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First described in 1918 by Krompecher, this lesion is believed to be a neural crest derived neoplasm. It’s placement in the PNET family of tumors is controversial. 2/3 of cases occur in the maxilla with other sites being the brain, mandible, and epididymis. Those in the brain have somewhat unique clinical features.
Microscopically, the tumors are composed of two cell types: 1) small neuroblast-like cells and 2) pigmented (melanin) epitheliod cells. The pigmented cells are usually arranged in nests or along clefts in the tumor
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