Hepatoblastoma and prune belly syndrome: a potential association
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ORIGINAL ARTICLE
Hepatoblastoma and prune belly syndrome: a potential association Brian Becknell & Priya Pais & Grace Onimoe & Hemalatha Rangarajan & Andrew L. Schwaderer & Kirk McHugh & Mark A. Ranalli & David S. Hains
Received: 31 January 2011 / Revised: 28 February 2011 / Accepted: 3 March 2011 / Published online: 20 May 2011 # IPNA 2011
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. Keywords Prune belly syndrome . Hepatoblastoma . Malignancy
Introduction Prune belly syndrome (PBS, Eagle-Barrett Syndrome, OMIM #100100) is a congenital anomaly affecting 3.8/
Electronic supplementary material The online version of this article (doi:10.1007/s00467-011-1874-1) contains supplementary material, which is available to authorized users B. Becknell (*) : G. Onimoe : A. L. Schwaderer : M. A. Ranalli : D. S. Hains Department of Pediatrics, Ohio State University College of Medicine, Columbus, OH 43205, USA e-mail: [email protected] P. Pais Division of Nephrology, Children’s Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI 53201, USA G. Onimoe : M. A. Ranalli Division of Hematology and Oncology, Nationwide Children’s Hospital, Columbus, OH 43205, USA H. Rangarajan Division of Hematology and Oncology, Children’s Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI 53201, USA
A. L. Schwaderer : D. S. Hains Division of Nephrology, Nationwide Children’s Hospital, Columbus, OH 43205, USA
K. McHugh Center for Molecular and Human Genetics, Research Institute at Nationwide Children’s Hospital, Columbus, OH 43205, USA B. Becknell : P. Pais : G. Onimoe : H. Rangarajan : A. L. Schwaderer : K. McHugh : M. A. Ranalli Center for Clinical and Translational Research, Columbus, OH 43205, USA
D. S. Hains Center for Clinical and Translational Research, Research Institute at Nationwide Children’s Hospital, Columbus, OH 43205, USA
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100,000 live male births in the USA. PBS is diagnosed based on the clinical triad of lax, “prune-like” abdominal wall musculature; bilateral cryptorchidism; and a dilated urinary tract, often associated with renal dysplasia. A diagnosis of PBS is associated with a mortality rate of 29% in the newborn period [1]. Furthermore, an estimated 50% of surviving patients with PBS develop chronic renal insufficiency and progress to end-stage renal disease (ESRD) [2]. Germ cell tumors have been described in PBS pa
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