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双侧同步性 Wilms 瘤伴肝转移。

Synchronous bilateral Wilms' tumor with liver metastasis.

机构信息

Department of Radiology and Radiotherapy, School of Medicine, Makerere University College of Health Sciences, Kampala, Uganda.

Department of Medicine, School of Medicine, Makerere University College of Health Sciences, Kampala, Uganda.

出版信息

BMC Urol. 2021 Jun 10;21(1):91. doi: 10.1186/s12894-021-00859-8.

Abstract

BACKGROUND

Wilms' tumor (nephroblastoma) is mostly unilateral; however, bilateral Wilms' tumors are seen in about 5-8% of patients. This can be synchronous or metachronous. It is uncommon to get liver metastasis from bilateral Wilms' tumor.

CASE PRESENTATION

An 8-year-old male Ugandan presented with a history of abdominal swelling and flank pains for 1 year. There was no history of hematuria. Both ultrasound and computed tomography of the abdomen demonstrated multiple solid lesions in both kidneys and a huge solid mass in segments V, VI, VII and VIII of the liver. Histological examination of renal biopsy specimen was favorable for chemotherapeutic regimens. However, following a multidisciplinary tumor board consensus, a nephron-sparing surgery was deemed unsuitable, and he was managed conservatively with chemotherapy (adriamycin and vincristine) with a palliative intent.

CONCLUSIONS

Metastatic bilateral Wilms' tumor has a particularly poor prognosis. There are no clear evidence-based guidelines for the management of this rare presentation. This patient benefited from early palliative care and symptom management.

摘要

背景

Wilms 瘤(肾母细胞瘤)大多为单侧;然而,双侧 Wilms 瘤在约 5-8%的患者中可见。这可以是同步的,也可以是异时的。从双侧 Wilms 瘤发生肝转移并不常见。

病例介绍

一名 8 岁的乌干达男性因腹部肿胀和侧腹痛 1 年就诊。无血尿史。腹部超声和 CT 均显示双侧肾脏多个实性病变,以及肝段 V、VI、VII 和 VIII 的巨大实性肿块。肾活检组织学检查对化疗方案有利。然而,经过多学科肿瘤委员会的共识,认为保肾手术不合适,他接受了姑息性化疗(阿霉素和长春新碱)治疗。

结论

转移性双侧 Wilms 瘤预后特别差。对于这种罕见的表现,没有明确的循证指南。该患者受益于早期姑息治疗和症状管理。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cdbe/8193872/f7748c3dc933/12894_2021_859_Fig1_HTML.jpg

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