Cystic-like liver tumours in children: two diseases with similar appearance but required different treatment approach

Abstract

PURPOSE/OBJECTIVE: Liver tumour only accounts for 0.5-2% of all tumours in children. Cystic-like hepatic tumours such as undifferentiated embryonal sarcoma (UES) and mesenchymal hamartoma (MH) are even rarer. Due to the differences in prognosis and treatment, timely and accurate diagnosis is essential. However, their closely resembling clinical characteristics make the initial diagnosis difficult. We reviewed our experience and tried to identify any unique clinical pattern. MATERIALS AND METHODS: This is a retrospective review of patients’ charts and database over a 15-yrs period. Clinical and imaging characteristics of indexed patients were analyzed. RESULTS: 2 cases each of UES & MH were identified and all were confirmed histopathologically. Their age of diagnoses were 10mos, 10yrs, 13yrs & 15yrs and 3 were girl. Both MH patients presented with abdominal distension without other symptoms. In contrary, one UES patient had abdominal distension and pain associated with anorexia and weight loss. There were no remarkable laboratory results except one MH patient had mild elevated liver parenchymal enzymes and alpha-fetoprotein. Both ultrasonography & CT-scan showed multiple cystic + / - solid lesions in all patients with MH and UES. The tumours of two cases with MH were gross totally excised and the ’cysts’ were actually myxoid tissue. Trisegmentectomy of liver was done due to rapidly mounting abdominal distension and pain in one UES patient. The other UES patient underwent hemi-hepatectomy. Both UES patients received chemotherapy (IRS-IV protocol). All patients achieved long term remission. Interestingly, both UES patients were found to have multiple hypo-enhancing liver lesions suggestive of metastasis in 6 to 12-months after completing chemotherapy but turned out to be focal nodular hyperplasia by biopsy. CONCLUSIONS::MH & UES had similar imaging appearance and possible pathological overlap; the presenting patterns may be more striking in some UES patients. However, surgical biopsy and excision remained mandatory to confirm the diagnosis and guide the subsequent treatment.