妇科癌的分化差异包括广泛的谱系,包括间充质,生殖细胞,高级神经内分泌,神经外胚层,和皮肤附件分化。在这里,我们介绍了一例卵巢子宫内膜样腺癌,伴有恶性黑素细胞分化(MMeD)。背景卵巢子宫内膜样腺癌显示局灶性异常β-catenin表达和与β-catenin激活相关的组织学模式,包括纺锤状元素和有绳和透明的焦点。有MMeD的区域既有纺锤状的,也有上皮样的形态,弥漫性异常β-catenin表达,黑素细胞标记物的表达(HMB45/Mart-1鸡尾酒,MITF,和S100),并且没有肌源性标记(SMA和结蛋白)或上皮标记(细胞角蛋白和E-钙黏着蛋白)的染色。INI1、BRG1、PMS2和MSH6被保留,p53呈野生型表达模式。尽管进行了大量采样,但仍未发现具有明确的癌肉瘤分化的区域。虽然在英语文献中报道了一例具有浆液性上皮成分且很少关注恶性黑色素瘤的妇科癌肉瘤,目前的情况代表了什么,据我们所知,在β-catenin激活的子宫内膜样腺癌的背景下出现的第一例MMeD。讨论了病因和鉴别诊断方面的考虑因素。
Divergent differentiation in gynecologic carcinomas encompasses a broad range of lineages, including mesenchymal, germ cell, high-grade neuroendocrine, neuroectodermal, and cutaneous adnexal differentiation. Here we present a
case of ovarian endometrioid adenocarcinoma with divergent malignant melanocytic differentiation (MMeD). The background ovarian endometrioid adenocarcinoma showed focally aberrant β-catenin expression and histologic patterns associated with β-catenin activation, including spindled elements and corded and hyalinized foci. The areas with MMeD had both spindled and epithelioid morphology, diffusely aberrant β-catenin expression, expression of melanocytic markers (an HMB45/Mart-1 cocktail, MITF, and S100), and no staining for myogenic markers (SMA and desmin) or epithelial markers (cytokeratins and E-cadherin). INI1, BRG1, PMS2, and MSH6 were retained, and p53 showed a wild-type expression pattern. No areas with definitive carcinosarcomatous differentiation were identified despite extensive sampling. While a single
case of gynecologic carcinosarcoma with a serous epithelial component and a small focus on malignant melanoma has been reported in the English literature, the current
case represents what is, to the best of our knowledge, the first
case of MMeD arising in the context of a β-catenin activated endometrioid adenocarcinoma. Pathogenetic and differential diagnostic considerations are discussed.