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Melanotic schwannoma of the vulva: A case report and review of the literature

The American Journal of Dermatopathology Jan 08, 2020

Cohen JN, et al. - Researchers presented the first clinicopathologic report of a vulvar melanotic schwannoma (pigmented tumor of peripheral nerve differentiation) in a 34-year-old woman and reviewed the literature concerning cutaneous exhibitions of melanotic schwannoma. In this case, microscopically, a dermal-based, heavily pigmented proliferation of plump spindled and epithelioid cells arrayed in nodules and fascicles was observed. They noted positive staining of lesional cells for S100, Melan-A, and BAP1 but negative staining for Prkar1α. As per next-generation sequencing of a panel of 480 cancer-associated genes, the tumor harbored a PRKAR1A p.S299fs truncating mutation and copy neutral loss of heterozygosity of chromosome 17q, the locus at which PRKAR1A resides. Based on combined histopathologic, immunohistochemical, and genetic characteristics, a diagnosis of melanotic schwannoma was provided in this case. Finding suggests that melanotic schwannian tumors belonging to the skin might behave more indolently in comparison to noncutaneous counterparts.
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