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]
1481 clinical trials for this condition and its sub-types, 33 tagged with Neurofibrosarcoma itself.
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New pill targets Hard-to-Treat cancers with missing gene
Disease control Recruiting nowThis study tests an experimental drug called TNG462 for people with advanced solid tumors that have a specific genetic deletion (MTAP). The drug is taken by mouth and aims to slow or stop tumor growth. The trial has two parts: first, finding the safest dose, and then testing that…
Phase 1/2 • Sponsor: Tango Therapeutics, Inc. • Aim: Disease control
Last updated Sep 10, 2026 00:00 UTC
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New drug targets hard-to-treat cancers with missing MTAP gene
Disease control Recruiting nowThis early-stage trial tests an experimental drug called MRTX1719 in adults with advanced solid tumors that have a missing MTAP gene. The study aims to find a safe dose and see if the drug can shrink tumors or slow their growth. About 336 participants with cancers like mesothelio…
Phase 1 • Sponsor: Bristol-Myers Squibb • Aim: Disease control
Last updated Sep 04, 2026 00:00 UTC
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New drug targets cancer spread in first human trial
Disease control Recruiting nowThis early-phase trial tests a new drug called metarrestin in people with advanced solid tumors that have spread (metastasized). The goal is to find a safe dose and see if it can shrink tumors. The study includes adults with pancreatic, breast, or other solid tumors, and children…
Phase 1 • Sponsor: National Cancer Institute (NCI) • Aim: Disease control
Last updated Aug 29, 2026 00:00 UTC
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New drug cocktail takes on rare nerve tumors
Disease control Recruiting nowThis early-phase trial tests a combination of three drugs—selumetinib, ZEN-3694, and durvalumab—in people with advanced sarcomas, including malignant peripheral nerve sheath tumors (MPNST) linked to NF1. The study first checks safety and dosing, then looks at whether the treatmen…
Phase 2 • Sponsor: University of Alabama at Birmingham • Aim: Disease control
Last updated Aug 26, 2026 00:00 UTC
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New drug targets rare nerve tumors in people with specific gene loss
Disease control Recruiting nowThis study tests an experimental drug called BMS-986504 in people with a rare cancer called malignant peripheral nerve sheath tumor (MPNST) that cannot be removed by surgery. Only people whose tumors are missing a gene called MTAP can join. The drug is taken daily by mouth, and t…
Phase 1/2 • Sponsor: Ankit Mangla, MD • Aim: Disease control
Last updated Aug 13, 2026 00:00 UTC
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Personalized cell therapy takes on childhood cancers
Disease control Recruiting nowThis study tests a new treatment called afamitresgene autoleucel in children and young adults aged 2 to 21 with advanced cancers that have a specific marker (MAGE-A4). The treatment uses the patient's own immune cells, modified to attack the cancer. The main goals are to check sa…
Phase 1/2 • Sponsor: USWM CT, LLC • Aim: Disease control
Last updated Aug 13, 2026 00:00 UTC
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New hope for rare childhood cancer: drug cocktail aims to shrink tumors and prevent return
Disease control Recruiting nowThis study is for children and young adults (up to age 30) with a rare type of soft tissue cancer called non-rhabdomyosarcoma. It tests whether adding the drugs pazopanib and selinexor to standard chemotherapy can improve outcomes. Participants receive treatment tailored to their…
Phase 1/2 • Sponsor: St. Jude Children's Research Hospital • Aim: Disease control
Last updated Aug 05, 2026 00:00 UTC
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Can a Two-Drug combo stop nerve tumors from turning cancerous?
Disease control Recruiting nowThis trial is testing whether combining two drugs—selumetinib and APG-115—can help people with neurofibromatosis type 1 (NF1) who have nerve sheath tumors that are either pre-cancerous or already cancerous. The study aims to find a safe dose and see if the combination can shrink …
Phase 1/2 • Sponsor: AeRang Kim • Aim: Disease control
Last updated Jul 29, 2026 00:00 UTC
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New pill for Hard-to-Treat cancers enters first human tests
Disease control Recruiting nowThis early-stage study tests a new oral drug, DEG6498, in about 100 adults with advanced solid tumors that have not responded to standard treatments. The main goals are to find a safe dose and understand side effects. Participants take the drug once daily and are closely monitore…
Phase 1 • Sponsor: Degron Therapeutics Co. • Aim: Disease control
Last updated Jul 02, 2026 00:00 UTC
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Engineered immune cells take aim at childhood cancers
Disease control Recruiting nowThis early-stage trial tests a new immunotherapy for children and young adults up to age 21 with solid tumors that have come back or not responded to standard treatment. The therapy uses the patient's own immune cells, which are modified in a lab to recognize and attack a protein…
Phase 1 • Sponsor: St. Jude Children's Research Hospital • Aim: Disease control
Last updated Jun 27, 2026 12:35 UTC
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Could a cancer drug that blocks a key protein help kids with relapsed wilms tumor?
