PEDBASE.org - The Pediatric Database - Detailed information of CONGENITAL MESOBLASTIC NEPHROMA

CONGENITAL MESOBLASTIC NEPHROMA

DEFINITION:

A benign renal neoplasm characterized usually by an abdominal mass in the first 6 months of life.

EPIDEMIOLOGY:

incidence: rare renal tumor (on differential for Wilms' Tumor)
peak age of onset:
- <6 months
risk factors:
- M > F

PATHOGENESIS:

1. Background

tumor origin - kidney
primary tumors tend to be massive, firm, infiltrative extending through the capsule but essentially benign
will rarely metastasize
arises from mesenchymal tissue
if a solid renal tumor is detected prenatally, it is most likely a Congenital Mesoblastic Nephroma (CMN)

CLINICAL FEATURES:

1. Primary Tumor

abdominal mass - asymptomatic

INVESTIGATIONS:

1. Imaging Studies

Abdominal X-Ray, Ultrasound, and/or CT
IVP

2. Pathology

1. Tumor

1. Electron Microscopy

fibroblasts or myofibroblasts

2. Light Microscopy

histology: conventional, atypical cellular, mixed

MANAGEMENT:

1. Surgury

clean wide excision; tumor friable and extremely prone to rupture

2. Chemo/Radiation

not indicated

Pediatric Database - CONGENITAL MESOBLASTIC NEPHROMA

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