Brady Kimberly L, Hurst Eva A
*Department of Dermatology, Roswell Park Cancer Institute, Buffalo, New York;†Division of Dermatology, Washington University School of Medicine, St. Louis, Missouri.
Dermatol Surg. 2017 Feb;43(2):281-286. doi: 10.1097/DSS.0000000000000943.
Sebaceous carcinoma is a rare and potentially aggressive adnexal neoplasm with historic data indicating high rates of recurrence, metastasis, and cancer-specific mortality.
To evaluate the incidence of local recurrence, metastasis, disease-specific mortality, and all-cause mortality and to identify work-up approaches.
PATIENTS AND METHODS/MATERIALS: Retrospective review of patients with sebaceous carcinoma treated with Mohs micrographic surgery between 2001 and 2013 at one institution.
Thirty-seven patients had 45 sebaceous carcinomas located on the periocular region (13%), non-periocular face (47%), scalp (7%), neck (4%), trunk (9%), and extremities (20%). The mean age was 66.1 years, and 24 (65%) patients were male. Five patients had Muir-Torre syndrome (MTS) or Lynch syndrome. Seven of 12 tumors showed loss of expression of ≥1 mismatch repair gene. The most common work-up involved taking a detailed personal and family medical history. No local recurrences, metastases, or disease-specific deaths occurred during an average follow-up of 3.6 years.
Mohs micrographic surgery is an effective treatment for sebaceous carcinoma. Detailed history taking, age-appropriate cancer screening, and immunohistochemical staining with MLH1, MSH2, or MSH6 is helpful in identifying which patients should be referred to a geneticist for work-up of MTS.
皮脂腺癌是一种罕见且具有潜在侵袭性的附属器肿瘤,历史数据表明其复发、转移及癌症特异性死亡率较高。
评估局部复发、转移、疾病特异性死亡率及全因死亡率的发生率,并确定检查方法。
患者与方法/材料:对2001年至2013年在一家机构接受莫氏显微外科手术治疗的皮脂腺癌患者进行回顾性研究。
37例患者有45处皮脂腺癌,位于眼周区域(13%)、非眼周面部(47%)、头皮(7%)、颈部(4%)、躯干(9%)和四肢(20%)。平均年龄为66.1岁,24例(65%)患者为男性。5例患者患有穆尔-托雷综合征(MTS)或林奇综合征。12个肿瘤中有7个显示≥1个错配修复基因表达缺失。最常见的检查方法是详细询问个人及家族病史。在平均3.6年的随访期间,未发生局部复发、转移或疾病特异性死亡。
莫氏显微外科手术是治疗皮脂腺癌的有效方法。详细询问病史、进行适合年龄的癌症筛查以及用MLH1、MSH2或MSH6进行免疫组化染色有助于确定哪些患者应转诊给遗传学家进行MTS检查。