aggressive fibromatosis

Summary

Summary: A childhood counterpart of abdominal or extra-abdominal desmoid tumors, characterized by firm subcutaneous nodules that grow rapidly in any part of the body but do not metastasize. The adult form of abdominal fibromatosis is FIBROMATOSIS, ABDOMINAL. (Stedman, 25th ed)

Top Publications

  1. Ferenc T, Sygut J, Kopczynski J, Mayer M, Latos Bielenska A, Dziki A, et al. Aggressive fibromatosis (desmoid tumors): definition, occurrence, pathology, diagnostic problems, clinical behavior, genetic background. Pol J Pathol. 2006;57:5-15 pubmed
    b>Aggressive fibromatosis, usually called desmoid tumor develops from muscle connective tissue, fasciae and aponeuroses. This neoplasm is composed of spindle (fibrocyte-like) cells...
  2. Lee J, Thomas J, Phillips S, Fisher C, Moskovic E. Aggressive fibromatosis: MRI features with pathologic correlation. AJR Am J Roentgenol. 2006;186:247-54 pubmed
    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.
  3. Micke O, Seegenschmiedt M. Radiation therapy for aggressive fibromatosis (desmoid tumors): results of a national Patterns of Care Study. Int J Radiat Oncol Biol Phys. 2005;61:882-91 pubmed
    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.
  4. Khanfir K, Guinebretiere J, Vanel D, Barreau Pouhaer L, Bonvalot S, Le Cesne A. Unusual problems in breast cancer and a rare lung cancer case. Case 2. Aggressive fibromatosis of the chest wall arising near a breast prosthesis. J Clin Oncol. 2003;21:2216-8 pubmed
  5. Gebert C, Hardes J, Kersting C, August C, Supper H, Winkelmann W, et al. Expression of beta-catenin and p53 are prognostic factors in deep aggressive fibromatosis. Histopathology. 2007;50:491-7 pubmed
    To determine the prognostic significance of beta-catenin in aggressive fibromatosis and to identify potential molecular markers for new targeted therapies.
  6. Kasper B, Dimitrakopoulou Strauss A, Strauss L, Hohenberger P. Positron emission tomography in patients with aggressive fibromatosis/desmoid tumours undergoing therapy with imatinib. Eur J Nucl Med Mol Imaging. 2010;37:1876-82 pubmed publisher
    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).
  7. Fen Li C, Kandel C, Baliko F, Nadesan P, Brunner N, Alman B. Plasminogen activator inhibitor-1 (PAI-1) modifies the formation of aggressive fibromatosis (desmoid tumor). Oncogene. 2005;24:1615-24 pubmed
    b>Aggressive fibromatosis is a mesenchymal neoplasm associated with mutations, resulting in beta-catenin-mediated transcriptional activation...
  8. Gronchi A, Casali P, Mariani L, Lo Vullo S, Colecchia M, Lozza L, et al. Quality of surgery and outcome in extra-abdominal aggressive fibromatosis: a series of patients surgically treated at a single institution. J Clin Oncol. 2003;21:1390-7 pubmed
    To explore prognostic factors in surgically treated aggressive fibromatosis (extra-abdominal desmoid tumor).
  9. Castellazzi G, Vanel D, Le Cesne A, Le Pechoux C, Caillet H, Perona F, et al. Can the MRI signal of aggressive fibromatosis be used to predict its behavior?. Eur J Radiol. 2009;69:222-9 pubmed publisher
    b>Aggressive fibromatosis is an invasive non-metastasizing soft-tissue tumor...

More Information

Publications62

  1. Constantinidou A, Jones R, Scurr M, Al Muderis O, Judson I. Pegylated liposomal doxorubicin, an effective, well-tolerated treatment for refractory aggressive fibromatosis. Eur J Cancer. 2009;45:2930-4 pubmed publisher
    b>Aggressive fibromatosis (AF) or desmoid tumour is a monoclonal proliferation which is locally invasive but does not metastasize...
  2. Tejpar S, Li C, Yu C, Poon R, Denys H, Sciot R, et al. Tcf-3 expression and beta-catenin mediated transcriptional activation in aggressive fibromatosis (desmoid tumour). Br J Cancer. 2001;85:98-101 pubmed
    b>Aggressive fibromatosis harbours mutations resulting in beta-catenin protein stabilization. Primary cell cultures demonstrate constitutive tcf activation in aggressive fibromatosis...
