Erginel Başak, Vural Sema, Akın Melih, Karadağ Cetin Ali, Sever Nihat, Yıldız Abdullah, Tanık Canan, Demir Arzu Ataman, Yanar Ozlem, Dokucu Ali İhsan
1Department of Pediatric Surgery, Sisli Etfal Education and Research Hospital, Istanbul, Turkey.
Pediatr Hematol Oncol. 2014 Aug;31(5):409-14. doi: 10.3109/08880018.2014.930767. Epub 2014 Jul 9.
Medical records of 71 children with Wilms' tumor at Sisli Etfal Education and Research Hospital between 1990 and 2014 were reviewed. Mean age at diagnosis was 3.11 years (2 days-7 years). Male to female ratio was M/F = 6/10. The incidence of associated anomaly was 16.9%. Clinical manifestations included abdominal mass (89%), hematuria (30%), hypertansion (25%), abdominal pain (15%), fever (5%), restlessness (2%), weight loss (2%), varicocele (1%). Ultrasound (USG) was the most often initial study in a child presenting with abdominal mass. Doppler USG was also made to evaluate the inferior vena cava (IVC) for the presence of tumor extension in children with renal mass. The left kidney was affected in 33 patients (46.5%), the right was affected in 31 patients (43.7%). Two patients was extrarenal (2.8%). And 5 patients (7.04%) were bilateral on the presentation. Preoperative chemotheraphy was done in 14 cases. In 63 patients with unilateral Wilm tm, unilateral radical nefrectomy is performed. In one patient with solitary kidney, nephron sparing surgery (NSS) is performed. In 3 patients with bilateral tm NSS is performed and in 2 patients with bilateral Wilms' tm NSS is performed in one side and nefrectomy on the other side. Out of 71 Wilms tumor (WT) patients, 17 of them has been out of our follow. And 4 of them are died. Ten of them has metastases. Forty children are under follow with no metastases. Patients with WT needs a multimodal, multidisiplinary treatment with the cooperation of pediatric oncologist and pediatric surgeon and needs close follow-up.
回顾了1990年至2014年间在锡斯利埃法尔教育与研究医院收治的71例肾母细胞瘤患儿的病历。诊断时的平均年龄为3.11岁(2天至7岁)。男女比例为M/F = 6/10。合并畸形的发生率为16.9%。临床表现包括腹部肿块(89%)、血尿(30%)、高血压(25%)、腹痛(15%)、发热(5%)、烦躁不安(2%)、体重减轻(2%)、精索静脉曲张(1%)。超声(USG)是出现腹部肿块的儿童最常进行的初始检查。对于有肾脏肿块的儿童,还进行多普勒超声检查以评估下腔静脉(IVC)是否存在肿瘤延伸。33例患者(46.5%)左肾受累,31例患者(43.7%)右肾受累。2例患者为肾外病变(2.8%)。5例患者(7.04%)初诊时为双侧病变。14例患者进行了术前化疗。63例单侧肾母细胞瘤患者进行了单侧根治性肾切除术。1例单肾患者进行了保留肾单位手术(NSS)。3例双侧肿瘤患者进行了NSS,2例双侧肾母细胞瘤患者一侧进行了NSS,另一侧进行了肾切除术。71例肾母细胞瘤(WT)患者中,17例失访。4例死亡。10例有转移。40例儿童在随访中无转移。WT患者需要儿科肿瘤学家和儿科外科医生合作进行多模式、多学科治疗,并需要密切随访。