Liver transplantation for pediatric liver tumors: a case report of hepatic angiosarcoma with literature review.
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BACKGROUND: Pediatric hepatic angiosarcoma (HAS) is poorly understood. The lack of effective therapies for unresectable pediatric liver tumors creates a clinical imperative, positioning liver transplantation (LT) as a pivotal modality for exploring the roadmap to prolonged survival. CASE DESCRIPTION: We report the first Chinese pediatric case of HAS treated with LT. A 2-year-old boy was admitted to hospital due to abdominal pain and distension. Imaging findings were suggestive of possible malignancy, but initial biopsy yielded a diagnosed infantile hepatic hemangioma (IHH). Following living-donor LT for unresectable disease, explant pathology revealed an angiosarcoma arising within a hemangioma. The patient ultimately died from metastasis 21 months after LT. Our review of pediatric LT for liver tumors over 15 years identified nine pediatric HAS cases, only one of whom was correctly diagnosed pre-LT, with survival ranging from 3 to 66 months. Regarding other tumors, six cases of IHH were reported prior to 2010, without survival data. Recent studies on hepatoblastoma (HB) report a 5-year overall survival (OS) of 80-90%, and the prognosis for hepatocellular carcinoma (HCC) has markedly improved, with a combined 5-year OS of 74%. In the largest reported study, the 5-year OS rates for hepatic epithelioid hemangioendothelioma (HEH) and hepatic undifferentiated embryonal sarcoma (HUES) were 60.6% and 90.0%, respectively. Evidence for biliary tract rhabdomyosarcoma (BT-RMS) and malignant rhabdoid tumor (MRT) of the liver are anecdotal, and no pediatric cases of primary hepatic kaposiform hemangioendothelioma (KHE) treated with LT have been reported. CONCLUSIONS: Pediatric HAS is rare and difficult to distinguish from IHH; contrast-enhanced computed tomography/magnetic resonance imaging (CT/MRI) aids differentiation. For suspected malignant transformation, contrast-enhanced ultrasound (CEUS) and ultrasound-guided multi-site biopsy are recommended. No standardized treatment exists for pediatric HAS. Given the poor long-term survival, LT or combined chemotherapy remains exploratory but is the only option for unresectable tumors. The high survival rates of LT for HB and HCC offer a promising blueprint. With advances in surgical techniques and increased living donor availability, pediatric LT deserves more attention.