Perilobar Nephroblastomatosis: Natural History and Management. (9th July 2014)

• Record Type: Journal Article
• Authors: Stabouli, S.; Printza, N.; Dotis, J.; Matis, A.; Koliouskas, D.; Gombakis, N.; Papachristou, F.
• Other Names: Cozzi Denis A. Academic Editor.
• Is Part Of: Case reports in pediatrics. Volume 2014(2014)
• Publication Date: 2014-07-09
• Subjects: Pediatrics -- Periodicals; Pediatrics; Pediatrics; Electronic journals; Periodicals; Case Reports; Periodicals; Case Reports; Fulltext; Internet Resources; Periodicals; 618.92
• DOI: 10.1155/2014/756819
• Languages: English
• ISSNs: 2090-6803

Abstract

Abstract : Nephroblastomatosis (NB) has been considered as a precursor of Wilms tumor (WT). The natural history of NB seems to present significant variation as some lesions may regress spontaneously, while others may grow and expand or relapse and develop into WT later in childhood. Although, most investigators suggest adjutant chemotherapy, the effect and duration of treatment are not well established. Children with diffuse perilobar NB, Beckwith-Wiedemann syndrome, and hemihypertrophy seem to particularly benefit from treatment. We discuss our experience on two cases of NB and we review the literature for the management of this rare condition.