| A Case of Retroperitoneal Extrarenal Wilms' Tumor with Metastasis to Lung and Bone |
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Jea Heon Jeong1, Weon Cheol Han2, Du Young Choi1 |
1Department of Pediatrics, School of Medicine, Wonkwang University, Iksan, Korea
2Department of Pediatrics, School of Medicine, Wonkwang University, Iksan, Korea |
| 폐와 골 전이를 동반한 후복막강 내 신외 윌름 종양 1례 |
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정재헌1, 한원철2, 최두영1 |
1원광대학교 의과대학 소아과학교실
2원광대학교 의과대학 병리학교실 |
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| Abstract |
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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. |
| Key Words:
Extrarenal Wilms' tumor, Retroperitonium |
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