Wilms Tumor in the Setting of Bilateral Nephroblastomatosis

Sethi, Ami Trivedi; Narla, Lakshmana Das; Fitch, Sarah; Frable, William J.

doi:10.1148/rg.305095022

Cited by 13 publications

(14 citation statements)

References 11 publications

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“…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].…”

Section: Discussionmentioning

confidence: 99%

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

Janeczko-Czarnecka¹,

Niedzielska-Łoś²,

Bronowicki³

et al. 2019

polp

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Nephroblastomatosis (NB) is the presence of multifocal residues of embryonic kidney localised in the kidney capsule, the cortex, or in the renal core. A characteristic picture of lesions in imaging studies (ultrasound, computed tomography, magnetic resonance imaging) allows us to establish the diagnosis and start chemotherapy. We present a course of treatment in three patients with baseline diagnosis of NB. We emphasise that due to the lack of initial histopathological examination diagnosis of NB only by means of imaging studies can be both a diagnostic and a therapeutic challenge. Close cooperation of specialists in the fields of radiology, surgery, and paediatric oncology is crucial.

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Section: Discussionmentioning

confidence: 99%

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

Janeczko-Czarnecka¹,

Niedzielska-Łoś²,

Bronowicki³

et al. 2019

polp

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“…They are further classified based on their location within the kidney as perilobar or intralobar [ 3 ]. Perilobar rests are restricted to the peripheral renal cortex and are associated with fetal overgrowth disorders [ 4 ] while intralobar rests occur randomly throughout the renal parenchyma as focal lesions [ 5 , 6 ]. Hyperplastic perilobar nephroblastomatosis is generally bilateral and the renal cortical surface consists partially or entirely of hyperplastic nephroblastic tissue in DHPLN [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

Case report: Diffuse hyperplastic perilobar nephroblastomatosis complicated by a unilateral Wilms tumour: diagnosis, treatment and follow-up

et al. 2018

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BackgroundNephroblastomatosis is an uncommon pathologic process characterized by the presence of persistent embryonic nephrogenic rests. Progression to Wilms tumour occurs in an estimated 35% of patients. Cure rates are based on histologic findings and disease stage and have improved from 10% in the 1920s to over 90% today.Case presentationWe report a case of a 9-month-old female presenting with a 2-month history of abdominal distension. Ultrasonographic and computed tomographic assessments demonstrated features consistent with bilateral, diffuse, hyperplastic perilobar nephroblastomatosis (DHPLNB) for which she underwent chemotherapy. Magnetic resonance imaging 6 weeks following commencement of chemotherapy revealed a mass concerning for unilateral Wilms tumor for which she underwent partial nephrectomy. Pathology confirmed DHPLNB with a unilateral Wilms tumor.Conclusion3.5 year radiographic follow up demonstrates complete recovery. To our knowledge, there are no similar cases with imaging depiction recently published. With potential for malignant transformation into Wilms tumour and low survival rate for late diagnosed Wilms tumors, it is important to recognize nephroblastomatosis early, both clinically and radiographically to improve overall patient prognosis.

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“…Perilobar rests are associated with Beckwith–Wiedemann syndrome and hemihypertrophy. Contrast-enhanced CT scan is the imaging of choice, which demonstrates hypoattenuating to isoattenuating, well-demarcated to ill-defined nonenhancing nodules with lobulations in the superficial multifocal type, and mild renomegaly with confluent, peripheral, nonenhancing areas in the superficial diffuse type [14].…”

Section: Discussionmentioning

confidence: 99%

Bilateral perirenal space fibromatosis with renal infiltration: case report and review of literature

Sabale

Pralhadan

Kalidos

et al. 2016

Radiology Case Reports

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Fibromatosis and/or desmoid tumors which constitute less than 1% of all neoplasms and 3.0% of all soft-tissue tumors are pathologically benign proliferations of the fibroblasts but are locally aggressive with infiltrative type of growth and tendency toward recurrence. Bilateral symmetrical perirenal involvement has been described in many conditions which can be renal, subcapsular, or perirenal in origin. However, bilateral perirenal fibromatosis as an isolated presentation was very uncommon. We report an exceptionally rare case of bilateral perirenal fibromatosis with renal infiltration.

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Wilms Tumor in the Setting of Bilateral Nephroblastomatosis

Cited by 13 publications

References 11 publications

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

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

Case report: Diffuse hyperplastic perilobar nephroblastomatosis complicated by a unilateral Wilms tumour: diagnosis, treatment and follow-up

Bilateral perirenal space fibromatosis with renal infiltration: case report and review of literature

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