the management of desmoid tumors.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Calcitriol / therapeutic use. Fibroma / drug therapy


35. Comandone A, Boglione A, Pochettino P, Berno E, Inguì M, Papotti M, Borasio P, Maggi G, Brach Del Prever E, Gino G: Primary sarcomas of the lungs and mediastinum: Clinicopathological study and therapy results of Piedmontese Group for Sarcomas. J Clin Oncol; 2009 May 20;27(15_suppl):e21509

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

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  • (PMID = 27963441.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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36. 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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37. Gebert C, Hardes J, Kersting C, August C, Supper H, Winkelmann W, Buerger H, Gosheger G: Expression of beta-catenin and p53 are prognostic factors in deep aggressive fibromatosis. Histopathology; 2007 Mar;50(4):491-7
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  • [Title] Expression of beta-catenin and p53 are prognostic factors in deep aggressive fibromatosis.
  • AIMS: To determine the prognostic significance of beta-catenin in aggressive fibromatosis and to identify potential molecular markers for new targeted therapies.
  • METHODS AND RESULTS: A tissue microarray of 37 cases of deep aggressive fibromatosis was constructed and subjected to immunohistochemical analysis for beta-catenin, p53, smooth muscle actin (SMA), desmin, Ki67, c-erbB2, epidermal growth factor receptor (EGFR), c-kit, CD34 and S100.
  • Nuclear beta-catenin expression was associated with an increased rate of local tumour recurrence (60.0% 1-year and 0% 5-year event-free survival; P < 0.05).
  • Furthermore, p53 expression was associated with an increased risk of tumour recurrence (50% 1-year event-free survival rate and 0% 5-years event-free survival rate, P < 0.05).
  • CONCLUSIONS: The overexpression of beta-catenin and p53 is associated with a decreased event-free survival in deep aggressive fibromatosis.
  • Further studies are required to establish whether these findings can lead to an improvement in the treatment of this rare neoplasm.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Tumor Suppressor Protein p53 / biosynthesis. beta Catenin / biosynthesis
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Humans. Infant. Middle Aged. Prognosis. Tissue Array Analysis


38. Shi B, Zhu Y, Xu Z, Liu Y, Zheng B, Qi T: Aggressive fibromatosis in the urological system. Report of two adult patients and review of the literature. Urol Int; 2007;78(1):93-6
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  • [Title] Aggressive fibromatosis in the urological system. Report of two adult patients and review of the literature.
  • Aggressive fibromatoses (AF) are locally aggressive neoplasms that do not metastasize but are frequently associated with one or more recurrences and subsequent associated morbidity.
  • AF in the urological system is quite rare and has mainly been described in single case reports or as isolated cases in a large series of extra-abdominal desmoid tumors.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Genital Neoplasms, Male / diagnosis. Scrotum. Urinary Bladder Neoplasms / diagnosis
  • [MeSH-minor] Adult. Cystoscopy. Diagnosis, Differential. Follow-Up Studies. Humans. Male. Tomography, X-Ray Computed. Urography. Urologic Surgical Procedures, Male / methods


39. Lee JC, Thomas JM, Phillips S, Fisher C, Moskovic E: Aggressive fibromatosis: MRI features with pathologic correlation. AJR Am J Roentgenol; 2006 Jan;186(1):247-54
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  • [Title] Aggressive fibromatosis: MRI features with pathologic correlation.
  • OBJECTIVE: We present the MRI features with pathologic correlation of aggressive fibromatosis, incorporating 203 cases over a 5-year period from the Royal Marsden Hospital Sarcoma Unit database.
  • MATERIALS AND METHODS: Sixty patients had imaging available for retrospective review of which 29 had preoperative MRI and final histopathologic diagnosis of aggressive fibromatosis.
  • RESULTS: The average age at diagnosis was 41.3 years with a female-to-male sex ratio of 1.2:1.
  • Twenty lesions were extraabdominal; six, intraabdominal; and three, in the abdominal wall (classic desmoid).
  • The average tumor size was 6.4 cm (range, 2.2-13.7 cm).
  • Intraabdominal aggressive fibromatosis produced the largest tumors, averaging 9.5 cm.
  • The lesions crossed major fascial boundaries in 31% of cases overall and in 66% of patients referred for recurrent disease.
  • Patients referred for recurrent disease were most likely to have a recurrence after surgery.
  • CONCLUSION: Accurate diagnosis and staging of aggressive fibromatosis by MRI have important treatment and prognostic implications.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Magnetic Resonance Imaging / methods
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Child. Female. Fibromatosis, Abdominal / pathology. Humans. Male. Middle Aged. Prognosis. Recurrence

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  • (PMID = 16357411.001).
  • [ISSN] 0361-803X
  • [Journal-full-title] AJR. American journal of roentgenology
  • [ISO-abbreviation] AJR Am J Roentgenol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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40. Stengel G, Metze D, Dörflinger B, Luger TA, Böhm M: Treatment of extra-abdominal aggressive fibromatosis with pegylated interferon. J Am Acad Dermatol; 2008 Aug;59(2 Suppl 1):S7-9
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  • [Title] Treatment of extra-abdominal aggressive fibromatosis with pegylated interferon.
  • Aggressive fibromatosis (desmoid tumor) is a very rare neoplasm arising from the musculoaponeurotic structures.
  • It is characterized by locally aggressive growth, and a tendency to relapse but not to metastasize.
  • We report on a young woman with a large desmoid tumor of the left foot.
  • Long-term immunointervention with pegylated interferon alfa-2b, however, led to marked clinical improvement of the patient's condition and a radiologically proven stabilization of the disease.
  • [MeSH-major] Antineoplastic Agents / administration & dosage. Fibromatosis, Aggressive / drug therapy. Foot. Interferon-alpha / administration & dosage. Soft Tissue Neoplasms / drug therapy
  • [MeSH-minor] Adult. Biopsy. Female. Humans. Neoplasm Recurrence, Local / drug therapy. Polyethylene Glycols. Recombinant Proteins. Severity of Illness Index

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  • (PMID = 18625396.001).
  • [ISSN] 1097-6787
  • [Journal-full-title] Journal of the American Academy of Dermatology
  • [ISO-abbreviation] J. Am. Acad. Dermatol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Interferon-alpha; 0 / Recombinant Proteins; 0 / peginterferon alfa-2b; 30IQX730WE / Polyethylene Glycols; 99210-65-8 / interferon alfa-2b
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41. Dufresne A, Bertucci F, Penel N, Le Cesne A, Bui B, Tubiana-Hulin M, Ray-Coquard I, Cupissol D, Chevreau C, Perol D, Goncalves A, Jimenez M, Bringuier PP, Blay JY: Identification of biological factors predictive of response to imatinib mesylate in aggressive fibromatosis. Br J Cancer; 2010 Aug 10;103(4):482-5
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  • [Title] Identification of biological factors predictive of response to imatinib mesylate in aggressive fibromatosis.
  • BACKGROUND: Imatinib induces responses and disease stabilisations in non-resectable patients with aggressive fibromatosis (AF).
  • Pre-treatment lymphopenia (<1500/microl) and tumour size >120 mm correlated with shorter PFS in univariate and multivariate analyses.
  • CONCLUSION: Our findings show that a baseline biological characteristic of the patient is the major parameter influencing response to imatinib in aggressive fibromatosis.
  • Tumour characteristics, including the presence of a KIT exon 10 M541L variant, may influence tumour control but this needs to be confirmed and better explained.
  • [MeSH-major] Biomarkers, Tumor / analysis. Fibromatosis, Aggressive / drug therapy. Proto-Oncogene Proteins c-kit / analysis


42. Roeder F, Timke C, Oertel S, Hensley FW, Bischof M, Muenter MW, Weitz J, Buchler MW, Lehner B, Debus J, Krempien R: Intraoperative electron radiotherapy for the management of aggressive fibromatosis. Int J Radiat Oncol Biol Phys; 2010 Mar 15;76(4):1154-60
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  • [Title] Intraoperative electron radiotherapy for the management of aggressive fibromatosis.
  • PURPOSE: We analyzed our experience with intraoperative electron radiotherapy (IOERT) followed by moderate doses of external beam radiotherapy (EBRT) after organ-sparing surgery in patients with primary or recurrent aggressive fibromatosis.
  • Median tumor size was 9 cm.
  • RESULTS: After a median follow-up of 32 months (range, 3-139 months), no disease-related deaths occurred.
  • CONCLUSION: Introduction of IOERT into a multimodal treatment approach in patients with aggressive fibromatosis is feasible with low toxicity and yielded good local control rates even in patients with microscopical or gross residual disease.
  • [MeSH-major] Electrons / therapeutic use. Fibromatosis, Aggressive / radiotherapy
  • [MeSH-minor] Adolescent. Adult. Feasibility Studies. Female. Humans. Intraoperative Period. Male. Middle Aged. Neoplasm Recurrence, Local / mortality. Neoplasm Recurrence, Local / surgery. Neoplasms, Multiple Primary / surgery. Radiotherapy Dosage. Salvage Therapy / methods. Survival Rate. Young Adult

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  • [Copyright] Copyright 2010 Elsevier Inc. All rights reserved.
  • (PMID = 19647952.001).
  • [ISSN] 1879-355X
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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43. 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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  • [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 / drug therapy. Fibromatosis, Aggressive / radionuclide imaging. Piperazines / therapeutic use. Positron-Emission Tomography. Pyrimidines / therapeutic use

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  • [CommentIn] Eur J Nucl Med Mol Imaging. 2011 Feb;38(2):406-7 [21052660.001]
  • (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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44. Castellazzi G, Vanel D, Le Cesne A, Le Pechoux C, Caillet H, Perona F, Bonvalot S: Can the MRI signal of aggressive fibromatosis be used to predict its behavior? Eur J Radiol; 2009 Feb;69(2):222-9
MedlinePlus Health Information. consumer health - MRI Scans.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Can the MRI signal of aggressive fibromatosis be used to predict its behavior?
  • PURPOSE: Aggressive fibromatosis is an invasive non-metastasizing soft-tissue tumor.
  • The aim of this study was to analyze and correlate the size and MR imaging signal features of aggressive fibromatosis with its behavior in order to choose the best treatment.
  • CONCLUSION: Fibromatoses are a group of soft-tissue tumors with variable characteristics on MRI, but it is not possible to predict their behavior based on the MRI signal.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Magnetic Resonance Imaging / methods. Soft Tissue Neoplasms / diagnosis

