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Hepatoblastoma and Wolf–Hirschhorn syndrome: Coincidence or a new feature of a rare disease?

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Wolf–Hirschhorn syndrome (WHS) is a chromosomal disorder characterized by multiple congenital anomalies. Deletion of the distal part of chromosome 4 (4p 16.3) is the underlying cause of the disease. Typically… Click to show full abstract

Wolf–Hirschhorn syndrome (WHS) is a chromosomal disorder characterized by multiple congenital anomalies. Deletion of the distal part of chromosome 4 (4p 16.3) is the underlying cause of the disease. Typically patients have a wide nasal bridge continuing to the forehead, giving the face the appearance of a Greek warrior helmet. Patients are small for gestational age, and weight gain and growth are limited after birth. Patients usually have seizures until 3 years of age, and majority of them have mental or motor retardation. Cancer is not a frequent feature of the syndrome, but hematopoietic system malignancies and hepatic adenoma have been reported in a few cases. Herein, we report a pediatric patient with WHS who developed hepatoblastoma.

Keywords: feature; hirschhorn syndrome; disease; wolf hirschhorn; hepatoblastoma

Journal Title: Pediatrics International
Year Published: 2017

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