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

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, s...

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Bibliographic Details
Published in:Pediatric blood & cancer 2019-08, Vol.66 (8), p.e27804-n/a
Main Authors: Ochiai, Kayo, Yamada, Ai, Kimoto, Yasuhiro, Imamura, Hideaki, Ikeda, Toshio, Matsukubo, Makoto, Ieiri, Satoshi, Moritake, Hiroshi
Format: Article
Language:English
Subjects:
Aneuploidy
bilateral nephrectomy
bilateral Wilms tumor
Chemotherapy
Child, Preschool
Chromatids
Chromatids - pathology
Chromosome Disorders - complications
Chromosome Disorders - pathology
Chromosome Disorders - therapy
Dactinomycin
Hematology
Humans
Kidney Neoplasms - complications
Kidney Neoplasms - pathology
Kidney Neoplasms - therapy
Male
Metastases
Mosaicism
Nephrectomy
Nephrectomy - mortality
Oncology
PCS/MVA syndrome
Pediatrics
Peritoneal dialysis
Peritoneal Dialysis - mortality
Peritoneum
Prognosis
Quality of Life
Remission
Remission Induction
Rhabdomyosarcoma
Survival Rate
Tumors
Wilms Tumor - complications
Wilms Tumor - pathology
Wilms Tumor - therapy
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Summary: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.
ISSN:1545-5009
1545-5017
DOI:10.1002/pbc.27804