Korean J Gastroenterol.  2015 Apr;65(4):229-235. 10.4166/kjg.2015.65.4.229.

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

• Hur CJ
• Min BR
• Lee YJ
• Jang BK
• Hwang JS
• Kim ES
• Park KS
• Cho KB
• Kang YN
• Chung WJ

• KMID: 2373214
• DOI: http://doi.org/10.4166/kjg.2015.65.4.229

Abstract

BACKGROUND/AIMS
Hepatic angiosarcoma, a rare and aggressive liver malignancy, is difficult to diagnose because of a lack of specific clinical features. The clinical and radiological features of patients with histologically confirmed hepatic angiosarcoma were examined.
METHODS
Among 2,336 patients diagnosed with primary hepatic carcinoma at Keimyung University Dongsan Medical Center (Daegu, Korea) between May 2002 and February 2012, eight (0.03%) with histologically confirmed primary hepatic angiosarcoma were included. The patterns of disease diagnosis, tumor characteristics, treatment responses, and prognoses were reviewed retrospectively.
RESULTS
Median age was 66 years-old (range, 41-80 years). Four patients were male. Five patients were compulsive drinkers. All patients had no HBsAg and anti-HCV. Initial radiologic diagnoses revealed primary hepatic angiosarcoma (n=2), hepatocellular carcinoma (n=2), hemangioma (n=2), and hepatic metastatic carcinoma (n=2). Definitive diagnoses were made by percutaneous needle biopsies in seven patients and surgical resection in one patient. At the time of the initial diagnosis, extrahepatic metastases were detected in three patients (37.5%). Metastatic sites included the spleen and lung, pericardium, and bone, in one patient each. Two patients underwent conservative treatments. The remaining patients underwent surgical resection (n=1), transcatheter arterial chemoembolization (n=1), and systemic chemotherapy (n=4). The median survival period was 214 days (range, 21-431 days).
CONCLUSIONS
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.

Keyword

Hepatic angiosarcoma; Liver neoplasms

MeSH Terms

Adult
Aged
Aged, 80 and over
Antineoplastic Agents/therapeutic use
Embolization, Therapeutic
Female
Hemangiosarcoma/*diagnosis/pathology/therapy
Humans
Immunohistochemistry
Liver Neoplasms/*diagnosis/pathology/therapy
Male
Middle Aged
Neoplasm Metastasis
Prognosis
Retrospective Studies
Survival Rate
Tomography, X-Ray Computed
Antineoplastic Agents

Figure

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  Fig. 1. CT findings of a typical hepatic angiosarcoma. A huge hypervascular enhancing mass with a central necrotic portion can be seen in the right hepatic lobe. Most nodular lesions are hypoattenuated and have enhanced foci. The enhancement is less than that of aorta, and some nodular lesions show irregular or ring enhancement. A progressive enhancement pattern can be seen in the delayed phase CT.

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  Fig. 2. CT findings of an atypical hepatic angiosarcoma. Multiple peripheral enhancing nodules with a hypoattenuated center are scattered in the liver.

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  Fig. 3. Cut surface and microscopic findings of the liver. (A) Surgically resected right lobe, measuring up to 14.0×11.5×5.5 cm, showed an ill-defined large heterogeneous hepatic mass with solid, cystic nature. Diffusely scattered areas of hemorrhage and necrosis were seen in the mass. (B) Needle biopsy of the hepatic mass showed diffuse growth of pleomorphic hyperchromatic tumor cells with anastomosing vascular channels (H&E, ×200). (C) Immunohistochemical staining for CD31 showed diffuse, strong, positive reactivity in the atypical tumor cells (×200), demonstrating a vascular-type tumor originating from endothelial cells.

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  Fig. 4. Survival time of the hepatic angiosarcoma patients. The median survival period was 214 days (range, 21–431 days).

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