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Hyperplastic perilobar nephroblastomatosis: Long‐term survival of 52 patients by Elizabeth J. Perlman; Paulo Faria; Andreia Soares; Fred Hoffer; Simone Sredni; Michael Ritchey; Robert C. Shamberger; Daniel Green; J.B. Beckwith is a Medicine article available to read on EtoBox.

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## Abstract ## Background This study provides insight into the clinical behavior, diagnostic complexities, and long‐term management of patients with hyperplastic perilobar nephroblastomatosis (HPLN). ## Procedure Fifty‐two patients with HPLN with available long‐term follow‐up were retrospectively analyzed for pathologic, radiologic, and clinical features. ## Results The mean age at diagnosis was 16 months; the lesions were bilateral in 49 patients. Of 33 patients who initially underwent diagnostic biopsy and adjuvant chemotherapy, 18 (55%) developed Wilms tumor (WT) at a mean of 35 months from diagnosis. Of 16 patients whose initial therapy included nephrectomy and adjuvant therapy, three (19%) developed WT at a mean of 36 months from diagnosis. All three patients who underwent initial diagnostic biopsy and received no adjuvant therapy during their initial course developed WT 4, 4, and 10 months following diagnosis. 24/52 patients developed either a single (13 patients) or multiple (11 patients) WT throughout their course; 8/24 (33%) of WT were anaplastic. The time from initial diagnosis to the development of the last WT ranged from 13 to 116 months (mean 42 months). Three children

Who reads Hyperplastic perilobar nephroblastomatosis: Long‐term survival of 52 patients?

Author

Elizabeth J. Perlman; Paulo Faria; Andreia Soares; Fred Hoffer; Simone Sredni; Michael Ritchey; Robert C. Shamberger; Daniel Green; J.B. Beckwith

Publisher

John Wiley and Sons; Wiley (John Wiley & Sons); John Wiley & Sons Inc.; Wiley (ISSN 1545-5009)

Published

2005

Language

EN

Field

Medicine (Health Sciences)