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Aggressive fibromatosis or desmoid tumor is a rare condition. Desmoid tumors are a type of fibromatosis and related to sarcoma, though without the ability to spread throughout the body (metastasize). The tumors arise from cells called fibroblasts, which are found throughout the body and provide structural support, protection to the vital organs ...
Desmoplastic small-round-cell tumor (DSRCT) is an aggressive and rare cancer that primarily occurs as masses in the abdomen. [4] Other areas affected may include the lymph nodes, the lining of the abdomen, diaphragm, spleen, liver, chest wall, skull, spinal cord, large intestine, small intestine, bladder, brain, lungs, testicles, ovaries, and the pelvis.
The white tumor infiltrates the adjacent skeletal muscle (red tissue – lower left) and fat (yellow tissue – upper left). This tendency for invasion of adjacent normal tissues and structures is the reason that desmoid-type fibromatosis has a relatively high rate of local recurrence, even after surgical removal. Specialty: Oncology
The FDA approval was supported by data from a late-stage trial, which tested 142 adults with progressing desmoid tumors, where the treatment showed a 71% lower risk of disease progression or death ...
The progression-free survival results were also supported by an assessment of patient-reported pain favoring the nirogacestat arm. [2] As of 2021, nirogacestat was in phase II clinical trials for unresectable desmoid tumors.
The Desmoid Tumor Research Foundation (DTRF) is an American non-profit organization which funds research on desmoid tumors (aggressive fibromatosis), a rare soft-tissue tumor affecting 5–6 people per million per year. [1] Its goal is to improve treatments and find a cure. [2] The organization was founded in 2005 by Marlene Portnoy and Jeanne ...
Gardner syndrome is an autosomal dominant form of polyposis characterized by the presence of multiple polyps in the colon together with tumors outside the colon. [3] The extracolonic tumors may include osteomas of the skull, thyroid cancer, epidermoid cysts, fibromas, [4] as well as the occurrence of desmoid tumors in approximately 15% of ...
Post-treatment local recurrences and metastases were observed in 15% and 38% of cases; 5- and 10-year overall survival rates were 60% and 48%, respectively; Overall median survival time were 10.1 years; and patients with tumors ≥10 cm in longest diameter had an almost 6-fold higher rate of developing metastases than patients with tumors 4 cm ...