Neurofibrosarcoma
MONDO:0002675A malignant tumor that arises from small cutaneous nerves, is locally aggressive, and has a potential for metastasis. Characteristic histopathologic features include proliferating atypical spindle cells with slender wavy and pointed nuclei, hypocellular areas, and areas featuring organized whorls of fibroblastic proliferation. The most common primary sites are the extremities, retroperitoneum, and trunk. These tumors tend to present in childhood, often in association with neurofibromatosis 1. (From DeVita et al., Cancer: Principles & Practice of Oncology, 5th ed, p1662; Mayo Clin Proc 1990 Feb;65(2):164-72)
Also known as: neurosarcoma [obs]
1475 clinical trials for this condition and its sub-types, 33 tagged with Neurofibrosarcoma itself.
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New combo therapy shows promise for rare sarcomas
Disease control CompletedThis study looked at adding the drug pazopanib to standard radiation or chemoradiation before surgery for patients with certain soft tissue sarcomas. The goal was to see if the combination is safe and helps shrink tumors more effectively. About 140 adults and children with newly …
Phase 2/3 • Sponsor: National Cancer Institute (NCI) • Aim: Disease control
Last updated Jun 27, 2026 14:00 UTC
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Immunotherapy boosts chemo against rare sarcomas?
Disease control CompletedThis study tested whether adding an immunotherapy drug called APX005M to standard chemotherapy (doxorubicin) could help shrink tumors in people with advanced soft tissue sarcoma. 27 adults with certain sarcoma types took part. The goal was to see if the combination was safe and w…
Phase 2 • Sponsor: Alexander Z. Wei, MD • Aim: Disease control
Last updated Jun 27, 2026 13:07 UTC
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New drug combo shows promise for rare nerve tumors
Disease control CompletedThis phase 2 trial tested two drugs, selumetinib and sirolimus, in 21 people with advanced malignant peripheral nerve sheath tumors (MPNST), a rare cancer often linked to neurofibromatosis type 1. The goal was to see if the combination could shrink or stabilize tumors. The study …
Phase 2 • Sponsor: Sarcoma Alliance for Research through Collaboration • Aim: Disease control
Last updated Jun 27, 2026 12:38 UTC
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New scan and genetic tests aim to catch cancer early in NF1 patients
Knowledge-focused CompletedThis study looked at people with neurofibromatosis type 1 (NF1) to better understand how harmless tumors (plexiform neurofibromas) turn into a rare cancer called MPNST. Ten participants had MRI, PET scans, and tumor biopsies. Researchers tested whether a special PET scan (FLT PET…
Phase 1 • Sponsor: National Cancer Institute (NCI) • Aim: Knowledge-focused
Last updated Aug 06, 2026 00:00 UTC