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Malignant nerve sheath tumor containing endocrine cells.

T F Warner, R Louie, G R Hafez

    The American Journal of Surgical Pathology
    |September 1, 1983
    PubMed
    Summary

    A rare malignant nerve sheath tumor in a patient with von Recklinghausen's neurofibromatosis showed unique glandular and endocrine features. This complex tumor contained somatostatin-immunoreactive cells, suggesting a neural crest origin.

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    Area of Science:

    • Oncology
    • Pathology
    • Neuroscience

    Background:

    • Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive neoplasms.
    • Von Recklinghausen's neurofibromatosis (NF1) is a genetic disorder predisposing to MPNSTs.
    • Glandular differentiation in MPNSTs is exceptionally rare.

    Observation:

    • A 29-year-old male with NF1 presented with a thigh mass.
    • Histopathological examination revealed a malignant nerve sheath tumor.
    • The tumor exhibited chondroid foci, rhabdomyoblasts, mucus-containing acini, and argyrophil cells.

    Findings:

    • Ultrastructural analysis identified endocrine-type granules in epithelial cells.
    • Somatostatin-immunoreactive cells were detected within the acinar epithelium.

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  • This case represents a unique "glandular schwannoma" with somatostatin expression.

    • Implications:

    • The presence of somatostatin-immunoreactive cells adds a novel dimension to glandular schwannomas.
    • Suggests a complex neural crest origin for this specific type of tumor.
    • Highlights the diverse differentiation potential of nerve sheath tumors in NF1.