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Two cases of hepatic adenomas in patients with wolf-hirschhorn syndrome: A new rare complication?

Authors

  • Giulia Prunotto,

    1. Pediatric Genetic Unit, Pediatric Department of Monza Brianza per il Bambino e la sua Mamma (MBBM) Foundation, S.Gerardo Hospital, Monza, Italy
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  • Paola Cianci,

    1. Pediatric Genetic Unit, Pediatric Department of Monza Brianza per il Bambino e la sua Mamma (MBBM) Foundation, S.Gerardo Hospital, Monza, Italy
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  • Anna Cereda,

    1. Pediatric Genetic Unit, Pediatric Department of Monza Brianza per il Bambino e la sua Mamma (MBBM) Foundation, S.Gerardo Hospital, Monza, Italy
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  • Agnese Scatigno,

    1. Pediatric Genetic Unit, Pediatric Department of Monza Brianza per il Bambino e la sua Mamma (MBBM) Foundation, S.Gerardo Hospital, Monza, Italy
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  • Chiara Fossati,

    1. Pediatric Genetic Unit, Pediatric Department of Monza Brianza per il Bambino e la sua Mamma (MBBM) Foundation, S.Gerardo Hospital, Monza, Italy
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  • Silvia Maitz,

    1. Pediatric Genetic Unit, Pediatric Department of Monza Brianza per il Bambino e la sua Mamma (MBBM) Foundation, S.Gerardo Hospital, Monza, Italy
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  • Andrea Biondi,

    1. Pediatric Hematology-Oncology Unit, Pediatric Department of MBBM Foundation, S.Gerardo Hospital, Monza, Italy
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  • Angelo Selicorni

    Corresponding author
    • Pediatric Genetic Unit, Pediatric Department of Monza Brianza per il Bambino e la sua Mamma (MBBM) Foundation, S.Gerardo Hospital, Monza, Italy
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  • Giulia Prunotto and Paola Cianci equally contributed to this study.
  • Conflict of interest: none.

Correspondence to:

Dr. Angelo Selicorni, Pediatric Genetic Unit, Pediatric Department of MBBM Foundation, S.Gerardo Hospital, Monza, Italy.

E-mail: angelo.selicorni@gmail.com

Abstract

Wolf–Hirschhorn syndrome (WHS) is a rare microdeletion syndrome associated with a characteristic facial appearance, failure to thrive, psychomotor delays, and various major malformations of internal organs; many medical complications have been described (feeding difficulties, epilepsy, hearing problems). Benign or malignant oncologic problems are not a typical feature of the natural history of these patients. We report on two patients with WHS patients in whom hepatic adenoma (HA) were diagnosed during adolescence. The clinical evolution of liver involvement was different between the two. We discuss the possibility of considering HA as a rare medical problem in the follow-up of WHS patients. © 2013 Wiley Periodicals, Inc.

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