Tan Wei Lun, Rizuana Iqbal Hussain, Lee Bang Rom, Fam Xeng Inn
Department of Surgery, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia.
Department of Radiology, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia.
Front Oncol. 2025 Sep 9;15:1602774. doi: 10.3389/fonc.2025.1602774. eCollection 2025.
Primary renal Ewing sarcoma/primitive neuroectodermal tumor (PNET) is a very rare and aggressive malignant tumor. We report a case of a young adult who presented with painless hematuria and acute urinary retention and was subsequently diagnosed with localized left renal Ewing sarcoma following robotic-assisted retroperitoneal partial nephrectomy. Histopathology revealed a tumor composed of nests and lobules of monotonous tumor cells with round nuclei, indistinct nucleoli, and scanty cytoplasm, associated with a vascular-rich to hyalinized stroma and fibrillary neural matrix. Moderate nuclear pleomorphism, scattered necrosis, and pseudorosette formation were noted. Immunohistochemical studies demonstrated tumor cell positivity for CD99 and CD117. The patient was subjected to chemotherapy. Early diagnosis and multimodality treatment play an important role in improving survival rate.
原发性肾尤文肉瘤/原始神经外胚层肿瘤（PNET）是一种非常罕见且侵袭性强的恶性肿瘤。我们报告一例年轻成人病例，该患者表现为无痛性血尿和急性尿潴留，在机器人辅助腹膜后部分肾切除术后被诊断为局限性左肾尤文肉瘤。组织病理学显示肿瘤由巢状和小叶状的单一肿瘤细胞组成，细胞核圆形，核仁不明显，细胞质稀少，伴有血管丰富至玻璃样变的间质和纤维状神经基质。可见中度核异型性、散在坏死和假菊形团形成。免疫组织化学研究显示肿瘤细胞CD99和CD117呈阳性。该患者接受了化疗。早期诊断和多模式治疗对提高生存率起着重要作用。