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  • (PMID = 19046842.001).
  • [ISSN] 1872-7727
  • [Journal-full-title] European journal of radiology
  • [ISO-abbreviation] Eur J Radiol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Ireland
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45. Zhu Z, Li F, Zhuang H, Yan J, Wu C, Cheng W: FDG PET/CT detection of intussusception caused by aggressive fibromatosis. Clin Nucl Med; 2010 May;35(5):370-3
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  • [Title] FDG PET/CT detection of intussusception caused by aggressive fibromatosis.
  • [MeSH-major] Fibromatosis, Aggressive / complications. Fluorodeoxyglucose F18. Intussusception / etiology. Intussusception / radionuclide imaging. Positron-Emission Tomography. Tomography, X-Ray Computed

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  • (PMID = 20395719.001).
  • [ISSN] 1536-0229
  • [Journal-full-title] Clinical nuclear medicine
  • [ISO-abbreviation] Clin Nucl Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0Z5B2CJX4D / Fluorodeoxyglucose F18
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46. Heinrich MC, McArthur GA, Demetri GD, Joensuu H, Bono P, Herrmann R, Hirte H, Cresta S, Koslin DB, Corless CL, Dirnhofer S, van Oosterom AT, Nikolova Z, Dimitrijevic S, Fletcher JA: Clinical and molecular studies of the effect of imatinib on advanced aggressive fibromatosis (desmoid tumor). J Clin Oncol; 2006 Mar 1;24(7):1195-203
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Clinical and molecular studies of the effect of imatinib on advanced aggressive fibromatosis (desmoid tumor).
  • PURPOSE: To determine the clinical efficacy of imatinib in patients with advanced aggressive fibromatosis (AF) and to identify the molecular basis of response/nonresponse to this agent.
  • Tumor specimens were analyzed for mutations of KIT, PDGFRA, PDGFRB, and CTNNB1 (beta-catenin).
  • Tumor expression of total and activated KIT, PDGFRA, and PDGFRB were assessed using immunohistochemistry and immunoblotting techniques.
  • RESULTS: Three of 19 patients (15.7%) had a partial response to treatment, with four additional patients having stable disease that lasted more than 1 year (overall 1 year tumor control rate of 36.8%).
  • AF tumors expressed minimal to null levels of KIT and PDGFRA but expressed levels of PDGFRB that are comparable with normal fibroblasts.
  • [MeSH-major] Antineoplastic Agents / pharmacology. Fibromatosis, Aggressive / drug therapy. Fibromatosis, Aggressive / genetics. Piperazines / pharmacology. Protein Kinase Inhibitors / pharmacology. Protein-Tyrosine Kinases / antagonists & inhibitors. Pyrimidines / pharmacology
  • [MeSH-minor] Adolescent. Adult. Benzamides. Clinical Trials, Phase II as Topic. Disease-Free Survival. Female. Gene Expression Regulation, Neoplastic / drug effects. Humans. Imatinib Mesylate. Immunoblotting. Immunohistochemistry. Male. Middle Aged. Mutation / drug effects. Platelet-Derived Growth Factor / metabolism. Positron-Emission Tomography. Proto-Oncogene Proteins c-kit / genetics. Proto-Oncogene Proteins c-sis. Receptor, Platelet-Derived Growth Factor alpha / genetics. Receptor, Platelet-Derived Growth Factor beta / genetics. Tomography, X-Ray Computed. beta Catenin / genetics

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  • [CommentIn] J Clin Oncol. 2006 Aug 1;24(22):3714-5; author reply 3715 [16877745.001]
  • (PMID = 16505440.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / 5P30 CA69533-04
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzamides; 0 / CTNNB1 protein, human; 0 / Piperazines; 0 / Platelet-Derived Growth Factor; 0 / Protein Kinase Inhibitors; 0 / Proto-Oncogene Proteins c-sis; 0 / Pyrimidines; 0 / beta Catenin; 0 / platelet-derived growth factor A; 0 / platelet-derived growth factor BB; 8A1O1M485B / Imatinib Mesylate; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit; EC 2.7.10.1 / Receptor, Platelet-Derived Growth Factor alpha; EC 2.7.10.1 / Receptor, Platelet-Derived Growth Factor beta
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47. Hu T, Jing G, Lv K: Aggressive fibromatosis in the maxilla. Br J Oral Maxillofac Surg; 2009 Mar;47(2):129-31
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Aggressive fibromatosis in the maxilla.
  • We present a rare case of recurrent aggressive fibromatosis of the maxilla in a 61-year-old woman, who was treated by resection of the left maxilla.
  • Adjuvant treatments, particularly radiotherapy, are valuable if the tumour recurs.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Maxillary Neoplasms / pathology
  • [MeSH-minor] Female. Humans. Middle Aged. Neoplasm Recurrence, Local / surgery

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  • (PMID = 18639958.001).
  • [ISSN] 1532-1940
  • [Journal-full-title] The British journal of oral & maxillofacial surgery
  • [ISO-abbreviation] Br J Oral Maxillofac Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Scotland
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48. 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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49. Meazza C, Bisogno G, Gronchi A, Fiore M, Cecchetto G, Alaggio R, Milano GM, Casanova M, Carli M, Ferrari A: Aggressive fibromatosis in children and adolescents: the Italian experience. Cancer; 2010 Jan 1;116(1):233-40
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Aggressive fibromatosis in children and adolescents: the Italian experience.
  • BACKGROUND: Aggressive fibromatosis (AF) is a rare tumor of intermediate malignancy that has a strong potential for local invasiveness and recurrence.
  • METHODS: The authors retrospectively analyzed 94 patients aged < or =21 years, including 23 patients who underwent complete surgery (Group I), 42 patients who underwent incomplete surgery with microscopic residual tumor (Group II), and 29 patients who underwent either biopsy or macroscopically incomplete surgery (Group III).
  • Two of 7 patients with abdominal disease died of tumor progression, whereas none of the patients with extra-abdominal AF died of their disease.
  • Systemic treatment was given to 15 patients as first-line treatment and to 34 patients at time the time they developed recurrent disease: The response rate was 47% in the former patients and 50% in the latter patients.
  • The completeness of initial resection was the main factor that influenced EFS, whereas disease control after marginal resection was much the same as that achieved after intralesional surgery/biopsy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Fibromatosis, Aggressive / drug therapy
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Female. Humans. Infant. Male. Methotrexate / administration & dosage. Neoplasm Recurrence, Local. Prognosis. Survival Rate. Vinblastine / administration & dosage. Vinblastine / analogs & derivatives

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  • [Copyright] Copyright 2010 American Cancer Society.
  • (PMID = 19950127.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 5V9KLZ54CY / Vinblastine; Q6C979R91Y / vinorelbine; YL5FZ2Y5U1 / Methotrexate
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50. Sun G, Xu M, Huang X: Treatment of aggressive fibromatosis of the head and neck. J Craniofac Surg; 2010 Nov;21(6):1831-3
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Treatment of aggressive fibromatosis of the head and neck.
  • OBJECTIVE: The purpose of this study was to assess the diagnosis, treatment, and prognosis of aggressive fibromatosis in the head and neck.
  • METHODS: This is a retrospective study of patients with aggressive fibromatosis who underwent surgical interventions during the 9-year period from 2001 to 2010.
  • Aggressive fibromatosis is treated by resection with an adequate safety margin.
  • Radiotherapy is given to patient with tumor that cannot be completely removed.
  • RESULTS: Four patients with aggressive fibromatosis were identified under the 2005 World Health Organization classification guidelines, which, here reported, have been surgically treated.
  • Then, they were all treated by aggressive local resection.
  • One patient who had a partially resected tumor underwent radiotherapy.
  • CONCLUSIONS: For the cases of tumor that can be completely removed, the prognosis is excellent.
  • Otherwise, the tumor is vulnerable to relapse.
  • We recommend radiotherapy in patients who have unresectable or partially resected tumor when the tumor is adjacent to vital structures.
  • Patients with aggressive fibromatosis should remain in long-term follow-up.
  • [MeSH-major] Fibromatosis, Aggressive / surgery. Head and Neck Neoplasms / surgery
  • [MeSH-minor] Adult. Aged. Female. Follow-Up Studies. Humans. Male. Middle Aged. Mouth Mucosa / surgery. Mouth Neoplasms / surgery. Neoadjuvant Therapy. Neoplasm Recurrence, Local / surgery. Neoplasm, Residual. Radiotherapy, Adjuvant. Retrospective Studies. Tongue Neoplasms / surgery. Treatment Outcome

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  • (PMID = 21119433.001).
  • [ISSN] 1536-3732
  • [Journal-full-title] The Journal of craniofacial surgery
  • [ISO-abbreviation] J Craniofac Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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51. Ferenc T, Stalińska L, Turant M, Sygut J, Tosik D, Dziki A, Kulig A: Analysis of TGF-beta protein expression in aggressive fibromatosis (desmoid tumor). Pol J Pathol; 2006;57(2):77-81
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Analysis of TGF-beta protein expression in aggressive fibromatosis (desmoid tumor).
  • Aggressive fibromatosis, usually termed desmoid tumor, develops from muscle connective tissue, fasciae and aponeuroses.
  • Aggressive fibromatosis located in various parts of the body demonstrates differentiated biological behavior.
  • Abnormalities in TGF-beta expression are very common in many disease processes, including neoplasms.
  • Immunohistochemical analysis employing a monoclonal antibody against TGF-beta was performed on archival material, consisting of 38 cases of aggressive fibromatosis, among which 23 represented abdominal, 11 extra-abdominal and 4 intra-abdominal localizations.
  • The average percentage of cells positively stained for TGF-beta protein was 40.2% in the group of extra-abdominal, 58.5% in the group of abdominal and 72.8% in the group of intra-abdominal localizations.
  • There were significant differences observed between the analyzed groups of desmoid tumor (p<0.05).
  • A positive cytoplasmic reaction for TGF-beta was noted in 65.8% (25/38) of the aggressive fibromatoses.
  • Overexpression of TGF-beta protein was noted in 39.5% (15/38) of the aggressive fibromatoses.
  • High expression noticed in desmoid fibroblasts might indicate that this protein plays a crucial role in the development of aggressive fibromatosis.
  • [MeSH-major] Abdominal Neoplasms / metabolism. Fibromatosis, Aggressive / metabolism. Transforming Growth Factor beta / metabolism
  • [MeSH-minor] Biomarkers, Tumor / metabolism. Cell Count. Cytoplasm / metabolism. Cytoplasm / pathology. Fibroblasts / metabolism. Fibroblasts / pathology. Humans. Image Processing, Computer-Assisted. Immunoenzyme Techniques

