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Sebaceous gland carcinomas are rare malignant cutaneous adnexal tumors with sebocytic differentiation. The typical predilection area is the head and neck region, where sebaceous gland carcinomas are the most common malignant adnexal tumors of the skin. According to their localization a distinction is made between periocular and extraocular sebaceous gland carcinomas. Muir-Torre syndrome (MTS) should always be ruled out if it is suspected. In terms of prognosis, sebaceous gland carcinomas are potentially aggressive tumors with a clear tendency to recur and metastasize. Only small extraocular sebaceous gland carcinomas that have been completely resected have a very good prognosis. Sebaceous gland carcinomas most frequently metastasize lymphogenously to regional or distant lymph nodes; organ metastasis occurs less frequently. Periocular sebaceous gland carcinomas have a higher metastasis rate (up to 15%) than extraocular sebaceous gland carcinomas (up to 2%). Complete micrographically controlled surgery (MCS) of the primary tumor is the therapy of first choice, regardless of periocular or extraocular localization. Adjuvant or therapeutic radiotherapy may be considered. There is currently no established standard therapy for advanced, inoperable, or metastatic sebaceous gland carcinomas. Local procedures and systemic therapies such as chemotherapy or immunotherapy can be considered. The procedure should be determined individually by an interdisciplinary tumor board. Close follow-up care is recommended for these potentially aggressive carcinomas., (© 2024 The Authors. Journal der Deutschen Dermatologischen Gesellschaft published by Wiley‐VCH GmbH on behalf of Deutsche Dermatologische Gesellschaft.)

Purpose: To report the frequency and clinical features of internal visceral malignancy in an unselected series of patients with sebaceous gland carcinoma (SGC)., Methods: A non-comparative retrospective case series of consecutive patients with a histologically proven diagnosis of periocular sebaceous cell carcinoma treated at the University Eye Hospital, University of Erlangen-Nürnberg between 1981 and 2000., Results: Twenty-three patients were identified, each with one tumor. Men and women were almost equally affected with a mean age at diagnosis of 62 years (range 37-83 years). The majority of tumors were located in the upper lid, but five tumors were situated in the lower lid or caruncle. In 22 of the 23 patients the diagnosis at referral was either incorrect or not suspected. In all cases, the SGC was treated by surgical excision. In the systems review, 11 of the 23 patients (48%) had evidence of previous systemic malignancy, the most common being colorectal cancer followed by liver and bronchial carcinoma. Forty-five percent of patients with SGC and internal visceral malignancy eventually died as a result of their tumors., Conclusion: Periocular SGC remains an underdiagnosed entity for which a high level of clinical suspicion is critical for early recognition. Almost half the patients additionally had an internal visceral malignancy. Internal visceral malignancy in unselected patients with SGC may be more common and have a more guarded prognosis than has been appreciated.