Department of Pathology, First Faculty of Medicine, Charles University in Prague and General University Hospital in Prague, Studničkova 2, Prague 128 00, Czech Republic.
Pathol Oncol Res. 2010 Dec;16(4):601-8. doi: 10.1007/s12253-010-9249-7. Epub 2010 Mar 5.
We report four cases of uterine tumors with neuroectodermal differentiation. One tumor had neuroectodermal component only; in the three other tumors, the neuroectodermal component was admixed with another component, namely rhabdomyosarcoma (1 case), and endometrioid adenocarcinoma (2 cases). Histologically, the neuroectodermal component consisted of small to medium sized cells arranged in diffuse sheets. The tumor cells had round nuclei with stippled to coarsely granular chromatin, mostly with non-prominent nucleoli, and scant eosinophilic or amphophilic cytoplasm. Immunohistochemically, 4/4 tumors showed expression of vimentin, synaptophysin and CD56; 3/4 tumors were CD99 and NSE positive; 2/4 tumors showed focal expression of S-100 protein; and 1/4 tumors had focal dot-like cytoplasmic positivity for cytokeratin AE1/AE3. FLI-1 was negative in all cases. FISH examination was performed and none of the tumors showed rearrangement of EWSR1 gene. Uterine tumors with neuroectodermal differentiation are rare; to the best of our knowledge only 44 cases have been reported in the literature to date, referred to as Ewing sarcoma, peripheral PNET (pPNET), PNET (not otherwise specified) and uterine tumors with neuroendocrine differentiation.
我们报告了 4 例具有神经外胚层分化的子宫肿瘤。1 例肿瘤仅具有神经外胚层成分；在另外 3 例肿瘤中，神经外胚层成分与另一种成分混合，即横纹肌肉瘤（1 例）和子宫内膜样腺癌（2 例）。组织学上，神经外胚层成分由排列成弥漫片状的小至中等大小的细胞组成。肿瘤细胞具有圆形核，染色质呈点彩状至粗颗粒状，大多数核仁不明显，胞质稀少嗜酸性或嗜碱性。免疫组织化学染色显示，4/4 例肿瘤表达波形蛋白、突触素和 CD56；3/4 例肿瘤 CD99 和 NSE 阳性；2/4 例肿瘤 S-100 蛋白局灶性表达；1/4 例肿瘤细胞胞质局灶点状阳性表达细胞角蛋白 AE1/AE3。所有病例均为 FLI-1 阴性。进行了 FISH 检查，没有肿瘤显示 EWSR1 基因重排。具有神经外胚层分化的子宫肿瘤很少见；据我们所知，迄今为止文献中仅报道了 44 例，分别称为尤文肉瘤、外周 PNET（pPNET）、PNET（未特指）和具有神经内分泌分化的子宫肿瘤。
