Clinical Courses of Primary Hepatic Angiosarcoma: Retrospective Analysis of Eight Cases

Hur, Chang Jae; Min, Bo Ram; Lee, Yoo Jin; Jang, Byoung Kuk; Kim, Eun Soo; Park, Kyung Sik; Cho, Kwang Bum; Kang, Yu Na; Chung, Woo Jin

doi:10.4166/kjg.2015.65.4.229

Cited by 11 publications

(10 citation statements)

References 23 publications

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“…All patients invariably died after a median of six/seven months due to tumour recurrence. Similar results have been shown in and outside the transplant setting (61,(68)(69)(70)(71)(72)(73)(74). Both European and American experiences confirm that HHS is an absolute contra-indication to LT (11,72).…”

Section: Hhssupporting

confidence: 75%

Vascular tumours of the liver: a particular story

Lerut

Iesari

2018

Transl. Gastroenterol. Hepatol

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Vascular tumours of the liver represent an underrated chapter of medical and surgical hepatology. These tumours cover a wide spectrum ranging from the frequent and most benign hepatic haemangioma (HH), via the rare and intermediately aggressive hepatic epithelioid haemangioendothelioma (HEHE) to the rare and most malignant hepatic haemangiosarcoma (HHS). In contrast to the treatment algorithms for hepatocellular and cholangiocellular cancer, the diagnostic and therapeutic approaches to HEHE and HHS are not well developed. The related uncertainty is explained by their rare occurrence and their protean clinical, morphological (imaging) and histopathological presentation and behaviour. This article gives an update about these particular tumours based on the analysis of the recent literature and of the studies on vascular tumours published by the European Liver Intestine Transplantation Association (ELITA)-European Liver Transplant Registry (ELTR). It focuses also on the place of liver transplantation (LT) in the respective therapeutic algorithms. The differential diagnosis between these vascular and other tumour types may be very difficult. Correct diagnosis is of utmost importance and is based on a high index of clinical suspicion and on the integration of clinical, radiological, histological [including immunohistochemistry (IHC) and molecular biology findings]. Surgery, be it partial or total hepatectomy (LT), should be proposed whenever possible, because it is the therapeutic mainstay. In HEHE, LT provides excellent results, with long-term diseasefree survivals (DFS) reaching 75%. Good results can be obtained even in case of (frequent) extrahepatic spread. Based on the extensive ELITA-ELTR study a HEHE-LT prognostic score has been proposed in order to estimate the risk of recurrence after LT. In contrast, results of surgery and LT are extremely poor for HHS, for the almost invariably rapid recurrence (within 6 months) and related death within 2 years. LT remains a contraindication for HHS. Due to the still important recurrence rate after surgical resection (25% in HEHE and almost 100% in HHS), there is an urgent need to develop pharmacological treatments targeting angiogenic and non-VEGF angiogenic pathways. To date, some prospective pilot studies and case reports have shown some short-term stabilisation of the disease in small groups of patients. In order to make progress, combination of surgery, anti-angiogenic and immunotherapy seems worthwhile. To complete the panel of vascular liver tumours, infantile haemangioendothelioma, haemangiopericytoma, nodular regenerative hyperplasia (NRH) and hepatic small vessel neoplasms (HSVN) are also discussed.

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Section: Hhssupporting

confidence: 75%

Vascular tumours of the liver: a particular story

Lerut

Iesari

2018

Transl. Gastroenterol. Hepatol

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“…Metastasis typically occurs hematogeneously most frequently to the lungs. Both cutaneous lesions as well as visceral manifestations exhibit wide possible differential diagnosis and similarly hepatic manifestation of angiosarcoma is radiologically difficult to differentiate from other liver tumors such as hepatocellular carcinoma, liver metastasis, or even hemangioma [68], so that biopsy of suspect lesions is required for correct diagnosis.…”

Section: Diagnostic Imaging Workupmentioning

confidence: 99%

Vascular tumors in infants and adolescents

et al. 2019

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Malignant vascular tumors as part of the vascular anomalies spectrum are extremely rare in children and young adults. Instead, benign vascular neoplasias are frequently encountered in the pediatric patient population. While vascular malformations are congenital vascular lesions, originating from a mesenchymal stem cell defect, vascular tumors are neoplastic transformations of endothelial and other vascular cells. The appropriate differential diagnosis and nomenclature according to the classification of the International Society for the Study of Vascular Anomalies (ISSVA) is decisive to initiate correct therapy. While infantile hemangioma can be routinely diagnosed by clinical means and rarely require therapy, more rare vascular tumors are frequently difficult to diagnose, require dedicated cross-sectional imaging, and benefit from an interdisciplinary treatment approach. The focus of this review is to provide an overview over the spectrum of vascular tumors, typical imaging characteristics, and summarize treatment options including interventional radiology approaches.

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“…At autopsy, it was initially doubtful whether the primary neoplastic lesion was in the heart or liver. The factors that suggested a primary cardiac tumor with liver metastasis were: 1) our patient’s younger age, since hepatic angiosarcomas are more frequent in subjects older than 60 years, sometimes with exposure to agents such as thorium oxide, vinyl chloride, arsenic, and anabolic steroids;15 2) single heart lesion X multiple hepatic lesions; 3) the location in the right atrium is a mark of cardiac angiosarcomas;6 , 7 4) the most common sites of metastatic dissemination of liver angiosarcoma are lungs and spleen, when intra-cardiac involvement is rare,15 , 16 and 5) the liver is commonly involved in the metastatic spread of cardiac angiosarcomas 3 - 6…”

Section: Discussionmentioning

confidence: 99%

Multiple hepatic metastases of cardiac angiosarcoma

et al. 2018

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The differential diagnosis of hepatic focal lesions is challenging because the etiology can be inflammatory, infectious, and even neoplastic. A rare cause of metastatic liver nodules is cardiac angiosarcoma. We report a case of this tumor, which was diagnosed only after autopsy.A 26-year-old Caucasian man was admitted for progressive dyspnea and cough over the past 3 weeks. Physical examination showed only hypophonetic heart sounds. Laboratory analysis demonstrated anemia and elevated inflammatory markers, despite normal biochemical parameters and liver function. Transthoracic echocardiography revealed massive pericardial effusion. Abdomen computed tomography (CT) showed multiple hepatic nodules, the largest of which measured 3 cm, but the percutaneous biopsy revealed only lobular necrosis and perisinusoidal fibrosis without granulomas or neoplastic cells. During hospitalization, the patient had fever and night sweats with weight loss, and empiric treatment for extrapulmonary tuberculosis associated with corticosteroids was initiated. The outpatient follow-up revealed complete improvement of the pericardial effusion, but maintenance of the liver lesions. After 2 months of hospital discharge, the patient was readmitted with hemorrhagic shock due to bleeding liver lesions, which were evidenced by CT. Embolization of the right hepatic artery was performed, but the patient soon died. The autopsy revealed a primary cardiac angiosarcoma with multiple hepatic metastases, rupture of the Glisson’s capsule and laceration of the liver. The case shows how important and difficult the diagnosis of focal liver lesions is, since it may result in an unexpected fatal outcome.

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Clinical Courses of Primary Hepatic Angiosarcoma: Retrospective Analysis of Eight Cases

Abstract: Hepatic angiosarcoma is a highly progressive disease with a poor prognosis. Detailed studies including histological examinations are essential to facilitate early diagnosis of the disease.

Cited by 11 publications

References 23 publications

Vascular tumours of the liver: a particular story

Vascular tumours of the liver: a particular story

Vascular tumors in infants and adolescents

Multiple hepatic metastases of cardiac angiosarcoma

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