Cylindroma

Cylindroma
TURBAN TUMOUR (CYLINDROMA). A rapidly growing fungating tumour on the scalp.
SpecialtyOncology, dermatology Edit this on Wikidata

Cylindroma is a rare, slow-growing, benign adnexal tumor of the skin. It mostly affects the face, scalp, and neck regions.

Types include:

Signs and symptoms

Grossly, the tumors are smooth, firm, pink to crimson in hue, commonly pedunculated, and often numerous. Tumors can sometimes cause pain. The scalp and surrounding skin are the most common sites, and the masses can coalesce to form a "turban tumor," in a hat-like arrangement. When pedunculated, the tumors may be nearly hairless, although the smaller lesions produce dermal nodules with minimal hair loss over them.

Microscopically, cylindromas can be seen with islands of basaloid cells that fit together as if in a jigsaw puzzle.

Photomicrograph of a dermal eccrine cylindroma under H&E stain. Note the islands of basaloid cells that fit together.

Pathogenesis

Cylindromas can be sporadic or inherited. Inherited or familial cylindromas are associated with inactivating mutations in the CYLD tumor suppressor gene, while sporadic cylindromas are associated with MYB-NFIB fusion transcripts.

However, despite being named after the tumor, mutation in the CYLD gene does not exclusively result in cylindromas or cylindromatosis. Other diseases, like trichoepitheliomas and Brooke-Spiegler syndrome, are also associated with the CYLD gene.

Diagnosis

Computed tomography or magnetic resonance imaging provide the best representation of the tumour's extension.

Treatment

As cylindromas are benign, they can be fully treated by surgical excision.

See also

References

  1. 1 2 3 V., Lakshmi Priya; Johnson, Thanka; S., Srismitha; Balakrishnan, Shobana (2022-12-16). "Solitary Dermal Cylindroma in an Uncommon Site: A Rare Case Report". Cureus. 14 (12) e32614. doi:10.7759/cureus.32614. ISSN 2168-8184. PMC 9841088. PMID 36654640.
  2. ↑ Akgul, Gokhan Giray; Yenidogan, Erdinc; Dinc, Soykan; Pak, Isin; Colakoglu, Muhammet Kadri; Gulcelik, Mehmet Ali (January 2013). "Malign cylindroma of the scalp with multiple cervical lymph node metastasis—A case report". International Journal of Surgery Case Reports. 4 (7): 589–592. doi:10.1016/j.ijscr.2013.02.027. ISSN 2210-2612. PMC 3679426. PMID 23702364.
  3. ↑ Guruprasad, Yadavalli; Chauhan, DineshSingh (2012). "Dermal cylindroma of the scalp". National Journal of Maxillofacial Surgery. 3 (1). Medknow: 59–61. doi:10.4103/0975-5950.102163. ISSN 0975-5950. PMC 3513812. PMID 23251061.
  4. ↑ McGhee, Kennedye; Spethman, Caroline; Muhammad, Hamad; Woodard, Stefanie; Guo, Hua; Yalniz, Ceren (November 2024). "Breast cylindroma: A rare and unexpected diagnosis". Radiology Case Reports. 19 (11): 5144–5148. doi:10.1016/j.radcr.2024.07.175. PMC 11387543. PMID 39263500.
  5. ↑ Wollina, Uwe (2023-05-13). "Dermal cylindroma – An update and a series of uncommon clinical presentations". Cosmoderma. 3 77. doi:10.25259/CSDM_87_2023. ISSN 2768-6620.
  6. ↑ Nardi, Leonardo; Bicker, Frank; Maier, Jannik; Waisman, Ari; Schmeisser, Michael J. (March 2025). "Role of CYLD in brain physiology and pathology". Journal of Molecular Medicine. 103 (3): 255–263. doi:10.1007/s00109-025-02521-4. ISSN 0946-2716. PMC 11880164. PMID 39945824.
  7. ↑ Bowen, Sarah; Gill, Melissa; Lee, David A.; Fisher, Galen; Geronemus, Roy G.; Vazquez, MariaLuisa Espinel; Celebi, Julide Tok (May 2005). "Mutations in the CYLD gene in Brooke–Spiegler Syndrome, Familial Cylindromatosis, and Multiple Familial Trichoepithelioma: Lack of Genotype–Phenotype Correlation". Journal of Investigative Dermatology. 124 (5): 919–920. doi:10.1111/j.0022-202X.2005.23688.x. hdl:20.500.12648/7844. PMID 15854031.
  8. ↑ Friedrich, Reinhard E. (2010-05-01). "Dermal Cylindroma of the Scalp (Turban Tumour) and Subjacent Calvarian Defects". Anticancer Research. 30 (5). International Institute of Anticancer Research: 1793–1797. ISSN 0250-7005. PMID 20592381. Retrieved 2024-04-03.

Further reading