Locally aggressive granular cell tumor causing priapism of the crus of the clitoris. A light and ultrastructural study, with observations concerning the pathogenesis of fibrosis of the corpus cavernosum in priapism.
A case of focal priapism of the clitoris caused by a microscopic granular cell tumor (GCT) is described. This neoplasm is considered locally aggressive because it invades the lumens of peripheral cavernous sinuses of the crus of the clitoris. Caverns adjacent to those invaded by tumor exhibit stasis, telangiectasia, and necrosis of the smooth muscle of the trabecular wall. These alterations lead to telescoping collapse and compression of the cavernous spaces and culminate in fibrosis. Ultrastructurally, replicated basal lamina is found surrounding clusters of granular cells. We suspect that the multilayered lamina, in addition to being produced by tumor cells, is derived from the trabecular endothelium surrounding the caverns invaded by the GCT. The replication of the basal lamina may be provoked by cycles of injury and repair to these vessels caused by repeated episodes of prolonged vascular stasis. A peculiar large vein with perforating branches was observed in the center of the cavernous spaces of the crus. This vein is not found in normal crura and, therefore, represents a morphologic adaptation created to drain the cavernous spaces.
D000328:Adult; D002987:Clitoris; D005260:Female; D006801:Humans; D007124:Immunoenzyme Techniques; D008297:Male; D008875:Middle Aged; D009379:Neoplasms, Muscle Tissue / Q000150:complications / Q000276:immunology / Q000473:pathology*; D011317:Priapism / Q000209:etiology*; D014846:Vulvar Neoplasms / Q000150:complications / Q000276:immunology / Q000473:pathology*