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  • (PMID = 17019969.001).
  • [ISSN] 1233-9687
  • [Journal-full-title] Polish journal of pathology : official journal of the Polish Society of Pathologists
  • [ISO-abbreviation] Pol J Pathol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Poland
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Transforming Growth Factor beta
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52. Lips DJ, Barker N, Clevers H, Hennipman A: The role of APC and beta-catenin in the aetiology of aggressive fibromatosis (desmoid tumors). Eur J Surg Oncol; 2009 Jan;35(1):3-10
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The role of APC and beta-catenin in the aetiology of aggressive fibromatosis (desmoid tumors).
  • BACKGROUND: Aggressive fibromatosis (syn. desmoid tumor) is a sporadically occurring neoplastic proliferation of fibroblasts originating from musculoaponeurotic planes, forming invasively growing masses without the capability to metastasize.
  • Better understanding of the aetiology of aggressive fibromatosis is needed to be able to develop new treatment strategies to cope with the high recurrence rates.
  • The following search terms were used: 'aggressive fibromatosis', 'desmoid tumor', 'adenomatous polyposis coli', 'APC', 'beta-catenin', 'Wnt', 'Wingless' and 'Wnt/Wingless'.
  • RESULTS: The neoplastic nature of aggressive fibromatosis and the role of the adenomatous polyposis coli (APC) and beta-catenin signaling cascade in driving the onset and progression of this disease are discussed.
  • CONCLUSION: Mutations in either the APC or beta-catenin genes are likely to be a major driving force in the formation of these desmoid tumors.
  • [MeSH-major] Adenomatous Polyposis Coli Protein / genetics. Fibromatosis, Aggressive / genetics. Fibromatosis, Aggressive / metabolism. Signal Transduction / genetics. beta Catenin / genetics

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  • (PMID = 18722078.001).
  • [ISSN] 1532-2157
  • [Journal-full-title] European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology
  • [ISO-abbreviation] Eur J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Adenomatous Polyposis Coli Protein; 0 / beta Catenin
  • [Number-of-references] 66
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53. Fen Li C, Kandel C, Baliko F, Nadesan P, Brünner N, Alman BA: Plasminogen activator inhibitor-1 (PAI-1) modifies the formation of aggressive fibromatosis (desmoid tumor). Oncogene; 2005 Feb 24;24(9):1615-24
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Plasminogen activator inhibitor-1 (PAI-1) modifies the formation of aggressive fibromatosis (desmoid tumor).
  • Aggressive fibromatosis is a mesenchymal neoplasm associated with mutations, resulting in beta-catenin-mediated transcriptional activation.
  • We found that plasminogen activator inhibitor-1 (PAI-1) was upregulated fourfold in aggressive fibromatosis.
  • To determine the role of PAI-1 in vivo, a mouse containing a targeted deletion in Pai-1 was crossed with a mouse that develops aggressive fibromatosis and gastrointestinal tumors (Apc/Apc1638N mouse).
  • Pai-1 deficiency reduced the number of aggressive fibromatosis tumors formed, but not the number of gastrointestinal tumors.
  • Deficiency of Pai-1 reduced tumor cell proliferation and motility rate.
  • Our study found that, although PAI-1 has cellular effects that could inhibit or enhance tumor growth, on balance, it acts as a tumor enhancer in aggressive fibromatosis.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Gene Expression Regulation, Neoplastic. Plasminogen Activator Inhibitor 1 / genetics


54. Sachs SA: Aggressive fibromatosis.. J Oral Maxillofac Surg; 2006 Aug;64(8):1325-6
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  • [Title] Aggressive fibromatosis..
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Fibromatosis, Aggressive / surgery. Mandibular Neoplasms / pathology. Mandibular Neoplasms / surgery

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  • (PMID = 16860240.001).
  • [ISSN] 0278-2391
  • [Journal-full-title] Journal of oral and maxillofacial surgery : official journal of the American Association of Oral and Maxillofacial Surgeons
  • [ISO-abbreviation] J. Oral Maxillofac. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] United States
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55. Tanaka H, Harasawa A, Furui S: Usefulness of MR imaging in assessment of tumor extent of aggressive fibromatosis. Radiat Med; 2005 Mar;23(2):111-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Usefulness of MR imaging in assessment of tumor extent of aggressive fibromatosis.
  • PURPOSE: We evaluated the usefulness of MR imaging for aggressive fibromatoses in order to determine tumor extent.
  • RESULTS: The tumor had a smooth margin in 41% of cases, while an irregular margin was present in 59%.
  • CONCLUSION: Detection of LSBs on MR images is important for the determination of tumor extent, which allows the surgical method to be decided and postoperative recurrence predicted.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Magnetic Resonance Imaging
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Contrast Media. Fascia / pathology. Female. Follow-Up Studies. Gadolinium DTPA. Humans. Image Enhancement / methods. Male. Middle Aged. Neoplasm Invasiveness. Retrospective Studies. Soft Tissue Neoplasms / diagnosis. Tendons / pathology

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  • (PMID = 15827528.001).
  • [ISSN] 0288-2043
  • [Journal-full-title] Radiation medicine
  • [ISO-abbreviation] Radiat Med
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Contrast Media; 84F6U3J2R6 / gadodiamide; K2I13DR72L / Gadolinium DTPA
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56. Indelicato DJ, Keole SR, Shahlaee AH, Morris CG, Gibbs CP, Scarborough MT, Islam S, Marcus RB: Ewing tumors of the chest wall: Local control and long-term outcomes. J Clin Oncol; 2009 May 20;27(15_suppl):e21501

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

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  • (PMID = 27963390.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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57. Watzinger F, Turhani D, Wutzl A, Fock N, Sinko K, Sulzbacher I: Aggressive fibromatosis of the mandible: a case report. Int J Oral Maxillofac Surg; 2005 Mar;34(2):211-3
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  • [Title] Aggressive fibromatosis of the mandible: a case report.
  • An extensive tumour in a 7-year-old girl, leading to severe disfigurement, proved to be an aggressive fibromatosis on histological examination.
  • Eighteen months after surgery there was no evidence of recurrent disease.
  • This suggests that tumour resection and reconstruction of the mandible had been successful.
  • Contrary to some reports, tumour resection led to curative therapy whereas radiotherapy failed.
  • [MeSH-major] Fibromatosis, Aggressive / surgery. Mandibular Neoplasms / surgery

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  • (PMID = 15695054.001).
  • [ISSN] 0901-5027
  • [Journal-full-title] International journal of oral and maxillofacial surgery
  • [ISO-abbreviation] Int J Oral Maxillofac Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Denmark
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58. Ghidirim G, Mishin I, Gagauz I, Vozian M, Zastavnitsky G, Iakovleva I: Sporadic retroperitoneal aggressive fibromatosis: report of a case. Zentralbl Chir; 2010 Feb;135(1):79-82
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Sporadic retroperitoneal aggressive fibromatosis: report of a case.
  • We describe herein a case of sporadic retroperitoneal aggressive fibromatosis.
  • A 54-year-old man with a palpable abdominal mass was referred to our hospital for investigation and treatment.
  • Abdominal ultrasonography and computed tomography revealed a solid mass with relatively well-defined borders in the left abdominal retroperitoneum.
  • At surgery, a large tumor (14 x 13 x 11 cm) was found, arising from the retroperitoneal space and involving the wall of jejunum.
  • Histological examination of the resected specimen revealed the presence of changes consistent with aggressive fibromatosis.
  • Two years after surgery, the patient is without any signs of recurrent disease.
  • Although extremely rare, aggressive fibromatosis should be considered in the differential diagnosis of retroperitoneal masses.
  • [MeSH-major] Fibromatosis, Abdominal / surgery. Fibromatosis, Aggressive / surgery. Retroperitoneal Neoplasms / surgery
  • [MeSH-minor] Diagnosis, Differential. Follow-Up Studies. Humans. Male. Middle Aged. Tomography, X-Ray Computed

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  • [Copyright] Georg Thieme Verlag Stuttgart, New York.
  • (PMID = 19424942.001).
  • [ISSN] 1438-9592
  • [Journal-full-title] Zentralblatt für Chirurgie
  • [ISO-abbreviation] Zentralbl Chir
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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59. Chattopadhyay A, Biswas SK, Dutta M: Pedicled omental split skin graft: A novel method for reconstruction of full-thickness abdominal wall defect. J Indian Assoc Pediatr Surg; 2010 Oct;15(4):142-4

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pedicled omental split skin graft: A novel method for reconstruction of full-thickness abdominal wall defect.
  • Although rare in children, aggressive fibromatosis or desmoid tumors require wide surgical excision for durable relief.
  • The authors report reconstruction of such a wound using a pedicled omental split skin graft, which resulted from the excision of a locally recurrent desmoid tumor.

