The patient is an 86-year-old woman who presents for evaluation of a growth on her scalp. The lesion has been present for several years and occasionally bleeds when traumatized by combing. Past medical history is positive for hypothyroidism and chronic obstructive pulmonary disease, and she is a former smoker. Examination reveals a 1.2-cm erythematous, well-demarcated nodule.
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Cylindroma is a rare neoplasm that most commonly occurs on the head and neck of middle-aged and elderly women. The lesion is asymptomatic and may range in hue from flesh-colored to erythematous. Solitary cylindromas present sporadically, while multiple tumors are observed in an autosomal dominantly inherited manner; the condition is referred to as familial cylindromatosis.1
This genodermatosis results from a mutation in the CYLD gene on chromosome 16q, which encodes a tumor suppressor enzyme.2 The histopathology of cylindroma is characteristic, and lesions demonstrate irregular isles comprising basaloid cells surrounded by a hyaline eosinophilic sheath.3 Immunohistochemical markers indicate derivation from eccrine and apocrine glands.
Malignant transformation is rare and more commonly seen as a complication of the multiple variant.4 Excision is curative.
Stephen Schleicher, MD, is an associate professor of medicine at the Commonwealth Medical College in Scranton, Pennsylvania, and an adjunct assistant professor of dermatology at the Perelman School of Medicine at the University of Pennsylvania in Philadelphia. He practices dermatology in Hazleton, Pennsylvania.
- Lian F, Cockerell CJ. Cutaneous appendage tumors: familial cylindromatosis and associated tumors update. Adv Dermatol. 2005;21:217-234.
- Bignell GR, Warren W, Seal S, et al. Identification of the familial cylindromatosis tumour-suppressor gene. Nat Genet. 2000;25(2):160-165.
- Klein W, Chan E, Seykora JT. Tumors of the epidermal appendages. In: Elder DE, Elenitsas R, Johnson BL, et al, eds. Lever’s Histopathology of the Skin. 9th ed. Philadelphia: Lippincott Williams & Wilkins; 2005:897-898.
- Gerretsen AL, van der Putte SC, Deenstra W, van Vloten WA. Cutaneous cylindroma with malignant transformation. Cancer. 1993;72(5):1618-1623.