Brooke-Spiegler Syndrome: A Rare Entity
نویسندگان
چکیده
Brooke-Spiegler syndrome is a rare entity. It is an autosomal dominant syndrome in which multiple trichoepitheliomas, cylindromas, or other adnexal tumors are seen. Very few cases of Brooke-Spiegler syndrome are reported in the literature. We came across a 40 -year-old female in which multiple trichoepitheliomas and cylindromas were seen on scalp. In view of clinical history and histopathological examination it was diagnosed as Brooke-Spiegler syndrome. We report this case because of its rarity.
منابع مشابه
Brooke-Spiegler Syndrome: a case report
Brooke-Spiegler syndrome is a rare autosomal recessive disease characterized by adnexal neoplasms, particularly trichoepithelioma, cylindroma, and occasionally spiradenoma, which usually develop in second to third decades of life. We report this syndrome in a 16-year-old woman with tumors on face and scalp.
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Page 19 Abstract Brooke-Spiegler syndrome (BSS) is a rare, inherited, autosomal-dominant genodermatosis characterized by the development of multiple adnexal cutaneous tumors including spiradenomas, cylindromas, spiradenocylindromas, trichoepitheliomas, epidermoid cysts, and milia. We present a case of Brooke-Spiegler syndrome with possible malignant transformation of a benign tumor. Brooke-Spie...
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BrookeSpiegler syndrome is an uncommon autosomal dominant disorder, only few cases have been reported. Herein, we report a patient with this rare condition. A 26 year old woman with Brooke-Spiegler syndrome (BSS) has multiple cutaneous adenxal neoplasms on her face and scalp. Clinical features, histopathogical spectrum, pathogenesis, malignant transformation and treatment modalities are reviewe...
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Malignant cylindroma (cylindromatous carcinoma, cylindrocarcinoma) is the malignant counterpart of benign cylindroma. It is a rare neoplasm, more often developing in the setting of multiple preexisting benign neoplasms. Herein we present an additional case of malignant transformation of the cylindroma diagnosed in an 83-year-old patient with known Brooke-Spiegler syndrome.
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Brooke-Spiegler Syndrome (BSS) is a rare genodermatosis characterized by the progressive formation of adnexal skin tumors in the scalp and face, mainly trichoepitheliomas, cylindromas, and spiradenomas. It has also been associated with salivary glands neoplasms. It is due to mutations in the tumor suppressor gene cylindromatosis (CYLD gene) localized on chromosome 16q12-q13. Around 93 mutations...
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عنوان ژورنال:
دوره 2014 شماره
صفحات -
تاریخ انتشار 2014