Active clinical trials for "Fibromatosis, Aggressive"

The purpose of this study is to constitute the French largest Aggressive fibromatosis cohort.

Desmoid-type fibromatosis (or desmoid tumor) represents an intermediate grade neoplasm with a striking predilection for locally invasive growth and recurrence following resection. It occurs in children as well as young adults. As a typically localized disease, the historical standard of care for treatment has been surgical resection, with or without ionizing radiation. In some cases where surgical resection or radiation is not feasible, chemotherapy has been used. Two clinical trials conducted in the Pediatric Oncology Group (POG) and the Children's Oncology Group (COG) evaluated the role for either low intensity or non-cytotoxic chemotherapy for children with desmoid tumor that is not amenable to standard therapy. These were largely empirical treatment strategies or based on somewhat anecdotal observations. By better understanding desmoid tumor biology, even more effective therapy targeting a particular protein that is central to the disease can be developed. Desmoid tumor is well-known to be associated with deregulation of the Adenomatous Polyposis Cell/beta-catenin (APC/β-catenin pathway). This is true of familial cases associated with Gardner's Syndrome and also in sporadic desmoid tumor, nearly all of which display histological or molecular evidence of Adenomatous Polyposis Cell/beta-catenin (APC β-catenin) pathway activation (Alman et al., 1997; Lips et al., 2009). Several new pieces of evidence support the concept that deregulation of the mammalian target of rapamycin (mTOR) cell proliferation/survival pathway may play an important role in tumor biology when the APC/β-catenin pathway is disrupted. Sirolimus, a drug that inhibits mammalian target of rapamycin (mTOR), is currently being evaluated as an anti-cancer agent in a variety of tumor types, but it has not been previously studied in desmoid tumor. The investigators are conducting this pilot study to begin to explore whether mTOR inhibition may be beneficial for children and young adults with desmoid tumor.

RATIONALE: Sunitinib may stop the growth of tumor cells by blocking some of the enzymes needed for cell growth and by blocking blood flow to the tumor. PURPOSE: This phase II trial is studying how well sunitinib works in treating patients with metastatic, locally advanced, or locally recurrent sarcomas.

The objective of the present study is to evaluate the activity and safety of imatinib in patients with aggressive fibromatosis who, after receiving the standard therapy, show an inoperable recurrent tumor or disease not readily controllable by surgery or radiotherapy.

RATIONALE: Imatinib mesylate may stop the growth of tumor cells by blocking some of the enzymes needed for cell growth. PURPOSE: This phase I/II trial is studying the side effects of imatinib mesylate and to see how well it works in treating patients with recurrent or refractory aggressive fibromatosis.

This phase II trial is studying how well giving sulindac together with tamoxifen works in treating patients with desmoid tumor. Sulindac may stop the growth of cancer cells by blocking the enzymes necessary for cancer cell growth. Hormone therapy using tamoxifen may fight cancer by blocking the use of estrogen. Combining sulindac with tamoxifen may kill more cancer cells.

This is a multicenter, interventional, randomized study among adult patients recently diagnosed with a rare tumor (<12 months). The study will aim to compare compliance with the personalized post-treatment surveillance plan, established for each patient according to national guidelines, when the surveillance is conducted in person by a hospital-based physician (control arm) or remotely by a trained nurse (experimental arm).

There is research supporting treatment of superficial fibromatoses (palmar fibromatosis and keloids) with triamcinolone acetonide injections. These lesions are histologically similar to deep fibromatoses (desmoid tumors). Currently there is little literature evaluating the response of desmoid tumors to injections of triamcinolone acetonide. The investigators aim to perform a pilot study evaluating the response of desmoid tumors to intralesional triamcinolone. If positive results are observed (based on RECIST criteria), then a phase II study will be initiated.

Aggressive fibromatosis (AF, also known as desmoid tumor) is a fibroproliferative neoplasm that typically arises in the abdomen but can develop at other anatomic sites, most commonly in the extremities. These tumors have a relatively high local failure rate after primary treatment using surgery and/or radiotherapy, and although rarely giving rise to distant metastases, can be multifocal and, therefore, not surgically resectable. Moreover, tumor may recur adjacent to the site of surgical resection, underscoring the limitations of surgery in the palliative setting. Therefore, effective medical therapies for AF are needed to maintain quality of life and prolong survival.The goal of the current study was to better define the activity of imatinib in the treatment of AF and to determine the molecular basis for response/nonresponse

Phase I, open-label, non-randomized study to evaluate safety of BC2059 administered intravenously to subjects with proven primary or recurrent desmoid tumor that is unresectable and symptomatic or progressive.