Clinical Cooperation Unit Dermato-Oncology of the German Cancer Research Center (DKFZ) Heidelberg and the Department of Dermatology, Venereology and Allergology, Medical Faculty Mannheim, Ruprecht Karl University Heidelberg, Mannheim, Germany.
DKFZ Hector Cancer Institute, University Medical Center Mannheim, Mannheim, Germany.
J Dtsch Dermatol Ges. 2024 May;22(5):730-747. doi: 10.1111/ddg.15405. Epub 2024 Apr 28.
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.
皮脂腺癌是一种罕见的具有皮脂分化的恶性皮肤附属器肿瘤。典型的好发部位是头颈部，皮脂腺癌是皮肤最常见的恶性附属器肿瘤。根据其定位，可以区分眼周和眼外皮脂腺癌。如果怀疑存在穆尔-托雷综合征（MTS），则应始终排除该综合征。就预后而言，皮脂腺癌是具有明显复发和转移倾向的潜在侵袭性肿瘤。只有完全切除的小的眼外皮脂腺癌具有非常好的预后。皮脂腺癌最常通过淋巴途径转移至局部或远处淋巴结；器官转移则较少发生。眼周皮脂腺癌的转移率（高达 15%）高于眼外皮脂腺癌（高达 2%）。无论眼周或眼外定位，完整的显微镜下控制手术（MCS）切除原发肿瘤都是首选的治疗方法。辅助或治疗性放疗可能是可以考虑的。对于晚期、不可手术或转移性的皮脂腺癌，目前尚无标准的治疗方法。局部手术和全身治疗，如化疗或免疫治疗，可以考虑。这些潜在侵袭性的癌症应通过多学科肿瘤委员会来确定具体的治疗方案。建议对这些肿瘤进行密切随访。
