Turban Tumor

A 79-year-old man was brought to the emergency department with mental status changes. A CT scan revealed a lacunar infarct. Numerous asymptomatic nodules had been present for many years on the patient’s scalp and forehead (A). The 6-mm to 3-cm pink lesions were nontender, smooth, and rubbery; they did not invaginate when pressed. Based on the clinical presentation, Drs Darron D. DiGiulio and Yelva Lynfield of Far Rockaway, NY, diagnosed turban tumor—multiple cylindromas that involve the scalp. Disease severity can range from a few isolated nodules to diffuse lesions that disfigure the entire scalp. It is thought that a cylindroma represents an immature sweat gland epithelioma of eccrine and apocrine origin.^l Typically, cylindromas are smooth, rubbery, domeshaped nodules that vary in size from a few millimeters to several centimeters.² Surface telangiectases are often present. Cylindromas commonly occur initially on the scalp and face of young adults; the trunk and extremities may also be involved. Single lesions are most common and are not inherited. ³ The manifestation of multiple lesions that characterizes the turban tumor is an autosomal dominant disorder with nearly 100% penetrance in adulthood.⁴ This patient’s brother had the same scalp condition. Multiple cylindromas may be associated with multiple trichoepithelioma, milia, and eccrine spiradenoma. The differential diagnosis includes epidermoid cyst, trichoepithelioma, syringoma, basal cell carcinoma, and neurofibroma. A biopsy specimen from a nodule (B) shows dermal tumor islands of epithelial cells that are surrounded and penetrated by a characteristic hyaline sheath. The islands, which appear to fit together like pieces of a jigsaw puzzle, are composed of 2 cell types⁵:

• Small cells with dark nuclei that occur at the periphery and often palisade.
• Larger cells with lighter nuclei that occur more centrally.

Lesions increase in size and number throughout life. Treatment consists of surgical excision. Cylindromas are considered benign; however, rare cases of malignant degeneration in single and multiple lesions have been reported.⁶