  3. Altmann S, Lenz Scharf O, Schneider W. [Therapeutic options for aggressive fibromatosis]. Handchir Mikrochir Plast Chir. 2008;40:88-93 pubmed publisher
    b>Aggressive fibromatosis, e.g., desmoid tumour, is a rare neoplasm of the connective tissue with local infiltrative growth...
  4. Tolg C, Poon R, Fodde R, Turley E, Alman B. Genetic deletion of receptor for hyaluronan-mediated motility (Rhamm) attenuates the formation of aggressive fibromatosis (desmoid tumor). Oncogene. 2003;22:6873-82 pubmed
    b>Aggressive fibromatosis (desmoid tumor) is a locally invasive soft tissue neoplasm associated with mutations resulting in beta-catenin-mediated transcriptional activation...
  5. Ferenc T, Wroński J, Kopczynski J, Kulig A, Sidor M, Stalinska L, et al. Analysis of APC, alpha-, beta-catenins, and N-cadherin protein expression in aggressive fibromatosis (desmoid tumor). Pathol Res Pract. 2009;205:311-24 pubmed publisher
    ..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 ..
  6. De Pas T, Bodei L, Pelosi G, de Braud F, Villa G, Capanna R, et al. Peptide receptor radiotherapy: a new option for the management of aggressive fibromatosis on behalf of the Italian Sarcoma Group. Br J Cancer. 2003;88:645-7 pubmed
    The management of aggressive fibromatosis (AF) is problematic, and few options are available to patients unsuitable for surgery and resistant to external-beam radiation therapy (EBRT)...
  7. Sharma A, Ngan B, Sandor G, Campisi P, Forte V. Pediatric aggressive fibromatosis of the head and neck: a 20-year retrospective review. J Pediatr Surg. 2008;43:1596-604 pubmed publisher
    b>Aggressive fibromatosis in children is a rare, benign condition that is locally infiltrative and destructive. It often presents as a rapidly growing, painless lump in the head and neck region...
  8. Seinfeld J, Kleinschmidt Demasters B, Tayal S, Lillehei K. Desmoid-type fibromatoses involving the brachial plexus: treatment options and assessment of c-KIT mutational status. J Neurosurg. 2006;104:749-56 pubmed
    ..The authors describe their experience in treating four patients with desmoid-type fibromatoses involving the brachial plexus and report the results of immunohistochemical analysis of the tumors...
  9. Roeder F, Timke C, Oertel S, Hensley F, Bischof M, Muenter M, et al. Intraoperative electron radiotherapy for the management of aggressive fibromatosis. Int J Radiat Oncol Biol Phys. 2010;76:1154-60 pubmed publisher
    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.
  10. 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;133:533-8 pubmed
    ..one report available that focuses on the treatment with imatinib mesylate of two individual persons with aggressive fibromatosis. The authors concluded that after long-term treatment, for 9 and 11 months, with imatinib mesylate, both ..
  11. Bright Thomas R, Agrawal A, Hargest R. Preclinical studies of gene transfer for the treatment of desmoid disease in familial adenomatous polyposis. Br J Surg. 2002;89:1563-9 pubmed
    ..The aim of this study was to assess the potential for APC gene transfer into fibroblasts in vitro and in vivo as a basis for consideration of gene therapy in the prevention or treatment of desmoid tumours...
  12. Brueckl W, Ballhausen W, Förtsch T, Günther K, Fiedler W, Gentner B, et al. 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;48:1275-81 pubmed
    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...
  13. De Riu G, Meloni S, Raho M, Tullio A. Complications of mandibular reconstruction in childhood: Report of a case of Juvenile Aggressive Fibromatosis. J Craniomaxillofac Surg. 2006;34:168-72 pubmed
    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...
  14. Phillips S, A Hern R, Thomas J. Aggressive fibromatosis of the abdominal wall, limbs and limb girdles. Br J Surg. 2004;91:1624-9 pubmed
    b>Aggressive fibromatosis (AF) is a rare soft-tissue tumour. It is histologically benign but locally aggressive and destructive. Surgery is the mainstay of treatment...
  15. Mendenhall W, Zlotecki R, Morris C, Hochwald S, Scarborough M. Aggressive fibromatosis. Am J Clin Oncol. 2005;28:211-5 pubmed
    ..this article is to review the pertinent literature and to define the optimal treatment of patients with aggressive fibromatosis. Data indicate the likelihood of local recurrence after surgery is high, particularly if margins are ..
  16. Heinrich M, McArthur G, Demetri G, Joensuu H, Bono P, Herrmann R, et al. Clinical and molecular studies of the effect of imatinib on advanced aggressive fibromatosis (desmoid tumor). J Clin Oncol. 2006;24:1195-203 pubmed
    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.
