Llombart B, Requena C, Cruz J
Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, España.
Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, España.
Actas Dermosifiliogr. 2017 Mar;108(2):108-119. doi: 10.1016/j.ad.2016.07.022. Epub 2016 Oct 19.
Merkel cell carcinoma (MCC) is a rare, highly aggressive tumor, and local or regional disease recurrence is common, as is metastasis. MCC usually develops in sun-exposed skin in patients of advanced age. Its incidence has risen 4-fold in recent decades as the population has aged and immunohistochemical techniques have led to more diagnoses. The pathogenesis of MCC remains unclear but UV radiation, immunosuppression, and the presence of Merkel cell polyomavirus in the tumor genome seem to play key roles. This review seeks to update our understanding of the epidemiology, etiology, pathogenesis, and clinical features of MCC. We also review histologic and immunohistochemical features required for diagnosis. MCC staging is discussed, given its great importance in establishing a prognosis for these patients.
默克尔细胞癌(MCC)是一种罕见的、侵袭性很强的肿瘤,局部或区域疾病复发很常见,转移情况也如此。MCC通常发生在老年患者暴露于阳光下的皮肤中。近几十年来,随着人口老龄化以及免疫组化技术带来了更多诊断,其发病率上升了4倍。MCC的发病机制尚不清楚,但紫外线辐射、免疫抑制以及肿瘤基因组中默克尔细胞多瘤病毒的存在似乎起着关键作用。本综述旨在更新我们对MCC的流行病学、病因、发病机制和临床特征的认识。我们还回顾了诊断所需的组织学和免疫组化特征。鉴于MCC分期对这些患者预后评估的重要性,也对其进行了讨论。