Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts, USA.
Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts, USA; Ophthalmic Plastic Surgery Service, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA.
Surv Ophthalmol. 2019 Sep-Oct;64(5):659-667. doi: 10.1016/j.survophthal.2019.03.002. Epub 2019 Mar 11.
Merkel cell carcinoma (MCC) is a rare, aggressive tumor of both epithelial and neuroendocrine origin, which carries a mortality rate of up to 40%. MCC tumors typically present as painless, expanding nodules on the sun-exposed skin areas of older, white patients. Eyelid and periocular tumors comprise approximately 2.5% of all cases of MCC and may be mistaken for chalazia or basal cell carcinomas. Immunosuppression is a significant risk factor, particularly in solid-organ-transplant recipients, patients with chronic lymphocytic leukemia, and patients with HIV. Sentinel lymph node biopsy is often used for accurate staging of head and neck MCC. Treatment includes wide local excision, commonly with the addition of radiotherapy for improved locoregional disease control. Historically, adjuvant chemotherapy had been reserved for metastatic disease, but immunotherapy and targeted chemotherapies are currently being investigated for use in primary disease. The clinical characteristics of all available published cases of eyelid MCC are summarized in this article.
默克尔细胞癌（Merkel cell carcinoma，MCC）是一种罕见的、具有上皮和神经内分泌起源的侵袭性肿瘤，死亡率高达 40%。MCC 肿瘤通常表现为无痛、扩张性结节，出现在老年白人患者的阳光暴露皮肤区域。眼睑和眶周肿瘤约占所有 MCC 病例的 2.5%，可能被误诊为睑板腺囊肿或基底细胞癌。免疫抑制是一个重要的危险因素，特别是在实体器官移植受者、慢性淋巴细胞白血病患者和 HIV 患者中。前哨淋巴结活检常用于头颈部 MCC 的准确分期。治疗包括广泛的局部切除，通常联合放疗以提高局部区域疾病的控制率。历史上，辅助化疗一直保留用于转移性疾病，但免疫疗法和靶向化疗目前正在研究用于原发性疾病。本文总结了所有已发表的眼睑 MCC 病例的临床特征。