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  • (PMID = 21170199.001).
  • [ISSN] 1998-3891
  • [Journal-full-title] Journal of Indian Association of Pediatric Surgeons
  • [ISO-abbreviation] J Indian Assoc Pediatr Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] India
  • [Other-IDs] NLM/ PMC2995941
  • [Keywords] NOTNLM ; Abdominal wall defect / aggressive fibromatosis / desmoid tumor / pedicled omental split skin graft
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60. Ferenc T, Sygut J, Kopczyński J, Mayer M, Latos-Bieleńska A, Dziki A, Kulig A: Aggressive fibromatosis (desmoid tumors): definition, occurrence, pathology, diagnostic problems, clinical behavior, genetic background. Pol J Pathol; 2006;57(1):5-15
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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 (desmoid tumors): definition, occurrence, pathology, diagnostic problems, clinical behavior, genetic background.
  • Aggressive fibromatosis, usually called desmoid tumor develops from muscle connective tissue, fasciae and aponeuroses.
  • This neoplasm is composed of spindle (fibrocyte-like) cells.
  • As regards the site, aggressive fibromatoses can be divided into: extra-abdominal in the area of the shoulder and pelvic girdle or chest and neck wall; abdominal in abdominal wall muscles; intra-abdominal concerning pelvis, mesentery connective tissue or retroperitoneal space.
  • Desmoid tumor is a neoplasm which rarely turns malignant and is non-metastasizing but demonstrates ability to local infiltration into tissue and is characterized by high risk of recurrence (25-65%) after surgical treatment.
  • Desmoid tumor etiology is uncertain.
  • This neoplasm occurs in sporadic (idiopathic) form and is also associated with some familial neoplastic syndromes.
  • Most sporadic cases of aggressive fibromatosis contain a somatic mutation in either the adenomatous polyposis coli (APC) or beta-catenin genes.
  • Sporadic tumors are more frequent in women than in men from 2 : 1 to 5 : 1.
  • In about 10-15 per cent of patients with familial adenomatous polyposis (FAP), aggressive fibromatosis is a parenteral manifestation of this familial syndrome conditioned by APC gene mutation.
  • Abdomen injury--most frequently due to surgery is said to play an important role in the initiation of fibrous tissue proliferative process in the cases of abdominal and intra abdominal forms.
  • High cells growth potential with relatively high local malignancy is observed in about 10% of cases with sporadic tumors as well as in those FAP-associated.
  • [MeSH-major] Fibromatosis, Aggressive. Mesoderm / pathology

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  • (PMID = 16739877.001).
  • [ISSN] 1233-9687
  • [Journal-full-title] Polish journal of pathology : official journal of the Polish Society of Pathologists
  • [ISO-abbreviation] Pol J Pathol
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Poland
  • [Number-of-references] 93
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61. Ferenc T, Sygut J, Tosik D, Kopczyński J, Sidor M, Góźdź S, Kulig A, Dziki A, Turant M, Stalińska L: Analysis of p27KIP1 protein and Ki-67 expression in aggressive fibromatosis (desmoid tumor). Pol J Pathol; 2006;57(4):187-91
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Analysis of p27KIP1 protein and Ki-67 expression in aggressive fibromatosis (desmoid tumor).
  • Aggressive fibromatosis (desmoid tumor) is an uncommon locally invasive non-metastasizing neoplasm lesion.
  • Desmoid tumor consists of fibroblasts, miofibroblasts and a significant amount of extracellular matrix. p27KIP1 (p27) protein is a member of the universal cyclin-dependent kinase inhibitor (CDKI) family that regulates progression through the cell cycle.
  • There were analysed 42 specimens of aggressive fibromatosis, in which there were 24 abdominal and 18 extra-abdominal cases.
  • There was no statistically significant difference between Ki-67 or p27 expression in abdominal and extra-abdominal location.
  • Analysis of p27 and Ki-67 expression levels might indicate that low proliferating activity of desmoid fibroblasts is connected with another mechanism than the one, in which p27 takes part.
  • [MeSH-major] Cyclin-Dependent Kinase Inhibitor p27 / metabolism. Fibromatosis, Abdominal / metabolism. Ki-67 Antigen / metabolism

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  • (PMID = 17285761.001).
  • [ISSN] 1233-9687
  • [Journal-full-title] Polish journal of pathology : official journal of the Polish Society of Pathologists
  • [ISO-abbreviation] Pol J Pathol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Poland
  • [Chemical-registry-number] 0 / Ki-67 Antigen; 147604-94-2 / Cyclin-Dependent Kinase Inhibitor p27
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62. Hara R, Matsuguma H, Suzuki H, Ishikawa Y, Nakahara R, Yamaguchi T, Hirabayashi K: Desmoid tumor presenting as a superior sulcus tumor: a unique bone change in the vertebral body. Ann Thorac Surg; 2007 Nov;84(5):1752-4
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  • [Title] Desmoid tumor presenting as a superior sulcus tumor: a unique bone change in the vertebral body.
  • Desmoid tumor, also referred to as aggressive fibromatosis, is a relatively rare, locally infiltrative, histologically benign tumor.
  • This report details a case of desmoid tumor presented as a superior sulcus tumor, which showed a unique manifestation in the vertebral bodies on computed tomography.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Lung Neoplasms / pathology. Spine / pathology

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  • (PMID = 17954107.001).
  • [ISSN] 1552-6259
  • [Journal-full-title] The Annals of thoracic surgery
  • [ISO-abbreviation] Ann. Thorac. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
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63. Kiverniti E, Cilasun U, Singh A, Kazi R, Clarke PM, Archer DJ: Aggressive fibromatosis of the oropharynx: a multidisciplinary approach to a benign disease. Ear Nose Throat J; 2009 May;88(5):930-4
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  • [Title] Aggressive fibromatosis of the oropharynx: a multidisciplinary approach to a benign disease.
  • We present the case of a 23-year-old woman with aggressive fibromatosis of the oropharynx that was initially treated elsewhere as a peritonsillar abscess.
  • We discuss the characteristics of this rare tumor and review the literature, stressing the importance of postoperative follow-up for peritonsillar abscesses to avoid missing other important diagnoses, such as the one described here.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Oropharynx / pathology. Palatal Neoplasms / pathology
  • [MeSH-minor] Adult. Female. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Peritonsillar Abscess / diagnosis. Treatment Outcome

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  • (PMID = 19444791.001).
  • [ISSN] 1942-7522
  • [Journal-full-title] Ear, nose, & throat journal
  • [ISO-abbreviation] Ear Nose Throat J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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64. Gallucci GL, Boretto JG, De Carli P: Desmoid tumor of the forearm. Reconstructive surgery and functional result. Chir Main; 2009 Oct;28(5):326-9
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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 of the forearm. Reconstructive surgery and functional result.
  • Desmoid tumors, also known as aggressive fibromatosis, are benign locally aggressive tumors with a high rate of recurrence.
  • Achieving margins of normal tissue around an upper extremity lesion without creating significant functional compromise is frequently difficult.
  • Therefore, functional reconstructive surgery is important, considering that for most patients treated for these tumors, the life expectancy is high and considering also that wide resection can affect the function and the aesthetics as well.
  • We present a case of aggressive fibromatosis in the proximal third of the forearm treated by wide resection and reconstructive surgery in one single procedure, with an acceptable functional result with no evidence of recurrence at 3 years of follow-up.
  • [MeSH-major] Fibromatosis, Aggressive / surgery. Forearm. Soft Tissue Neoplasms / surgery

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  • (PMID = 19766035.001).
  • [ISSN] 1769-6666
  • [Journal-full-title] Chirurgie de la main
  • [ISO-abbreviation] Chir Main
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] France
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65. Jeblaoui Y, Bouguila J, Haddad S, Helali M, Zaïri I, Zitouni K, Mokhtar M, Adouani A: [Mandibular aggressive fibromatosis]. Rev Stomatol Chir Maxillofac; 2007 Apr;108(2):153-5
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  • [Title] [Mandibular aggressive fibromatosis].
  • [Transliterated title] Fibromatose agressive mandibulaire.
  • INTRODUCTION: Aggressive fibromatosis is a rare histologically benign fibrous tumor with a potential for locoregional aggression.
  • Surgical treatment is the reference, chemotherapy, radiotherapy and homonotherapy being proposed as complementary treatment or for inoperable tumors.
  • CASE REPORT: A three-year-old patient underwent surgery for removal of a mandibular tumor.
  • Pathology reported aggressive fibromatosis.
  • DISCUSSION: Conservative surgery for aggressive mandibular fibromatosis appears to be preferable to radical mutilating surgery which would have a major impact on facial growth in children.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Mandibular Neoplasms / pathology

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  • (PMID = 17350660.001).
  • [ISSN] 0035-1768
  • [Journal-full-title] Revue de stomatologie et de chirurgie maxillo-faciale
  • [ISO-abbreviation] Rev Stomatol Chir Maxillofac
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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66. Stalinska L, Turant M, Tosik D, Sygut J, Kulig A, Kopczynski J, Dziki A, Ferenc T: Analysis of pRb, p16INK4A proteins and proliferating antigens: PCNA, Ki-67 and MCM5 expression in aggressive fibromatosis (desmoid tumor). Histol Histopathol; 2009 03;24(3):299-308
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  • [Title] Analysis of pRb, p16INK4A proteins and proliferating antigens: PCNA, Ki-67 and MCM5 expression in aggressive fibromatosis (desmoid tumor).
  • Aggressive fibromatosis (desmoid tumor) is a mesenchymal lesion originating from fascial, aponeurotic and muscular connective tissue.
  • In this study we analyzed the cell cycle regulation proteins: pRb, p16, and proliferating antigens: Ki-67, PCNA, MCM5 with immunohistochemical method in archival material derived from 27 extra-abdominal (E-AD), 18 abdominal (AD) and 5 intra-abdominal (I-AD) cases of desmoid tumor.
  • None of the examined cases (n=50) of aggressive fibromatosis was pRb-immunonegative.
  • The noted levels of pRb and p16 expression in desmoid cells reflect their function in cell cycle regulation.
  • Probably the unsettled cell cycle progression, especially in G1 phase, is not the cause of aggressive fibromatosis pathogenesis.
  • [MeSH-major] Cell Cycle Proteins / analysis. Cyclin-Dependent Kinase Inhibitor p16 / analysis. Fibromatosis, Abdominal / metabolism. Ki-67 Antigen / analysis. Proliferating Cell Nuclear Antigen / analysis. Retinoblastoma Protein / analysis

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  • (PMID = 19130399.001).
  • [ISSN] 1699-5848
  • [Journal-full-title] Histology and histopathology
  • [ISO-abbreviation] Histol. Histopathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Spain
  • [Chemical-registry-number] 0 / Cell Cycle Proteins; 0 / Cyclin-Dependent Kinase Inhibitor p16; 0 / Ki-67 Antigen; 0 / MCM5 protein, human; 0 / Proliferating Cell Nuclear Antigen; 0 / Retinoblastoma Protein
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67. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Intra-abdominal desmoid tumor].
  • [Transliterated title] Tumor desmoide intraabdominal.
  • Aggressive fibromatosis (desmoid tumor) are rare connective tissue tumors that occur sporadically or in association with familial adenomatous polyposis.
  • Biopsy is required to establish the diagnosis.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Fibromatosis, Aggressive / surgery. Retroperitoneal Neoplasms / pathology. Retroperitoneal Neoplasms / surgery

