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Case of the Month



Vulvar Neurofibroma

The patient is a 41-year-old woman who is seen by her gynecologist for a vulvar lesion which has been present for 6 months. The lesion is polypoid, flesh-colored, nonkeratinizing, and intact. A simple excisional biopsy is performed and the specimen is submitted to pathology. Histologic examination shows a well-circumscribed, nonencapsulated, plexiform proliferation of slender spindle cells in a myxoid stroma. The lesion demonstrates variable cellullity. Scattered throughout the lesion are multinucleate giant cells with nuclei arranged in a wreath-like or linear pattern. Immunoperoxidase stains are positive for S-100 and negative for panCK, SMA, and CD34. The diagnosis is neurofibroma. Literature suggests that the presence of floret-like giant cells in neurofibromas might be a clue to the diagnosis of neurofibromatosis type 1 (NF1).