  17. Meyerson S, D Amico T. Intrathoracic desmoid tumor: brief report and review of literature. J Thorac Oncol. 2008;3:656-9 pubmed publisher
    ..A comprehensive review of the literature was undertaken to identify the prevalence and characteristics of true intrathoracic desmoid tumors...
  18. Abbas A, Deschamps C, Cassivi S, Nichols F, Allen M, Schleck C, et al. Chest-wall desmoid tumors: results of surgical intervention. Ann Thorac Surg. 2004;78:1219-23; discussion 1219-23 pubmed
    ..We are analyzing our experience with treatment of desmoid tumors of the chest wall and adjacent structures...
  19. Jeblaoui Y, Bouguila J, Haddad S, Helali M, Zairi I, Zitouni K, et al. [Mandibular aggressive fibromatosis]. Rev Stomatol Chir Maxillofac. 2007;108:153-5 pubmed
    b>Aggressive fibromatosis is a rare histologically benign fibrous tumor with a potential for locoregional aggression. Treatment is not well defined and several therapeutic approaches have been proposed...
  20. Li C, MacDonald J, Wei R, Ray J, Lau K, Kandel C, et al. 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 pubmed
    ..In the mesenchymal neoplasm, aggressive fibromatosis, subtractive hybridization identified sterile alpha motif domain 9 (SAMD9) as a substantially down ..
  21. Zlotecki R, Scarborough M, Morris C, Berrey B, Lind D, Enneking W, et al. External beam radiotherapy for primary and adjuvant management of aggressive fibromatosis. Int J Radiat Oncol Biol Phys. 2002;54:177-181 pubmed
    To review a large single-institution experience in the management of aggressive fibromatosis to determine the effectiveness of external beam radiotherapy (EBRT) and identify the presentation and treatment variables predictive of ..
  22. Tjandra S, Hsu C, Goh Y, Goh I, Gurung A, Poon R, et al. IFN-{beta} signaling positively regulates tumorigenesis in aggressive fibromatosis, potentially by modulating mesenchymal progenitors. Cancer Res. 2007;67:7124-31 pubmed
    b>Aggressive fibromatosis (also called desmoid tumor) is a benign, locally invasive, soft tissue tumor composed of cells with mesenchymal characteristics...
  23. Skapek S, Hawk B, Hoffer F, Dahl G, Granowetter L, Gebhardt M, et al. Combination chemotherapy using vinblastine and methotrexate for the treatment of progressive desmoid tumor in children. J Clin Oncol. 1998;16:3021-7 pubmed
    ..We report the treatment of 10 children for progressive desmoid tumor not amenable to standard surgical or radiation therapy with the use of vinblastine (VBL) and methotrexate (MTX)...
  24. Lackner H, Urban C, Benesch M, Raith J, Moser A, Sovinz P, et al. Multimodal treatment of children with unresectable or recurrent desmoid tumors: an 11-year longitudinal observational study. J Pediatr Hematol Oncol. 2004;26:518-22 pubmed
    ..Weekly administration of vinblastine and methotrexate seems to be safe and effective in these children...
  25. Mace J, Sybil Biermann J, Sondak V, McGinn C, Hayes C, Thomas D, et al. Response of extraabdominal desmoid tumors to therapy with imatinib mesylate. Cancer. 2002;95:2373-9 pubmed
    ..Imatinib mesylate appears to demonstrate inhibitory activity against multiple class 3 receptor tyrosine kinases, including platelet-derived growth factor receptor (PDGFR)-alpha and PDGFR-beta, as well as c-kit...
  26. Sørensen A, Keller J, Nielsen O, Jensen O. Treatment of aggressive fibromatosis: a retrospective study of 72 patients followed for 1-27 years. Acta Orthop Scand. 2002;73:213-9 pubmed
    ..local recurrence-free survival, including expression of estrogen receptors, after surgical treatment of aggressive fibromatosis in 72 patients (53 women) having primary tumors between 1970 and 1998...
  27. Azzarelli A, Gronchi A, Bertulli R, Tesoro J, Baratti D, Pennacchioli E, et al. Low-dose chemotherapy with methotrexate and vinblastine for patients with advanced aggressive fibromatosis. Cancer. 2001;92:1259-64 pubmed
    ..Phase II study was undertaken to assess the activity of methotrexate plus vinblastine in the treatment of patients with inoperable aggressive fibromatosis (AF) and to observe the evolution of the disease after such low-dose chemotherapy.
