CASE REPORT
A 2-year-old boy was diagnosed with CCSK, which relapsed 4 times until
he yielded to disease at the age of 7 years (Fig. 1, Supplementary Fig.
S1). At diagnosis, the primary tumor site was the right kidney
(Stage-I). He received chemotherapy consisting of actinomycin D,
vincristine and, doxorubicin (DD-4A) followed by radiotherapy
(10.8Gy/6Fr) after a right partial nephrectomy. This combination therapy
successfully induced complete remission. However, 4 months after the
completion of treatment, first relapse occurred at his left lung. He
received 6 courses of chemotherapy consisting of ifosfamide,
carboplatin, and etoposide (ICE) with radiotherapy (12Gy/8Fr) after a
left upper lobectomy, and then achieved second remission. At the age of
5 years, second relapse developed at the abdominal lymph nodes. A total
excision was done after three courses of chemotherapy consisting of
cyclophosphamide, vincristine, pirarubicin, and cisplatin. Postoperative
chemoradiotherapy induced third remission. At the age of 6 years, he
suffered from third relapse at the para-aortic lymph nodes. The patient
underwent a subtotal excision of these lymph nodes followed by high dose
chemotherapy (busulfan and melpharan) with autologous hematopoietic stem
cell transplantation (aHSCT). The patient achieved fourth remission;
however, four months later, fourth relapse occurred at the ascending
colon, small intestine, duodenum, pancreas, and right hepatic lobe, and
he yielded to the advanced disease.