Cylindrospiradenomas in Brooke-Splegler syndrome

Brooke-Spiegler syndrome is a rare autosomal-dominant inherited disease, ch aracterized by the development of multiple cylindromas and trichoepitheliom as. We report a case in which tumors were found on, the head showing histol ogical features of both cylindromas and spiradenomas. These changes were in continuity within the same lesions. Since cylindromas are thought to be ap ocrine in nature, whereas spiradenomas are eccrine, a contiguous growth wit hin the same neoplasm indicates a conflict concerning their histogenesis. T he available histological ultrastructural and immunohistochemical studies h ave not produced any criteria to allow an unequivocal classification, sugge sting instead that both tumors arise from similarly differentiated cells pe rhaps from pluripotent epithelial cells. In our opinion,it seems impractica l to strictly classify cylindromas and spiradenomas into tumors of apocrine or eccrine differentiation. Moreover, at least for those cases of Brooke-S piegler syndrome,where both tumors are found in Immediate vicinity to each other, we propose the term cylindrospiradenoma.