CASE #1: Cylindroma

A cylindroma is a benign neoplasm of adnexal origin. The lesion’s precise origin is a matter of debate; sometimes cylindromas are considered eccrine in origin, but most often, they are thought to be of apocrine derivation.

Cylindromas can occur singly (as was the case in our patient), but they can also present as multiple nodules. The most common location is the head and neck area, particularly the scalp, but other locations, such as the trunk or genitalia, have been reported. Cylindromas are more commonly seen in females than males. When multiple cylindromas form on the scalp, they can coalesce and create an appearance that has been referred to as a “turban” tumor. No clinical features are considered pathognomonic of a cylindroma, and oftentimes, the diagnosis is made after biopsy. 

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Multiple cylindromas can be seen in Brooke-Spiegler syndrome, an autosomal dominant condition caused by a defect in the CYLD gene, a negative regulator of nuclear factor-kB signaling. In Brooke-Spiegler syndrome, cylindromas occur in association with trichoepitheliomas and spiradenomas. 

Another disorder with multiple cylindromas is Rasmussen syndrome. This autosomal dominant condition consists of multiple cylindromas, trichoepitheliomas, and milia. 

When subjected to pathologic examination, cylindromas have a distinctive appearance: Sharply circumscribed basaloid nodules are closely apposed in a pattern described as “jigsaw puzzlelike.” A rim of eosinophilic, basement membranelike material that stains positive for periodic acid-Schiff (PAS) surrounds each of the basaloid nests. In addition, the basaloid nests frequently contain eosinophilic droplets, which also stain positive for PAS.

Treatment consists of complete excision of the tumor. Although our patient had experienced recurrence after the initial tumor was removed, recurrence is uncommon. The tumor was excised, with good results.