Bilaterally multicentric synchronous Wilms' tumor: Successful conservative treatment despite persistence of nephrogenic rests

J. J. Regalado, M. M. Rodriguez, S. Toledano

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11 Scopus citations

Abstract

We report a case of synchronous bilateral Wilms' tumor in a girl with incomplete Beckwith-Wiedemann syndrome and hemihypertrophy. Multiple small tumors were present in both kidneys. The initial diagnostic biopsy showed stage 1 monophasic blastematous Wilms' tumor of favorable histology, with multiple perilobar nephrogenic rests (nephroblastomatosis). By flow cytometry, tumor was diploid, with an S-phase fraction of 13.9%. Dactinomycin and vincristine were begun as per the National Wilms' Tumor Study IV (18 week course). After 1 month, only a single small lesion was evident, which persisted unchanged. Excisional biopsy 5 months after beginning chemotherapy showed entirely necrotic tumor but apparently unaltered nephrogenic rests. After completing chemotherapy, the child has done well, with normal renal function and no evidence of disease 3 years after diagnosis. Management of stage V Wilms' tumor is tailored to the individual case, being as conservative as possible to spare renal parenchyma. Given the high incidence of coexisting nephrogenic rests in bilateral Wilms' tumor, careful follow-up is required, as these potentially pre-malignant rests may resist chemotherapy.

Original languageEnglish (US)
Pages (from-to)420-423
Number of pages4
JournalMedical and Pediatric Oncology
Volume28
Issue number6
DOIs
StatePublished - May 28 1997

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Keywords

  • Beckwith-Wiedemann syndrome
  • hemihypertrophy
  • nephroblastomatosis
  • nephrogenic rests
  • nephromegaly
  • Wilms' tumor

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Oncology
  • Cancer Research

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