title: Brooke-Spiegler syndrome: report of a case with combined lesions containing cylindromatous, spiradenomatous, trichoblastomatous, and sebaceous differentiation.
abstract: Brooke-Spiegler syndrome is an autosomal dominantly inherited disease with predisposition to cutaneous adnexal neoplasms, most commonly cylindromas and trichoepitheliomas. We report a patient in whom 11 lesions were removed from the scalp and face for various reasons over a period of 3 years. The histopathological survey revealed a plethora of benign adnexal neoplasms showing apocrine, follicular, and sebaceous differentiation occurring independently and conjointly. The histopathological spectrum in our patient included cylindromas, spiradenomas, trichoepitheliomas, small nodular trichoblastomas, and lymphadenomas. Many lesions had hybrid features of two or more neoplasms. By far the most common composite tumor was spiradenocylindroma. Some spiradenocylindromas demonstrated prominent sebaceous or trichoblastomatous differentiation or both. We suggest the terms "sebaceous spiradenocylindroma" and "trichospiradenocylindroma" for these lesions. The occurrence of sebaceous and trichoblastic differentiation in spiradenocylindromas is a further proof that spiradenoma and cylindroma are not eccrine tumors but neoplasms of the folliculosebaceousapocrine unit.
authors.affiliation: Sikl's Department of Pathology, Charles University, Medical Faculty Hospital, Pilsen, Czech Republic.
authors.forename: Dmitry V
authors.initials: DV
authors.lastname: Kazakov
authors.forename: Roman
authors.initials: R
authors.lastname: Soukup
authors.forename: Petr
authors.initials: P
authors.lastname: Mukensnabl
authors.forename: Ludmila
authors.initials: L
authors.lastname: Boudova
authors.forename: Michal
authors.initials: M
authors.lastname: Michal
mesh_terms: D006607:Adenoma, Sweat Gland / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D000368:Aged; D014408:Biomarkers, Tumor / Q000378:metabolism; D003528:Carcinoma, Adenoid Cystic / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D018280:Carcinoma, Skin Appendage / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D006201:Hair Diseases / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D018859:Hair Follicle / Q000378:metabolism / Q000473:pathology; D006801:Humans; D007124:Immunoenzyme Techniques; D008297:Male; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D009386:Neoplastic Syndromes, Hereditary / Q000473:pathology*; D012627:Sebaceous Glands / Q000378:metabolism / Q000473:pathology; D012878:Skin Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D013577:Syndrome
chemical_list: D014408:Biomarkers, Tumor