Aggressive Fibromatosis (Desmoid)

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461 relevant articles (29 outcomes, 41 trials/studies) found for this Disease

Description: 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)

Also Known As:

Desmoid; Fibromatosis, Aggressive; Aggressive Fibromatoses; Desmoids; Fibromatoses, Aggressive

Relationship Network

Disease Context: Research Results

Neoplasms: 387640

Neoplasms by Histologic Type: 1

Connective and Soft Tissue Neoplasms

Connective Tissue Neoplasms: 6

Fibrous Tissue Neoplasms

Fibroma: 749

Aggressive Fibromatosis: 461

Related Diseases

1.	Neoplasms (Cancer)
2.	Retroperitoneal Fibrosis
3.	Adenomatous Polyposis Coli (Familial Adenomatous Polyposis)
4.	Sarcoma (Soft Tissue Sarcoma)
5.	Fibroma (Fibromatosis)

Experts

1.	Alman, Benjamin A: 4 articles (01/2007 - 05/2002)
2.	Marinucci, Lorella: 3 articles (09/2008 - 03/2005)
3.	Stabellini, Giordano: 3 articles (09/2008 - 03/2005)
4.	Balducci, Chiara: 3 articles (09/2008 - 03/2005)
5.	Locci, Paola: 3 articles (09/2008 - 03/2005)
6.	Lilli, Cinzia: 3 articles (09/2008 - 03/2005)
7.	Lumare, Eleonora: 2 articles (09/2008 - 02/2007)
8.	Calastrini, Carla: 2 articles (09/2008 - 02/2007)
9.	Becchetti, Ennio: 2 articles (09/2008 - 02/2007)
10.	Dolci, Claudia: 2 articles (09/2008 - 02/2007)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Aggressive Fibromatosis:

