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Sebaceous Carcinoma, Syringocystadenoma Papilliferum, and Sebaceoma Arising in a Nevus Sebaceus of Jadassohn: A Case Report and Review of the Literature

Authors

Fatima Zahraa Sassine, Chourouq Mustapha Eid, Ilyass Anouar, Youssef Zemmez, Rachid Frikh, Naoufal Hjira

Journal Information

Journal: Medpeer Publisher

ISSN: 3066-2737

Volume: 3

Issue: 6

Date of Publication: 2026/06/13

DOI: 10.70780/medpeer.000QGTK

Abstract

Nevus sebaceus (NS), also known as nevus sebaceus of Jadassohn, is a congenital hamartoma of the pilosebaceous unit, most commonly located on the head and neck. Its natural course is characterized by progressive morphological changes and the potential development of secondary tumors, which are usually benign and more rarely malignant, particularly in adulthood.
We report the case of a 45-year-old man with a congenital nevus sebaceus of the scalp complicated by the exceptional association of three secondary tumors: syringocystadenoma papilliferum, sebaceoma, and sebaceous carcinoma. The patient presented with a recently enlarging, mildly painful, and oozing nodule arising within the pre-existing lesion. Clinical and dermoscopic examination revealed features suggestive of malignant transformation. Histopathological analysis confirmed the diagnosis of sebaceous carcinoma associated with syringocystadenoma papilliferum and sebaceoma within the excision specimen. Immunohistochemical staining for mismatch repair proteins showed preserved expression, ruling out Muir-Torre syndrome. At 12-month follow-up, there was no evidence of local recurrence or metastatic disease.
To our knowledge, this is the first reported case of the simultaneous occurrence of sebaceous carcinoma, syringocystadenoma papilliferum, and sebaceoma within a single nevus sebaceus. This case underscores the importance of lifelong surveillance of nevus sebaceus and provides dermoscopic clues that may aid in the early detection of malignant transformation.

Keywords

nevus sebaceus; sebaceous carcinoma; syringocystadenoma papilliferum; sebaceoma; dermoscopy; Muir-Torre syndrome

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