Pathak Niramya J, Singh Abhishek G, Surwase Pavan J, Agrawal Sahil A, Ganpule Arvind P, Sabnis Ravindra B, Desai Mahesh R
Department of Urology, MPUH, Nadiad, Gujarat, India.
Indian J Urol. 2024 Jul-Sep;40(3):185-190. doi: 10.4103/iju.iju_175_23. Epub 2024 Jul 1.
We aim to share the experience of a single center in the management of eight cases of renal primitive neuroectodermal tumor (PNET) which are uncommon, aggressive tumors. The objectives were to study the presentation of the disease, the treatment offered and its outcomes, and the comparison of the treatment with published literature.
The single-center renal PNET data of all patients were retrospectively reviewed from 2011 to 2022. Renal PNET was seen in eight patients. Minimum follow-up period of 1 year was required.
Male-to-female ratio was 7:1. The mean age was 26.5 years. All were locally advanced tumors on presentation. One patient had an inferior vena cava thrombus, one patient had metastases on presentation, and two patients had tumor extending to paranephric space. The diagnosis was made by histopathology supported by immunohistochemistry showing CD99 positivity. All patients were treated with radical nephrectomy, followed by chemotherapy in all and radiotherapy in three patients. Two patients expired at 3½ and 6 years after surgery, the remaining six are alive at a median follow-up period of 34.5 months.
Renal PNET is an uncommon renal tumor which is aggressive and requires multimodal therapy for prolonged survival.
我们旨在分享单一中心管理8例肾原发性神经外胚层肿瘤（PNET）的经验，这是一种罕见的侵袭性肿瘤。目的是研究该疾病的表现、所提供的治疗及其结果，并将治疗方法与已发表的文献进行比较。
回顾性分析2011年至2022年该单一中心所有患者的肾PNET数据。8例患者被诊断为肾PNET。要求最短随访期为1年。
男女比例为7:1。平均年龄为26.5岁。所有患者初诊时均为局部晚期肿瘤。1例患者有下腔静脉血栓形成，1例患者初诊时已有转移，2例患者肿瘤延伸至肾旁间隙。通过组织病理学诊断，并经免疫组织化学支持，显示CD99阳性。所有患者均接受了根治性肾切除术，之后所有患者均接受化疗，3例患者接受放疗。2例患者分别在术后3.5年和6年死亡，其余6例患者在中位随访期34.5个月时存活。
肾PNET是一种罕见的肾肿瘤，具有侵袭性，需要多模式治疗以延长生存期。
