Journal List > Korean J Gastroenterol > v.65(4) > 1007359

Hur, Min, Lee, Jang, Hwang, Kim, Park, Cho, Kang, and Chung: Clinical Courses of Primary Hepatic Angiosarcoma: Retrospective Analysis of Eight Cases

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.

References

1. Molina E, Hernandez A. Clinical manifestations of primary hepatic angiosarcoma. Dig Dis Sci. 2003; 48:677–682.
2. Oe A, Habu D, Kawabe J, et al. A case of diffuse hepatic angiosarcoma diagnosed by FDG-PET. Ann Nucl Med. 2005; 19:519–521.
crossref
3. Locker GY, Doroshow JH, Zwelling LA, Chabner BA. The clinical features of hepatic angiosarcoma: a report of four cases and a review of the English literature. Medicine (Baltimore). 1979; 58:48–64.
crossref
4. Bioulac-Sage P, Laumonier H, Laurent C, Blanc JF, Balabaud C. Benign and malignant vascular tumors of the liver in adults. Semin Liver Dis. 2008; 28:302–314.
crossref
5. Almogy G, Lieberman S, Gips M, et al. Clinical outcomes of surgical resections for primary liver sarcoma in adults: results from a single centre. Eur J Surg Oncol. 2004; 30:421–427.
crossref
6. Holden CA, Spittle MF, Jones EW. Angiosarcoma of the face and scalp, prognosis and treatment. Cancer. 1987; 59:1046–1057.
crossref
7. Maddox JC, Evans HL. Angiosarcoma of skin and soft tissue: a study of forty-four cases. Cancer. 1981; 48:1907–1921.
crossref
8. Timaran CH, Grandas OH, Bell JL. Hepatic angiosarcoma: long-term survival after complete surgical removal. Am Surg. 2000; 66:1153–1157.
9. Ozden I, Bilge O, Erkan M, Cevikbaş U, Acarli K. Five years and 4 months of recurrence-free survival in hepatic angiosarcoma. J Hepatobiliary Pancreat Surg. 2003; 10:250–252.
10. Arima-Iwasa S, Chijiiwa K, Makino I, Tanabe R, Ohuchida J, Kondo K. A case of hepatic angiosarcoma surviving for more than 16 months after hepatic resection. Hepatogastroenter-ology. 2007; 54:533–535.
11. Zhou YM, Li B, Yin ZM, et al. Results of hepatic resection for primary hepatic angiosarcoma in adults. Med Sci Monit. 2010; 16:CR61–CR66.
12. Louagie YA, Gianello P, Kestens PJ, Bonbled F, Haot JG. Vinylchloride induced hepatic angiosarcoma. Br J Surg. 1984; 71:322–323.
crossref
13. Ludwig J, Hoffman HN. Hemangiosarcoma of the liver. Spectrum of morphologic changes and clinical findings. Mayo Clin Proc. 1975; 50:255–263.
14. Kojiro M, Nakashima T, Ito Y, Ikezaki H, Mori T, Kido C. Thorium dioxide-related angiosarcoma of the liver. Pathomorphologic study of 29 autopsy cases. Arch Pathol Lab Med. 1985; 109:853–857.
15. Koyama T, Fletcher JG, Johnson CD, Kuo MS, Notohara K, Burgart LJ. Primary hepatic angiosarcoma: findings at CT and MR imaging. Radiology. 2002; 222:667–673.
crossref
16. Fayette J, Martin E, Piperno-Neumann S, et al. Angiosarcomas, a heterogeneous group of sarcomas with specific behavior de-pending on primary site: a retrospective study of 161 cases. Ann Oncol. 2007; 18:2030–2036.
crossref
17. Alrenga DP. Primary angiosarcoma of the liver. Review article. Int Surg. 1975; 60:198–203.
18. Park YS, Kim JH, Kim KW, et al. Primary hepatic angiosarcoma: imaging findings and palliative treatment with transcatheter arterial chemoembolization or embolization. Clin Radiol. 2009; 64:779–785.
crossref
19. Buetow PC, Buck JL, Ros PR, Goodman ZD. Malignant vascular tumors of the liver: radiologic-pathologic correlation. Radio-graphics. 1994; 14:153–166.
crossref
20. White PG, Adams H, Smith PM. The computed tomographic ap-pearances of angiosarcoma of the liver. Clin Radiol. 1993; 48:321–325.
crossref
21. Yang KF, Leow VM, Hasnan MN, Manisekar KS. Primary hepatic angiosarcoma: difficulty in clinical, radiological, and pathological diagnosis. Med J Malaysia. 2012; 67:127–128.
22. Heo SH, Jeong YY, Shin SS, Chung TW, Kang HK. Solitary small hepatic angiosarcoma: initial and follow-up imaging findings. Korean J Radiol. 2007; 8:180–183.
crossref
23. Drinković I, Brkljacić B. Two cases of lethal complications following ultrasound-guided percutaneous fine-needle biopsy of the liver. Cardiovasc Intervent Radiol. 1996; 19:360–363.
crossref
24. Peterson MS, Baron RL, Rankin SC. Hepatic angiosarcoma: findings on multiphasic contrast-enhanced helical CT do not mimic hepatic hemangioma. AJR Am J Roentgenol. 2000; 175:165–70.
25. Hertzanu Y, Peiser J, Zirkin H. Massive bleeding after fine needle aspiration of liver angiosarcoma. Gastrointest Radiol. 1990; 15:43–46.
crossref
26. Kim YB. Epithelioid hemangioendothelioma of the liver. Korean J Hepatol. 2008; 14:399–404.
crossref
27. Mark RJ, Poen JC, Tran LM, Fu YS, Juillard GF. Angiosarcoma. A report of 67 patients and a review of the literature. Cancer. 1996; 77:2400–2406.
crossref
28. Maluf D, Cotterell A, Clark B, Stravitz T, Kauffman HM, Fisher RA. Hepatic angiosarcoma and liver transplantation: case report and literature review. Transplant Proc. 2005; 37:2195–2199.
crossref
29. Seok JY, Kim YB. Primary angiosarcoma of the liver. Korean J Hepatol. 2009; 15:216–221.
crossref