1.	Tamoxifen FDA LinkGeneric 06/01/1997 - "A few cases responded well to tamoxifen, a drug which was already used in therapy of desmoid tumours" 04/01/2007 - "Tamoxifen therapy for aggressive fibromatosis of the posterior triangle of the neck." 08/01/2005 - "Long-term follow-up of a recurrent multifocal desmoid tumour treated with tamoxifen: a case report." 11/01/2004 - "On an empirical basis, tamoxifen has been used to pharmacologically treat desmoid tumours" 02/01/2000 - "The case report of a young woman with recurrent pelvic desmoid tumour successfully treated with tamoxifen is described" Order ALL the reference details at left...
2.	Sulindac (Copal)FDA LinkGeneric 01/28/2000 - "To suppress growth of the desmoid tumour Sulindac, a nonsteroid anti-inflammatory drug, was administered" 04/01/1994 - "Moreover, growth of desmoid cells from one of the patients was inhibited by sulindac" 04/01/1997 - "She was treated with sulindac, NSAID, at 300 mg/day, the diagnosis being intra-abdominal desmoid tumors" 01/01/1992 - "When using sulindac for the treatment of desmoid tumors, this phenomenon should be considered." 12/01/1998 - "This complete macroscopic regression of a desmoid tumor after sulindac therapy emphasizes again the interest of this treatment for mesenteric fibromatosis." Order ALL the reference details at left...
3.	Toremifene (Fareston)FDA Link 09/01/2008 - "The reduction in receptor number only in desmoid cells suggests that toremifene may reduce TGFbeta1's affinity for its receptors" 09/01/2008 - "Toremifene reduced TGFbeta1 receptors only in desmoid fibroblasts, with no effect on the changes in type I, II, and III receptors" 11/01/2001 - "Synthesis and secretion of transforming growth factor-beta1 by human desmoid fibroblast cell line and its modulation by toremifene." 10/01/1999 - "Treatment of mesenteric desmoid tumours with the anti-oestrogenic agent toremifene: case histories and an overview of the literature." 02/01/1994 - "Management of desmoid tumours including a case report of toremifene." Order ALL the reference details at left...
4.	Estrogens (Estrogen)FDA Link 02/01/2005 - "This case illustrates the probable contribution of estrogens towards desmoid tumor development, the durability of abdominal wall reconstruction when subjected to the extraordinary strain of both a gravid uterus and labor, as well as the safety and efficacy of aggressive surgical therapy during pregnancy." 04/01/2000 - "Desmoid tumors may be estrogen-dependent, and estrogen-blocking drugs are part of the usual treatment of these tumors" 05/01/1986 - "Clinical and experimental evidence suggests a role for estrogen in the natural history of desmoid tumors (DT)" 05/01/1997 - "BACKGROUND: Clinical and experimental evidence suggest that estrogen has a role in the natural history of desmoid tumor (DT) and colorectal carcinoma" 11/01/2001 - "Toremifene mediated its effects in desmoid fibroblasts via an estrogen receptor-independent pathway" Order ALL the reference details at left...
5.	InterferonsIBA 09/01/2004 - "Interferon-induced remission of rapidly growing aggressive fibromatosis in the temporal fossa." 01/01/1999 - "Interferon-induced remission in aggressive fibromatosis of the lower extremity." 08/01/2008 - "Treatment of extra-abdominal aggressive fibromatosis with pegylated interferon." Order ALL the reference details at left...
6.	imatinib (Gleevec)FDA Link 03/01/2006 - "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" 11/01/2005 - "C-KIT was detectable in one abdominal case only.CONCLUSIONS: The data from this immunohistochemical study show that the published effects of antioestrogens and imatinib mesylate in the treatment of aggressive fibromatoses may not be attributable to oestrogen receptor alpha or c-KIT expression." 08/01/2007 - "OBJECTIVE: There has been only one report available that focuses on the treatment with imatinib mesylate of two individual persons with aggressive fibromatosis" 08/01/2007 - "Control of aggressive fibromatosis by treatment with imatinib mesylate" 12/01/2006 - "Therapy with Imatinib has been proposed as a therapeutic option, although in most reports desmoid tumours are reported to be c-kit-" Order ALL the reference details at left...
7.	Estrogen Receptor Modulators (Antiestrogen)IBA 07/15/1997 - "BACKGROUND: Antiestrogens and nonsteroidal antiinflammatory drugs have been shown to be effective in adult patients with unresectable or recurrent desmoid tumors" 08/01/2000 - "CONCLUSIONS: Although recent reports demonstrate selective regression of desmoid tumors with antiestrogen therapy, management of symptomatic pelvic desmoid tumors in young women may necessitate an operation" 11/01/1993 - "Further investigative work must be undertaken before considering routine use of antiestrogen therapy even in this subset of patients with desmoid tumors." 08/01/1988 - "Desmoid tumors: report of a case responsive to antiestrogen and review of the literature." 01/01/1976 - "Extraabdominal desmoid tumor: one case of successful treatment with antiestrogens (author's transl)]" Order ALL the reference details at left...
8.	Raloxifene (Evista)FDA Link 07/01/2003 - "CONCLUSIONS: Although the number of patients included in the study is limited and in spite of some limitations, the available results support that, in the evaluation of response, daily therapy with raloxifene decreases desmoid tumor and mesenteric fibromatosis size and symptoms and does not cause side effects" 07/01/2003 - "Treatment of desmoids and mesenteric fibromatosis in familial adenomatous polyposis with raloxifene." 11/01/2004 - "Recently, raloxifene, a second-generation SERM, has been used in the treatment of familial adenomatous polyposis patients affected by desmoid tumour" 07/01/2003 - "METHODS: We studied the effects of 120 mg daily of raloxifene, a non-steroidal benzothiophene, on progressive desmoid tumors and mesenteric fibromatosis by evaluation of lesion size and symptoms in 13 patients with familial adenomatous polyposis, selected on the basis of intra-abdominal localization of the lesion, on refractoriness to other medical treatments, and on estrogen receptor-alpha expression" 07/15/2006 - "Although surgery is widely accepted as the first-line treatment for extra-abdominal and abdominal wall desmoids, a proportion of cases are successfully palliated with either estrogen antagonists (tamoxifen, toremifene, and raloxifene) or nonsteroidal anti-inflammatory drugs" Order ALL the reference details at left...
9.	pirfenidoneIBA 08/01/2003 - "A placebo-controlled trial is needed to determine whether there is a subset of patients for whom pirfenidone may result in partial shrinkage of desmoid tumors, because the natural history of desmoid tumors is not predictable or understood." 08/01/2003 - "METHODS: We enrolled 10 women and four men with extensive familial adenomatous polyposis (FAP)-associated desmoid disease in a 2-yr open-label treatment trial with oral pirfenidone" 08/01/2003 - "Some patients with FAP/desmoid tumors treated with pirfenidone had regression of tumors, some had progression, and some had no response" 08/01/2003 - "CONCLUSIONS: Pirfenidone is well tolerated by patients with FAP-associated desmoid tumors" 08/01/2003 - "Desmoid tumors in familial adenomatous polyposis: a pilot project evaluating efficacy of treatment with pirfenidone." Order ALL the reference details at left...
10.	Doxorubicin (Adriamycin)FDA LinkGeneric 04/01/1993 - "To the authors' knowledge, this is the first report of the successful treatment of desmoids with doxorubicin used as a single agent" 04/01/1993 - "A young woman with an extensive abdominal desmoid had a prolonged response to doxorubicin after she did not respond to more conventional treatments" 11/01/2003 - "Doxorubicin treatment of an intra-abdominal desmoid tumour in a patient with familial adenomatous polyposis." 04/01/1993 - "Successful treatment of a desmoid tumor with doxorubicin." 03/01/2008 - "Finally, when the desmoid occupied the pelvic space, we gave the patient dacarbazine (DTIC) and doxorubicin (DOX)" Order ALL the reference details at left...

