Liver Transplant as Treatment Modality for Inflammatory Myofibroblastic Tumor of the Liver

A 3-year-old African female child with a noncontributory birth, past and family history presented with progressively increasing abdominal distension for 3 months associated with intermittent fever, abdominal pain and cough. On her initial evaluation, contrastenhanced computed tomography (CECT) abdomen revealed a mass in the right hepatic lobe with thrombus in the inferior vena cava (IVC) and right ventricle with enlarged aortocaval, para aortic and celiac lymph nodes with mild ascites. Liver biopsy was deferred, as the patient was sick upon presentation. Suspecting it to be hepatoblastoma, she was given 4 cycles of Doxorubicin and Carboplatin. Tumor markers carcinoembryonic antigen (CEA), Beta human chorionic gonadotropin (hCG) and Alpha-fetoprotein (AFP) were found to be negative. In view of poor response to chemotherapy and persistent symptoms, she was brought to our hospital in New Delhi, India. On presentation, she was hemodynamically stable and had firm hepatomegaly and pallor. Positron emission tomography–computed tomography (PET CT) abdomen revealed hypertrophied left lobe of the liver, 18F-fluorodeoxyglucose (FDG) avid large heterogenous arterial enhancing lesion in the right lobe liver involving segment VII, VIII, VI with extension into IVC, right atrium and ventricle. There was bilateral moderate pleural effusion with atelectasis. She underwent an ultrasound guided biopsy of the lesion which was suggestive of inflammatory Myofibroblastic tumor (bundles of oval to spindle shaped fibroblastic cells in a collagenized stroma with cells showing mild pleomorphism). Immunohistochemical stain for Anaplastic Lymphoma Kinase (ALK) was positive but stain for SMA, CD34 and CD31 were negative. Crizotinib (ALK inhibitor) was started for reduction of the tumor volume and patient improved.