Wahyudi Irfan, Aritonang Johannes, Hamid Agus Rizal A H, Situmorang Gerhard R, Mirza Hendy, Widia Fina, Rodjani Arry
Department of Urology, Cipto Mangunkusumo National General Hospital, Faculty of Medicine Universitas Indonesia, Indonesia.
Department of Urology, Cipto Mangunkusumo National General Hospital, Faculty of Medicine Universitas Indonesia, Indonesia.
Int J Surg Case Rep. 2020;77:53-56. doi: 10.1016/j.ijscr.2020.10.057. Epub 2020 Oct 19.
Wilms' tumour remains the most common renal tumour in children (6% of all pediatric malignancies) and present as one of the most challenging tasks for paediatric urologists as its management requires an advanced procedure. The ultimate goal in these cases is to preserve as much renal parenchyma as possible whilst still achieving complete tumour resection.
Here we present a six year follow up report of a bilateral Wilms' tumour case in a 19-months old boy. This patient underwent neoadjuvant chemotherapy regimen, followed by right partial nephrectomy and left radical nephrectomy. Adjuvant radiotherapy was performed following the surgery. Follow-up imaging 5 months afterward revealed a firmly heterogeneous cystic lesion consist of fat and calcification at the upper pole of the right kidney, none of which created any problem for the patient. MRI was later performed on the 19 month after the surgery, showing marked decrease in the size of the cyst.
According to SIOP and NWTSG classification, the patient presented as stage V of the disease. The patient was on neoadjuvant chemotherapy (Regimen I) as recommended by NWTSG. This strategy was shown to be effective, as the tumour on the left kidney was reduced to less than 70% of the initial size. A routine follow-up using chest x-ray, abdominal ultrasonography (USG), and contrast studies such as MRI and MSCT scan, was performed in our reports.
From our experience, the combination of neo-adjuvant chemotherapy, renal salvage surgery and adjuvant radiotherapy is a feasible, safe and effective option for bilateral Wilms' tumour cases.
肾母细胞瘤仍是儿童最常见的肾肿瘤（占所有儿科恶性肿瘤的6%），由于其治疗需要先进的手术，因此对儿科泌尿科医生来说是最具挑战性的任务之一。这些病例的最终目标是在实现肿瘤完全切除的同时，尽可能保留更多的肾实质。
在此，我们报告一名19个月大男孩双侧肾母细胞瘤的六年随访情况。该患者接受了新辅助化疗方案，随后进行了右肾部分切除术和左肾根治性切除术。术后进行了辅助放疗。术后5个月的随访影像显示右肾上极有一个由脂肪和钙化组成的坚实的异质性囊性病变，对患者没有造成任何问题。术后19个月进行了MRI检查，显示囊肿大小明显减小。
根据国际小儿肿瘤学会（SIOP）和美国国立威尔姆斯瘤研究组（NWTSG）的分类，该患者为疾病V期。患者按照NWTSG的建议接受了新辅助化疗（方案I）。这一策略被证明是有效的，因为左肾肿瘤缩小到初始大小的70%以下。在我们的报告中，采用胸部X光、腹部超声（USG）以及MRI和多层螺旋CT扫描等对比检查进行常规随访。
根据我们的经验，新辅助化疗、保留肾手术和辅助放疗相结合，对于双侧肾母细胞瘤病例是一种可行、安全且有效的选择。
