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[双侧肾母细胞瘤]

[Bilateral Wilms tumor].

作者信息

Presedo A, Martínez Ibáñez V, Marqués A, Sánchez de Toledo J, Boix Ochoa J

机构信息

Departamento de Cirugía Pediátrica, Hospital Materno Infantil Vall d'Hebron, Barcelona.

出版信息

Cir Pediatr. 1997 Jul;10(3):108-11.

PMID:9376233
Abstract

The incidence of bilateral involvement it is generally estimated to be 5% to 10%. It shows association with certain congenital anomalies and it has an increased occurrence of familial cases. The records of 9 children (5 boys, 4 girls) diagnosed at Vall d'Hebron Hospital with bilateral Wilms tumor between 1976-1995 were analyzed. Six patients had synchronous tumors and 3 had metachronous lesions. Genitourinary malformations were present in 4 children and another had hemihypertrophy. Two children were brothers. Eight patients underwent pre-operative radiation therapy and/or chemotherapy. Five patients had nephrectomy on one side (3 of them had metachronous presentation) and partial nephrectomy on the other side. The other children had bilateral partial nephrectomy or tumorectomy. Seven out of the nine patients are alive (78%). The two children who died presented with stage IV tumors and high grade malignant. One boy suffers cardiomyopathy. All survivors have normal renal function. With the proven efficacy of chemotherapy, bilateral renal salvage procedures were demonstrated to be effective in controlling disease without compromising renal function or survival. The innovative approaches developed for the treatment of bilateral Wilms tumor may influence the treatment of unilateral Wilms.

摘要

双侧受累的发生率一般估计为5%至10%。它与某些先天性异常有关,且家族性病例的发生率有所增加。对1976年至1995年间在瓦尔德希布伦医院诊断为双侧肾母细胞瘤的9名儿童(5名男孩,4名女孩)的记录进行了分析。6例患者为同步肿瘤,3例为异时性病变。4名儿童存在泌尿生殖系统畸形,另1名有半身肥大。2名儿童是兄弟。8例患者接受了术前放疗和/或化疗。5例患者一侧行肾切除术(其中3例为异时性表现),另一侧行部分肾切除术。其他儿童接受了双侧部分肾切除术或肿瘤切除术。9例患者中有7例存活(78%)。死亡的2名儿童表现为IV期肿瘤且恶性程度高。1名男孩患有心肌病。所有幸存者的肾功能均正常。随着化疗已证实的疗效,双侧肾脏挽救手术被证明在不影响肾功能或生存率的情况下有效控制疾病。为治疗双侧肾母细胞瘤而开发的创新方法可能会影响单侧肾母细胞瘤的治疗。

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