  28. Schulz Ertner D, Zierhut D, Mende U, Harms W, Branitzki P, Wannenmacher M. The role of radiation therapy in the management of desmoid tumors. Strahlenther Onkol. 2002;178:78-83 pubmed
    ..To investigate the role of radiation therapy (RT) in the management of desmoid tumors...
  29. Abdelkader M, Riad M, Williams A. Aggressive fibromatosis of the head and neck (desmoid tumours). J Laryngol Otol. 2001;115:772-6 pubmed
    ..A review of cases of desmoid tumours of the head and neck presented in the last 10 years is reported in this paper. Complete surgical excision of desmoid tumours is considered to be the only effective method of cure by most authorities...
  30. Mohos E, Kovacs T, Brittig F, Nagy A. [Desmoid tumors in three patients]. Magy Seb. 2001;54:387-92 pubmed
  31. Buitendijk S, van de Ven C, Dumans T, den Hollander J, Nowak P, Tissing W, et al. Pediatric aggressive fibromatosis: a retrospective analysis of 13 patients and review of literature. Cancer. 2005;104:1090-9 pubmed
    b>Aggressive fibromatosis (AF) is a soft tissue tumor and is rare in childhood, with high potential for local invasiveness and recurrence. General recommendations for the clinical management of pediatric patients with AF remain undetermined.
  32. Raguse J, Gath H, Oettle H, Bier J. Interferon-induced remission of rapidly growing aggressive fibromatosis in the temporal fossa. Int J Oral Maxillofac Surg. 2004;33:606-9 pubmed
    b>Aggressive fibromatosis is the name for uncommon soft-tissue neoplasms arising within musculoaponeurotic tissue. They show benign histologic features but have an aggressive local behaviour and frequently recur after surgery or radiation...
  33. Meazza C, Bisogno G, Gronchi A, Fiore M, Cecchetto G, Alaggio R, et al. Aggressive fibromatosis in children and adolescents: the Italian experience. Cancer. 2010;116:233-40 pubmed publisher
    b>Aggressive fibromatosis (AF) is a rare tumor of intermediate malignancy that has a strong potential for local invasiveness and recurrence. To date, there are no general recommendations for the clinical management of pediatric AF.
  34. Skubitz K, Manivel J, Clohisy D, Frolich J. 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;64:635-40 pubmed publisher
    b>Aggressive fibromatosis (AF) is usually a slowly growing locally invasive tumor, but may exhibit a much more aggressive phenotype. The role of chemotherapy in AF is not well defined, but can be useful in some cases...
  35. Lips D, 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;35:3-10 pubmed publisher
    b>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...
  36. Stengel G, Metze D, Dörflinger B, Luger T, Bohm M. Treatment of extra-abdominal aggressive fibromatosis with pegylated interferon. J Am Acad Dermatol. 2008;59:S7-9 pubmed publisher
    b>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...
  37. Dufresne A, Bertucci F, Penel N, Le Cesne A, Bui B, Tubiana Hulin M, et al. Identification of biological factors predictive of response to imatinib mesylate in aggressive fibromatosis. Br J Cancer. 2010;103:482-5 pubmed publisher
    Imatinib induces responses and disease stabilisations in non-resectable patients with aggressive fibromatosis (AF). The precise target of imatinib in AF and predictive factors for response to treatment are unknown.
  38. Gega M, Yanagi H, Yoshikawa R, Noda M, Ikeuchi H, Tsukamoto K, et al. Successful chemotherapeutic modality of doxorubicin plus dacarbazine for the treatment of desmoid tumors in association with familial adenomatous polyposis. J Clin Oncol. 2006;24:102-5 pubmed
    ..Here, we prospectively investigated the efficacy of a chemotherapeutic regimen involving doxorubicin (DOX) and dacarbazine (DTIC) for inoperable FAP-associated desmoid tumors...
  39. Bertario L, Russo A, Sala P, Eboli M, Giarola M, D Amico F, et al. Genotype and phenotype factors as determinants of desmoid tumors in patients with familial adenomatous polyposis. Int J Cancer. 2001;95:102-7 pubmed
    ..9; 95% CI 1.1-3.4). Our results indicate that integrating genetic and clinical data is helpful in defining subgroups of patients at higher risk for desmoids, who may benefit from specific prevention programs...
  40. Kotiligam D, Lazar A, Pollock R, Lev D. Desmoid tumor: a disease opportune for molecular insights. Histol Histopathol. 2008;23:117-26 pubmed publisher
    ..This knowledge will eventually result in the development of patient and tumor tailored therapies and assist in the control and eradication of this disease...
