胎儿横纹肌瘤型肾母细胞瘤

Fetal rhabdomyomatous nephroblastoma

目的 探讨胎儿横纹肌瘤型肾母细胞瘤 (FRN)特点和恰当的治疗方法。方法 从195 5～ 1996年 36 9例肾母细胞瘤中检出FRN 10例。回顾性分析其临床表现、病理组织学特点和预后。结果 10例FRN均因腹部肿大或腹部肿物入院。经术前放、化疗肿瘤不缩小。 6例单侧病变做瘤肾切除 ,5例获随访均长期存活。 4例双侧病变中 1例做双侧单纯肿瘤切除 ,3例做一侧瘤肾切除 ,对侧肾部分切除或单纯肿瘤切除 ,随访存活 2例。结论 FRN是一种少见的特殊类型的肾母细胞瘤。对术前放、化疗不敏感 ,但侵袭性小 ,较少发生转移 ,应按预后好的组织结构化疗。对双侧病变单纯肿瘤切除可更多的保留肾组织 ,提高生存质量。

Objective To review the morphological and clinic features of fetal rhabdomyomatous nephroblastoma (FRN) and evaluate the treatment for this rare variant of Wilms' tumor. Methods The pathological specimens of 369 Wilms' tumors managed in Beijing Children's Hospital during the period of 1955 to 1996 were reviewed. Ten of them were classified as FRN. Retrospective analysis of histopathological and clinical data of these 10 cases were carried out. Results The 10 patients all presented with gross abdominal mass. Preoperative radiotherapy and chemotherapy were not effective. Nephrectomy was performed on 6 unilateral cases and 5 of them received postoperative chemotherapy. These 5 patients were followed-up. Long-term survival ranging from 8 to 25 years was achieved. In 4 patients with bilateral tumors, 3 received unilateral nephrectomy and contralateral partial nephrectomy or tumor enucleation and one patient underwent bilateral tumor enucleation. In the bilateral cases, long-term survival of 2 to 14 years was achieved in 2 patients. The overall survival rate of the 9 patients who were followed-up was 77.7%. Conclusions FRN is a rare variant of Wilms' tumor with less aggressive clinical behavior and less metastasis. It is not responsive to preoperative chemotherapy and radiotherapy. FRN belongs to a favorable histology type of Wilms' tumor and similar treatment is adopted. Tumor enucleation with postoperative chemotherapy may be reserved more renal tissue for patients with bilateral diseases.