Search

PHA in the paediatric population is an extremely rare and aggressive malignant soft tissue neoplasm, with less than 50 cases published worldwide. The prognosis is dismal. If the tumour is unresectable, one treatment option is LT. In this article, the current available literature is reviewed and additionally, three cases of paediatric patients with PHA who underwent LT at Karolinska University Hospital, Sweden, are presented. Based on the literature and our own experience, there is undoubtedly possible good outcome of LT due to PHA. On the contrary, no patients have survived PHA without LT. PHA in paediatric patients should be recommended to LT in selected patients. Effect of modern adjuvant chemo and RT should be evaluated further based on international registry for such rare cases of PHA.

Introduction. Angiosarcomas are malignant tumors of vascular endothelium that may arise from different locations. Although primary hepatic angiosarcoma accounts for only 1.8% of primary liver tumors, it is the most common malignant mesenchymal tumor of the liver. We report a case of primary hepatic angiosarcoma with melena as an unusual initial manifestation of this extremely rare tumor. Case report. Forty-four-years old patient with melena was referred to our Clinic because melena was not resolved after repeated argon plasma coagulation of bleeding lesions during esophagogastroduodenoscopy in the regional hospital. Abdominal ultrasound and multislice computed tomography (MSCT) revealed enlarged liver, with focal lesion 6 cm in diameter localized in the left lobe with multiple satellite lesions in both liver lobes, enlarged spleen and extremely dilated and long umbilical vein. Double-balloon enteroscopy and video capsule endoscopy detected the multiple bleeding vascular lesions in the small bowel. Histopathological examination and immunohistochemistry of the small bowel lesions revealed malignant mesenchymal proliferation with vascular/endothelium differentiation of neoplastic cells. The patient was diagnosed with metastatic angiosarcoma probably of hepatic origin with metastasis in the small bowel, that caused melena, and in the lumbar spine, causing back pain. Conclusion. Rare causes of melena include bleeding from primary or metastatic hemangiosarcoma localized in the gastrointestinal tract, especially small bowel.

Purpose Primary hepatic angiosarcoma is a very rare and highly malignant tumor with poor prognosis. It is difficult to diagnose because of the lack of typical clinical features, and the treatment protocols for PHA are also not clear. Therefore, this study wants to find out the clinical characteristics and surgical treatments of primary hepatic angiosarcoma. Methods Among 8990 patients diagnosed with primary malignant tumor of the liver from January 2000 to December 2019 in our hospital, only four patients were diagnosed with primary hepatic angiosarcoma. The demographics, clinical manifestation, past history, serology test results, MRI features, pathology, treatment modality and prognosis of four patients were collected and analyzed. Results Three of four patients had no clinical symptoms, while one patient's symptom was abdominal pain. The levels of tumor markers of all four patients were within the normal reference range and serological tests were negative for hepatitis B and C virus. The MRI imaging findings of all four patients were mixed mass with highly disordered vascular characteristics. All four patients were misdiagnosed preoperatively. One patient who underwent hepatic lobectomy was still alive for about 18 months after surgery. One patient who underwent hepatic lobectomy has survived for only 6 months due to severe pneumonia. The other two patients who received transarterial chemoembolization survived 16 months and 11 months respectively. Conclusion The clinical symptoms of primary hepatic angiosarcoma are not typical, and primary hepatic angiosarcoma is easily misdiagnosed. The typical imaging manifestations are structural disorder and heterogeneous tumor. Hepatic lobectomy and transarterial chemoembolization may be important surgical treatments to improve the prognosis of patients.

Primary hepatic angiosarcoma was diagnosed in a 59-year-old woman who presented an arthralgia of limbs and dry cough for 6 weeks. Physical examination revealed digital clubbing. A Tc-MDP bone scintigraphy showed diffusely increased uptake along the cortical margins of long bones, suggesting hypertrophic osteoarthropathy.

Background/aim Epithelioid hemangio-endothelioma (EHE) of the liver is an uncommon vascular tumor with variable clinical courses ranging from stable disease to fatal outcome. EHE can mimic epithelioid angiosarcoma, which has a more aggressive behavior, especially in a small biopsy sample. EHEs are known to have the WWTR1-CAMTA1 fusion gene, and nuclear expression of CAMTA1 by immunohistochemistry (IHC) has been reported in about 90% of EHEs in multiple organs. Our study aimed to validate the diagnostic utility of CAMTA1 expression in EHEs, especially in the liver. Patients and methods IHC was performed using anti-CAMTA1 antibody in 34 tumors (24 hepatic EHEs and 10 angiosarcomas). In CAMTA1-negative EHEs, TFE3 IHC was performed. Results Of the 24 hepatic EHEs, 22 (91.6%) showed nuclear staining for CAMTA1. One of two CAMTA1-negative cases showed TFE3 positivity. The other case was negative for TFE3. Meanwhile, all 10 angiosarcoma cases had no CAMTA1 expression. Conclusion CAMTA1 is a highly sensitive and specific marker for diagnosis of hepatic EHE. It is helpful for differentiation of hepatic EHE and angiosarcoma, especially in small biopsy samples.

