关键词: S1 guideline cancer oncology sebaceous carcinoma sebaceous gland

Mesh : Sebaceous Gland Neoplasms / pathology therapy diagnosis Humans Muir-Torre Syndrome / pathology diagnosis therapy Prognosis Adenocarcinoma, Sebaceous / pathology therapy diagnosis Dermatology / standards Germany Mohs Surgery Practice Guidelines as Topic

来  源:   DOI:10.1111/ddg.15405

Abstract:
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.
摘要:
皮脂腺癌是罕见的恶性皮肤附件肿瘤,伴有皮脂腺分化。典型的好发区域是头颈部,皮脂腺癌是最常见的皮肤附件恶性肿瘤。根据它们的定位,眼周皮脂腺癌和眼外皮脂腺癌之间有区别。如果怀疑Muir-Torre综合征(MTS),应始终排除。在预后方面,皮脂腺癌是潜在的侵袭性肿瘤,具有明显的复发和转移趋势。只有完全切除的小眼外皮脂腺癌的预后非常好。皮脂腺癌最常通过淋巴途径转移到区域或远处淋巴结;器官转移发生频率较低。眼周皮脂腺癌的转移率(高达15%)高于眼外皮脂腺癌(高达2%)。原发肿瘤的完全显微控制手术(MCS)是首选的治疗方法,无论眼周或眼外定位。可以考虑辅助或治疗性放疗。目前还没有确定的晚期标准疗法,无法操作,或转移性皮脂腺癌。可以考虑局部程序和全身疗法,例如化疗或免疫疗法。该程序应由跨学科肿瘤委员会单独确定。建议对这些潜在的侵袭性癌进行密切随访。
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