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An 81-year-old woman requests removal of a lesion on her left leg. She states that a growth had been removed from this site about 7 years ago and “grew back.” She was told that the pathology report for the lesion was “benign” and she denies a history or family history of skin cancer. Examination reveals a 1.5cm firm nodule with a faint bluish coloration and a central linear scar. No similar lesions are noted elsewhere.
Dermal cylindroma is a benign neoplasm of eccrine sweat glands found primarily on the head and neck.1 Some cases have been linked to mutations in CYLD, a tumor-suppressor gene.2 Cutaneous cylindroma syndromes associated with this mutation include Brook-Spielger syndrome, familial cylindromatosis, and multiple...
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Dermal cylindroma is a benign neoplasm of eccrine sweat glands found primarily on the head and neck.1 Some cases have been linked to mutations in CYLD, a tumor-suppressor gene.2 Cutaneous cylindroma syndromes associated with this mutation include Brook-Spielger syndrome, familial cylindromatosis, and multiple familial trichoepitheliomas.3 Some sporadic cylindromas contain an overexpressed oncoprotein, fusion of MYB-NFIB genes.4
Cylindromas present as reddish to blue smooth surfaced papules or nodules that can reach a diameter of several centimeters. On the scalp, multiple lesions may coalesce to form a large mass referred to as a “turban tumor.” Diagnosis is made by biopsy, which reveals tumor islands surrounded by a hyaline sheath creating a characteristic “jigsaw puzzle” appearance.5,6 Malignant transformation is rare; warning signs include rapid enlargement, increased tenderness, ulceration, or bleeding. 7
Excision is the preferred treatment modality and inadequate margins often result in recurrence as evidenced by this case. Multiple coalesced tumors are difficult to manage; a recent case report documented improvement following electrochemotherapy with bleomycin.8
Sidney Lampert is a medical student at the Drexel University College of Medicine, in Philadelphia. 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.
References
- Chauhan DS, Guruprasad Y. Dermal cylindroma of the scalp. Natl J Maxillofac Surg. 2012;3(1):59-61. doi:10.4103/0975-5950.102163
- Dubois A, Hodgson K, Rajan N. Understanding inherited cylindromas: clinical implications of gene discovery. Dermatol Clin. 2017;35(1):61-71. doi:10.1016/j.det.2016.08.002
- Nagy N, Dubois A, Szell M, Rajan N. Genetic testing in CYLD cutaneous syndrome: an update. Appl Clin Genet. 2021;14:427-444. doi:10.2147/TACG.S288274
- Rajan N, Andersson MK, Sinclair N, et al. Overexpression of MYB drives proliferation of CYLD-defective cylindroma cells. J Pathol. 2016;239(2):197-205. doi:10.1002/path.4717
- Tallon B. Cylindroma pathology. DermNet; 2022. https://dermnetnz.org/topics/cylindroma-pathology.
- V LP, Johnson T, S S, Balakrishnan S. Solitary dermal cylindroma in an uncommon site: a rare case report. Cureus. 2022;14(12):e32614. doi:10.7759/cureus.32614
- Kuklani RM, Glavin FL, Bhattacharyya I. Malignant cylindroma of the scalp arising in a setting of multiple cylindromatosis: a case report. Head Neck Pathol. 2009;3(4):315-319. doi:10.1007/s12105-009-0138-x
- Bonadies A, Iorio A, Silipo V, et al. Bleomycin electrochemotherapy of dermal cylindroma as an alternative treatment in a rare adnexal neoplasm: a case report and literature review. Biomedicines. 2023;11(10):2667. doi:10.3390/biomedicines11102667
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