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 10/01/2006 - "Effective treatment of multifocal aggressive fibromatosis with low-dose chemotherapy." 01/01/2003 - "and methods: Patients with desmoids for whom radical resection was impossible or related to extensive mutilation were treated with chemotherapy" 01/01/2003 - "Purpose: To evaluate the tolerance of a low dose chemotherapy regimen for desmoid tumours" 07/20/2002 - "Chemotherapy is indicated in patients with progressive desmoid tumours when non-cytotoxic treatment has failed" 03/01/2000 - "Symptomatic improvement was noted after chemotherapy in six of ten patients with extremely complex desmoids" Order ALL the reference details at left...
2.	Colectomy 10/01/2007 - "Female patients who had an early colectomy are at significantly greater risk of developing a desmoid compared with female patients who had a colectomy in adulthood" 02/01/2008 - "Most desmoids develop after colectomy" 10/01/2007 - "These results suggest that delayed colectomy might be considered in young female patients with FAP to decrease the chances of developing desmoids." 10/01/2007 - "Female patients who had an early colectomy (</=18 years) were 2.5 times more likely to develop desmoids, compared with male patients who had a late colectomy (P = .05)" 10/01/2007 - "Early colectomy did not increase risk of developing a desmoid in male patients (P = .42)" Order ALL the reference details at left...
3.	Brachytherapy 10/01/2004 - "MATERIALS AND METHODS: A 28-year-old patient was treated with postoperative interstitial brachytherapy boost followed by external radiotherapy after the 11th recurrence of a desmoid tumour in the forearm" 01/01/2004 - "Furthermore, patients with recurrent desmoid tumors involving the bony structures of the hand or feet are poor candidates for brachytherapy alone" 01/01/1997 - "METHODS: Twelve patients over a 3-year period had resection for localized soft-tissue sarcomas and desmoids with insertion of intra-operative brachytherapy implants combined with resection for localized soft-tissue sarcomas" 06/01/1986 - "This experience suggests that surgery and brachytherapy treatment for desmoid tumor results in higher local control than expected from surgery alone in this selected group of patients." 06/01/1986 - "From 1977 to 1982, fourteen patients with desmoid tumors underwent surgery and brachytherapy" Order ALL the reference details at left...
4.	Limb Salvage 11/01/1999 - "The high rate of limb salvage achieved by isolated limb perfusion (ILP) with tumor necrosis factor (TNF) and melphalan for extensive, high-grade soft tissue sarcoma led us to implement this modality in difficult cases of limb desmoids" 02/01/1995 - "Important advances discussed over this period include: establishment of sarcoma teams in major oncology centers; staging systems for both soft tissue and osseous sarcomas; demonstration of genetic determinants in the development of, at least, some of the sarcomas; the revolutionary change in quality of diagnostic imaging by the introduction of CT and MRI; use of immunohistochemistry in diagnostic pathology; the drastic gains in survival of patients with osteogenic sarcoma, Ewing's sarcoma and rhabdomyosarcoma due to the efficacy of multi-drug and multi-cycle chemotherapy protocols; major advances in surgical techniques which have made limb salvage practical; cell lines derived from human sarcomas have been shown to have in vitro radiation sensitivity comparable to that of cell lines from epithelial tumors; the combination of conservative surgery and moderate doses of radiation yields local control and survival results equivalent to that of radical surgery with a much improved functional and cosmetic outcome; intra-operative electron beam radiation therapy improves the outcome of patients with retroperitoneal sarcomas when given after grossly complete resection combined with external beam radiation therapy (pre- or postoperatively); radiation is a highly effective alternative to extensive surgery for desmoid tumors; local control of giant cell tumors by modern radiation techniques is approximately 80% and the incidence of radiation induced tumors at 10 years is approximately 3%; to decrease the incidence of radiation induced sarcoma, resection has replaced radiation in the management of selected patients with primary Ewing's sarcoma when the response to chemotherapy has been excellent and the morbidity/functional decrement consequent upon the surgery judged reasonable; proton beam radiation therapy has been accepted as being superior to conventional external beam radiation therapy for chondrosarcoma and chordoma of the skull base; and attempts to utilize brachytherapy for sarcomas of the spine/sacrum appear to offer promise" Order ALL the reference details at left...
5.	Neoadjuvant Therapy 05/01/2007 - "Increased neoadjuvant treatments may be associated with improved desmoid patient outcomes" 12/01/2004 - "There are descriptions and discussion of the surgical techniques, advantages and disadvantages of various types and combinations of adjuvant and neoadjuvant therapies, therapy in case of nonresectability, and procedures for desmoid tumors" Order ALL the reference details at left...

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