Millar Alastair J W, Cox Sharon, Davidson Alan
Emeritus Professor of Paediatric Surgery, Division of Paediatric Surgery, University of Cape Town and Red Cross War Memorial Children's Hospital, Klipfontein Road, Rondebosch, Cape Town, 7700, South Africa.
Associate Professor and Head of Clinical Unit, Division of Paediatric Surgery, University of Cape Town and Red Cross War Memorial Children's Hospital, Klipfontein Road, Rondebosch, Cape Town, 7700, South Africa.
Pediatr Surg Int. 2017 Apr;33(4):461-469. doi: 10.1007/s00383-016-4047-2. Epub 2017 Jan 4.
Wilms tumour is named after Max Wilms. It is an embryonal tumour derived from the metanephros. It is the commonest childhood renal tumour and the third commonest paediatric malignancy. Synchronous bilateral Wilms tumours (BWT) represent 4-7% of all Wilms tumours (WT) and present at a younger age than unilateral Wilms tumours. At least 10% of synchronous BWTs have unfavourable histology, and up to 22% are associated with genitourinary abnormalities, aniridia, WAGR syndrome, Denys-Drash Syndrome, hemihypertrophy, or one of the other overgrowth syndromes. The long-term disease-free survival (DFS) rate for patients with unilateral Wilms' tumours is approaching 90%, and is around 70% for those with metastatic disease. For both synchronous and metachronous Wilms tumours the prognosis is less favourable with reported cure rates approaching 80% in the best centres and lower in resource poor settings. There is potential for a reduced quality of life due to renal insufficiency and the possible need for renal transplantation. The major clinical challenge in BWTs is preservation of functioning renal tissue using nephron sparing surgical techniques, while achieving cure with minimum therapy-related morbidity. Mortality is generally associated with progressive disease of anaplastic tumours. Chemotherapy followed by nephron sparing surgery has been able, in most cases, to eradicate the tumour while preserving renal function. Radiotherapy has largely been avoided because of fears of long term radiation injury to the residual functioning renal mass. Patient selection, appropriate pre- and post-operative chemotherapy and skilled surgical techniques all contribute to excellent outcomes where these are achievable.
肾母细胞瘤是以马克斯·威尔姆斯的名字命名的。它是一种源自后肾的胚胎性肿瘤。它是儿童期最常见的肾肿瘤，也是儿科第三大常见恶性肿瘤。双侧同时性肾母细胞瘤（BWT）占所有肾母细胞瘤（WT）的4 - 7%，发病年龄比单侧肾母细胞瘤小。至少10%的双侧同时性BWT具有不良组织学特征，高达22%与泌尿生殖系统异常、无虹膜、WAGR综合征、迪尼-德拉斯综合征、半侧肥大或其他过度生长综合征之一相关。单侧肾母细胞瘤患者的长期无病生存率（DFS）接近90%，而转移性疾病患者的这一比例约为70%。对于双侧同时性和异时性肾母细胞瘤，预后较差，据报道，在最好的中心治愈率接近80%，在资源匮乏地区则更低。由于肾功能不全以及可能需要肾移植，患者的生活质量可能会下降。双侧同时性肾母细胞瘤的主要临床挑战是使用保留肾单位的手术技术保留有功能的肾组织，同时以最低的治疗相关发病率实现治愈。死亡率通常与间变性肿瘤的进展性疾病相关。在大多数情况下，化疗后进行保留肾单位的手术能够在保留肾功能的同时根除肿瘤。由于担心长期辐射对残留的有功能肾组织造成损伤，放疗在很大程度上已被避免。在能够实现这些目标的情况下，患者选择、适当的术前和术后化疗以及熟练的手术技术都有助于取得良好的治疗效果。
