title: Hepatoblastoma and prune belly syndrome: a potential association.
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.
authors: Department of Pediatrics, Ohio State University College of Medicine, Columbus, OH 43205, USA. Michael.Becknell@nationwidechildrens.org
authors: Brian
authors: B
authors: Becknell
authors: Priya
authors: P
authors: Pais
authors: Grace
authors: G
authors: Onimoe
authors: Hemalatha
authors: H
authors: Rangarajan
authors: Andrew L
authors: AL
authors: Schwaderer
authors: Kirk
authors: K
authors: McHugh
authors: Mark A
authors: MA
authors: Ranalli
authors: David S
authors: DS
authors: Hains
references: Pediatr Blood Cancer. 2007 Sep;49(3):357-9
references: 16283630
references: Clin Genet. 1990 Dec;38(6):410-4
references: 2289313
references: J Pediatr Surg. 1990 Aug;25(8):924
references: 2169528
references: J Pediatr. 1998 Mar;132(3 Pt 1):398-400
references: 9544889
references: Am J Epidemiol. 2006 May 1;163(9):818-28
references: 16510543
references: Clin Pediatr (Phila). 1980 Jul;19(7):485-8
references: 6103774
references: Clin Dysmorphol. 2002 Oct;11(4):293-4
references: 12401997
references: Pediatrics. 2009 Jul;124(1):96-104
references: 19564288
references: Arch Pediatr Adolesc Med. 1995 Jan;149(1):70-6
references: 7827665
references: J Pediatr Hematol Oncol. 2009 Jul;31(7):527-9
references: 19564751
references: Am J Med Genet A. 2004 Aug 30;129A(2):198-200
references: 15316976
references: Eur Urol. 1991;19(1):79-80
references: 1672521
references: Urology. 2010 Jul;76(1):44-8
references: 20381841
references: Am J Med. 1986 Nov;81(5):895-7
references: 2877580
references: Br J Cancer. 2008 May 6;98(9):1570-3
references: 18392049
references: Pediatrics. 2010 Jul;126(1):e235-8
references: 20547648
references: Cancer. 1989 Sep 1;64(5):1169-76
references: 2547509
references: Cancer. 2004 Mar 1;100(5):1070-6
references: 14983504
references: Cancer. 1987 May 15;59(10):1776-80
references: 3030527
references: Br J Urol. 1983 Oct;55(5):580-1
references: 6626914
references: Pediatr Int. 2001 Jun;43(3):302-5
references: 11380930
references: Pediatr Nephrol. 2010 Jun;25(6):1185-9
references: 20084400
references: Pathologe. 2000 Nov;21(6):456-9
references: 11148826