Department of Urology, Zhengzhou Children's Hospital, Children's Hospital Affiliated to Zhengzhou University, Zhengzhou, 450018, China.
Department of Urology, Beijing Children's Hospital Affiliated to Capital Medical University, 56# Nanlishi Road, Beijing, 100045, China.
Int Urol Nephrol. 2021 Apr;53(4):619-625. doi: 10.1007/s11255-020-02706-5. Epub 2020 Nov 27.
According to the guidelines of International Society of Pediatric Oncology (SIOP) and National Wilms Tumor Study (NWTS), Wilms tumor with preoperative rupture should be classified as at least stage III. Few clinical reports can be found about preoperative Wilms tumor rupture. The purpose of this study was to investigate our experience on the diagnosis, treatment and prognosis of preoperative Wilms tumor rupture.
Patients with Wilms tumor who underwent treatment according to the NWTS or SIOP protocol from January 2008 to September 2017 in Beijing Children's Hospital were reviewed retrospectively. The clinical signs of preoperative tumor rupture were acute abdominal pain, and/or fall of hemoglobin. The radiologic signs of preoperative tumor rupture are as follows: (1) retroperitoneal and/or intraperitoneal effusion; (2) acute hemorrhage located in the sub-capsular and/or perirenal space; (3) tumor fracture communicating with peritoneal effusion; (4) bloody ascites. Patients with clinical and radiologic signs of preoperative tumor rupture were selected. Patients having radiologic signs without clinical symptoms were also selected. The clinical data, treatments and outcomes were analyzed. Meanwhile, patients without preoperative Wilms tumor rupture during the same period were collected and analyzed.
565 Patients with Wilms tumor were registered in our hospital. Of these patients, 45 patients were diagnosed with preoperative ruptured Wilms tumor. All preoperative rupture were confirmed at surgery. Spontaneous tumor rupture occurred in 41 patients, the other 4 patients had traumatic history. Of the 45 patients, 41 were classified as stage III, 3 patients with pulmonary metastases were classified as stage IV, and one patient with bilateral tumors were classified as stage V. Of these patients with preoperative tumor rupture at stage III, 30 patients had clinical and radiologic signs of tumor rupture, the other 11 patients had radiologic signs without clinical symptoms. Among the 41 patients at stage III, 13 patients had immediate surgery without preoperative chemotherapy (immediate group), and 28 patients had delayed surgery after preoperative chemotherapy (delayed group). In immediate group, 12 patients had localized rupture, 1 patient underwent emergency surgery because of continuous bleeding. In delayed group, 4 had inferior vena cava tumor embolus (1 thrombus extended to inferior vena cava behind the liver, three thrombi got to the right atrium), 4 crossed the midline with large tumors, 20 had extensive rupture without localization. In immediate group, tumor recurrence and metastasis developed in 2 patients, and no death occurred. In the delayed group, tumor recurrence and metastasis developed in 8 patients, and 7 patients died. During the same period, 41 patients were classified as stage III without preoperative rupture. In the non-ruptured group, tumor recurrence and metastasis developed in 3 patients, and 4 patients died. The median survival time in the ruptured group (both immediate group and delayed group) and non-ruptured group were (85.1 ± 7.5) and (110.3 ± 5.6) months, and the 3-year cumulative survival rates were 75.1% and 89.6%, respectively. The overall survival rate between the ruptured and non-ruptured groups showed no statistic difference (P = 0.256). However, there was significant difference in recurrence or metastasis rate between the ruptured and non-ruptured groups (24.4% vs 7.3%; P = 0.031).
