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Brooke-Spiegler Syndrome

Report of a Case with Combined Lesions Containing Cylindromatous, Spiradenomatous, Trichoblastomatous, and Sebaceous Differentiation
Authors: Dmitry V, Kazakov; Roman, Soukup; Petr, Mukensnabl; Ludmila, Boudova; Michal, Michal;
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.

Related Organizations
Keywords

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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Powered by OpenAIRE graph
citations
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
103
Top 10%
Top 1%
Top 10%