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MeSH Review

Fibromatosis, Aggressive

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Disease relevance of Fibromatosis, Aggressive


High impact information on Fibromatosis, Aggressive


Chemical compound and disease context of Fibromatosis, Aggressive


Biological context of Fibromatosis, Aggressive


Anatomical context of Fibromatosis, Aggressive


Gene context of Fibromatosis, Aggressive

  • These data show that APC truncating mutations give aggressive fibromatosis cells a proliferative advantage through beta-catenin and suggest that beta-catenin acts to transactivate transcription [25].
  • Breeding mice with Cox-2-/- mice resulted in no difference in number of aggressive fibromatoses formed, but in a smaller tumor size, while there was a decrease in number of GI lesions by 50% [26].
  • In addition, the desmoid tumor showed deregulation between PCNA and p21WAF1/CIP1 because the normal inverse relation between these two was not apparent [27].
  • Importantly, exogenous p16(INK4a) introduced by cotransfection is sufficient to reduce GR activity in FS cells, without affecting GR activity in p16-positive aggressive fibromatosis cells [28].
  • We found that plasminogen activator inhibitor-1 (PAI-1) was upregulated fourfold in aggressive fibromatosis [29].
  • 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 [30].

Analytical, diagnostic and therapeutic context of Fibromatosis, Aggressive


  1. Somatic mutation of APC gene in desmoid tumour in familial adenomatous polyposis. Sen-Gupta, S., Van der Luijt, R.B., Bowles, L.V., Meera Khan, P., Delhanty, J.D. Lancet (1993) [Pubmed]
  2. Tumorigenesis in the multiple intestinal neoplasia mouse: redundancy of negative regulators and specificity of modifiers. Halberg, R.B., Katzung, D.S., Hoff, P.D., Moser, A.R., Cole, C.E., Lubet, R.A., Donehower, L.A., Jacoby, R.F., Dove, W.F. Proc. Natl. Acad. Sci. U.S.A. (2000) [Pubmed]
  3. Severe Gardner syndrome in families with mutations restricted to a specific region of the APC gene. Davies, D.R., Armstrong, J.G., Thakker, N., Horner, K., Guy, S.P., Clancy, T., Sloan, P., Blair, V., Dodd, C., Warnes, T.W. Am. J. Hum. Genet. (1995) [Pubmed]
  4. Characterization of molecular abnormalities in human fibroblastic neoplasms: a model for genotype-phenotype association in soft tissue tumors. Hoos, A., Lewis, J.J., Antonescu, C.R., Dudas, M.E., Leon, L., Woodruff, J.M., Brennan, M.F., Cordon-Cardo, C. Cancer Res. (2001) [Pubmed]
  5. Expression of the common acute lymphoblastic leukemia antigen (CD10) in mesenchymal tumors. Mechtersheimer, G., Möller, P. Am. J. Pathol. (1989) [Pubmed]
  6. Effect of progesterone on desmoid tumors (aggressive fibromatosis). Lanari, A. N. Engl. J. Med. (1983) [Pubmed]
  7. The type of somatic mutation at APC in familial adenomatous polyposis is determined by the site of the germline mutation: a new facet to Knudson's 'two-hit' hypothesis. Lamlum, H., Ilyas, M., Rowan, A., Clark, S., Johnson, V., Bell, J., Frayling, I., Efstathiou, J., Pack, K., Payne, S., Roylance, R., Gorman, P., Sheer, D., Neale, K., Phillips, R., Talbot, I., Bodmer, W., Tomlinson, I. Nat. Med. (1999) [Pubmed]
  8. Apc1638N: a mouse model for familial adenomatous polyposis-associated desmoid tumors and cutaneous cysts. Smits, R., van der Houven van Oordt, W., Luz, A., Zurcher, C., Jagmohan-Changur, S., Breukel, C., Khan, P.M., Fodde, R. Gastroenterology (1998) [Pubmed]
  9. A transition in transcriptional activation by the glucocorticoid and retinoic acid receptors at the tumor stage of dermal fibrosarcoma development. Vivanco, M.D., Johnson, R., Galante, P.E., Hanahan, D., Yamamoto, K.R. EMBO J. (1995) [Pubmed]
  10. beta-Catenin stabilization dysregulates mesenchymal cell proliferation, motility, and invasiveness and causes aggressive fibromatosis and hyperplastic cutaneous wounds. Cheon, S.S., Cheah, A.Y., Turley, S., Nadesan, P., Poon, R., Clevers, H., Alman, B.A. Proc. Natl. Acad. Sci. U.S.A. (2002) [Pubmed]
  11. Combination chemotherapy using vinblastine and methotrexate for the treatment of progressive desmoid tumor in children. Skapek, S.X., Hawk, B.J., Hoffer, F.A., Dahl, G.V., Granowetter, L., Gebhardt, M.C., Ferguson, W.S., Grier, H.E. J. Clin. Oncol. (1998) [Pubmed]
  12. Treatment of intra-abdominal and abdominal wall desmoid tumors with drugs that affect the metabolism of cyclic 3',5'-adenosine monophosphate. Waddell, W.R. Ann. Surg. (1975) [Pubmed]
  13. Response of multicentric desmoid tumours to tamoxifen. Procter, H., Singh, L., Baum, M., Brinkley, D. The British journal of surgery. (1987) [Pubmed]
