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    A case of fibroblastic low-grade malignant peripheral nerve sheath tumor--a true neurofibrosarcoma.

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    Authors
    Houreih, Mohammad Adib
    Eyden, Brian P
    Deolekar, Madhuri
    Banerjee, Saumitra S
    Affiliation
    Department of Histopathology, Christie Hospital, Manchester, UK.
    Issue Date
    2007
    
    Metadata
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    Abstract
    The authors report a case of low-grade retroperitoneal malignant peripheral nerve sheath tumor (MPNST) showing Schwannian and fibroblastic differentiation in individual tumor cells. The tumor was detected in a 29-year-old male and posed diagnostic difficulty because of the unusual morphologic and immunophenotypic features. Morphologic examination of the H&E sections revealed a rather circumscribed, highly vascular, moderately cellular spindle cell tumor. The neoplastic cells were arranged in vague, short fascicles, distributed haphazardly amid hemangiopericytoma-like vascular channels, and showed occasional whorls. Myxoid stroma and keloid-like collagen bundles were frequently seen. There were satellite nodules outside the main tumor mass and low mitotic activity but no necrosis. The tumor cells stained strongly and diffusely for both S-100 protein and CD34. Electron microscopy revealed cells with processes and focal lamina, and prominent rough endoplasmic reticulum. Although the capacity of MPNST to exhibit divergent differentiation is well known, fibroblastic differentiation is generally poorly and inconsistently documented. The present case represents an unambiguous demonstration of the co-expression within individual tumor cells of Schwannian and fibroblastic differentiation in a low-grade MPNST. The literature on this subject is reviewed.
    Citation
    A case of fibroblastic low-grade malignant peripheral nerve sheath tumor--a true neurofibrosarcoma., 31 (5):347-56 Ultrastruct Pathol
    Journal
    Ultrastructural Pathology
    URI
    http://hdl.handle.net/10541/70044
    DOI
    10.1080/01913120701577827
    PubMed ID
    17963184
    Type
    Article
    Language
    en
    ISSN
    1521-0758
    ae974a485f413a2113503eed53cd6c53
    10.1080/01913120701577827
    Scopus Count
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