Primary Hepatic Angiosarcoma Research Articles

Hepatic angiosarcoma presenting as acute liver failure in young adults. Report of two cases and review of literature

Background: Hepatic angiosarcoma is a rare tumor of endothelial origin that accounts for up to 2% of all primary neoplasms of the liver. It is uncommon in young adults and acute liver failure is a very unusual presentation of this disease. Case Presentation: We report the cases of two young male adults who presented with acute liver failure. One of them was diagnosed with primary hepatic angiosarcoma after transplantation based on the complete evaluation of the heaptectomy specimen; while the other was diagnosed through a liver biopsy which was done as part of the work-up for transplantation. Both patients had a rapidly worsening clinical course and died within 2 months of diagnosis. Conclusion: Hepatic angiosarcoma is a poor prognosis disease with high and early mortality. Therefore, a high level of suspicion should be present to promptly diagnose it, especially when dealing with patients with a rapidly worsening liver disease.

Hepatic angiosarcoma: cross-sectional imaging findings in seven patients with emphasis on dynamic contrast-enhanced and diffusion-weighted MRI

Primary hepatic angiosarcoma is a very rare and aggressive malignancy of vascular origin. We describe cross-sectional imaging findings of this entity with emphasis on dynamic contrast-enhanced (DCE) and diffusion-weighted (DWI) MR imaging. Seven cases of pathologically confirmed hepatic angiosarcoma were retrospectively reviewed (CT and MRI examinations were available in seven and six patients, respectively). Two radiologists evaluated lesion growth patterns, attenuation, signal intensity characteristics, contrast enhancement patterns, and apparent diffusion coefficients (ADCs). Multifocal hepatic disease was present in six patients by means of a mixed pattern of large dominant masses and multiple small nodules; one patient had a solitary large mass. Unenhanced images depicted hemorrhagic areas and a markedly heterogeneous internal architecture within large tumors. Contrast-enhanced early phase images showed variable patterns including patchy peripheral or bizarre shaped intralesional foci of enhancement, peripheral rim enhancement, and small lesions without enhancement. On DCE images, the majority of lesions presented with varying degrees of progressive enhancement. Small nodules frequently displayed homogeneous enhancement on delayed phase images due to complete fill-in. DWI revealed a high interlesional variability of ADC values (range 0.57-2.41 × 10(-3 )mm(2)/s, mean 1.37 × 10(-3 )mm(2)/s). Cross-sectional imaging findings of hepatic angiosarcoma reflect the varied histopathological composition of the tumors. Multifocal disease, hemorrhage within large lesions, as well as progressive enhancement on DCE images are typical features of hepatic angiosarcoma. The mean ADC of lesions was found to be slightly elevated in comparison with other hepatic malignancies.

Primary hepatic angiosarcoma: A retrospective analysis of 6 cases

The aim of this study was to define the clinical features and surgical treatment outcomes of patients with primary hepatic angiosarcoma. Data of the 6 patients diagnosed with primary hepatic angiosarcoma in Tianjin Medical University Cancer Institute and Hospital from January 1999 to December 2005 were retrospectively reviewed. The median age of the patients was 49 years (range 45-78 years) with a male predominance. Laboratory tests showed a mild elevation of α-fetoprotein in 2 patients, and 2 had both hepatitis B and C. Liver resection was performed in all patients. For the 5 patients who received curative liver resection, the median follow-up duration was 41 months (range 23-84 months) and the overall 1-year, 3-year and 5-year survival rates were 100.0%, 80.0% and 40.0%, respectively. One patient who underwent a palliative operation died of tumor progression a month after operation. Early diagnosis is necessary and complete surgical resection is the key to improve prognosis.