Hepatic angiosarcoma is a rare malignant vascular tumor presenting unique treatment challenges. The aim of the present study was to determine the treatment and prognosis of this entity.A systematic literature search was conducted using PubMed, Embase and Chinese Biomedical Literature database, to identify articles published from January 1980 to July 2017. Search terms were "hepatic angiosarcoma" and "liver angiosarcoma". Additional articles were retrieved through manual search of bibliographies of the relevant articles. Pooled individual data concerning the prognosis following various therapeutic modalities were analyzed.A total of 75 articles involving 186 patients were eligible for inclusion. The median overall survival (OS) was 8 months, with 1-, 3-, and 5-year OS rates of 36.6%, 22.3%, and 12.0%, respectively. The median OS after partial hepatectomy (n = 86), chemotherapy (n = 36), liver transplantation (n = 17), and supportive care (n = 46) were 15, 10, 5 and 1.3 months, respectively. Small tumor size (10 cm) was the only significant favorable factor for OS after partial hepatectomy (P = 0.012).Despite the dismal prognosis, partial hepatectomy could prolong the survival of hepatic angiosarcoma patients, particularly those with tumors10 cm. Chemotherapy could be an option for unresectable disease. Liver transplantation is not a recommendable option for the management of this malignancy.

Primary hepatic angiosarcoma is a rare tumor originating from endothelial cells in the liver and accounts for approximately 1% of all hepatic malignant tumors. It is difficult to diagnose due to the lack of specific symptoms or tumor markers. No effective treatment exists, but complete surgical resection may achieve a good outcome. Since most primary hepatic angiosarcomas are already at an advanced stage at diagnosis, few reports describe tumors smaller than 2 cm. We report a case of surgery for a 1.7-cm sized primary hepatic angiosarcoma. Further studies are required to improve the preoperative diagnosis of primary hepatic angiosarcoma.

Hepatic angiosarcoma is a mesenchymal tumor originating from liver sinusoidal endothelial cells. It is an extremely rare malignant neoplasm accounting for less than 1% of primary malignant liver tumors. The deregulated coagulopathy that can be seen in hepatic angiosarcoma fulfills the clinical diagnostic criteria of disseminated intravascular coagulation. However, the mechanism that governs this coagulopathy has been poorly understood. This case report provides histological evidence of the consumption of coagulation factors along with trapped platelets occurring within the tumor, which is the foundation for the concept of Kasabach-Merritt syndrome (KMS). KMS is characterized by thrombocytopenia and hyperconsumption of coagulation factors within a vascular tumor. However, KMS associated with angiosarcoma has not been well recognized. This case report describes, for the first time, the histological evidence of KMS that occurred in an extremely rare mesenchymal malignant tumor of the liver.

Primary hepatic angiosarcoma (PHA) is a rare malignant tumor that occurs mainly in the elderly, with almost no specific symptoms or tumor markers. Information on the imaging characteristics of this tumor is limited due to its rarity. Therefore, it is difficult to diagnose PHA. So far, its definite diagnosis depends on histopathologic examination combined with immunohistochemical results. Patients with PHA have a poor prognosis in spite of surgical resection of this tumor. In this case report, we present a 72-year-old woman with PHA, focusing on the imaging features of this tumor, especially its enhancement pattern on contrast-enhanced ultrasound images. Contrast-enhanced ultrasound provided helpful information for diagnosis.