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  • (PMID = 16420952.001).
  • [ISSN] 0009-739X
  • [Journal-full-title] Cirugía española
  • [ISO-abbreviation] Cir Esp
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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68. Harish K: Recurrent large thoracic desmoid. Singapore Med J; 2008 Nov;49(11):e322-4

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Recurrent large thoracic desmoid.
  • Desmoid tumours, also termed aggressive fibromatosis, are rare soft tissue neoplasms derived from fascial or musculo-aponeurotic structures.
  • These tumours are histologically benign, but may behave aggressively.
  • We report a 37-year-old man with a large recurrent thoracic desmoid which attained a very large size.
  • [MeSH-major] Fibromatosis, Aggressive / surgery. Thoracic Neoplasms / surgery
  • [MeSH-minor] Adult. Fatal Outcome. Humans. Male. Recurrence. Soft Tissue Neoplasms / diagnosis. Soft Tissue Neoplasms / pathology. Soft Tissue Neoplasms / surgery. Thorax / pathology. Treatment Outcome

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  • (PMID = 19037541.001).
  • [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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69. Röpke M, Kalinski T, Wördehoff H, Aumann V, Bürger T: [Multicentric extra-abdominal fibromatosis: a rare case]. Z Orthop Ihre Grenzgeb; 2006 Mar-Apr;144(2):223-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Multicentric extra-abdominal fibromatosis: a rare case].
  • [Transliterated title] Multizentrische extraabdominale Fibromatose -- ein seltener Fall.
  • Extra-abdominal aggressive fibromatosis is a benign fibroblastic neoplasia with an infiltrative nature and a high tendency of local recurrence.
  • Here, we report on a very rare case of multicentric fibromatosis.
  • Low complaints led to considerable size of the tumours.
  • [MeSH-major] Fibroma / diagnosis. Fibroma / surgery. Leg / surgery. Limb Salvage. Soft Tissue Neoplasms / diagnosis. Soft Tissue Neoplasms / surgery
  • [MeSH-minor] Abdomen. Adult. Chemotherapy, Adjuvant. Combined Modality Therapy. Female. Humans. Radiotherapy, Adjuvant. Rare Diseases / diagnosis. Rare Diseases / surgery. Treatment Outcome

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  • (PMID = 16625455.001).
  • [ISSN] 0044-3220
  • [Journal-full-title] Zeitschrift für Orthopädie und ihre Grenzgebiete
  • [ISO-abbreviation] Z Orthop Ihre Grenzgeb
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Germany
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70. Oda D: Soft-tissue lesions in children. Oral Maxillofac Surg Clin North Am; 2005 Nov;17(4):383-402

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Soft-tissue lesions in children.
  • This article reviews some of the benign and malignant oral soft-tissue swellings that occur in children, with an emphasis on their clinical presentation, etiology, histopathology, and treatment.
  • These lesions include single and multiple nodules, reactive lesions, and benign and malignant neoplasms.
  • Diseases discussed include reactive gingival swelling, generalized gingival fibromatosis, melanotic neuroectodermal tumor of infancy, fibromas, vascular lesions, salivary gland lesions, and infantile rhabdomyomas.
  • Also covered are lesions that may present in multiples, such as neuromas, multiple endocrine neoplasia type 2b, neurofibromatosis, and human papilloma virus-related benign epithelial lesions.
  • Benign but locally aggressive and malignant neoplasms are discussed, such as aggressive fibromatosis, myofibromatosis, fibrosarcoma, and rhabdomyosarcoma.

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  • (PMID = 18088794.001).
  • [ISSN] 1042-3699
  • [Journal-full-title] Oral and maxillofacial surgery clinics of North America
  • [ISO-abbreviation] Oral Maxillofac Surg Clin North Am
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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71. Knechtel G, Stoeger H, Szkandera J, Dorr K, Beham A, Samonigg H: Desmoid tumor treated with polychemotherapy followed by imatinib: a case report and review of the literature. Case Rep Oncol; 2010;3(2):287-93

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Desmoid tumor treated with polychemotherapy followed by imatinib: a case report and review of the literature.
  • Desmoid tumors, also known as aggressive fibromatosis, are tumors of intermediate dignity, which grow slowly but are locally aggressive.
  • These tumors do not metastasize but can be potentially life threatening when infiltrating vital structures.
  • We report on a 40-year-old male patient with advanced fibromatosis of the neck who has been treated with 7 cycles of polychemotherapy (adriablastin, ifosfamide and dacarbazine) followed by targeted therapy with imatinib.
  • Tumor response was evaluated clinically and by magnetic resonance imaging.
  • The tumor decreased significantly after the first cycle of chemotherapy and tumor-related symptoms declined.
  • The best treatment for this rare tumor remains under discussion.
  • We included ifosfamide in our therapy, which is standard in the treatment of soft tissue tumors.
  • We conclude that combination chemotherapy including doxorubicin, ifosfamide and dacarbazine in the treatment of aggressive fibromatosis should be considered for patients suffering from unresectable, advanced disease and clinical symptoms which require a rapid response to therapy.

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  • (PMID = 21347195.001).
  • [ISSN] 1662-6575
  • [Journal-full-title] Case reports in oncology
  • [ISO-abbreviation] Case Rep Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
  • [Other-IDs] NLM/ PMC3042021
  • [Keywords] NOTNLM ; Aggressive fibromatosis / Chemotherapy / Desmoid tumor / Imatinib
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72. Huang PW, Tzen CY: Prognostic factors in desmoid-type fibromatosis: a clinicopathological and immunohistochemical analysis of 46 cases. Pathology; 2010 Feb;42(2):147-50
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Prognostic factors in desmoid-type fibromatosis: a clinicopathological and immunohistochemical analysis of 46 cases.
  • AIMS: To determine risk factors for recurrence of desmoid-type fibromatosis (aggressive fibromatosis).
  • METHODS: Forty-six cases of desmoid-type fibromatosis in Taiwanese patients were analysed for an association between tumour recurrence and clinical features, pathology, and the presence of p53 protein and beta-catenin on immunohistochemical staining.
  • The only factor significantly associated with tumour recurrence was positive surgical margin (p = 0.035).
  • CONCLUSIONS: A positive surgical margin is a risk factor for recurrence of desmoid-type fibromatosis.
  • [MeSH-major] Fibromatosis, Abdominal / pathology. Fibromatosis, Aggressive / pathology
  • [MeSH-minor] Adult. Biomarkers, Tumor / metabolism. Female. Humans. Immunohistochemistry. Male. Neoplasm Recurrence, Local. Prognosis. Risk Factors. Tumor Suppressor Protein p53 / metabolism. beta Catenin / metabolism

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  • (PMID = 20085516.001).
  • [ISSN] 1465-3931
  • [Journal-full-title] Pathology
  • [ISO-abbreviation] Pathology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / CTNNB1 protein, human; 0 / Tumor Suppressor Protein p53; 0 / beta Catenin
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73. Zampieri N, Cecchetto M, Zorzi MG, Pietrobelli A, Ottolenghi A, Camoglio F: An unusual case of extra-abdominal desmoid tumour. Eur J Cancer Care (Engl); 2010 May;19(3):410-2

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

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  • (PMID = 19709174.001).
  • [ISSN] 1365-2354
  • [Journal-full-title] European journal of cancer care
  • [ISO-abbreviation] Eur J Cancer Care (Engl)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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74. Chung KH, Charlton A, Arbuckle S, Chaseling R, Owler BK: Metachronous multifocal desmoid-type fibromatoses along the neuraxis with adenomatous polyposis syndrome. J Neurosurg Pediatr; 2010 Oct;6(4):372-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Metachronous multifocal desmoid-type fibromatoses along the neuraxis with adenomatous polyposis syndrome.
  • Desmoid-type fibromatosis, aggressive fibromatosis, or desmoid tumor is an uncommon benign but locally aggressive fibroblastic lesion.
  • Although intraabdominal desmoid-type fibromatoses are well described in association with adenomatous polyposis syndrome, their occurrence along the neuraxis is extremely rare.
  • The authors report the case of a 14-year-old boy with metachronous intracranial and spinal desmoid-type fibromatoses with preceding medulloblastoma.
  • This is the first reported case of spinal desmoid-type fibromatosis in association with adenomatous polyposis syndrome.
  • The identification of an underlying genetic instability allows for screening to detect lesions and institute measures to avoid preventable mortality from nonneurological tumors.
  • [MeSH-major] Adenomatous Polyposis Coli / pathology. Cerebral Ventricle Neoplasms / pathology. Fibromatosis, Aggressive / pathology. Genes, APC. Neoplasms, Multiple Primary / pathology

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  • (PMID = 20887112.001).
  • [ISSN] 1933-0715
  • [Journal-full-title] Journal of neurosurgery. Pediatrics
  • [ISO-abbreviation] J Neurosurg Pediatr
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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75. Engelhardt TO, Jeschke J, Piza-Katzer H: [About the self-reported quality of life after amputation of the hand in patients with upper extremity tumors]. Handchir Mikrochir Plast Chir; 2008 Feb;40(1):23-30
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [About the self-reported quality of life after amputation of the hand in patients with upper extremity tumors].
  • PURPOSE/BACKGROUND: Amputation proximal to the wrist may be oncologically dictated in cases of upper extremity soft tissue sarcoma and aggressive fibromatosis.
  • PATIENTS/METHODS: Between 1999 and 2007, 14 patients (6 females/8 males; average age: 25 years) were operated because of a soft tissue sarcoma (n = 11) or aggressive fibromatosis (n = 3).
  • Local recurrence following forequarter amputation due to aggressive fibromatosis required reoperation in another patient.
  • [MeSH-major] Amputation. Amputees / psychology. Arm. Artificial Limbs. Fibromatosis, Aggressive / surgery. Hand / surgery. Quality of Life. Sarcoma / surgery

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  • (PMID = 18322896.001).
  • [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] Case Reports; Comparative Study; English Abstract; Evaluation Studies; Journal Article
  • [Publication-country] Germany
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76. Huang CC, Ko SF, Yeh MC, Ng SH, Huang HY, Lee CC, Lee TY: Aggressive fibromatosis of the chest wall: sonographic appearance of the fascial tail and staghorn patterns. J Ultrasound Med; 2009 Mar;28(3):393-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Aggressive fibromatosis of the chest wall: sonographic appearance of the fascial tail and staghorn patterns.
  • [MeSH-major] Fibroma / ultrasonography. Thoracic Neoplasms / ultrasonography. Thoracic Wall / ultrasonography. Ultrasonography / methods

