ReviewCurrent treatment options in oncology2021
Systemic Options for Malignant Peripheral Nerve Sheath Tumors.
Review in Current treatment options in oncology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
21 citing papers in PubMed, 31 citations in OpenAlex.
- Article
- A case report of malignant transformation in neurofibromatosis type 1: pain and rapid growth as key indicators for early biopsy.Frontiers in oncology · 2026Article
- Transformation of a gluteal type 1 plexiform neurofibroma into a high-grade malignant peripheral nerve sheath tumor: a rare case report.Frontiers in oncology · 2026Article
- The Role of Pre-Operative Biopsy in Malignant Peripheral Nerve Sheath Tumours: A Review and Retrospective Series with a Management Algorithm from a Single-Center Experience.Neurology international · 2025Article
- Simultaneous malignant peripheral nerve sheath tumour and malignant melanoma of the anorectal region.International cancer conference journal · 2025Article
- Schwann cells in regeneration and cancer: an epithelial-mesenchymal transition perspective.Open biology · 2025Review
- A Rare Case of Hodgkin Lymphoma With Isolated Epitrochlear Lymphadenopathy. Our Experience and Main Differential Diagnosis.Case reports in medicine · 2025Article
- Phase II Study of Pexidartinib Plus Sirolimus in Unresectable Malignant Peripheral Nerve Sheath Tumors Identifies M2 Macrophage Activation.JCO oncology advances · 2025Article
- Current state of spinal nerve sheath tumor management and future advances.Neuro-oncology advances · 2024Review
- Specifics of spinal neuropathology in the molecular age.Neuro-oncology advances · 2024Review
- Contemporary Approach to Neurofibromatosis Type 1-Associated Malignant Peripheral Nerve Sheath Tumors.American Society of Clinical Oncology educational book. American Society of Clinical Oncology. Annual Meeting · 2024Review
- PRRX1-TOP2A interaction is a malignancy-promoting factor in human malignant peripheral nerve sheath tumours.British journal of cancer · 2024Article
- Chronic kidney disease with malignant peripheral nerve sheath tumor of the ureter: a case report.Frontiers in oncology · 2024Article
- Management of Central and Peripheral Nervous System Tumors in Patients with Neurofibromatosis.Current oncology reports · 2023Review
- Case Report of a Patient With Malignant Peripheral Nerve Sheath Tumor Treated With Wide Resection.Cureus · 2023Article
- Surgical management of craniospinal axis malignant peripheral nerve sheath tumors: a single-institution experience and literature review.World journal of surgical oncology · 2023Review
- RRM2 as a novel prognostic and therapeutic target of NF1-associated MPNST.Cellular oncology (Dordrecht, Netherlands) · 2023Article
- Single-cell multiomics identifies clinically relevant mesenchymal stem-like cells and key regulators for MPNST malignancy.Science advances · 2022Article
- Eliciting an immune-mediated antitumor response through oncolytic herpes simplex virus-based shared antigen expression in tumors resistant to viroimmunotherapy.Journal for immunotherapy of cancer · 2021Article
- Cranial nerve and intramedullary spinal malignant peripheral nerve sheath tumor associated with neurofibromatosis-1.Surgical neurology international · 2021Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
3 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
opinion statementMalignant peripheral nerve sheath tumors (MPNSTs) are rare mesenchymal neoplasms that represent a profound therapeutic challenge due to their high proclivity for recurrence and metastasis and relatively poor response to systemic therapy regimens. While our understanding of the pathophysiology of MPNST is growing, including loss of the tumor suppressor gene neurofibromin and subsequent activation of the Ras pathway, targeted therapy to modify the poor prognosis seen in MPNST patients has thus far been without success. Correspondingly, MPNST patients are treated as per soft tissue sarcoma treatment algorithms with anthracycline-based therapy as the front-line therapy of choice for patients with unresectable, locally advanced, or metastatic MPNST. Beyond first-line anthracycline-based therapy, other standard cytotoxic chemotherapy agents used in advanced MPNST include the alkylating agent ifosfamide and the topoisomerase II inhibitor etoposide. Notably, soft tissue sarcoma regimens are used in MPNST despite distinct systemic therapy sensitivity and prognosis. This is particularly notable for neurofibromatosis type 1 (NF1)-associated MPNST, which is associated with poorer response to systemic therapy and prognosis than sporadic MPNST. As such, NF1-associated MPNST is a particular area in need of novel therapeutic strategies. Given the lack of benefit in the targeting of unique aspects of MPNST disease biology thus far, pre-clinical studies to identify novel rational therapies are critical to inform future clinical trials.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.