Malignant transformation of infantile hepatic hemangioendothelioma to hepatic angiosarcoma: report of one case and review of literatures

Objective To investigate the possibility of infantile hepatic hemangioendothelioma （IHHE） progressing into hepatic angiosarcoma and the timing of liver transplantation. Methods Clinical characteristics, pathological features of one IHHE case progressing into malignant angiosarcoma after liver transplantation admitted to our hospital were retrospectively analyzed. The morphology and immuno-histochemical features of IHHE and angiosarcoma were analyzed．Literature review related to IHHE progressing into angiosarcoma was performed from PubMed, SinoMed, CNKI, Wanfang data and Chongqing VIP databases by using the searching words of “liver transplantation”, “infantile hepatic hemangioendothelioma”, “angiosarcoma” amd “clinicopathology”. Results The 4-year-old boy diagnosed with IHHE was admitted to our hospital due to chief complaints of abdominal distention and systemic and scleral yellowing at more than 2 years after comprehensive treatment. MRI showed the recurrence of the tumor. The patient underwent “allograft orthotopic transplantation”. Pathological biopsy demonstrated that the tumor was composed of two types of structures including typical angiosarcoma and IHHE-like morphological features. However, the tissue structure and cellular heterogeneity were more complicated compared with conventional IHHE. The transition was observed between two regions. The patient died from lung infection and heart failure at 17 d after liver transplantation. In the literature review, 3 cass of IHHE transforming into angiosarcoma all died due to disease aggravation after steroids, interferon or chemotherapy. Conclusions IHHE can progress into angiosarcoma. long-term follow-up, accurate diagnosis and effective treatment should be delivered for the affected children. The specific timing of liver transplantation should be determined by surgeons and pathologists. Whether the indications of liver transplantation should be widened to obtain better clinical prognosis remains to be elucidated.