Abstract
Malignant cylindroma (MC) is an extremely rare adnexal tumor, firstly described by Wiedemann in 1929 with less than 40 documented cases in literature. It is a high-grade neoplasm with aggressive behavior, characterized by locally infiltrative growth and metastatic potential. Histopathologically, the diagnosis is possible only on the basis of the presence of foci of benign preexisting cylindroma. Malignant transformation may arise in solitary cylindromas or, more commonly, in the setting of the autosomal dominant Brooke-Spiegler syndrome, characterized by the development of adnexal tumors, mostly cylindromas, but also trichoepitheliomas and spiradenomas. Etiopathogenesis is controversial, but some reported cases have been described following radiotherapy, and mutations of the tumor suppressor gene CYLD have been demonstrated in Brooke-Spiegler syndrome.
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Caputo, V., Rongioletti, F. (2015). Malignant Cylindroma. In: Rare Malignant Skin Tumors. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-2023-5_20
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DOI: https://doi.org/10.1007/978-1-4939-2023-5_20
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