Epithelioid hemangioendothelioma (EHE) of liver is an extremely rare malignant tumour of vascular origin, representing less than 1 % of all vascular tumours. Nearly 260 cases have been reported in ...English literature. Radiologically it is seen as multifocal lesions. It can be seen at different sites like lungs, bones, lymph nodes, breast and soft tissue. Often it is misdiagnosed with metastases, cholangiocarcinoma or angiosarcoma. No definite treatment protocol is available due to its rarity, however these malignancy are treated by radical resection of the tumour or liver transplant and /or chemotherapy. Here we present a primary hepatic epithelioid hemangioendothelioma (HEHE) which was mimicking metastases in a 42 year old male who was treated with chemotherapy and radiotherapy. Sadly patient expired after 1 year of complete course of treatment. Imaging features can help to improve the diagnostic accuracy of this tumour.
Adult hepatoblastoma: Learning from children Rougemont, Anne-Laure; McLin, Valérie A; Toso, Christian ...
Journal of hepatology,
06/2012, Letnik:
56, Številka:
6
Journal Article
Recenzirano
Odprti dostop
Summary Hepatoblastoma is the most common malignant liver tumour in infants and young children. Its occurrence in the adult population is debated and has been questioned. The aim of this paper is to ...review the histological and clinical features of adult hepatoblastoma as described in the adult literature, and to compare the findings with those of paediatric hepatoblastoma. The developmental and molecular aspects of hepatoblastoma are reviewed and their potential contribution to diagnosis of adult hepatoblastoma discussed. Case reports of adult hepatoblastoma identified by a PubMed search of the English, French, German, Italian, and Spanish literature through March 2011 were reviewed. Forty-five cases of hepatoblastoma were collected. Age at presentation was variable. Survival was uniformly poor, except for the rare patients who presented with the relatively differentiated, foetal type. The common denominator between adult and paediatric cases is the occurrence of embryonal or immature aspect of the tumours. Whether the adult cases of hepatoblastoma represent blastemal tumours, stem cell tumours, or unusual differentiation patterns in otherwise more frequent adult liver tumours remains to be established. Adult tumours labelled as hepatoblastoma are characterised by malignant appearing mesenchymal components. Surgical management is the cornerstone of therapy in children and also appears to confer an improved prognosis in adults. Whether adult hepatoblastoma exists, remains controversial. Indeed, several features described in adult cases are markedly different from hepatoblastoma as it is understood in children, and other differential diagnoses should also be entertained. Nonetheless, hepatoblastoma should be considered in adults presenting with primary liver tumours in the absence of pre-existing liver disease. Adult and paediatric patients with immature hepatoblastoma appear to have worse outcomes, and adults presenting with presumed hepatoblastoma have an overall poorer prognosis than children with hepatoblastoma. In all patients, surgery should be the treatment of choice, neoadjuvant chemotherapy is advisable.
PDF