Some multifocal nephrogenic rests may become hyperplastic, which may produce a thick rind of blastemal or tubular cells that enlarge the kidney. Nephroblastomatosis may be diagnosed radiographically by magnetic resonance imaging, in which the homogeneity of the hypointense rind-like lesion on contrast-enhanced imaging differentiates it from Wilms tumor. Biopsy often cannot discriminate Wilms tumor from these hyperplastic nephrogenic rests. Differentiation may occur after chemotherapy is administered.
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Current treatment includes vincristine and dactinomycin until nearly complete resolution, as determined by imaging.
Even with treatment (vincristine and dactinomycin), about one-half of children diagnosed with nephroblastomatosis will develop Wilms tumor within 36 months. In a series of 52 patients, 3 patients died of recurrent Wilms tumor. In treated children, as many as one-third of Wilms tumors are anaplastic, probably as a result of selection of chemotherapy-resistant tumors, so early detection is critical. Patients are monitored by imaging at a maximum interval of 3 months, for a minimum of 7 years. Given the high incidence of bilaterality and the subsequent Wilms tumors, renal-sparing surgery is indicated.
These patients are eligible for treatment on the COG-AREN0534 trial with vincristine and dactinomycin.
Perlman EJ, Faria P, Soares A, et al.: Hyperplastic perilobar nephroblastomatosis: long-term survival of 52 patients. Pediatr Blood Cancer 46 (2): 203-21, 2006.
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May 28, 2015
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