Solid liver tumours with cystic appearance: do they have the same outcome?

Abstract

Objective: Paediatric undifferentiated embryonal sarcoma (UES) and mesenchymal hamartoma (MH) of liver have similar imaging cystic imaging characteristic but have totally different clinical behaviour. We reviewed ours experience and compared that with the literature. Methods: Our patients' database over the past 25 years and all articles available from PubMed up to December 2016 were searched. Case reports recorded with demographic data, clinical information and investigations were recruited. The diagnosis of all cases had to be confirmed by histopathology. The data was analysed by SPSS 11.0 software. Results: We identified 2 cases each of UES and MH in our centre, and from the literature, there were a total of 219 cases of UES and 138 cases of MH. The age at diagnosis for UES ranged from 1-83 years but 39.73% were between 6-10 years, whereas 65.22% of MH were found at <2 years (range, newborn-73 years). The chief complaint for UES was abdominal distension with or without abdominal pain and 50.81% of them had accompanied systemic symptoms. In contrary, only 9.57% of MH had systemic symptoms. Interestingly, both tumours were more commonly found in the right hepatic lobe. The prognosis of these two entities was quite different, 41/103 (39.81%) of UES died, whereas only 7/110 (6.36%) cases with MH died. UES required additional chemotherapy whereas MH did not. With the limited information, some authors suggested that the ultrasonography findings were better concordant with the histology than computed tomography or magnetic resonance imaging. 54.9% UES patients' tumour was solid and only 30.36% were cystic. For MH, there was no significant difference in the nature of tumour. Conclusion: Similar imaging appearance and possible pathological overlap between UES and MH makes the initial diagnosis difficult to be established. Surgical biopsy and excision remained mandatory to confirm the diagnosis and to guide the subsequent treatment option. © 2018, Medcom Limited. All rights reserved.