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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Immunotherapy duo takes on rare tumors
Disease control OngoingThis phase 2 trial is testing a combination of two immunotherapy drugs, nivolumab and ipilimumab, in 798 people with over 50 types of rare cancers. The goal is to see if these drugs can shrink tumors by helping the immune system attack cancer cells. Participants receive the drugs…
Phase 2 • Sponsor: National Cancer Institute (NCI) • Aim: Disease control
Last updated Jun 27, 2026 14:00 UTC
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New drug hope for rare nerve cancer: tazemetostat under study
Disease control OngoingThis phase 2 trial tests the drug tazemetostat in 10 people with recurrent, treatment-resistant, or metastatic malignant peripheral nerve sheath tumors (MPNST), a rare and aggressive cancer. Participants take tazemetostat pills twice daily for up to 2 years. The main goal is to s…
Phase 2 • Sponsor: University of Florida • Aim: Disease control
Last updated Jun 27, 2026 13:03 UTC
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Engineered immune cells take aim at childhood cancers in early trial
Disease control OngoingThis early-phase study tests a personalized cell therapy for children and young adults (up to age 26) with solid tumors that have come back or not responded to standard treatment. The therapy uses the patient's own immune cells, modified to recognize and attack a protein called B…
Phase 1 • Sponsor: Seattle Children's Hospital • Aim: Disease control
Last updated Jun 27, 2026 12:25 UTC
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Experimental combo aims to boost immune attack on sarcoma before surgery
Disease control OngoingThis early-phase study tests the safety of combining two immunotherapy drugs—nivolumab and BO-112—given before surgery for soft tissue sarcoma that can be removed. About 14 adults with certain sarcoma types will receive these drugs to see if they help the immune system fight the …
Phase 1 • Sponsor: Jonsson Comprehensive Cancer Center • Aim: Disease control
Last updated Jun 27, 2026 11:00 UTC
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Engineered immune cells take on childhood cancers in first human test
Disease control OngoingThis early-stage trial tests a new type of immunotherapy for children and young adults with solid tumors that have not responded to standard treatments. The therapy uses the patient's own immune cells (T cells) that are genetically modified to recognize and attack cancer cells ca…
Phase 1 • Sponsor: Seattle Children's Hospital • Aim: Disease control
Last updated Jun 27, 2026 07:59 UTC
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New hope for tough chest tumors: targeted radiation trial launches
Disease control By invitation onlyThis study tests a new type of radiation therapy called BNCT for people with chest tumors that have come back and cannot be removed by surgery or treated with standard methods. About 30 participants will receive a special boron drug followed by neutron radiation to target cancer …
Phase 1/2 • Sponsor: Stella Pharma Corporation • Aim: Disease control
Last updated Jun 27, 2026 07:58 UTC
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10-Year NF1 study aims to unlock secrets of rare genetic disorder
Knowledge-focused OngoingThis natural history study follows 259 children, adolescents, and adults with Neurofibromatosis Type 1 (NF1) for up to 10 years. Researchers will track tumor growth, monitor quality of life, and perform genetic testing to better understand how the disease changes over time. The g…
Sponsor: National Cancer Institute (NCI) • Aim: Knowledge-focused
Last updated Sep 20, 2026 00:00 UTC
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Blood test could spot rare nerve tumors years earlier in NF1 patients
Knowledge-focused By invitation onlyThis study is testing whether a blood test (liquid biopsy) can detect a rare but serious nerve tumor called MPNST earlier than current methods in adults with Neurofibromatosis Type 1 (NF1). Researchers will compare the timing of tumor detection by the blood test versus standard i…
Sponsor: David Miller • Aim: Knowledge-focused
Last updated Sep 02, 2026 00:00 UTC
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New study aims to predict cancer risk in NF1 patients before it strikes
Knowledge-focused OngoingThis study follows 80 people with Neurofibromatosis type 1 (NF1) who are at high risk for developing malignant peripheral nerve sheath tumors (MPNST). Researchers will use yearly whole-body MRI scans, blood samples, and checkups to track changes in nerve lesions over time. The go…
Sponsor: Johns Hopkins University • Aim: Knowledge-focused
Last updated Jun 27, 2026 08:10 UTC