2007 Volume 43 Issue 7 Pages 933-937
Abdominal distension and a mass in a 12-month-old boy were noticed by a local doctor. The tumor was about 15cm in diameter in the left abdomen with a smooth and hard surface on palpation. In the diagnostic imaging, a large mass originated from the left kidney. Another mass, 4cm in diameter with same properties as the left tumor, was also pointed out in the lower pole of the right kidney. An abdominal open biopsy on both sides was performed. The diagnosis was nephroblastoma on the right and fetal rhabdomyomatous nephroblastoma (FRN) on the left side. We started chemotherapy with ActD and VCR to reduce the right tumor. One month later, the tumor on the right was reduced in size but the left became almost double in a diagnostic imaging. Then, he underwent left nephrectomy and enucleation of the right tumor. Most of the right kidney was able to be preserved. Pathological diagnosis was FRN on both sides. He recovered well after the operation and following chemotherapy. He has been disease free for 1 year after the treatment. FRN is a rare type of nephroblastoma with abundant striated muscle components. Chemotherapy and radiation therapy are not effective and in bilateral and large cases, their prognosis is poor. This case might provide some suggestions regarding the treatment strategy for FRN.