Contrast-enhanced computed tomography (CT) and ultrasonography (US) are of major value in the diagnosis of preoperative tumor rupture, and immediate surgery or delayed surgery are available therapeutic methods. The treatment plan was based on patients' general conditions, tumor size, position and impairment degree of tumor rupture, extent of invasion and experience of a multidisciplinary team (including surgeon and anesthesiologists). In our experience, for ruptured preoperative tumor diagnosed with stage III, the criteria for immediate surgery are as follows: tumor not acrossing the midline, tumor without inferior vena cava thrombus, localized rupture, being capable of complete resection. Selection criteria for delayed surgery after preoperative chemotherapy are as follows: large tumors, long inferior vena cava tumor thrombus, tumors infiltrating to surrounding organs, unlocalized rupture, tumors can not being resected completely. Additionally, patients with preoperative Wilms tumor rupture had an increased risk of postoperative recurrence or metastasis.
根据国际小儿肿瘤学会（SIOP）和国家肾母细胞瘤研究（NWTS）指南，术前破裂的肾母细胞瘤应至少归类为 III 期。关于术前肾母细胞瘤破裂的临床报告很少。本研究旨在探讨我们在术前肾母细胞瘤破裂的诊断、治疗和预后方面的经验。
回顾性分析 2008 年 1 月至 2017 年 9 月在北京儿童医院根据 NWTS 或 SIOP 方案治疗的肾母细胞瘤患儿的临床资料。术前肿瘤破裂的临床征象为急性腹痛和/或血红蛋白下降。术前肿瘤破裂的影像学征象如下：（1）腹膜后和/或腹腔积液；（2）位于包膜下和/或肾周空间的急性出血；（3）与腹腔积液相通的肿瘤骨折；（4）血性腹水。选择有临床和影像学征象的术前肿瘤破裂的患者。选择有影像学征象而无临床症状的患者。分析其临床资料、治疗方法和结果。同时，收集同期无术前肾母细胞瘤破裂的患儿进行分析。
我院共登记 565 例肾母细胞瘤患儿，其中 45 例诊断为术前破裂的肾母细胞瘤。所有术前破裂均在手术中证实。自发性肿瘤破裂 41 例，外伤性病史 4 例。45 例中，III 期 41 例，有肺转移的 III 期 3 例，双侧肿瘤的 V 期 1 例。III 期术前肿瘤破裂的 30 例患者有临床和影像学征象的肿瘤破裂，另外 11 例患者有影像学征象而无临床症状。在 41 例 III 期患者中，13 例无术前化疗立即手术（立即组），28 例术前化疗后延迟手术（延迟组）。立即组 12 例为局限性破裂，1 例因持续出血行急诊手术。延迟组 4 例下腔静脉肿瘤栓子（1 例血栓延伸至肝后下腔静脉，3 例血栓进入右心房），4 例肿瘤越过中线，20 例无定位的广泛破裂。立即组肿瘤复发转移 2 例，无死亡。延迟组肿瘤复发转移 8 例，死亡 7 例。同期无术前破裂的 III 期患者 41 例，肿瘤复发转移 3 例，死亡 4 例。破裂组（立即组和延迟组）和未破裂组的中位生存时间分别为（85.1±7.5）和（110.3±5.6）个月，3 年累积生存率分别为 75.1%和 89.6%。破裂组和未破裂组的总生存率无统计学差异（P=0.256）。但破裂组和未破裂组的复发或转移率差异有统计学意义（24.4%比 7.3%；P=0.031）。
增强 CT 和超声对术前肿瘤破裂的诊断具有重要价值，立即手术或延迟手术是可行的治疗方法。治疗方案取决于患者的一般情况、肿瘤大小、位置和肿瘤破裂损伤程度、侵犯程度以及多学科团队（包括外科医生和麻醉师）的经验。根据我们的经验，对于术前诊断为 III 期破裂的肿瘤，立即手术的标准为：肿瘤未越过中线、肿瘤无下腔静脉血栓、局限性破裂、能够完全切除。术前化疗后延迟手术的选择标准为：肿瘤较大、下腔静脉肿瘤栓子较长、肿瘤侵犯周围器官、无定位破裂、肿瘤不能完全切除。此外，术前肾母细胞瘤破裂的患儿术后复发或转移的风险增加。
