Multiple familial trichoepitheliomas: a folliculosebaceous-apocrine genodermatosis.
We reviewed the pathologic findings on a family with multiple hereditary trichoepitheliomas. Although the majority of the lesions were trichoepitheliomas, basal cell carcinomas, spiradenomas, and spiradenomas with cylindromatous foci (spiradenocylindroma) were present, representing a spectrum of lesions exhibiting folliculosebaceous (trichoepithelioma, basal cell carcinoma) and apocrine (spiradenoma, spiradenocylindroma) differentiation. Multiple familial trichoepitheliomas may be a syndrome whereby tumors develop from undifferentiated germinative cells of the folliculosebaceous-apocrine unit. Published findings regarding the genetics of this syndrome and solitary trichoepitheliomas are reviewed; although the molecular basis for the tumors has yet to be determined, current data suggest that a tumor suppressor gene may be involved.
D001050:Apocrine Glands / Q000473:pathology*; D002280:Basal Cell Carcinoma / Q000235:genetics / Q000473:pathology; D004439:Eccrine Glands / Q000473:pathology*; D005192:Family Health; D005260:Female; D018859:Hair Follicle / Q000473:pathology; D006801:Humans; D008297:Male; D018294:Neoplasms, Adnexal and Skin Appendage / Q000235:genetics / Q000473:pathology*; D009378:Neoplasms, Multiple Primary / Q000235:genetics / Q000473:pathology*; D012878:Skin Neoplasms / Q000235:genetics / Q000473:pathology*