Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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A Case of Retroperitoneal Extrarenal Wilms' Tumor with Metastasis to Lung and Bone

폐와 골 전이를 동반한 후복막강 내 신외 윌름 종양 1례

  • Jeong, Jae Heon (Department of Pediatrics, School of Medicine, Wonkwang University) ;
  • Han, Weon Cheol (Department of Pathology, School of Medicine, Wonkwang University) ;
  • Choi, Du Young (Department of Pediatrics, School of Medicine, Wonkwang University)
  • 정재헌 (원광대학교 의과대학 소아과학교실) ;
  • 한원철 (원광대학교 의과대학 병리학교실) ;
  • 최두영 (원광대학교 의과대학 소아과학교실)
  • Received : 2004.09.03
  • Accepted : 2004.10.14
  • Published : 2005.01.15
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Abstract

Extrarenal Wilms' tumor is a very exceptional tumor which is defined as Wilms' tumor found anywhere other than in the kidneys. Until now less than 60 cases have been reported in the English literature. The tumor can be located in the retroperitoneum, inguinal canal, uterus, cervix, testes, skin and even in the thorax. The diagnosis is almost always made after surgical intervention. Distant metastasis of this tumor has only been reported in the case of a 6-year-old girl who developed Wilms' tumor in the inguinal canal with lung involvement. In this paper we report a case of extrarenal Wilms' tumor in retroperitoneum with distant metastasis to lungs, ribs and pelvic bone in a 6-year old male. The tumor was successfully removed without tumor cell spillage. The patient was diagnosed as favorable histologic group, in which the tumor consists of epithelial, blastemal and mesenchymal components without teratomatous elements. He received combination chemotherapy according to NWTS-IV guidelines and radiation to involved lungs, and has been alive and well for the last 6 months without severe complication or relapse.

저자들은 8세 남아에서 교통사고 후 우연히 발견된 혈흉과 복부 종물의 진단 과정에서 확진된 폐 및 골 전이를 동반한 후복강 내 신외 윌름 종양 1례를 경험하였기에 문헌 고찰과 함께 보고 하고자 한다.

Keywords

Acknowledgement

Supported by : 원광대학교

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