brooke-spiegler syndrome
نویسندگان
چکیده
introduction : brooke-spiegler syndrome (bss) is a rare autosomal dominant disease characterized by the development of multiple adnexal cutaneous tumors such as cylindromas, trichoepitheliomas and spiradenomas. case description: a 49 years old female patient presented with a history of gradual appearance of multiple nodules situated predominantly on the scalp and face. pathologic examination of lesions revealed cylindroma with foci of spiradenoma. discussion: brooke-spiegler syndrome (bss) caused by various mutations of the cylindromatosis gene (cyld), a tumor suppressor gene resulting in multiple cylindromas and trichoepitheliomas. malignant transformation may develop within solitary cylindromas, but more common in multiple variant and requires careful followup surveillance.
منابع مشابه
Brooke-spiegler syndrome
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...
متن کاملBrooke-Spiegler syndrome.
The Brooke-Spiegler syndrome (BSS) is an uncommon autosomal dominant disorder characterized by a high affinity to form multiple adnexal neoplasia (skin appendage tumors), especially trichoepitheliomas and cylindromas, and occasionally spiradenomas, which usually appear in the second or third decade of life. To date, only a few cases with this syndrome have been reported. This case report descri...
متن کاملBrooke-Spiegler Syndrome: A Case Report
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...
متن کاملBrooke-Spiegler Syndrome: A Rare Entity
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 histopathologic...
متن کاملBrooke-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.
متن کاملMalignant cylindroma in a patient with Brooke-Spiegler syndrome
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.
متن کاملمنابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
basic and clinical cancer researchجلد ۳، شماره ۱، صفحات ۶۵-۶۹
میزبانی شده توسط پلتفرم ابری doprax.com
copyright © 2015-2023