Contrast-enhanced ultrasound study of primary hepatic angiosarcoma: A pitfall of non-enhancement

ObjectiveTo investigate the contrast-enhanced ultrasound (CEUS) characteristics of primary hepatic angiosarcoma (PHA). MethodsThe sonographic findings and CEUS images of PHA in three patients were retrospectively analyzed. ResultsIn our study, 3 cases of PHA (2 multiple nodules and 1 solitary mass) showed similar enhancement pattern on CEUS, characterized by remarkable central non-enhancement and peripheral irregular enhancement in the arterial and portal phase, and complete wash-out in the late phase. Furthermore, we unexpectedly found that abundant neoplastic tissues were present in the central area of non-enhancement on pathological evaluation. Based on literature review, we supposed that the unusual finding may be associated with the very low velocity of blood flow in the central region of tumors. ConclusionCEUS could well depict PHA with some common features, which may provide valuable clues in diagnosis of this rare disease. And non-necrotic tumor tissue of PHA could also demonstrate non-enhancement on CEUS, which warrant further investigations.

Hepatic angiosarcoma five years following spontaneous intraperitoneal bleed of a hepatic mass

Primary hepatic angiosarcoma is a rare and rapidly fatal disease. We present the highly unusual identification of this lesion five years after the initial clinical presentation.In 2003, a 32-year-old man presented with abdominal pain, tachycardia, and evidence of hemorrhage. A CT scan showed a hepatic mass with intralesional hemorrhage, intraperitoneal blood, and splenomegaly. The patient was stabilized clinically. Laparoscopic core biopsies demonstrated no malignancy, only findings consistent with an old hemorrhage. Contralateral lobe biopsies revealed normal liver tissue. A metastatic workup was negative and the decision was made to observe the patient clinically with radiographic follow-up, given his suspected portal hypertension based on thrombocytopenia and splenomegaly.Sequential imaging demonstrated a decrease in the size of the mass from 12.0 cm in 2003 to 3.0 cm in 2007. Subsequent newly identified esophageal varices prompted a reevaluation of the case. A repeat biopsy demonstrated a neoplasm of vascular etiology and uncertain malignant potential. By early 2008 the lesion had increased to 4.8 cm and was resected via a left hepatic lobectomy. An extremely vascular lesion with surrounding dense fibrosis was identified and pathologic examination demonstrated a high-grade angiosarcoma.We are unaware of any previous reports suggesting such a prolonged natural history of hepatic angiosarcoma. This case may represent the possibility of malignant transformation of a lower grade vascular neoplasm such as hepatic epithelioid hemangioendothelioma to an angiosarcoma.

Primary hepatic angiosarcoma: imaging findings and palliative treatment with transcatheter arterial chemoembolization or embolization

To describe the image findings and results of transcatheter arterial chemoembolization (TACE) or transcatheter arterial embolization (TAE) for treating primary hepatic angiosarcoma. A retrospective review of the electronic medical database from 2002 to 2007, revealed six patients with primary hepatic angiosarcoma confirmed by percutaneous liver biopsy. The computed tomography (CT) and angiography imaging findings, the TACE or TAE results, and the post-procedure course were evaluated in all patients. On CT and angiography, each tumour appeared as a solitary mass or as multiple nodules or masses with heterogeneously early and progressive enhancement. One of the two patients with tumour response to TACE died 8 months after initial presentation, and the remaining patient was still alive at the last follow-up 12 months after initial presentation. However, two patients with no response to TACE and two patients who underwent emergent TAE for tumour rupture died 1 week to 5 months (mean 2.1 months) after initial presentation. Primary hepatic angiosarcoma appears as a solitary or multiple, hypervascular lesions with heterogeneously early and progressive enhancement on CT and angiography. Although TAE may be the primary procedure for achieving emergent bleeding control caused by the rupture of hepatic angiosarcomas, TACE may be effective for treating patients with a dominant hepatic angiosarcoma with or without intrahepatic metastases.

