학술논문

A fatal case of an infant with malignant rhabdoid tumor of the kidney that was refractory to multidisciplinary treatment / 集学的治療にも関わらず致死的経過を辿った乳児腎悪性ラブドイド腫瘍の一例
Document Type
Journal Article
Source
日本小児血液・がん学会雑誌 / The Japanese Journal of Pediatric Hematology / Oncology. 2018, 55(3):315
Subject
INI-1
hematuria
malignant rhabdoid tumor of the kidney
renal tumor
腎悪性ラブドイド腫瘍
腎腫瘍
血尿
Language
Japanese
ISSN
2187-011X
2189-5384
Abstract
Malignant rhabdoid tumor of the kidney (MRTK) in infants often shows poor prognosis. Here, we report a fatal case of an infant with MRTK that was refractory to multidisciplinary treatment. An eight-month-old male infant initially presented with an abdominal mass and gross hematuria. Computed tomography revealed a mass 75 mm in diameter in the right kidney without metastasis. He was tentatively diagnosed as having stage I right renal tumor and underwent one-stage surgical resection followed by postoperative chemotherapy. However, postoperative histopathological findings confirmed MRTK with renal capsule invasion. Despite receiving focal abdominal irradiation and three courses of chemotherapy comprising vincristine, doxorubicin, and cyclophosphamide, the patient developed peritoneal metastasis and died on day 130. MRTK shows rapid progression with a fatal outcome, mainly in infants. Development of novel therapeutic agents based on the pathology and biology of MRTK is warranted to improve the treatment outcome of MRTK.

Online Access