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  • (PMID = 19244079.001).
  • [ISSN] 1550-9613
  • [Journal-full-title] Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine
  • [ISO-abbreviation] J Ultrasound Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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77. Gandolfo L, Guglielmino S, Lorenzetti P, Fiducia G, Scenna G, Bosco V: [Chest wall fibromatosis after mammary prosthesis implantation. A case report and review of the literature]. Chir Ital; 2006 Sep-Oct;58(5):655-60
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Chest wall fibromatosis after mammary prosthesis implantation. A case report and review of the literature].
  • [Transliterated title] Fibromatosi del torace dopo impianto di protesi mammaria. Caso clinico e revisione della letteratura.
  • Desmoid tumour of the breast is a rare lesion.
  • Only 9 cases in which the origin of the tumour was linked to a breast implant had been published up to 2004.
  • We report a case of aggressive fibromatosis which developed on the thoracic wall two years after implantation of a mammary prosthesis.
  • This tumour was locally aggressive.
  • [MeSH-major] Breast Implantation / adverse effects. Breast Neoplasms / etiology. Fibromatosis, Aggressive / etiology. Silicones / adverse effects

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  • (PMID = 17069197.001).
  • [ISSN] 0009-4773
  • [Journal-full-title] Chirurgia italiana
  • [ISO-abbreviation] Chir Ital
  • [Language] ita
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Silicones
  • [Number-of-references] 25
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78. Kraft SM, Singh V, Sykes KJ, Gamis A, Manalang MA, Wei JL: Differentiating between congenital rhabdomyosarcoma versus fibromatosis of the pediatric tongue. Int J Pediatr Otorhinolaryngol; 2010 Jul;74(7):781-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Differentiating between congenital rhabdomyosarcoma versus fibromatosis of the pediatric tongue.
  • Fibromatosis of the tongue is also rare, and very difficult to distinguish from the spindle cell variant of rhabdomyosarcoma.
  • For fibromatosis, complete surgical excision is usually adequate without additional therapy, although some cases of aggressive fibromatosis also require chemotherapy.
  • [MeSH-major] Fibroma / pathology. Rhabdomyosarcoma / pathology. Tongue Neoplasms / pathology
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Diagnosis, Differential. Glossectomy. Humans. Immunohistochemistry. Infant. Magnetic Resonance Imaging. Male. Microscopy

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  • [Copyright] Copyright 2010 Elsevier Ireland Ltd. All rights reserved.
  • (PMID = 20435354.001).
  • [ISSN] 1872-8464
  • [Journal-full-title] International journal of pediatric otorhinolaryngology
  • [ISO-abbreviation] Int. J. Pediatr. Otorhinolaryngol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Ireland
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79. Eren S: A sporadic abdominal desmoid tumour case presenting with intermittent intestinal obstruction. Eur J Pediatr Surg; 2007 Aug;17(4):285-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A sporadic abdominal desmoid tumour case presenting with intermittent intestinal obstruction.
  • Desmoid tumours, also known as aggressive fibromatosis, are rare lesions with an intermediate biological behaviour between benign fibrous lesions and fibrosarcomas.
  • Although abdominal desmoids have an increased incidence in Gardner's syndrome, they are rarely found in an isolated form.
  • We report the findings of a barium study, ultrasound, computed tomography and magnetic resonance imaging in a nine-year-old boy with intermittent nausea and vomiting, diagnosed as having a desmoid tumour.
  • Although intra-abdominal desmoids are usually detected as a solitary lesion in sporadic cases, the presented case had two mesenteric lesions in the left upper quadrant.
  • [MeSH-major] Abdominal Neoplasms / complications. Digestive System Surgical Procedures / methods. Fibromatosis, Aggressive / complications. Intestinal Obstruction / etiology
  • [MeSH-minor] Child. Diagnosis, Differential. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Male. Tomography, X-Ray Computed

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  • (PMID = 17806029.001).
  • [ISSN] 0939-7248
  • [Journal-full-title] European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift für Kinderchirurgie
  • [ISO-abbreviation] Eur J Pediatr Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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80. Karadeniz T, Baran C, Topsakal M: Giant desmoid tumor in a case of ileal neobladder. Can J Urol; 2010 Feb;17(1):5038-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Giant desmoid tumor in a case of ileal neobladder.
  • Aggressive fibromatosis or desmoid tumor, is a histologically benign entity with unknown etiology that may present a serious clinical course.
  • Due to its high tendency to recur and local aggressive behavior and as there is no established effective medical treatment, complete surgical excision remains the sole management.
  • To our knowledge, we describe the first case of giant desmoid tumor which arised from ileal neobladder mesenterium in a bladder cancer patient with orthotopic substitution.
  • [MeSH-major] Fibromatosis, Aggressive. Neoplasms, Second Primary. Urinary Reservoirs, Continent

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  • (PMID = 20156390.001).
  • [ISSN] 1195-9479
  • [Journal-full-title] The Canadian journal of urology
  • [ISO-abbreviation] Can J Urol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Canada
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81. Hosalkar HS, Fox EJ, Delaney T, Torbert JT, Ogilvie CM, Lackman RD: Desmoid tumors and current status of management. Orthop Clin North Am; 2006 Jan;37(1):53-63

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

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  • (PMID = 16311111.001).
  • [ISSN] 0030-5898
  • [Journal-full-title] The Orthopedic clinics of North America
  • [ISO-abbreviation] Orthop. Clin. North Am.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 51
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82. Duffaud F, Le Cesne A: Imatinib in the treatment of solid tumours. Target Oncol; 2009 Jan;4(1):45-56
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Imatinib in the treatment of solid tumours.
  • The extraordinary success of imatinib in gastrointestinal stromal tumors (GIST) represents a model for molecularly targeted therapy for other solid tumors.
  • In this article, we review recent advances in the clinical management of patients with GISTs treated with imatinib, but also of patients with dermatofibrosarcoma protuberans, chordoma, aggressive fibromatosis, and some other common solid tumors treated with this drug.
  • We reviewed the knowledge of the molecular mechanisms that are basic to imatinib effects in these tumors.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Chordoma / drug therapy. Dermatofibrosarcoma / drug therapy. Gastrointestinal Stromal Tumors / drug therapy. Skin Neoplasms / drug therapy
  • [MeSH-minor] Adjuvants, Immunologic / administration & dosage. Benzamides. Clinical Trials as Topic. Fibromatosis, Aggressive / drug therapy. Fibromatosis, Aggressive / pathology. Fusion Proteins, bcr-abl / antagonists & inhibitors. Fusion Proteins, bcr-abl / genetics. Humans. Imatinib Mesylate. Mutation. Piperazines / administration & dosage. Piperazines / pharmacology. Protein-Tyrosine Kinases / antagonists & inhibitors. Proto-Oncogene Proteins c-kit / genetics. Proto-Oncogene Proteins c-kit / metabolism. Pyrimidines / administration & dosage. Pyrimidines / pharmacology. Receptor, Macrophage Colony-Stimulating Factor / antagonists & inhibitors. Receptors, Platelet-Derived Growth Factor / antagonists & inhibitors. Receptors, Platelet-Derived Growth Factor / genetics. Treatment Outcome

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  • (PMID = 19343301.001).
  • [ISSN] 1776-260X
  • [Journal-full-title] Targeted oncology
  • [ISO-abbreviation] Target Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Adjuvants, Immunologic; 0 / Benzamides; 0 / Piperazines; 0 / Pyrimidines; 0 / abl-bcr fusion protein, human; 8A1O1M485B / Imatinib Mesylate; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit; EC 2.7.10.1 / Receptor, Macrophage Colony-Stimulating Factor; EC 2.7.10.1 / Receptors, Platelet-Derived Growth Factor; EC 2.7.10.2 / Fusion Proteins, bcr-abl
  • [Number-of-references] 146
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83. Khanfir K, Zouhair A: Review: aggressive fibromatosis and breast implant. Clin Adv Hematol Oncol; 2008 Sep;6(9):693-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Review: aggressive fibromatosis and breast implant.
  • [MeSH-major] Breast Implants / adverse effects. Breast Neoplasms / pathology. Fibromatosis, Aggressive / pathology

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  • [CommentOn] Clin Adv Hematol Oncol. 2008 Sep;6(9):687-94 [18827791.001]
  • (PMID = 18827792.001).
  • [ISSN] 1543-0790
  • [Journal-full-title] Clinical advances in hematology & oncology : H&O
  • [ISO-abbreviation] Clin Adv Hematol Oncol
  • [Language] eng
  • [Publication-type] Comment; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Anti-Inflammatory Agents; 0 / Antineoplastic Agents
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84. Callahan KS, Eberhardt SC, Fechner RE, Cerilli LA: Desmoplastic fibroma of bone with extensive cartilaginous metaplasia. Ann Diagn Pathol; 2006 Dec;10(6):343-6
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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 with extensive cartilaginous metaplasia.
  • Desmoplastic fibroma of bone is a rare tumor demonstrating the same histologic and biologic features of its soft tissue counterpart, aggressive fibromatosis.
  • We report the second case of desmoplastic fibroma of bone with extensive chondroid metaplasia.
  • The tumor arose in the left ischium of a 51-year-old male, with extension into adjacent musculature as a pseudoencapsulated mass.
  • The infiltrating growth and quality of the fibrous component are characteristic of desmoplastic fibroma, and in addition, abrupt transitions into bland hyalin cartilage were frequent.
  • Discriminating features of this lesion from other bone tumors capable of biphasic expression of fibrous and chondroid elements are discussed.
  • [MeSH-major] Bone Neoplasms / pathology. Cartilage / pathology. Fibroma, Desmoplastic / pathology. Metaplasia / pathology

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  • (PMID = 17126252.001).
  • [ISSN] 1092-9134
  • [Journal-full-title] Annals of diagnostic pathology
  • [ISO-abbreviation] Ann Diagn Pathol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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85. Morris LG, Sikora AG, Kuriakose MA, DeLacure MD: Tamoxifen therapy for aggressive fibromatosis of the posterior triangle of the neck. Otolaryngol Head Neck Surg; 2007 Apr;136(4):674-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Tamoxifen therapy for aggressive fibromatosis of the posterior triangle of the neck.
  • [MeSH-major] Antineoplastic Agents, Hormonal / therapeutic use. Fibromatosis, Aggressive / drug therapy. Head and Neck Neoplasms / drug therapy. Tamoxifen / therapeutic use

