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Case Reports
. 2011 Aug;26(8):1269-73.
doi: 10.1007/s00467-011-1874-1. Epub 2011 May 20.

Hepatoblastoma and prune belly syndrome: a potential association

Brian Becknell  1 , Priya Pais, Grace Onimoe, Hemalatha Rangarajan, Andrew L Schwaderer, Kirk McHugh, Mark A Ranalli, David S Hains
Affiliations
Case Reports

Hepatoblastoma and prune belly syndrome: a potential association

Brian Becknell et al. Pediatr Nephrol. 2011 Aug.

Abstract

Prune belly syndrome (PBS) is a congenital anomaly characterized by the clinical triad of lax abdominal musculature, bilateral cryptorchidism, and abnormalities of the kidney and urinary tract. Previous reports of malignancy in patients with PBS have been limited to germ cell tumors. Hepatoblastoma (HBL) is the most common hepatic malignancy of childhood, affecting approximately 100 children each year in the USA. We describe a set of 4 pediatric patients with PBS and HBL. All individuals were born after 2002. These subjects lacked genetic, natal, or environmental factors known to confer risk of HBL. The occurrence of PBS and HBL in these patients constitutes a novel potential association.

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