The Japanese Journal of Pediatric Hematology / Oncology
Online ISSN : 2189-5384
Print ISSN : 2187-011X
ISSN-L : 2187-011X
Case Report
A case of blastemal predominant type of Wilms tumor with focal anaplasia resistant to standard adjuvant chemotherapy
Shojiro HanakiYasuo NakaharaSoichi NakadaYusuke TakahashiTakahiro OhkuraSyuichi IshibashiKohsuke HitomiAsami Ukida
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2021 Volume 58 Issue 1 Pages 45-49

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Abstract

A 4-year-old girl presented with an abdominal mass to her previous physician. She had right kidney tumor as revealed by CT and underwent tumor resection. Pathologically, the tumor had features of both focal anaplasia and blastemal predominant type of Wilms tumor, and had a para-aortic lymph node metastasis. She was diagnosed as having stage 3 Wilms tumor and received adjuvant chemotherapy with the DD4A regimen and abdominal irradiation with JWiTS-2. During the treatment with the DD4A regimen, masses were newly documented in the tumor excision site and liver and were judged as recurrence. Two cycles of salvage chemotherapy with the ICE regimen were effective, and she received additional chemotherapy with Regimen I of JWiTS-2. She is alive without evidence of disease 18 months after the completion of therapy. Although focal anaplasia and blastemal predominant types are not clearly defined as high-risk subtypes in the JWiTS protocol, there is room for consideration in the choice of the chemotherapeutic regimen for patients with Wilms tumor.

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© 2021 The Japanese Society of Pediatric Hematology / Oncology
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