Premium
Long‐term remission of bilateral Wilms tumors that developed from premature separation of chromatids/mosaic variegated aneuploidy syndrome due to bilateral nephrectomy and peritoneal dialysis
Author(s) -
Ochiai Kayo,
Yamada Ai,
Kimoto Yasuhiro,
Imamura Hideaki,
Ikeda Toshio,
Matsukubo Makoto,
Ieiri Satoshi,
Moritake Hiroshi
Publication year - 2019
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.27804
Subject(s) - medicine , nephrectomy , wilms' tumor , peritoneal dialysis , bilateral nephrectomy , chemotherapy , rhabdomyosarcoma , surgery , vincristine , aneuploidy , sarcoma , kidney , pathology , cyclophosphamide , biochemistry , chemistry , chromosome , gene
We report a 38‐month‐old Japanese male with premature chromatid separation/mosaic variegated aneuploidy syndrome bearing biallelic BUB1B germline mutations who suffered from bilateral Wilms tumor. After right nephrectomy, dactinomycin monotherapy was administered for the left Wilms tumor; however, severe adverse reaction prevented the patient from receiving further chemotherapy. Left nephrectomy was then performed without postoperative chemotherapy. The patient survived for 15 months after bilateral nephrectomy without peritoneal relapse, metastasis of Wilms tumor, or the occurrence of rhabdomyosarcoma and maintained a good quality of life while receiving peritoneal dialysis at home.