Liver angiosarcoma, a rare liver malignancy, presented with intraabdominal bleeding due to rupture- a case report
© Chien et al; licensee BioMed Central Ltd. 2012
Received: 12 November 2011
Accepted: 26 January 2012
Published: 26 January 2012
Liver angiosarcoma is a rare disease, however it still ranks as the third of most common primary liver maligancies. The prognosis of liver angiosarcoma is very poor with almost all patients with this kind of disease die within 2 years after diagnosis. No specific symptoms and signs are closely associated with this disease. Here, we report a case presenting shock status at first due to rupture of liver angiosarcoma- induced internal bleeding. After emergent transarterial embolization (TAE), she received partial hepatectomy two weeks later. 4 months after operation, she is still with a good performance status without obvious recurrence or metastasis identified.
Keywordsangiosarcoma liver angiosarcoma hepatectomy embolization
Hepatic angiosarcoma is a very rare disease, accounting for only 2% of primary liver malignancy [1–3]; however, it still ranks as the third place in the list of most common primary liver malignancies [2, 3]. Hepatic angiosarcoma originates from endothelial cells and usually presents as an abdominal mass with unspecific symptoms and signs , making it difficult to diagnose in the early stage. The survival of hepatic angiosarcoma is very poor, which is attributable to its rapid progress, high recurrence rate, and resistant to traditional chemotherapy and radiotherapy [5–7]. Even liver transplantation could not benefit patients with liver angiosarcoma . To date, the therapeutic guideline for liver angiosarcoma has not been set up; partial liver resection to remove tumor radically still remains to be the cornerstone of treatment options. Here we reported a case with primary liver angiosarcoma presenting with hemoperitoneum due to tumor rupture and active bleeding treated by TAE and subsequent partial liver resection.
After operation, this patient recovered very well and was discharged two weeks later. Adjuvant chemotherapy and radiation were not given after taking the effectiveness of them and her old age into consideration. No local recurrence or distant metastasis has been found at the time of writing, 4 months after operation. This patient is now still with a good performance status.
Angiosarcoma, a subtype of soft tissue sarcoma, is an aggressive malignant disease deriving from endothelium, lymphatics, or blood vessels. Angiosarcoma occurs most commonly in head and neck, followed by breast. Liver angiosarcoma is ranked as fifth in the list of most common seen sites of angiosarcoma  and is very rare.
Liver angiosarcoma occurs in the elder mainly . Although the symptoms are usually nonspecific, abdominal distension and discomfort, weight loss, and fatigue are commonly found [13, 14]. Others like jaundice, ascites, or hepatomegaly are usually associated with advanced liver angiosarcoma [13, 15]. The abnormal liver functions found in advanced liver angiosarcoma are attributable to the replacement of normal liver parenchema by tumor .
Due to the hypervascular characteristic of liver angiosarcoma, it is difficult to differentiate liver angiosarcoma from other vascular tumors in liver, such as hepatoma or adenoma, radiologically. As shown in the present case, CT and angiogram all indicated a hypervascular mass, which was in line with the image findings of hepatoma. After resection, pathologically, the resected liver showed a tumor composed of anastomosing vascular channels infiltrating surrounding hepatic tissue in a destructive fashion. The channels were lined by atypical endothelial cells with enlarged hyperchromatic nuclei. Immunohistochemically, the tumor was positive for CD31, CD34, and Vimentin, a mesenchymal marker, and negative for HAS and GPC-3, which excluded the possibility of hepatocyte origin. Taken together, the liver angiosarcoma was confirmed as the final diagnosis [9–12].
The survival of patients with liver angiosarcoma is very poor with media survival of 6 months without treatment. Even after treatment, only 3% of patients were reported to live longer than 2 years . The reasons contributing to this are complicated. First, due to its nonspecific symptoms, the diagnosis is usually too late, which excludes the chance of complete resection of tumor in most cases. Second, liver angiosarcoma usually presents with early metastases to other organs, such as lung, spleen, or bone, which further worsens its prognosis. At present, the standard treatment for liver angiosarcoma remains surgical resection. Others such as chemotherapy or radiotherapy fails to reach a conclusive benefit on survival of liver angiosarcoma [5–7], although chemotherapy is the critical part of treatments of soft tissue sarcoma and radiotherapy plays a palliative role in liver metastasis ; Liver transplantation is also not deemed feasible to treat liver angiosarcoma thanks to its high recurrent rate and still poor survival after transplantation .
Liver angiosarcoma also easily induces severe intraabdominal hemorrhage due to spontaneous rupture, leading to subsequent tumor cell spillage and peritoneal tumor seeding, which further complicates the prognosis [18, 19]. Once liver angiosarcoma-induced internal bleeding happens, TAE is usually the first choice to stop the bleeding and to get the patients stable [18, 19]. Otherwise, in relatively stable patients, conservative treatment may also work in this situation . However, due to the rare incidence of liver angiosarcoma, how to treat acute bleeding of liver angiosarcoma has not yet reached a definite conclusion at present. As shown in the present case, spontaneous liver angiosarcoma rupture made her present with a shock status at first. After embolizaton and resuscitation, her condition got stabilized and she received partial hepatectomy two weeks later. For this patient, the poor prognosis is expected according to previous reports. However, given that this patient is already 83 years old and no effective strategies against liver angiosarcoma are available so far, no more adjuvant therapy has been given to this patient. No recurrence or metastasis has been identified at the time of writing and she is still with a good performance status so far, 4 months after operation.
