PS01ECTOPIC NEPHROGENIC RESTS IN CHILDREN: THE CLINICO-SURGICAL IMPLICATIONS

• Published in: ANZ journal of surgery Vol. 79; no. s1; p. A52
• Main Authors: Desphande, A. V., Lai hei, E. R., Kellie, S., Arbuckle, S., Cummins, G., Cooke, A. A.
• Format: Journal Article
• Language: English
• Published: Melbourne, Australia Blackwell Publishing Asia 01-05-2009; Blackwell Publishing Ltd
• Subjects: Cancer; Kidney diseases; Medical treatment; Pediatrics; Surgery
• ISSN: 1445-1433; 1445-2197
• DOI: 10.1111/j.1445-2197.2009.04925_1.x

Ectopic nephrogenic rests (ENR) are rare. The significance of ENR and their role in tumourgenesis is largely unknown, although they have been associated with Extrarenal Wilms' Tumours (ERWT). We review our experience with the surgical management of ENR and discuss the clinico-surgical implications of their incidental discovery. We reviewed the hospital records of patients with histopathologically confirmed ERWT and ENR treated at our hospital over a ten year period. Ninety-five children with WT were identified but only one case of ENR and ERWT. This patient was a fourteen month old boy who was incidentally found to have a mass in the left inguinal canal during orchiopexy. Following histology, a provisional diagnosis of ENR was made. Six months later the child went onto develop an ERWT at the same site. Periodic post surgical follow-up has been uneventful. The discovery of a site of ENR raises two distinct possibilities: either it is an isolated developmental abnormality or, of more clinical significance, the early stages of a neoplastic process. Our experience lends support for the theory that ENR are precursor lesions to the development of WT in ectopic sites. Histopathologically, ENR can be difficult to differentiate from ERWT and this adds to the uncertainty in identifying a natural clinical progression for these lesions and in labelling them as putative pre- cursor lesions to ERWT. Children with confirmed ENR should be considered as high risk for the development of ERWT and require long term post - operative follow up. [PUBLICATION ABSTRACT]