ArticleVirchows Archiv : an international journal of pathology2022
S100-negative epithelioid malignant peripheral nerve sheath tumor with possible perineurial differentiation.
Article in Virchows Archiv : an international journal of pathology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.
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Who cites it
6 citing papers in PubMed, 7 citations in OpenAlex.
- Case Report: Partial neurectomy and limb-sparing treatment for SOX-10 expressing epithelioid malignant nerve sheath tumour of the tibial nerve.Frontiers in veterinary science · 2026Article
- Rapidly progressive spinal epithelioid malignant peripheral nerve sheath tumor following subtotal resection surgery: a case report.Discover oncology · 2025Article
- From benign neurofibromas to malignant peripheral nerve sheath tumors (MPNST): a gaming among multiple factors.Cellular oncology (Dordrecht, Netherlands) · 2025Review
- Malignant perineurioma derived from the retroperitoneum with an aggressive clinical course: a case report.Surgical case reports · 2024Article
- Cardiac tumor comprising a malignant peripheral nerve sheath tumor and spontaneous atrial osseous metaplasia in a sheep.The Journal of veterinary medical science · 2024Article
- The intriguing perineurial cells - an updated overview of their origin, structure, functions and implication in pathology.Romanian journal of morphology and embryology = Revue roumaine de morphologie et embryologieReview
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Authors and funding
13 authors at 7 institutions in 1 country.
Funding
Abstract
Epithelioid malignant peripheral nerve sheath tumor (MPNST) is a rare subtype of MPNST composed of epithelioid cells with abundant cytoplasm. Currently, strong and diffuse immunostaining for S100 protein and SOX10 is generally regarded as a characteristic feature of epithelioid MPNST. However, malignant tumors with epithelioid morphology that arise from a peripheral nerve or a pre-existing benign nerve sheath tumor should be regarded as epithelioid MPNSTs when they do not show characteristic features that definitively lead to other specific diagnoses. Here, we describe 3 cases of epithelioid MPNST in the peripheral nerve or schwannoma that was negative for S100 protein and SOX10 expression. Instead, these tumors were positive for EMA, GLUT1, claudin 1, and cytokeratin to varying degrees, while all of them retained SMARCB1 and H3K27me3 by immunohistochemistry. EMA, GLUT1, and claudin 1 are known markers of perineurial cell differentiation; thus, they could possibly represent epithelioid MPNST with perineurial cell differentiation.
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Registered trials
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