Brooke-Spiegler Syndrome
pmid: 15677973
Brooke-Spiegler Syndrome
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.
- Charles University Czech Republic
Male, Skin Neoplasms, Adenoma, Sweat Gland, Carcinoma, Skin Appendage, Syndrome, Carcinoma, Adenoid Cystic, Immunoenzyme Techniques, Neoplasms, Multiple Primary, Sebaceous Glands, Neoplastic Syndromes, Hereditary, Biomarkers, Tumor, Humans, Hair Diseases, Hair Follicle, Aged
Male, Skin Neoplasms, Adenoma, Sweat Gland, Carcinoma, Skin Appendage, Syndrome, Carcinoma, Adenoid Cystic, Immunoenzyme Techniques, Neoplasms, Multiple Primary, Sebaceous Glands, Neoplastic Syndromes, Hereditary, Biomarkers, Tumor, Humans, Hair Diseases, Hair Follicle, Aged
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