Abstract

Mesenchymal hamartoma of liver is the third most common hepatic tumor in pediatric age group. Typically, it presents as a large multicystic mass in children. Gradually they get increases in size and some can undergo incomplete spontaneous regression and very rarely, few have shown malignant transformation to undifferentiated(embryonal) sarcoma. Here, we report a 6 year old female child with no relevant personal and family history, who presented with abdominal distension and vomiting. Radiology revealed a multiloculated large cystic lesion in the right lobe of liver with a possibility of hemangioendothelioma on ultrasonography and CT abdomen was suggestive of mesenchymal hamartoma of liver. Following worsening of symptoms, partial excision and marsupialisation of the cyst was performed. Histologically, it was diagnosed as mesenchynal hamartoma with undifferentiated embryonal sarcomatous component and immunohistochemical confirmation done.

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