A 66-year-old woman is requesting evaluation of a growth on her scalp, which she first noted approximately 1 year ago. Her medical history includes removal of several other facial and scalp lesions: 2 basal cell carcinomas, 1 cylindroma, and several trichoepitheliomas. Physical examination reveals a firm, pink-colored, 1.2-cm nodule on her scalp that is unmovable and compressed minimally on palpation.
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Cylindroma is a rare, benign lesion that most commonly occurs on the head and neck of middle-aged and elderly women. Diagnosis is made by histology, which reveals a characteristic pattern of irregular tumor islands composed of basaloid cells encased within a hyaline eosinophilic sheath. The irregular islands have been referred to as a jigsaw pattern.1 The origin of cylindroma has not been definitively established. Theories include a hair follicle derivation or a neoplasm that arises within primitive sweat glands.2
A solitary cylindroma usually presents as a firm to rubbery, flesh-colored to pinkish, papule or nodule on the scalp or face. Excision is the preferred treatment modality; inadequate margins may result in recurrence.3 Lesions present sporadically in contrast to multiple tumors that characterize familial cylindromatosis, which is transmitted as an autosomal dominant pattern with variable penetrance. These tumors are referred to as “turban tumors” and result from a mutation in the CYLD gene on chromosome 16q.4
Cutaneous cylindromas rarely undergo malignant transformation in either the solitary form or in familial cylindromatosis.5 Cylindromas may be a feature of the Brooke-Spiegler syndrome, a rare autosomal dominant condition that is made up of multiple skin lesions such as spiradenoma, trichoepithelioma, and cylindroma, is associated with spiradenomas and trichoepitheliomas.6
Stephen Schleicher, MD, is director of the DermDox Dermatology Centers, associate professor of medicine at Geisinger Commonwealth Medical College, and clinical instructor of dermatology at Arcadia University and Kings College.
1. Tallon B. Cylindroma pathology. DermNet. Accessed on November 15, 2022. https://dermnetnz.org/topics/cylindroma-pathology
2. Konkimalla A, Sakthivel P, Singh CA, Yogal R, Panda S, Sharma MC. Head and neck cylindroma masquerading as squamous cell carcinoma: a case report. JNMA J Nepal Med Assoc. 2020;58(228):611-613. doi:10.31729/jnma.5067.
3. Myers DJ, Fillman EP. Cylindroma. In: StatPearls [Internet]. StatPearls Publishing; 2022 Aug 8.
4. Bignell GR, Warren W, Seal S, et al. Identification of the familial cylindromatosis tumour-suppressor gene. Nat Genet. 2000;25(2):160-165. doi:10.1038/76006
5. Gerretsen AL, van der Putte SC, Deenstra W, van Vloten WA. Cutaneous cylindroma with malignant transformation. Cancer. 1993;72(5):1618-1623. doi: 10.1002/1097-0142(19930901)72:5<1618::aid-cncr2820720521>3.0.co;2-5
6. Neema S, Sandhu S, Radhakrishnan S. Brooke-Spiegler syndrome. Indian J Dermatol Venereol Leprol. 2021:1-2. doi:10.25259/IJDVL_355_20