Background & aimPrimary hepatic angiosarcoma is a rare tumor with poor prognosis. The aim of this study was to generate a new angiosarcoma model to improve research on hepatic angiosarcoma.MethodsPigs sus scrofa were treated with different regimens of diethylnitrosamine (DENA). Tissues were analyzed by histology and immunohistochemistry. Serum parameters were determined. Angiosarcoma tissue was investigated for chromosomal aberrations by aCGH analysis.ResultsAnimals of almost all different treatment regimens developed a multitude of variable liver lesions. Different tumor types such as granulation tissue type, cellular-like, hyalinization necrosis-like, angiosarcoma-like, dysplastic nodule-like, hepatocellular-like, glandular structure-like, and leiomyoma-like lesions were observed. Weekly treatment with 15 mg/kg for up to 52 weeks or a single shot of 200 mg/kg DENA led to the development of hepatic angiosarcomas. aCGH analysis of angiosarcoma tissue revealed increased alterations in tumors compared to non-tumorous tissue. Most of the chromosomal alterations were found on chromosomes 6, 7, 12, and 14.ConclusionIn this preliminary study treatment of sus scrofa with weekly injections of 15 mg/kg DENA results in a new model for primary hepatic angiosarcoma. This model may help to shed light on the pathomechanisms of primary hepatic angiosarcoma and might therefore open new treatment options.

Rationale: Hepatic angiosarcoma is a rare endothelial cell tumor that may lead to concurrent consumptive coagulopathies including disseminated intravascular coagulation (DIC). This report details a multifaceted approach to managing DIC in a patient with advanced-stage hepatic angiosarcoma, which continued to progress after a brief response to taxane-based chemotherapy. Patient concerns: A 55-year-old man with a recent history of hemorrhoids and hemarthroses presented with acute rectal bleeding. He was found to have concurrent hepatomegaly, abnormal liver function tests, anemia, thrombocytopenia, and coagulopathy. Diagnoses: DIC in the setting of hepatic angiosarcoma. Interventions: The patient's acute bleeding in the setting of DIC was controlled with a combination of antifibrinolytic agents to prevent clot breakdown, heparin products to prevent deposition of new clot, and romiplostim to increase platelet production. His angiosarcoma was treated with various combinations of chemotherapy, including taxane-based chemotherapy, doxorubicin, and pazopanib. Outcomes: The patient's DIC and acute bleeding on initial presentation improved following treatment with unfractionated heparin and low-molecular weight heparin maintenance therapy. It is unclear if the chemotherapy to treat the hepatic angiosarcoma played a significant role in the improvement of DIC. Lessons: Laboratory measurement of prothrombin fragment 1.2, a byproduct of prothrombin conversion to thrombin, proved to be a useful way to monitor this patient's DIC over time.

Background Primary hepatic angiosarcoma (PHA) is a rare tumor entity. Radical surgical resection is currently considered the best treatment choice. The aim of this analysis is to report our experience with surgery for PHA. Methods All resections of PHA from 01/2002 until 06/2017 were identified from our prospective institutional database. All cases were re-confirmed by a second pathologist. We analyzed completeness of resection, overall (OS) and disease-free survival (DFS). Results Nine patients with PHA underwent hepatic resection. Median follow-up after surgery was 15.5 months (range: 3–144). At last follow-up 4/9 patients were alive, three of them without recurrence 15, 21 and 144 months after surgery. Five patients developed PHA recurrence. Four of these died 3 to 17 months after surgery. One patient with PHA recurrence is alive 15 months after surgery. Another patient without PHA recurrence died 59 months after surgery from pancreatic cancer. Median OS and DFS after resection was 18 months (range: 3–144 months) and 10 months (range: 2–144 months), respectively. After R-0 resection (n = 8), the median OS and DFS was 59 and 11 months. Conclusions Resection of PHA is the only approach to achieve complete tumor removal and offers a chance for long-term survival and should be evaluated in cases of PHA.

A 76-year-old woman was referred to our hospital due to massive gingival bleeding following teeth extraction. Laboratory findings suggested disseminated intravascular coagulopathy (DIC). Enhanced computed tomography and magnetic resonance imaging disclosed multiple hypervascular liver masses of 2-6 cm in diameter, the largest of which displaying an irregular enhancement pattern. We considered that her DIC was caused by the multiple liver masses and commenced repeated erythrocyte/fresh frozen plasma infusion and gabexate mesilate administration. However, the DIC proved uncontrollable and trans-arterial embolization could not be attempted. The patient eventually died 4 months after admission due to spontaneous hepatic tumor rupture and hepatic failure. Post-mortem hepatic tumor biopsy led to a final diagnosis of hepatic angiosarcoma with Kasabach-Merritt phenomenon (KMP). Among the 7 cases of hepatic angiosarcoma representing KMP found in the literature, mortality occurred within 4 months of the appearance of bleeding tendency primarily due to abdominal bleeding and hepatic failure. The possibility of hepatic angiosarcoma should be considered in patients with DIC and hypervascular liver tumors. Since treatment is uncertain and prognosis is poor, novel diagnostic and therapeutic advances are needed for angiosarcoma.