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  • (PMID = 17418276.001).
  • [ISSN] 0194-5998
  • [Journal-full-title] Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery
  • [ISO-abbreviation] Otolaryngol Head Neck Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 094ZI81Y45 / Tamoxifen
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86. Ray ME, Lawrence TS: Radiation therapy for aggressive fibromatosis (desmoid tumor). J Clin Oncol; 2006 Aug 1;24(22):3714-5; author reply 3715
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Radiation therapy for aggressive fibromatosis (desmoid tumor).
  • [MeSH-major] Fibromatosis, Aggressive / radiotherapy. Fibromatosis, Aggressive / surgery

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  • [CommentOn] J Clin Oncol. 2006 Mar 1;24(7):1195-203 [16505440.001]
  • (PMID = 16877745.001).
  • [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] Comment; Letter
  • [Publication-country] United States
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87. Sleijfer S: Management of aggressive fibromatosis: can we unravel the maze of treatment options? Eur J Cancer; 2009 Nov;45(17):2928-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Management of aggressive fibromatosis: can we unravel the maze of treatment options?
  • [MeSH-major] Fibromatosis, Aggressive / therapy
  • [MeSH-minor] Evidence-Based Medicine / methods. Humans. Neoplasm Proteins / metabolism. Prognosis. beta Catenin / metabolism

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  • [CommentOn] Eur J Cancer. 2009 Nov;45(17):2930-4 [19767198.001]
  • (PMID = 19804964.001).
  • [ISSN] 1879-0852
  • [Journal-full-title] European journal of cancer (Oxford, England : 1990)
  • [ISO-abbreviation] Eur. J. Cancer
  • [Language] eng
  • [Publication-type] Comment; Editorial
  • [Publication-country] England
  • [Chemical-registry-number] 0 / CTNNB1 protein, human; 0 / Neoplasm Proteins; 0 / beta Catenin
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88. Li CF, Wei RY, Baliko F, Bapat B, Alman BA: An association between the 4G polymorphism in the PAI-1 promoter and the development of aggressive fibromatosis (desmoid tumor) in familial adenomatous polyposis patients. Fam Cancer; 2007;6(1):89-95
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] An association between the 4G polymorphism in the PAI-1 promoter and the development of aggressive fibromatosis (desmoid tumor) in familial adenomatous polyposis patients.
  • Aggressive fibromatosis is a mesenchymal neoplasm associated with mutations resulting in beta-catenin mediated transcriptional activation.
  • Plasminogen activator inhibitor-1 (PAI-1) is expressed at a high level in aggressive fibromatosis, and using transgenic mice, we found that PAI-1 plays an important role in aggressive fibromatosis tumor formation.
  • Familial adenomatous polyposis is associated with Adenomatous Polyposis Coli gene mutations resulting in beta-catenin mediated transcriptional activation, yet only some patients develop aggressive fibromatosis.
  • Since PAI-1 expression is influenced by a promoter 4G/5G polymorphism, we investigated the incidence of this polymorphism in familial adenomatous polyposis patients who did and who did not develop aggressive fibromatosis, as well as sporadic aggressive fibromatosis patients.
  • There was a trend towards association of the 4G allele (associated with high PAI-1 expression) with the development of aggressive fibromatosis in familial adenomatous polyposis patients (50% vs. 19%, P = 0.1).
  • In familial adenomatous polyposis patients who did not develop aggressive fibromatosis, there was a significantly lower proportion of patients with a 4G allele compared to the healthy control (19% vs. 51%, P = 0.0286).
  • The lower incidence of 4G polymorphism in the PAI-1 promoter may be preventive against the development of aggressive fibromatosis.
  • This data provides additional evidence supporting an important role for PAI-1 in the pathogenesis of aggressive fibromatosis.
  • [MeSH-major] Adenomatous Polyposis Coli / genetics. Fibromatosis, Aggressive / genetics. Gene Expression Regulation, Neoplastic. Plasminogen Activator Inhibitor 1 / genetics. Polymorphism, Genetic. Promoter Regions, Genetic / genetics
  • [MeSH-minor] Adult. Causality. Comorbidity. Genetic Predisposition to Disease. Humans. Incidence. Severity of Illness Index. Transcriptional Activation


89. Catalano F, Furci M, Fancello R, Costanzo M: Giant recurrent fibromatosis of the breast: a case report. Clinical features and implications for treatment. Chir Ital; 2006 Jul-Aug;58(4):538-43
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Giant recurrent fibromatosis of the breast: a case report. Clinical features and implications for treatment.
  • Fibromatosis of the breast is a benign but locally aggressive neoplasm, which has been described under various names including extra-abdominal desmoid tumour and aggressive fibromatosis.
  • It is a rare condition, accounting for approximately 0.2% of all solid tumours of the breast.
  • Clinically and radiologically the lesion mimics breast cancer and the definitive diagnosis is provided by histology.
  • A high risk of local recurrence is a characteristic of this disease.
  • Radical excision of the tumour with clear histological margins decreases the likelihood of recurrence.
  • The possible association between breast implants and mammary fibromatosis has been reported.
  • In this study we report a case of giant fibromatosis of the breast observed in a young patient and its extensive recurrence, involving all quadrants of the breast, the pectoralis major muscle, the rectus abdominis muscle sheath and the costal layer.
  • The main clinical and pathological characteristics of this rare disease are illustrated, emphasizing the difficulties encountered both in the diagnosis of the primary lesion and in the management of its giant recurrence, particularly as regards the possibility of obtaining a radical surgical excision and the option of performing breast reconstruction.
  • [MeSH-major] Breast Neoplasms / diagnosis. Breast Neoplasms / surgery. Fibromatosis, Aggressive / diagnosis. Fibromatosis, Aggressive / surgery
  • [MeSH-minor] Adult. Female. Humans. Mastectomy. Neoplasm Recurrence, Local / diagnosis. Neoplasm Recurrence, Local / surgery. Pectoralis Muscles / pathology. Pectoralis Muscles / surgery. Rectus Abdominis / pathology. Rectus Abdominis / surgery. Treatment Outcome

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  • (PMID = 16999161.001).
  • [ISSN] 0009-4773
  • [Journal-full-title] Chirurgia italiana
  • [ISO-abbreviation] Chir Ital
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
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90. Seper L, Bürger H, Vormoor J, Joos U, Kleinheinz J: Agressive fibromatosis involving the mandible--case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod; 2005 Jan;99(1):30-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Agressive fibromatosis involving the mandible--case report and review of the literature.
  • BACKGROUND: Aggressive fibromatosis (AF) involving the mandible is rare, and surgery is often complicated by a high recurrence rate.
  • Excisional biopsy revealed AF, and local excision of the tumor was performed.
  • The tumor was determined to be unresectable and the boy was treated with low-dose chemotherapy including methotrexate and vinblastine for 1 year.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Mandibular Neoplasms / pathology
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child, Preschool. Diagnosis, Differential. Follow-Up Studies. Humans. Male. Neoplasm Recurrence, Local / pathology

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  • [ErratumIn] Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2005 Feb;99(2):254
  • (PMID = 15599346.001).
  • [ISSN] 1079-2104
  • [Journal-full-title] Oral surgery, oral medicine, oral pathology, oral radiology, and endodontics
  • [ISO-abbreviation] Oral Surg Oral Med Oral Pathol Oral Radiol Endod
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 52
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91. Cassier PA, Dufresne A, El Sayadi H, Pissaloux D, Alberti L, Decouvelaere AV, Ranchere D, Ray-Coquard I, Blay JY: [Targeted therapy of sarcomas]. Bull Cancer; 2008 Oct;95(10):963-74
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  • Soft tissue sarcomas can be divided into 6 sub-types based on the underlying molecular biology of the disease:.
  • 2) tyrosine kinase receptor mutations (gastrointestinal stromal tumors);.
  • 3) tumor-suppressor gene deletion (type 1 neurofibromatosis, rhabdoid tumors);.
  • 5) sarcomas with more complex genetic alterations (leiomyosarcoma) and 6) abnormalities involving the cell-adhesion pathways (aggressive fibromatosis).
  • Together with the current development of numerous targeted therapies, these recent progress are the basis of tomorrow's personalised medicine for patients with soft tissue sarcoma.
  • [MeSH-major] Gastrointestinal Stromal Tumors / drug therapy. Sarcoma / drug therapy

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  • (PMID = 19004727.001).
  • [ISSN] 1769-6917
  • [Journal-full-title] Bulletin du cancer
  • [ISO-abbreviation] Bull Cancer
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 0 / Antineoplastic Agents; 0 / Protein Kinase Inhibitors
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92. Ayadi-Kaddour A, Ben Slama S, Braham E, Abid L, Ismail O, Smati B, Djilani H, El Mezni F: [Desmoplastic fibroma of the rib: two case reports]. Ann Pathol; 2005 Oct;25(5):398-401
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  • [Title] [Desmoplastic fibroma of the rib: two case reports].
  • [Transliterated title] Le fibrome desmoplastique de la côte: à propos de deux observations.
  • Desmoplastic fibroma is a very rare primary tumor of bone, closely related to aggressive fibromatosis of soft tissue.
  • Although considered a benign lesion, it can be very aggressive locally and has a high rate of local recurrence after incomplete surgical excision.
  • Rib involvement by desmoplastic fibroma is extremely rare.
  • We present two cases of desmoplastic fibroma involving this unusual location with lytic costal lesion and chest wall extension.
  • Histological examination after surgical resection revealed that the tumor consisted of spindle cells with small, elongated nuclei in a background of numerous collagen fibers and infiltrating lamellar bone.
  • We also discuss histological differential diagnosis as well as clinical features and the radiological and pathologic findings of this rare disease.
  • [MeSH-major] Bone Neoplasms / pathology. Fibroma, Desmoplastic / pathology. Ribs / pathology
  • [MeSH-minor] Biomarkers, Tumor / analysis. Collagen / analysis. Diagnosis, Differential. Female. Fibroblasts / pathology. Humans. Lung / pathology. Lung / surgery. Male. Middle Aged. Neoplasm Invasiveness. Neoplasm Proteins / analysis. Neoplasm Recurrence, Local / surgery. Pneumonectomy. Thoracic Wall / pathology

