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

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.

Hepatic mesenchymal hamartoma and undifferentiated embryonal sarcoma of the liver: a pathologic review

“…Surgical resection is the mainstay treatment for HMH, although recurrence may occur in the setting of positive resection margins [1,2,17]. Liver transplantation may be considered in unresectable cases [37,38]. Ultrasound-guided percutaneous cyst aspiration has been shown to improve the clinical course of prenatal cases [2,39].…”

Giant Mesenchymal Hepatic Hamartomas With Adrenal Involvement Precipitating Respiratory Failure: A Myxomatous Mystery in a Three-Month-Old

“…To our knowledge, this has only sparsely been described in the literature and may suggest a malignant potential in an otherwise benign lesion. Furthermore, this is one of only a few cases in the literature of a patient successfully undergoing a whole liver transplant for HMH as opposed to surgical resection or percutaneous drainage [12,13].…”

Fetal mesenchymal hamartoma of the liver: A case report and literature review

“…El Demellawy et al described an atypical HML with a tandem triplication of chromosomal segment at chromosomem 1q44 ( 32 ). Meanwhile, rare cases of HML in the setting of Beckwith-Wiedemann syndrome (BWS) have been reported ( 33 ). HML may also be part of the expanding spectrum of findings of BWS ( 34 ).…”

“…For some neonates with unstable vital signs, tumor vascular interventional therapy can be considered ( 43 ). Liver transplantation is the most effective treatment for unresectable symptomatic HML ( 33 , 44 ).…”