Liver transplantation as definitive treatment of an unresectable mesenchymal hamartoma in a child with Beckwith-Wiedemann Syndrome

Evelyn T. Pan, Dor Yoeli, Michael L. Kueht, N. Thao N. Galvan, Ronald T. Cotton, Christine A. O'Mahony, Abbas Rana, John A. Goss

Research output: Contribution to journalArticlepeer-review

Abstract

Mesenchymal liver hamartomas are benign tumors that can cause life-threatening abdominal distension and carry a risk for malignant transformation. In this case report, we describe a 13-month-old male with Beckwith-Wiedemann Syndrome (BWS) who presented with multiple mesenchymal liver hamartomas causing severe intra-abdominal mass effect. Imaging revealed six large multi-locular cystic lesions, ranging from 3.8 to 8.9 cm in diameter. The large size and spread of the tumors necessitated liver transplantation for complete removal. The patient successfully underwent cadaveric piggyback liver transplantation at 25 months of age. He was alive at 16-month follow-up without evidence of tumor recurrence or graft rejection. Histological examination of the hepatic masses revealed mucinous epithelial lining and abundant hepatocytes in varying stages of differentiation, supporting the diagnosis of mesenchymal hamartoma. To the best of our knowledge, this is the first reported case of liver transplantation in a patient with BWS as definitive treatment for unresectable mesenchymal liver hamartoma.

Original languageEnglish (US)
Article numberrjx167
JournalJournal of Surgical Case Reports
Volume2017
Issue number8
DOIs
StatePublished - Aug 1 2017
Externally publishedYes

ASJC Scopus subject areas

  • Surgery

Fingerprint

Dive into the research topics of 'Liver transplantation as definitive treatment of an unresectable mesenchymal hamartoma in a child with Beckwith-Wiedemann Syndrome'. Together they form a unique fingerprint.

Cite this