Disease control Recruiting nowThis phase II trial is testing the drug selinexor in people with Wilms tumor, rhabdoid tumor, MPNST, or other solid tumors that have returned or not responded to treatment. Selinexor works by blocking a protein called XPO1, which is often overactive in these cancers. The study ai…
Phase 2 • Sponsor: Memorial Sloan Kettering Cancer Center • Aim: Disease control
Last updated Jun 27, 2026 12:28 UTC
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New hope for rare cancers: immunotherapy drug targets tumors regardless of origin
Disease control Recruiting nowThis study tests the drug nivolumab in people with advanced rare cancers that have a specific marker (PD-L1). The goal is to see if the drug can shrink or control the tumors when other treatments have stopped working. Up to 28 adults will receive treatment for up to 12 months, an…
Phase 2 • Sponsor: Instituto do Cancer do Estado de São Paulo • Aim: Disease control
Last updated Jun 27, 2026 12:08 UTC
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New gene therapy offers hope for advanced cancers
Disease control Expanded accessThis expanded access program provides DNG64-CAR-V, a gene therapy, to patients with advanced pancreatic cancer, sarcoma, or breast cancer who have exhausted standard treatments. The therapy is given intravenously or directly into tumors, alone or with other cancer drugs. Early st…
Sponsor: Aveni Foundation • Aim: Disease control
Last updated Jun 27, 2026 09:10 UTC
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New hope for rare nerve cancer: drug combo targets genetic flaw
Disease control Recruiting nowThis study tests a drug called ASTX727 in people with a rare type of nerve tumor (MPNST) that has a specific genetic change (PRC2 loss). The drug combines two medicines to better attack cancer cells. About 25 adults and teens whose tumor has grown despite standard treatment will …
Phase 2 • Sponsor: Memorial Sloan Kettering Cancer Center • Aim: Disease control
Last updated Jun 27, 2026 09:02 UTC
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New hope for sarcoma patients: immunotherapy plus chemo shows promise
Disease control Recruiting nowThis study tests a new combination of drugs (tislelizumab, liposomal doxorubicin, and ifosfamide) as a first treatment for people with advanced soft tissue sarcoma that cannot be removed by surgery or has spread. The goal is to see if this combo shrinks tumors and is safe. About …
Phase 2 • Sponsor: Fudan University • Aim: Disease control
Last updated Jun 27, 2026 08:09 UTC
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Pen-Like device could help surgeons detect cancer in real time
Diagnosis Recruiting nowThis study tests whether a handheld device called the MasSpec Pen can tell the difference between cancerous MPNST, benign neurofibromas, and normal tissue during surgery. About 60 people with MPNST or neurofibroma will take part. The goal is to see if the device is safe and accur…
Sponsor: M.D. Anderson Cancer Center • Aim: Diagnosis
Last updated Sep 20, 2026 00:00 UTC
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New study hopes to spot cancer early in NF1 patients
Knowledge-focused Recruiting nowThis study is for people aged 3 and older with neurofibromatosis type 1 (NF1), a genetic condition that often causes noncancerous nerve tumors. About half of people with NF1 get these tumors, and sometimes they turn cancerous. Researchers want to test a new method to predict whic…
Sponsor: National Cancer Institute (NCI) • Aim: Knowledge-focused
Last updated Sep 04, 2026 00:00 UTC
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Global MPNST registry aims to unlock secrets of rare nerve cancer
Knowledge-focused Recruiting nowThis study is building a worldwide database of people with malignant peripheral nerve sheath tumors (MPNST), a rare cancer often linked to the genetic condition neurofibromatosis type 1. Researchers will collect medical records, tumor samples, and genetic data to find patterns th…
Sponsor: Washington University School of Medicine • Aim: Knowledge-focused
Last updated Jun 27, 2026 11:00 UTC