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A woman age 79 years is referred for evaluation of a growth on her leg. The lesion is asymptomatic and she is uncertain as to duration. History is negative for skin cancer or other malignancy. Examination reveals a 0.8-cm flesh-colored firm papule. No similar appearing lesions are noted elsewhere.
By Stephen Schleicher, MD
Dr. Schleicher is director of the DermDox Center for Dermatology in Hazleton, Pennsylvania, as well as an Associate Professor of Medicine at the Commonwealth Medical College and a Clinical Instructor of dermatology at Arcadia University in Glenside, Pennsylvania, and at Kings College in Wilkes-Barre, Pennsylvania.
A biopsy was performed, and histopathology revealed a cylindroma. Cylindromas present as papules or nodules most commonly arising on the scalp. Multiple growths in this location lead to cosmetic disfigurement and are referred to as turban tumors. The condition is...
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A biopsy was performed, and histopathology revealed a cylindroma. Cylindromas present as papules or nodules most commonly arising on the scalp. Multiple growths in this location lead to cosmetic disfigurement and are referred to as turban tumors. The condition is caused by a mutation in the CYLD gene and has variable penetrance.1,2 They may be seen in Brooke-Spiegler syndrome along with spiradenomas and trichoepitheliomas.3
Cylindromas occurring outside of the scalp are extremely rare.4 Malignant transformation, referred to as cylindrocarcinoma, is also rare. This neoplasm enlarges rapidly, ulcerates, and may metastasize.5,6 Surgical excision of an isolated cylindroma is curative.
References
- Brummelkamp TR, Nijman SM, Dirac AM, Bernards R. Loss of the cylindromatosis tumour suppressor inhibits apoptosis by activating NF-kappaB. Nature. 2003;424:797-801.
- Kovalenko A, Chable-Bessia C, Cantarella G, Israel A, Wallach D, Courtois G. The tumour suppressor CYLD negatively regulates NF-kappaB signalling by deubiquitination. Nature. 2003;424:801-805.
- Kazakov DV, Soukup R, Mukensnabl P, Boudova L, Michal M. Brooke-Spiegler syndrome: report of a case with combined lesions containing cylindromatous, spiradenomatous, trichoblastomatous, and sebaceous differentiation. Am J Dermatopathol. 2005;27:27-33.
- Baden HP. Cylindromatosis simulating neurofibromatosis. N Engl J Med. 1962;267:296-297.
- Gerretsen AL, van der Putte SC, Deenstra W, van Vloten WA. Cutaneous cylindroma with malignant transformation. Cancer. 1993;72:1618-1623.
- Borik L, Heller P, Shrivastava M, Kazlouskaya V. Malignant cylindroma in a patient with Brooke-Spiegler syndrome. Dermatol Pract Concept. 2015;5(2):61-65.
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