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  • (PMID = 16498294.001).
  • [ISSN] 0242-6498
  • [Journal-full-title] Annales de pathologie
  • [ISO-abbreviation] Ann Pathol
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Neoplasm Proteins; 9007-34-5 / Collagen
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93. Viriyaroj V, Yingsakmongkol N, Pasukdee P, Rermluk N: A large abdominal desmoid tumor associated with pregnancy. J Med Assoc Thai; 2009 Jun;92 Suppl 3:S72-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A large abdominal desmoid tumor associated with pregnancy.
  • Desmoid tumors are rare, benign tumors with locally aggressive behavior which originate from fascial or musculoaponeurotic structure.
  • The etiology of desmoid tumors are uncertain but may be related to operation, trauma or hormonal factors.
  • The authors report a 17-year old woman, gravida 1, para 1 with a mass at her lower abdominal wall during the fifth month of gestation.
  • She was biopsied during delivery in another hospital but was not given a definite diagnosis.
  • The tumor measured 28 x 21 x 18 centimeters in size and 4,900 g in weight.
  • Complete surgical excision was performed The pathological report was desmoid tumor (aggressive fibromatosis).
  • She had no post-operative complication and no recurrent tumor in the 8 months after operation.
  • [MeSH-major] Abdominal Neoplasms / diagnosis. Fibromatosis, Aggressive / diagnosis. Pregnancy Complications

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  • (PMID = 19702071.001).
  • [ISSN] 0125-2208
  • [Journal-full-title] Journal of the Medical Association of Thailand = Chotmaihet thangphaet
  • [ISO-abbreviation] J Med Assoc Thai
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Thailand
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94. Borzellino G, Minicozzi AM, Giovinazzo F, Faggian G, Iuzzolino P, Cordiano C: Intra-thoracic desmoid tumour in a patient with a previous aortocoronary bypass. World J Surg Oncol; 2006;4:43

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Intra-thoracic desmoid tumour in a patient with a previous aortocoronary bypass.
  • BACKGROUND: Intra-thoracic desmoid tumours with mediastinal invasion are very rare.
  • Although rare they have to be taken into account in the differential diagnosis of a thoracic mass and therapeutic options have to be weighted since surgical treatment may require wide excision.
  • The histological examination revealed aggressive fibromatosis.
  • CONCLUSION: Although technically demanding, radical surgical excision is actually the most indicated therapeutic approach for intra-thoracic desmoid tumours.

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  • [Cites] Eur J Cardiothorac Surg. 2001 Nov;20(5):1040-1 [11675201.001]
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  • [Cites] Am J Surg. 1986 Feb;151(2):230-7 [3946757.001]
  • (PMID = 16831227.001).
  • [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/ PMC1543629
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95. Skubitz KM, D'Adamo DR: Sarcoma. Mayo Clin Proc; 2007 Nov;82(11):1409-32
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  • They can be grouped into 2 general categories, soft tissue sarcoma and primary bone sarcoma, which have different staging and treatment approaches.
  • This review includes a discussion of both soft tissue sarcomas (malignant fibrous histiocytoma, liposarcoma, leiomyosarcoma, synovial sarcoma, dermatofibrosarcoma protuberans, angiosarcoma, Kaposi sarcoma, gastrointestinal stromal tumor, aggressive fibromatosis or desmoid tumor, rhabdomyosarcoma, and primary alveolar soft-part sarcoma) and primary bone sarcomas (osteosarcoma, Ewing sarcoma, giant cell tumor, and chondrosarcoma).
  • The 3 most important prognostic variables are grade, size, and location of the primary tumor.
  • The approach to a patient with a sarcoma begins with a biopsy that obtains adequate tissue for diagnosis without interfering with subsequent optimal definitive surgery.
  • Subsequent treatment depends on the specific type of sarcoma.
  • [MeSH-major] Bone Neoplasms / therapy. Sarcoma / therapy. Soft Tissue Neoplasms / therapy
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Chromosome Aberrations. Humans. Neoplasm Metastasis. Neoplasm Staging. Risk Factors

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  • (PMID = 17976362.001).
  • [ISSN] 0025-6196
  • [Journal-full-title] Mayo Clinic proceedings
  • [ISO-abbreviation] Mayo Clin. Proc.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 391
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96. Pickhardt PJ, Bhalla S: Unusual nonneoplastic peritoneal and subperitoneal conditions: CT findings. Radiographics; 2005 May-Jun;25(3):719-30
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Peritoneal disease can manifest at computed tomography (CT) as fluid accumulation within the peritoneal cavity (ascites) or soft-tissue infiltration of the various peritoneal ligaments and mesenteries.
  • For example, systemic or organ-based diseases that occasionally involve the peritoneum include eosinophilic gastroenteritis, amyloidosis, extramedullary hematopoiesis, Erdheim-Chester disease, sarcoidosis, and mesenteric cavitary lymph node syndrome.
  • Tumorlike conditions that may affect the peritoneum include aggressive fibromatosis (desmoid), inflammatory pseudotumor, retractile mesenteritis, and Castleman disease.
  • Atypical peritoneal infections include tuberculosis, actinomycosis, echinococcosis, Whipple disease, and mesenteric adenitis.

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  • (PMID = 15888621.001).
  • [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] 21
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97. Bonvalot S, Eldweny H, Haddad V, Rimareix F, Missenard G, Oberlin O, Vanel D, Terrier P, Blay JY, Le Cesne A, Le Péchoux C: Extra-abdominal primary fibromatosis: Aggressive management could be avoided in a subgroup of patients. Eur J Surg Oncol; 2008 Apr;34(4):462-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Extra-abdominal primary fibromatosis: Aggressive management could be avoided in a subgroup of patients.
  • OBJECTIVE: To evaluate the impact of surgery as first-line treatment on event-free survival (EFS) of primary aggressive fibromatosis.
  • Eighty-nine patients had initial resection of their primary tumour followed by postoperative radiotherapy in 13 cases.
  • Gender, age, tumour size, treatment period and strategy (surgery versus no-surgery) were not statistically significant.
  • Quality of resection according to margins and the tumour site were the only prognostic factors.
  • There was a significant correlation between tumour site and quality of surgery (p=0.0002).
  • CONCLUSIONS: A subset of patients with extra-abdominal fibromatosis could be managed with a nonaggressive policy, as growth arrest concerned 2/3 of nonoperated patients.
  • [MeSH-major] Fibromatosis, Aggressive / surgery. Soft Tissue Neoplasms / surgery

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  • (PMID = 17709227.001).
  • [ISSN] 1532-2157
  • [Journal-full-title] European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology
  • [ISO-abbreviation] Eur J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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98. Chummun S, McLean NR, Abraham S, Youseff M: Desmoid tumour of the breast. J Plast Reconstr Aesthet Surg; 2010 Feb;63(2):339-45
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Desmoid tumour of the breast.
  • Desmoid tumour of the breast is a rare fibroblastic tumour whose spectrum ranges from being locally inert to aggressive and destructive, and represents 0.2% of all breast tumours.
  • Ultrasound showed a well-defined, hypoechoic mass arising within the muscles of the anterior chest, deep beneath the implant and not involving the underlying rib.
  • A provisional diagnosis of scarring and fibroblastic proliferation was made.
  • A final diagnosis of aggressive fibromatosis was made.
  • Although the association of desmoid tumour and breast implants has been described, this case is unique as the FNA was highly suggestive of a silicone granuloma and the diagnosis of desmoid tumour was made on definitive pathology.
  • The aetiology of desmoid tumours is reviewed and current treatment modalities discussed.
  • [MeSH-major] Breast Neoplasms / diagnosis. Breast Neoplasms / surgery. Fibromatosis, Aggressive / diagnosis. Fibromatosis, Aggressive / surgery

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  • [Copyright] 2008 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.
  • (PMID = 19059821.001).
  • [ISSN] 1878-0539
  • [Journal-full-title] Journal of plastic, reconstructive & aesthetic surgery : JPRAS
  • [ISO-abbreviation] J Plast Reconstr Aesthet Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
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99. 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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100. Gouin F, Tesson A, Bertrand-Vasseur A, Cassagnau E, Rolland F: [Rating of tumoral growth in non-operated primary or recurrent extra-abdominal aggressive fibromatosis]. Rev Chir Orthop Reparatrice Appar Mot; 2007 Oct;93(6):546-54
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Rating of tumoral growth in non-operated primary or recurrent extra-abdominal aggressive fibromatosis].
  • [Transliterated title] Etude de la croissance tumorale des tumeurs desmoïdes extra-abdominales (FDEA) primitives ou récidivées non opérées.
  • PURPOSE OF THE STUDY: Extra-abdominal aggressive fibromatosis (EAAF) is a benign desmoid tumor with a potentially aggressive behavior.
  • For eight, the tumor was a recurrence after surgical removal.
  • MRI showed progression for two tumors (12%) but with a short follow-up since diagnosis (9 and 14 months), in one case despite medical treatment.
  • Three tumors regressed and twelve remained stable on successive MRI.
  • On average the tumor growth lasted ten months.
  • DISCUSSION: Tumor growth was never noted beyond 36 months.
  • This notion of an interruption in tumor growth is mentioned sporadically in reports on EAAF, which have generally included recurrent tumors.
  • To our knowledge this is the first series reporting tumors left in place a followed with modern imaging techniques.
  • The high rate of spontaneous interruption of tumor growth must be counterbalanced with the difficult task of local treatment: the risk of recurrence is particularly high after surgery and functional sequelae can be significant when wide resection is proposed in an anatomically difficult localization.
  • Further work is needed to define the useful contribution of simple surveillance of these benign tumors.
  • [MeSH-major] Fibromatosis, Aggressive / physiopathology. Neoplasm Recurrence, Local / physiopathology
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Biopsy. Chemotherapy, Adjuvant. Cohort Studies. Disease Progression. Female. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Neoplasm Invasiveness. Palliative Care. Prospective Studies. Radiotherapy, Adjuvant. Remission Induction. Treatment Outcome

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  • (PMID = 18065863.001).
  • [ISSN] 0035-1040
  • [Journal-full-title] Revue de chirurgie orthopédique et réparatrice de l'appareil moteur
  • [ISO-abbreviation] Rev Chir Orthop Reparatrice Appar Mot
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
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