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01.08.2011 | Original Article

Hepatoblastoma and prune belly syndrome: a potential association

verfasst von: Brian Becknell, Priya Pais, Grace Onimoe, Hemalatha Rangarajan, Andrew L. Schwaderer, Kirk McHugh, Mark A. Ranalli, David S. Hains

Erschienen in: Pediatric Nephrology | Ausgabe 8/2011

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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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Titel: Hepatoblastoma and prune belly syndrome: a potential association
verfasst von: Brian Becknell
Priya Pais
Grace Onimoe
Hemalatha Rangarajan
Andrew L. Schwaderer
Kirk McHugh
Mark A. Ranalli
David S. Hains
Publikationsdatum: 01.08.2011
Verlag: Springer Berlin Heidelberg
Erschienen in: Pediatric Nephrology / Ausgabe 8/2011
Print ISSN: 0931-041X
Elektronische ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-011-1874-1

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