Scintigraphic Imaging of Hepatic Epithelioid Hemangioendothelioma

Malignant hepatic epithelioid hemangioendothelioma (HEH) is a rare malignant tumor of vascular origin with unknown etiology and a variable natural course.The authors present a comprehensive review of the literature on HEH with a focus on clinical outcome after different therapeutic strategies. All published series on patients with HEH (n ¼ 434 patients) were analyzed from the first description in 1984 to the current literature. The reviewed parameters included demographic data, clinical manifestations, therapeutic modalities, and clinical outcome. The mean age of patients with HEH was 41.7 years, and the male-tofemale ratio was 2:3. The most common clinical manifestations were right upper quadrant pain, hepatomegaly, and weight loss. Most patients presented with multifocal tumor that involved both lobes of the liver. Lung, peritoneum, lymph nodes, and bone were the most common sites of extrahepatic involvement at the time of diagnosis. The most common management has been liver transplantation (LTx) (44.8% of patients), followed by no treatment (24.8% of patients), chemotherapy or radiotherapy (21% of patients), and liver resection (LRx) (9.4% of patients). The 1-year and 5-year patient survival rates were 96% and 54.5%, respectively, after LTx; 39.3% and 4.5%, respectively, after no treatment, 73.3% and 30%, respectively, after chemotherapy or radiotherapy; and 100% and 75%, respectively, after LRx. LRx has been the treatment of choice in patients with resectable HEH. However, LTx has been proposed as the treatment of choice because of the hepatic multicentricity of HEH. In addition, LTx is an acceptable option for patients who have HEH with extrahepatic manifestation. Highly selected patients may be able to undergo living-donor LTx, preserving the donor pool. The role of different adjuvant therapies for patients with HEH remains to be determined.

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Primary malignant hepatic epithelioid hemangioendothelioma

Mehrabi

Kashfi

Fonouni

et al. 2006

Cancer

424

295

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Pediatric liver neoplasms: a radiologic-pathologic correlation

et al. 1999

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Only 1-2 % of all pediatric tumors occur in the liver. Two thirds of these tumors are malignant and almost all of the tumors cause clinical symptoms due to their mass effects. Besides the poor prognosis in most of the malignant tumors, for further treatment the origin and nature of the neoplasm has to be known. Due to the mostly unimpeded growth into the peritoneal cavity, the origin of the tumors is primarily often unclear and can non-invasively only be determined by advanced imaging techniques. The display of the macro- and microhistological key features of primary pediatric liver neoplasms, including hepatoblastoma (HB), infantile hemangioendothelioma (IHE), mesenchymal hamartoma (MH), undifferentiated (embryonal) sarcoma (UES), and hepatocellular carcinoma (HCC), together with their imaging representation by ultrasound, computed tomography, and magnetic resonance imaging, may deepen the understanding of the underlying pathology and its imaging appearance. Furthermore, in many cases sufficient information may be provided not only to differentiate benign from malignant tumors, but also to guide for adequate treatment.

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Hemangioendothelioma

Türkmen

Rozanes

2008

Cancer Imaging

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Scintigraphic Imaging of Hepatic Epithelioid Hemangioendothelioma

Cited by 8 publications

References 7 publications

Primary malignant hepatic epithelioid hemangioendothelioma

Primary malignant hepatic epithelioid hemangioendothelioma

Pediatric liver neoplasms: a radiologic-pathologic correlation

Hemangioendothelioma

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