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Showing 1-16 of 16 trials
NCT07170644
This study is being conducted to characterize the efficacy and safety of nirogacestat in Japanese adults with progressing desmoid tumors/aggressive fibromatosis.
NCT07461246
RIPAF (Rete Italiana Poliposi Adenomatosa Familiare) is a national, multicenter observational registry designed to establish a coordinated Italian network for the management of Familial Adenomatous Polyposis (FAP) and related adenomatous polyposis syndromes. The registry includes patients with APC-related FAP (classic and attenuated forms), MUTYH-associated polyposis (MAP), and adenomatous polyposis not associated with APC or MUTYH mutations (NAMP), including cases linked to other susceptibility genes or without identified pathogenic variants. The study combines retrospective and prospective data collection across 28 Italian centers. Its primary purpose is to generate standardized, large-scale clinical data to better characterize disease presentation and evolution, evaluate current surveillance and surgical strategies, and assess oncological outcomes and quality-of-care indicators in real-world practice. The registry will collect detailed information on genotype-phenotype correlations, colorectal and upper gastrointestinal cancer incidence, desmoid tumor development, timing and type of prophylactic surgery, postoperative outcomes, and long-term survival. Additional objectives include evaluating adherence to surveillance guidelines, timing of genetic diagnosis, and preventive surgical uptake among at-risk relatives. By harmonizing data collection and promoting collaboration among referral centers, RIPAF aims to reduce variability in clinical management across Italy, improve risk stratification and decision-making, and create a national platform to support future multicenter research initiatives and international collaborations in hereditary colorectal cancer syndromes.
NCT04871282
The current study is designed to evaluate the efficacy and safety of AL102 in patients with progressive desmoid tumors.
NCT03190174
This study investigates the safety/toxicity and potential anti-tumor activity of sequential administration of nivolumab and escalating doses of the mammalian target of rapamycin (mTOR) inhibitor nab-rapamycin (ABI-009) in advanced Ewing's sarcoma, perivascular epithelioid cell tumor (PEComa), epithelioid sarcoma, desmoid tumor, chordoma, non-small cell lung cancer, small cell lung cancer, urothelial carcinoma, melanoma, renal cell carcinoma, squamous cell carcinoma of head and neck, hepatocellular carcinoma, classical Hodgkin's lymphoma, high microsatellite instability (MSI-H)/ mismatch repair deficient (dMMR) metastatic colorectal cancer, and tumors with genetic mutations sensitive to mTOR inhibitors.
NCT02076906
The purpose of this study is to determine if Magnetic Resonance guided High Intensity Focused Ultrasound ablative therapy is safe and feasible for children, adolescents, and young adults with refractory or relapsed solid tumors.
NCT01981551
Background: * Desmoid tumors (also known as aggressive fibromatosis), are rare, locally invasive, slow-growing soft-tissue tumors. The disease can be either asymptomatic or be associated with severe loss of organ function and significant morbidity. * Treatment with the selective small-molecule Gamma-secretase inhibitor PF-03084014 caused significant tumor shrinkage in patients with unresectable desmoid tumors in an early phase clinical trial. * The Notch pathway is a key regulator of cell differentiation, proliferation and apoptosis; aberrant signaling via the Notch pathway is associated with carcinogenesis. Objectives: * Primary: Determine the response rate (Complete Response (CR)+Partial Response (PR)) of PF-03084014 in patients with desmoid tumors/aggressive fibromatosis * Secondary: Assess symptom measures at baseline and on study; perform genotyping for germline and somatic mutations in adenomatous polyposis coli gene (APC) and catenin-beta 1 (CTNNB1) genes; correlate clinical response to therapy with genotyping data; and assess modulation of the Notch pathway by evaluating notch response genes in tumor biopsies at baseline and after drug administration Eligibility: * Age greater than or equal to18; histologically confirmed desmoid tumor not amenable to curative resection or definitive radiation therapy that has progressed after receiving at least one line of standard treatment; adequate organ function * Willingness to provide blood samples and 10 unstained slides or a tumor block for genetic research studies Study Design: * This is an open-label Phase II trial of PF-03084014; study drug will be administered orally at 150 mg twice a day in 21-day cycles * Optional tumor biopsies for research purposes will be performed at baseline prior to study treatment and at the beginning of cycle 7 (+/- one cycle) * Restaging scans (magnetic resonance imaging (MRI) with diffusion weighting) will be performed at baseline, at the end of cycles 1 and 6, and then every 6 cycles * Health-related quality of life (HRQOL)/symptom questionnaires will be administered at baseline and at each Clinical Center visit
NCT06195085
This study aimed to explore the long-term outcomes of CT-guided radioactive iodine-125 (I-125) seed implantation (RISI) for recurrent desmoid tumor. A multicenter retrospective study reviewed 38 recurrent desmoid tumor patients receiving RISI from July 2013 to July 2021.The clinical outcomes including overall response rate (ORR), disease control rate (DCR), local control time (LCT), overall survival (OS), adverse events (AEs), and symptom relief rate were statistically assessed.