  41. Signoroni S, Frattini M, Negri T, Pastore E, Tamborini E, Casieri P, et al. Cyclooxygenase-2 and platelet-derived growth factor receptors as potential targets in treating aggressive fibromatosis. Clin Cancer Res. 2007;13:5034-40 pubmed
    To explore the molecular bases of potential new pharmacologic targets in aggressive fibromatosis (desmoid tumor).
  42. Poon R, Smits R, Li C, Jagmohan Changur S, Kong M, Cheon S, et al. Cyclooxygenase-two (COX-2) modulates proliferation in aggressive fibromatosis (desmoid tumor). Oncogene. 2001;20:451-60 pubmed
    b>Aggressive fibromatosis is a locally invasive soft tissue lesion. Seventy-five per cent of cases harbor a somatic mutation in either the APC or beta-catenin genes, resulting in beta-catenin protein stabilization...
  43. Eccles D, van der Luijt R, Breukel C, Bullman H, Bunyan D, Fisher A, et al. Hereditary desmoid disease due to a frameshift mutation at codon 1924 of the APC gene. Am J Hum Genet. 1996;59:1193-201 pubmed
    ..We show that HDD segregates with an unusual germ-line chain-terminating mutation at the 3' end of the APC gene (codon 1924) with somatic loss of the wild-type allele leading to tumor development...
  44. Fallen T, Wilson M, Morlan B, Lindor N. Desmoid tumors -- a characterization of patients seen at Mayo Clinic 1976-1999. Fam Cancer. 2006;5:191-4 pubmed
    ..Using Bayesian analysis, we demonstrate how these findings can assist genetic professionals in their evaluation of patients with desmoid tumors by providing prior probabilities of FAP based upon clinical presentation...
  45. Shields C, Winter D, Kirwan W, Redmond H. Desmoid tumours. Eur J Surg Oncol. 2001;27:701-6 pubmed
    ..Failure to recognize the potential for malignant behaviour in this tumour renders desmoids susceptible to inadequate treatment. This distinct pathological entity is reviewed with a specific focus on aetiology and treatment...
  46. Cheon S, Cheah A, Turley S, Nadesan P, Poon R, Clevers H, et al. beta-Catenin stabilization dysregulates mesenchymal cell proliferation, motility, and invasiveness and causes aggressive fibromatosis and hyperplastic cutaneous wounds. Proc Natl Acad Sci U S A. 2002;99:6973-8 pubmed
    ..They range from hypertrophic scars to neoplasms such as aggressive fibromatosis. Cells from these disorders share cytologic similarity with fibroblasts present during the proliferative ..
  47. Fiore M, Rimareix F, Mariani L, Domont J, Collini P, Le Péchoux C, et al. Desmoid-type fibromatosis: a front-line conservative approach to select patients for surgical treatment. Ann Surg Oncol. 2009;16:2587-93 pubmed publisher
    ..The disease remained stable in more than half of patients. This study was designed to evaluate this approach on the natural history of the disease in a larger series of patients...
  48. Lessow A, Song P, Komisar A. Unusual fibromatosis of the head and neck. Otolaryngol Head Neck Surg. 2004;130:366-9 pubmed
  49. Arshad A, Normala B. Surgical management of large desmoid tumour of the anterior abdominal wall. Asian J Surg. 2008;31:90-5 pubmed publisher
    ..Closure under tension of the peritoneum did not seem to produce any morbidity...
  50. Dhingra M, Amladi S, Savant S, Nayak C. Juvenile hyaline fibromatosis and infantile systemic hyalinosis: divergent expressions of the same genetic defect?. Indian J Dermatol Venereol Leprol. 2008;74:371-4 pubmed
    ..The similarities and differences between these two described types of hyalinoses have been reviewed in reference to the present report...
  51. Kendi T, Erakar A, Saglik Y, Yildiz H, Erekul S. Desmoplastic fibroma of bone: case report. Clin Imaging. 2003;27:200-2 pubmed
    ..The radiograph, CT imaging features, radiological and pathological differential diagnosis of the case are described, and literature is briefly reviewed...
  52. Locci P, Balducci C, Lilli C, Marinucci L, Becchetti E, Dolci C, et al. Desmoid and fibroma tumors differently respond to TGFbeta(1) stimulus and ECM macromolecule accumulation. Biomed Pharmacother. 2007;61:131-6 pubmed
  53. Hardell L, Breivald M, Hennerdal S, Fernberg J, Strander H. Shrinkage of desmoid tumor with interferon alfa treatment: a case report. Cytokines Cell Mol Ther. 2000;6:155-6 pubmed
    ..This treatment modality may be considered as an alternative to mutilating surgery in patients with desmoid tumor...