Clinical features and treatment outcomes of advanced stage primary hepatic angiosarcoma

BackgroundPrimary hepatic angiosarcoma is a very rare malignancy with a poor prognosis. While surgical resection has been validated as curative choice, most cases are diagnosed too late for resection. Nonetheless, treatment protocols have not been established and also there are very few reports on the clinical features and treatment outcomes. Patients and methodsAmong 11939 patients diagnosed with primary hepatic tumors from January 1985 to December 2007 at two centers, five patients were diagnosed with primary hepatic angiosarcoma. We analyzed patients’ demographics, tumor characteristics, treatment modality, and outcomes using imaging, serology, and pathology. ResultsAll five patients were diagnosed at advanced stage with distant metastases. The most common symptom was abdominal pain. The levels of the tumor markers were within the normal range and serological tests were negative for hepatitis B and C viruses. Two of four patients who received chemotherapy died <3 months after diagnosis, but the other two patients survived >6 months. ConclusionsA combination of chemotherapy resulted in an improved outcome for two of four patients, suggesting the potential usefulness of palliative chemotherapy to improve survival. This case study may aid in planning chemotherapy for patients with advanced hepatic angiosarcoma.

Hepatic angiosarcoma manifested as recurrent hemoperitoneum

Angiosarcoma is a rare tumor that account for less than 1% of all sarcomas. Although hepatic angiosarcoma usually presents with unspecific symptoms, it rapidly progresses and has a high mortality. We report a rare case of primary hepatic angiosarcoma manifested as recurrent hemoperitoneum.

Primary Hepatic Angiosarcoma on Coregistered FDG PET and CT Images

4Department of Radiology, Kobe University Graduate School of Medicine, Kobe, Japan. ngiosarcoma is an uncommon mesenchymal malignant neoplasm of the vascular or lymphatic endothelium, accounting for 2% of all soft-tissue sarcomas [1–4], that can affect any organ but seems to have a predilection for the skin in the head and neck regions [3, 4]. Although primary hepatic angiosarcoma is rare and accounts for only 2% of primary hepatic tumor, it is the most common malignant mesenchymal tumor of the liver [5]. Various appearances of primary hepatic angiosarcoma on CT and on MRI have been noted [3, 5]. However, to the best of our knowledge, the FDG PET findings have not been described yet. Here, we present a case of primary hepatic angiosarcoma and the coregistered FDG PET and CT images that show the affected sites.

Spontaneous ruptured primary hepatic angiosarcoma coincident with Schistosoma Japonica liver fibrosis

We report herein a case of spontaneous ruptured primary hepatic angiosarcoma coincident with Schistosoma Japonica liver fibrosis and review the correlative literature. The resected specimen was examined by histopathological and immunohistochemical evaluation. The final diagnosis was spontaneous ruptured primary hepatic angiosarcoma coincident with Schistosoma Japonica liver fibrosis Conclusion: Considering the nature of primary hepatic angiosarcoma, in particular the ruptured hepatic angiosarcoma, it is obviously desirable to avoid any unnecessary delay or definitive surgical treatment. It is presumed that angiosarcoma in the liver has a possible association with S. japonicum and the deposition of ovae in liver.

Primary hepatic angiosarcoma: findings at CT and MR imaging.

To evaluate and describe cross-sectional imaging findings in patients with pathologically confirmed primary hepatic angiosarcoma. Findings from imaging examinations in 13 patients with pathologically confirmed primary hepatic angiosarcoma were retrospectively reviewed (computed tomographic [CT] images obtained in 10 patients and magnetic resonance [MR] images obtained in five patients were available for review). Two gastrointestinal radiologists evaluated lesion number, size, attenuation and signal intensity characteristics, and the pattern and degree of contrast material enhancement. Medical records were reviewed for clinical features associated with angiosarcoma. Angiosarcoma appeared as multiple nodules (n = 6), as dominant masses (n = 6), or as a diffusely infiltrating lesion (n = 1). Multiple nodules were hypoattenuating at unenhanced and contrast material--enhanced CT (six of six patients). When dominant masses were encountered at MR imaging, T2-weighted MR imaging demonstrated heterogeneous internal architecture (four of four patients) similar to that of hepatocellular carcinoma. Multiphase contrast-enhanced CT and MR images showed dominant masses to have heterogeneous and progressive enhancement (three of three patients). Clinical features associated with angiosarcoma included splenic metastases (six of 13 patients), thrombocytopenia (seven of 13 patients), disseminated intravascular coagulation (four of 13 patients), and hemolytic anemia (three of 13 patients). Primary hepatic angiosarcoma exhibits a spectrum of appearances that reflect its varied pathologic features.

