Wu Yuanyuan, Mao Aihong, Wang Jun
Department of Head and Neck Surgery, Gansu Provincial Cancer Hospital, Lanzhou 730050, China.
Gansu Provincial Academic Institute for Medical Research, Lanzhou 730050, China.
Oral Oncol. 2022 May;128:105818. doi: 10.1016/j.oraloncology.2022.105818. Epub 2022 Apr 1.
Ewing's sarcoma (ES) and primitive neuroectodermal tumor (PNET) are high grade malignant small round cell tumors presenting in both skeletal and extraskeletal anatomic locations. Actually, they are the same entity showing varying degrees of neuroectodermal differentiation. Very few cases of peripheral PNET (pPNET) of the tongue was reported in medical literature. Here, we first report a case of primary extraskeletal ES/pPNET in the tongue of a 9-years old girl. The result of immunohistochemical analysis showed the membranous positive for CD99 and Vimentin. The girl underwent radical surgical treatment and received six cycles of OPEC regimen adjuvantchemotherapy without radiotherapy, and now she is remaining tumor-free survival. The individual comprehensive therapy shows a good curative effect.
尤因肉瘤（ES）和原始神经外胚层肿瘤（PNET）是高级别恶性小圆细胞肿瘤，可出现在骨骼和骨骼外的解剖部位。实际上，它们是同一实体，表现出不同程度的神经外胚层分化。医学文献中报道的舌部外周PNET（pPNET）病例极少。在此，我们首次报告一例9岁女孩舌部原发性骨骼外ES/pPNET病例。免疫组化分析结果显示CD99和波形蛋白呈膜阳性。该女孩接受了根治性手术治疗，并接受了六个周期的OPEC方案辅助化疗，未进行放疗，目前她处于无瘤生存状态。个体化综合治疗显示出良好的疗效。