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.
kjg-65-229f1.tif
Fig. 2.
CT findings of an atypical hepatic angiosarcoma. Multiple peripheral enhancing nodules with a hypoattenuated center are scattered in the liver.
kjg-65-229f2.tif
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.
kjg-65-229f3.tif
Fig. 4.
Survival time of the hepatic angiosarcoma patients. The median survival period was 214 days (range, 21–431 days).
kjg-65-229f4.tif
Table 1.
Characteristics of Patients and Clinical Findings with Primary Hepatic Angiosarcoma
Patient Sex/age (yr) Chief complaint Comorbidity Growth pattern Typical nature Maximal diameter (mm)/tumor (n) Metastasis TNM stage Initial radiologic diagnosis Immunohistochemical stain positive Treatment Survival time (day)
1 F/61 Bone pain HTN Multiple nodules Atypical 70/8 Bone T2 N1 M1 Hepatic metastatic carcinoma CD31, factor VIIIRA Chemotherapy 431
2 M/64 Hemoptysis Alcoholic LC Mixed dominant mass withnodules Atypical 132/1 Spleen, lung T2 N1 M1 Hepatocellular carcinoma CD31, factor VIIIRA Chemotherapy 427
3 F/41 Dyspnea None Multiple nodules Typical 44/6 Pericardium, lung T1 N1 M1 Hemangioma CD31, factor VIIIRA Chemotherapy 375
4 M/62 Abdominal pain Alcoholic LC Mixed dominant mass withnodules Atypical 80/6 None T2 N0 M0 Angiosarcoma CD31 Chemotherapy 305
5 F/72 Abdominal pain HTN Mixed dominant mass withnodules Typical 76/2 None T2 N1 M0 Hemangioma CD31, factor VIIIRA Transcatheter arterial chemoembolization 123
6 M/68 Abdominal pain None Large solitary mass s Typical 120/1 None T2 N0 M0 Angiosarcoma CD31, CD34, factor VIIIRA Operation 74
7 F/72 Abdominal pain DM, HTN, CVA Mixed dominant mass withnodules Typical 140/1 None T2 N1 M0 Hepatocellular carcinoma CD31, CD34, factor VIIIRA Conservative 36
8 M/80 Abdominal pain Alcoholic LC, AMI Mixed dominant mass withnodules Typical 128/3 None T2 N0 M0 Hepatic metastatic carcinoma CD31, CD34 Conservative 21

HTN, hypertension; LC, liver cirrhosis; DM, diabetes mellitus; CVA, cerebrovascular attack; AMI, acute myocardial infarction; factor VIII RA, factor VIII related antigen.

TOOLS
Similar articles