Qureshi Sajid S, Bhagat Monica, Verma Kamlesh, Yadav Subhash, Prasad Maya, Vora Tushar, Chinnaswamy Girish, Amin Nayana, Smriti Vasundhara, Baheti Akshay, Laskar Siddharth, Khanna Nehal, Ramadwar Mukta, Shah Sneha
Division of Paediatric Surgical Oncology, Department of Surgical Oncology, Tata Memorial Hospital and Advanced Centre for Training Research and Education in Cancer (ACTREC), Tata Memorial Centre, Mumbai, India; Homi Bhabha National Institute (HBNI), Mumbai, India.
Division of Paediatric Surgical Oncology, Department of Surgical Oncology, Tata Memorial Hospital and Advanced Centre for Training Research and Education in Cancer (ACTREC), Tata Memorial Centre, Mumbai, India.
J Pediatr Urol. 2020 Aug;16(4):475.e1-475.e9. doi: 10.1016/j.jpurol.2020.05.168. Epub 2020 Jun 5.
Non-Wilms renal tumors represent a compelling subset of childhood renal tumors. However, their relative rarity renders accurate diagnosis, and therapy challenging which in some instance is inferred from their adult counterparts.
To describe the incidence and analyze the diagnostic challenges, therapies and, outcomes of non-Wilms renal tumors at the largest tertiary cancer centre in India.
All patients with histologically confirmed non-Wilms renal tumours diagnosed in the paediatric oncology unit of Tata Memorial Hospital between 2006 and 2019 were included. Data regarding clinical and radiological features and treatment outcomes were retrieved from the prospectively maintained institutional database. At the outset, histological types were categorised into a high and low-risk group depending on anticipated survival. Survival analysis was performed utilising the Kaplan-Meier method on SPSS software version 24.0.
Of the 569 patients with renal tumors, 109 (19%) patients with primary (n = 97) or recurrent (n = 12) non-Wilms renal tumors were included. Histological high-risk group included clear cell sarcoma (CCSK) (39.4%), renal cell carcinoma (RCC) (19.3%), malignant rhabdoid tumor (MRTK) (12.8%), Ewing's sarcoma (rES) (15.6%), synovial sarcoma (2%), and undifferentiated sarcoma (2%). The low-risk group comprised of congenital mesoblastic nephroma (CMN) (4.6%), cystic partially differentiated nephroblastoma (2%), and other rare tumors (3%). Diagnostic error occurred in 2 patients in the high-risk group. All low-risk tumours were treated with surgery alone and most (97%) high-risk tumors were operated either upfront (61.5%) or after preoperative chemotherapy (38.4%). Adjuvant therapy based on histology was offered to 70%. The recurrent tumors received various salvage treatments including chemotherapy; radiotherapy; surgery and immunotherapy, however, only 2 patients could be salvaged. The 3-year overall survival for the entire cohort with primary tumors was 59%, and the survival rates were 76.7%, 77.9%, 0.0%, and 52% for CCSK, RCC, MRTK, and rES (summary figure). Low-risk tumors had 100% survival while the recurrent tumors had a median survival of 10.5 months.
Non-Wilms renal tumors constitute a heterogeneous group of tumors, accounting for less than 20% of all renal tumors. Low-risk tumors are associated with excellent outcomes following surgery alone while the high-risk tumours have a variable outcome. MRTK and recurrent non-Wilms tumour have the worst survival. Favourable outcomes for CCSK and RCC and worst outcomes for MRTK were observed in this study. Renal ES have higher incidence of treatment failure and unsatisfactory outcomes. Recurrent non-Wilms tumours have an extremely poor outcome and more alternative or innovative approaches are needed for their treatment.
非肾母细胞瘤性肾肿瘤是儿童肾肿瘤中一个引人关注的子集。然而，它们相对罕见，这使得准确诊断和治疗具有挑战性，在某些情况下是从成人对应肿瘤推断而来。
描述印度最大的三级癌症中心非肾母细胞瘤性肾肿瘤的发病率，并分析其诊断挑战、治疗方法及结果。
纳入2006年至2019年期间在塔塔纪念医院儿科肿瘤科确诊的所有经组织学证实的非肾母细胞瘤性肾肿瘤患者。从前瞻性维护的机构数据库中检索有关临床和放射学特征及治疗结果的数据。一开始，根据预期生存率将组织学类型分为高风险组和低风险组。使用SPSS 24.0软件上的Kaplan-Meier方法进行生存分析。
在569例肾肿瘤患者中，纳入了109例（19%）原发性（n = 97）或复发性（n = 12）非肾母细胞瘤性肾肿瘤患者。组织学高风险组包括透明细胞肉瘤（CCSK）（39.4%）、肾细胞癌（RCC）（19.3%）、恶性横纹肌样瘤（MRTK）（12.8%）、尤因肉瘤（rES）（15.6%）、滑膜肉瘤（2%）和未分化肉瘤（2%）。低风险组包括先天性中胚层肾瘤（CMN）（4.6%）、囊性部分分化肾母细胞瘤（2%）和其他罕见肿瘤（3%）。高风险组中有2例发生诊断错误。所有低风险肿瘤仅接受手术治疗，大多数（97%）高风险肿瘤要么 upfront（61.5%）手术，要么术前化疗后（38.4%）手术。70%的患者接受了基于组织学的辅助治疗。复发性肿瘤接受了包括化疗、放疗、手术和免疫治疗在内的各种挽救治疗，然而，只有2例患者得以挽救。整个原发性肿瘤队列的3年总生存率为59%，CCSK、RCC MRTK和rES的生存率分别为76.7%、77.9%、0.0%和52%（汇总图）。低风险肿瘤的生存率为100%，而复发性肿瘤的中位生存期为10.5个月。
非肾母细胞瘤性肾肿瘤构成一组异质性肿瘤，占所有肾肿瘤的不到20%。低风险肿瘤仅手术治疗后预后良好，而高风险肿瘤预后不一。MRTK和复发性非肾母细胞瘤性肿瘤的生存率最差。本研究中观察到CCSK和RCC预后良好，MRTK预后最差。肾ES治疗失败的发生率较高，预后不理想。复发性非肾母细胞瘤性肿瘤的预后极差，需要更多替代或创新方法来治疗。