  14. Management of desmoid tumours including a case report of toremifene. Benson, J.R., Mokbel, K., Baum, M. Ann. Oncol. (1994) [Pubmed]
  15. Familial infiltrative fibromatosis (desmoid tumours) (MIM135290) caused by a recurrent 3' APC gene mutation. Scott, R.J., Froggatt, N.J., Trembath, R.C., Evans, D.G., Hodgson, S.V., Maher, E.R. Hum. Mol. Genet. (1996) [Pubmed]
  16. Genetic deletion of receptor for hyaluronan-mediated motility (Rhamm) attenuates the formation of aggressive fibromatosis (desmoid tumor). Tolg, C., Poon, R., Fodde, R., Turley, E.A., Alman, B.A. Oncogene (2003) [Pubmed]
  17. Upregulation of Wilms' tumor gene 1 (WT1) in desmoid tumors. Amini Nik, S., Hohenstein, P., Jadidizadeh, A., Van Dam, K., Bastidas, A., Berry, R.L., Patek, C.E., Van der Schueren, B., Cassiman, J.J., Tejpar, S. Int. J. Cancer (2005) [Pubmed]
  18. Tcf-3 expression and beta-catenin mediated transcriptional activation in aggressive fibromatosis (desmoid tumour). Tejpar, S., Li, C., Yu, C., Poon, R., Denys, H., Sciot, R., Van Cutsem, E., Cassiman, J.J., Alman, B.A. Br. J. Cancer (2001) [Pubmed]
  19. Chromosome changes in desmoid tumors developed in patients with familial adenomatous polyposis. Yoshida, M.A., Ikeuchi, T., Iwama, T., Miyaki, M., Mori, T., Ushijima, Y., Hara, A., Miyakita, M., Tonomura, A. Jpn. J. Cancer Res. (1991) [Pubmed]
  20. Coexistence of somatic and germ-line mutations of APC gene in desmoid tumors from patients with familial adenomatous polyposis. Miyaki, M., Konishi, M., Kikuchi-Yanoshita, R., Enomoto, M., Tanaka, K., Takahashi, H., Muraoka, M., Mori, T., Konishi, F., Iwama, T. Cancer Res. (1993) [Pubmed]
  21. Suppression of protein kinase C and the stimulation of glucocorticoid receptor synthesis by dexamethasone in human fibroblasts derived from tumor tissue. Gadson, P., McCoy, J., Wikström, A.C., Gustafsson, J.A. J. Cell. Biochem. (1990) [Pubmed]
  22. The desmoid syndrome. New aspects in the cause, pathogenesis and treatment of the desmoid tumor. Reitamo, J.J., Scheinin, T.M., Häyry, P. Am. J. Surg. (1986) [Pubmed]
  23. Successful low-dose chemotherapy using vinblastine and methotrexate for the treatment of an ileoanal pouch mesenteric desmoid tumor: report of a case. Kono, T., Tomita, I., Chisato, N., Matsuda, M., Kakisaka, A., Kasai, S. Dis. Colon Rectum (2004) [Pubmed]
  24. Comprehensive genetic and endoscopic evaluation may be necessary to distinguish sporadic versus familial adenomatous polyposis-associated abdominal desmoid tumors. Bandipalliam, P., Balmana, J., Syngal, S. Surgery (2004) [Pubmed]
  25. Adenomatous polyposis coli gene mutation alters proliferation through its beta-catenin-regulatory function in aggressive fibromatosis (desmoid tumor). Li, C., Bapat, B., Alman, B.A. Am. J. Pathol. (1998) [Pubmed]
  26. Cyclooxygenase-two (COX-2) modulates proliferation in aggressive fibromatosis (desmoid tumor). Poon, R., Smits, R., Li, C., Jagmohan-Changur, S., Kong, M., Cheon, S., Yu, C., Fodde, R., Alman, B.A. Oncogene (2001) [Pubmed]
  27. An aggressive desmoid tumor in a patient with familial adenomatous polyposis: immunohistochemical findings. Arai, N., Mitomi, H., Uesugi, H., Aihara, S., Ohtani, Y., Okayasu, I. Am. J. Gastroenterol. (1999) [Pubmed]
  28. Loss of p16INK4a results in increased glucocorticoid receptor activity during fibrosarcoma development. Roca, R., Kypta, R.M., Vivanco, M.M. Proc. Natl. Acad. Sci. U.S.A. (2003) [Pubmed]
  29. Plasminogen activator inhibitor-1 (PAI-1) modifies the formation of aggressive fibromatosis (desmoid tumor). Fen Li, C., Kandel, C., Baliko, F., Nadesan, P., Brünner, N., Alman, B.A. Oncogene (2005) [Pubmed]
  30. Cyclooxygenase-2 and platelet-derived growth factor receptors as potential targets in treating aggressive fibromatosis. 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, M.A., Pilotti, S. Clin. Cancer Res. (2007) [Pubmed]
  31. Evidence for genetic predisposition to desmoid tumours in familial adenomatous polyposis independent of the germline APC mutation. Sturt, N.J., Gallagher, M.C., Bassett, P., Philp, C.R., Neale, K.F., Tomlinson, I.P., Silver, A.R., Phillips, R.K. Gut (2004) [Pubmed]
  32. Long-term outcome of familial adenomatous polyposis patients after restorative coloproctectomy. Parc, Y., Piquard, A., Dozois, R.R., Parc, R., Tiret, E. Ann. Surg. (2004) [Pubmed]
  33. Intestinal transplantation for the treatment of desmoid tumors associated with familial adenomatous polyposis. Chatzipetrou, M.A., Tzakis, A.G., Pinna, A.D., Kato, T., Misiakos, E.P., Tsaroucha, A.K., Weppler, D., Ruiz, P., Berho, M., Fishbein, T., Conn, H.O., Ricordi, C. Surgery (2001) [Pubmed]
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