Bilateral universal nephroblastomatosis in an 8-month-old infant treated with chemotherapy

Abstract

We report the case of an 8-month-old female infant presenting with bilateral, diffusely enlarged kidneys. A diagnosis of bilateral, universal nephroblastomatosis was made on tissue biopsies from both kidneys after correlation with the radiological findings. As far as we know, this is the oldest patient reported with this diagnosis in the English literature (they are usually younger than 4 months). The patient was treated with chemotherapy with very good response and almost 1 year later she is showing no signs of recurrence of her disease.

An Approach to Renal Masses in Pediatrics

“…15 Microscopic findings range from benign small nests of blastemal cells to large areas with frank malignant transformation (WT), and is extremely difficult to differentiate between the 2 based on needle or even incisional biopsies alone. 90,91 Current recommendations are for treatment with chemotherapy (vincristine and dactinomycin) until nearly complete resolution as determined by imaging. 15 However, given the high incidence of bilaterality and the subsequent development of WT, renal sparing surgery is indicated (partial nephrectomy).…”

Is it really nephroblastomatosis? Analysis of diagnostic and therapeutic difficulties: case report of three patients

“…The authors thus proved that hyperplastic, perilobar NB must be regarded as a pre-neoplastic lesion, and accurate diagnosis in cooperation with radiologists and paediatric surgeons combined with proper treatment enables long-term survival of these patients. Close follow-up of NB patients during and after chemotherapy and readiness to perform therapeutic intervention are emphasised in the literature due to lack of predictability of clinical course [1,16,17].…”

“…Nephroblastomatosis (NB) is defined as the presence after the 36 th week of gestation of diffuse, multifocal nephrogenic rests (NR) located in the kidney capsule in the renal cortex or in the renal core [1]. Based on autopsy results, NR-like lesions are present in approximately 1% of newborns, and malignant transformation occurs in less than 1% of them [2].…”

Nephroblastomatosis and wilms tumor: dangerous liaisons

“…Most retrieved papers consisted either of multicentric retrospective reviews reporting on long periods of time (2,6) or case reports (1,(7)(8)(9)(10)(11)(12)(13)(14)(15). The most exhaustive paper available, a multicentric review from Perlman et al, reports on 52 cases/30 years (less than 2/year), while covering more than 90% of the cases registered in the USA (2).…”

“…Hyperplastic diffuse nephroblastomatosis (HDNBM), also called universal nephroblastomatosis (1), is a rare pre-malignant condition, associated with Wilms tumor (WT) in a third to half of the cases reported. Most agree that HCNBM should be treated with chemotherapy and followed-up closely, aiming to detect WT degeneration early.…”