Seeking a new strategy in addition to surgical resection to treat liver angiosarcoma should be prioritized due to its high recurrence rate and very dismal prognosis. Since traditional chemotherapy and radiotherapy could not benefit this kind of patients, combined with the rapid progress of current molecular biology, target therapy may be the further direction against liver angiosarcoma
This study has been approved by Chang Gung memorial hospital IRB board. The approved IRB number is 100-3732B. A copy of the approval of IRB is available for review by the Editor-in-Chief of this journal.
- Mani H, Van Thiel DH: Mesenchymal tumors of the liver. Clin Liver Dis. 2001, 5: 219-257. 10.1016/S1089-3261(05)70162-8. viiiView ArticlePubMedGoogle Scholar
- Molina E, Hernandez A: Clinical manifestations of primary hepatic angiosarcoma. Dig Dis Sci. 2003, 48: 677-682. 10.1023/A:1022868221670.View ArticlePubMedGoogle Scholar
- 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. 10.1097/00005792-197901000-00003.View ArticleGoogle Scholar
- Selby DM, Stocker JT, Ishak KG: Angiosarcoma of the liver in childhood: a clinicopathologic and follow-up study of 10 cases. Pediatr Pathol. 1992, 12: 485-498. 10.3109/15513819209024199.View ArticlePubMedGoogle Scholar
- Almogy G, Lieberman S, Gips M, Pappo O, Edden Y, Jurim O, Simon Slasky B, Uzieli B, Eid A: Clinical outcomes of surgical resections for primary liver sarcoma in adults: results from a single centre. Eur J Surg Oncol. 2004, 30: 421-427. 10.1016/j.ejso.2004.01.004.View ArticlePubMedGoogle Scholar
- Holden CA, Spittle MF, Jones EW: Angiosarcoma of the face and scalp, prognosis and treatment. Cancer. 1987, 59: 1046-1057. 10.1002/1097-0142(19870301)59:5<1046::AID-CNCR2820590533>3.0.CO;2-6.View ArticlePubMedGoogle Scholar
- Maddox JC, Evans HL: Angiosarcoma of skin and soft tissue: a study of forty-four cases. Cancer. 1981, 48: 1907-1921. 10.1002/1097-0142(19811015)48:8<1907::AID-CNCR2820480832>3.0.CO;2-T.View ArticlePubMedGoogle Scholar
- 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. 10.1016/j.transproceed.2005.03.060.View ArticlePubMedGoogle Scholar
- Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ: Angiosarcoma. Lancet Oncol. 2010, 11: 983-991. 10.1016/S1470-2045(10)70023-1.View ArticlePubMedGoogle Scholar
- Leader M, Collins M, Patel J, Henry K: Vimentin: an evaluation of its role as a tumour marker. Histopathology. 1987, 11: 63-72.View ArticlePubMedGoogle Scholar
- Lamps LW, Folpe AL: The diagnostic value of hepatocyte paraffin antibody 1 in differentiating hepatocellular neoplasms from nonhepatic tumors: a review. Adv Anat Pathol. 2003, 10: 39-43. 10.1097/00125480-200301000-00004.View ArticlePubMedGoogle Scholar
- Libbrecht L, Severi T, Cassiman D, Vander Borght S, Pirenne J, Nevens F, Verslype C, van Pelt J, Roskams T: Glypican-3 expression distinguishes small hepatocellular carcinomas from cirrhosis, dysplastic nodules, and focal nodular hyperplasia-like nodules. Am J Surg Pathol. 2006, 30: 1405-1411. 10.1097/01.pas.0000213323.97294.9a.View ArticlePubMedGoogle Scholar
- Timaran CH, Grandas OH, Bell JL: Hepatic angiosarcoma: long-term survival after complete surgical removal. Am Surg. 2000, 66: 1153-1157.PubMedGoogle Scholar
- Forbes A, Portmann B, Johnson P, Williams R: Hepatic sarcomas in adults: a review of 25 cases. Gut. 1987, 28: 668-674. 10.1136/gut.28.6.668.PubMed CentralView ArticlePubMedGoogle Scholar
- Poggio JL, Nagorney DM, Nascimento AG, Rowland C, Kay P, Young RM, Donohue JH: Surgical treatment of adult primary hepatic sarcoma. Br J Surg. 2000, 87: 1500-1505. 10.1046/j.1365-2168.2000.01564.x.View ArticlePubMedGoogle Scholar
- Jaffe BM, Donegan WL, Watson F, Spratt JS: Factors influencing survival in patients with untreated hepatic metastases. Surg Gynecol Obstet. 1968, 127: 1-11.PubMedGoogle Scholar
- Turek-Maischeider M, Kazem I: Palliative irradiation for liver metastases. JAMA. 1975, 232: 625-628. 10.1001/jama.1975.03250060021014.View ArticlePubMedGoogle Scholar
- Lee SW, Song CY, Gi YH, Kang SB, Kim YS, Nam SW, Lee DS, Kim JO: Hepatic angiosarcoma manifested as recurrent hemoperitoneum. World J Gastroenterol. 2008, 14: 2935-2938. 10.3748/wjg.14.2935.PubMed CentralView ArticlePubMedGoogle Scholar
- Leowardi C, Hormann Y, Hinz U, Wente MN, Hallscheidt P, Flechtenmacher C, Buchler MW, Friess H, Schwarzbach MH: Ruptured angiosarcoma of the liver treated by emergency catheter-directed embolization. World J Gastroenterol. 2006, 12: 804-808.PubMed CentralPubMedGoogle Scholar
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.