NCT06113094
Desmoid tumors, also called aggressive fibromatosis, are rare, locally invasive tumors with no potential for metastasis. The incidence is approximately 2 to 4 per million per year in the general population. The "watch and wait" policy is the standard of care for newly diagnosed patients in Europe. Patients who progress have first-line medical treatment, surgery is no longer the treatment because there is a high rate of recurrence (\> 60%) and radiotherapy is not very suitable because the patients are often young and the tumors are large. Cryoablation seems to be an effective therapeutic option that should be evaluated.
NCT00928525
The purpose of this study is to determine whether Imatinib Mesylate is active in diseases - such as Desmoid Tumor and Chondrosarcoma - expressing the receptor for the platelet-derived growth factor (PDGF) both in its isoform alpha and beta
NCT04289077
Rationale: Desmoid-type fibromatosis (DTF) is a rare, histologically benign, soft tissue tumour. Although incapable of metastasizing, the clinical course is unpredictable and can be aggressive because of local invasive growth. Various treatments are available including; surgical resection, radiotherapy, hormonal therapy and chemotherapy. Nowadays there is a trend towards a more conservative strategy with a wait and see policy because of high recurrence rates after surgical resection. Health-related quality of life (HRQL) is a corner stone in treatment choice and can be used during the disease for monitoring the impact of the disease on physical, psychological and social level. Additionally, HRQL can be in important endpoint for future clinical trials. Today, no HRQL-tools are available which capture the needs of DTF patients. The hypothesis is that patients with DTF have issues on several HRQL domains including physical, social and emotional well-being. For this reason we developed a list of items based on previous research. This study aims to evaluate HRQL issues experienced by DTF patients. Purpose: Multi-centre, cross-sectional, observational study to measure HRQL of DTF patients and to evaluate the prevalence of the experienced problems.
NCT05561036
The aim of this study was to evaluate the efficacy and safety of liposomal doxorubicin in the treatment of desmoid tumors. Unless the subject withdraws from the trial voluntarily, or the researcher considers that the subject is not suitable for further trial, each subject will be treated until the disease progresses or the toxic and side effects caused by the drug are intolerable, and then enter the survival follow-up period
NCT00068419
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.
NCT02955446
This is a single-center, open label, non randomized, compassionate use protocol in patients with advanced solid tumor malignancies who were previously enrolled in the phase I study (NCT00878189) of this agent.
NCT00474994
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.
NCT00030680
RATIONALE: Radiation therapy uses high-energy x-rays to damage tumor cells. PURPOSE: Phase II trial to study the effectiveness of radiation therapy in treating patients who have aggressive fibromatoses.
NCT00002595
RATIONALE: Estrogen can stimulate the growth of cancer cells. Hormone therapy using toremifene may fight the growth of desmoid tumors by reducing the production of estrogen. PURPOSE: Phase II trial to study the effectiveness of toremifene in treating patients with desmoid tumors.