Surgical treatment of adult primary hepatic sarcoma.

Primary sarcomas of the liver are extremely rare in adults. Optimal therapeutic approaches remain unclear. Twenty consecutive adult patients who had surgical treatment for primary hepatic sarcomas were reviewed. Patient age ranged from 23 to 80 years. Other than one patient with primary hepatic angiosarcoma who had a history of thorium dioxide colloid (Thorotrast) exposure 23 years before diagnosis, no predisposing causes were apparent. Nineteen patients had hepatic resection and one patient had an orthotopic liver transplant. No patient received neoadjuvant chemotherapy or radiotherapy but radiotherapy was delivered intraoperatively in one patient. Leiomyosarcoma was the most common histological type of sarcoma diagnosed (five of 20 patients), followed by malignant solitary fibrous tumour (four) and epithelioid haemangioendothelioma (three). Fourteen tumours were high-grade sarcomas and six were low grade malignancies. Thirteen patients developed a recurrence. Distant metastases (ten patients) and intrahepatic recurrence (six) were the predominant sites of initial treatment failure. Six patients received salvage chemotherapy. Histological grade was the only factor significantly associated with overall patient survival (P= 0.03). With complete resection, patients with high-grade tumours had a 5-year survival rate of 18 (95 per cent confidence interval 5-62) per cent compared with 80 (52-100) per cent for patients with low-grade tumours. The 5-year survival rate for all 20 patients was 37 (20-60) per cent. Surgical resection is the only effective therapy for primary hepatic sarcomas at present. Better adjuvant therapy is necessary, especially for high-grade malignancies, owing to the high failure rate with operation alone.

Hepatic hemangiosarcoma: imaging findings and differential diagnosis.

Primary hepatic angiosarcoma is a rare mesenchymal tumor of the liver that usually presents with nonspecific symptoms in elderly men. We present four cases of hepatic hemangiosarcoma and discuss the imaging characteristics of this entity. Our series shows that this tumor is not uncommon in younger patients with no associated risk factors such as previous exposure to thorotrast or vinyl chloride. Our experiences on a limited number of patients suggests that the combined use of angiography and dual-phase helical CT provides a better identification of the tumor and its complications. Analysis of imaging studies in patients with hepatic hemangiosarcoma reveals hypervascular lesions. Common complications were portal vein thrombosis, Budd-Chiari syndrome, as well as arterio-venous or arterio-portal shunts. Due to the vascularity of the tumor, percutaneous liver biopsy is hazardous.

The British Hepatic Angiosarcoma Register

A register of British cases of primary hepatic angiosarcoma (HAS) was established in 1974 to monitor the occurrence of cases from 1963 onwards. Details of cases dying in 1963-77 have been obtained. Thirty-five cases were agreed as HAS by panel of liver pathologists, and occupational and medical information was obtained in the majority of these. Two cases were attributable to VCM exposure, and eight others had received intra-arterial Thorotrast. In 1978-79, two more cases were confirmed in VCM polymerization workers.

The British hepatic angiosarcoma register.

A register of British cases of primary hepatic angiosarcoma (HAS) was established in 1974 to monitor the occurrence of cases from 1963 onwards. Details of cases dying in 1963-77 have been obtained. Thirty-five cases were agreed as HAS by panel of liver pathologists, and occupational and medical information was obtained in the majority of these. Two cases were attributable to VCM exposure, and eight others had received intra-arterial Thorotrast. In 1978-79, two more cases were confirmed in VCM polymerization workers.