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Liver transplantation as definitive treatment of an unresectable mesenchymal hamartoma in a child with Beckwith–Wiedemann Syndrome
- Source :
- Journal of Surgical Case Reports
- Publication Year :
- 2017
- Publisher :
- Oxford University Press, 2017.
-
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.
- Subjects :
- Pathology
medicine.medical_specialty
congenital, hereditary, and neonatal diseases and abnormalities
business.industry
medicine.medical_treatment
Mesenchymal stem cell
Beckwith–Wiedemann syndrome
Case Report
Abdominal distension
Liver transplantation
medicine.disease
Mesenchymal hamartoma
Malignant transformation
Transplant rejection
03 medical and health sciences
0302 clinical medicine
medicine.anatomical_structure
030220 oncology & carcinogenesis
medicine
Abdomen
030211 gastroenterology & hepatology
Surgery
medicine.symptom
business
Subjects
Details
- Language :
- English
- ISSN :
- 20428812
- Volume :
- 2017
- Issue :
- 8
- Database :
- OpenAIRE
- Journal :
- Journal of Surgical Case Reports
- Accession number :
- edsair.doi.dedup.....d433c3df2150f349dd10ed3c38ac74ed