Hyperplastic perilobar nephroblastomatosis: Long‐term survival of 52 patients
- 20 December 2005
- journal article
- research article
- Published by Wiley in Pediatric Blood & Cancer
- Vol. 46 (2), 203-221
- https://doi.org/10.1002/pbc.20386
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 with HPLN died of WT at 3, 5, and 6 years of age; 2/3 were anaplastic. Conclusions HPLN is a self-limited, pre-neoplastic proliferative process associated with a high risk of developing WT. The accurate diagnosis and the choices of therapy during the often-complex course of HPLN depend on the availability and accurate interpretation of a combination of pathologic, radiologic, and clinical information. When such information is appropriately obtained, the long-term survival of patients with HPLN is excellent.Keywords
This publication has 12 references indexed in Scilit:
- Severe Intrarenal Fibrosis, Infundibular Stenosis, Renal Cysts, and Persistent Perilobar Nephrogenic Rests in a Patient With Beckwith-Wiedemann Syndrome 27 years After Diffuse Nephroblastomatosis and Wilms Tumor: Natural Progression or a Consequence of Treatment?Journal of Pediatric Hematology/Oncology, 2002
- Management decisions in children with nephroblastomatosisMedical and Pediatric Oncology, 2000
- Nephrogenic rests and the pathogenesis of Wilms tumor: Developmental and clinical considerationsAmerican Journal of Medical Genetics, 1998
- US, CT and MR imaging characteristics of nephroblastomatosisPediatric Radiology, 1998
- Wilms tumor and nephroblastomatosis: imaging characteristics at gadolinium-enhanced MR imaging.Radiology, 1993
- Precursor lesions of Wilms tumor: Clinical and biological implicationsMedical and Pediatric Oncology, 1993
- Imaging of nephroblastomatosis: an overview.Radiology, 1992
- Nephrogenic Rests, Nephroblastomatosis, and the Pathogenesis of Wilms' TumorPediatric Pathology, 1990
- Wilms tumor arising from spontaneously regressing nephroblastomatosisAmerican Journal of Roentgenology, 1980
- Massive infantile nephroblastomatosis.A clinical, radiological, and pathological analysis of four casesCancer, 1977