To describe imaging characteristics of primary hepatic angiosarcoma on gadoxetate disodium-enhanced dynamic magnetic resonance imaging (MRI) and to determine features that differentiate angiosarcomas from similar-sized haemangiomas.The study included 15 patients with hepatic angiosarcomas and 35 patients with size-matched hepatic haemangiomas who underwent gadoxetate disodium-enhanced liver MRI. The number, size, growth pattern, signal intensity (SI) characteristics, and SI changes on dynamic scans were evaluated and compared between the two entities.Overall, hepatic angiosarcomas significantly more often showed lesion multiplicity (86.7%), capsular retraction (40%), prominent intratumoural vessels (66.7%), vascular invasion (20%), heterogeneous SI on T2-weighted (100%) and hepatobiliary phase images (80%), and intralesional haemorrhage (60%, all p0.05). On dynamic scans, angiosarcomas demonstrated enhancing foci of irregular or rim-like nodular/linear or bizarre (86.7%) shapes, with centrifugal or bizarre patterns of progressive enhancement (53.3%). Enhancement of angiosarcomas was less than that of the blood pool on visual grading, but the enhancement curves followed that of the aorta. Regardless of size, angiosarcomas showed heterogeneous T2 SI, intratumoural haemorrhage, and heterogeneity during the hepatobiliary phase, whereas these findings were more common in haemangiomas6 cm in diameter.Gadoxetate disodium-enhanced dynamic liver MRI is capable of depicting vascular hallmarks of hepatic angiosarcomas. Heterogeneous SI on T2-weighted and hepatobiliary phase images, multiplicity, and an enhancement curve following that of the aorta are also distinctive features that differentiate angiosarcomas from haemangiomas.

Hepatic angiosarcoma is a rare, aggressive, malignant neoplasm with fewer than 50 cases reported in children. Prognosis is poor, with a minority surviving beyond 2 years after diagnosis. We report eight cases of pediatric hepatic angiosarcoma, diagnosed at a mean age of 3 years. Seven were initially diagnosed with an infantile hepatic hemangioendothelioma (IHHE) or hemangioma and the eighth with a "vascular tumor." Two patients, who received liver transplant, survived. We suggest hepatic hemangiomas can rarely transform into angiosarcomas and a subset of IHHEs (Type II) are actually a low-grade form of angiosarcoma rather than a benign lesion.

Primary hepatic angiosarcoma (PHA) is a rare malignancy with a badly poor prognosis. The extremely low morbidity and untypical clinical manifestations conduce to a missed diagnosis. The present study reported a case of an adult male patient who was pathologically confirmed to be PHA, which mimicked intrahepatic cholangiocarcinoma on conventional ultrasound and contrast-enhanced ultrasound. Findings on various imaging examinations were carefully evaluated and the associated literatures were also reviewed.

AIM To analyse the computed tomography (CT) and magnetic resonance imaging (MRI) manifestations of hepatic angiosarcoma. MATERIALS AND METHODS Nineteen patients with hepatic angiosarcoma underwent preoperative abdominal unenhanced and contrast-enhanced CT (11 cases) or (eight cases) MRI. RESULTS The results of a coagulation examination showed varying degrees of abnormalities in 12 (63.16%) cases (most were prolonged prothrombin time and an increased proportion of prothrombin time), which were the most common abnormalities on the laboratory tests. Unenhanced CT of the lesions showed homogeneous or heterogeneous hypointense with hyperintense haemorrhagic lesions, contrast-enhanced CT was performed for six cases (6/11, 54.5%) with centripetal heterogeneous filling. All lesions showed heterogeneous intensity at MRI, including heterogeneous hypointense T1WI and homogeneous or heterogeneous hyperintense T2WI. Haemorrhage lesions showed higher hyperintensity with spot or patchy signals. Centripetal enhancement was found in six cases using contrast-enhanced imaging. Flaky patches of contrast enhancement were seen in the lesions. CONCLUSION The CT and MRI features of most of the hepatic angiosarcomas in the present study were relatively characteristic: the border of the mass was indistinct, the density was heterogeneous, and haemorrhage was frequently seen, with secondary calcification in a few cases, whereas enhanced imaging showed typical centripetal heterogeneous enhancement. In addition, highly malignant angiosarcoma could not be enhanced.

Background. Liver transplantation (LT) is performed for hemangiosarcoma (HAS) despite disappointing results. Methods. Retrospective study of 14 males and 8 females reported to the European Liver Transplant Registry. In view of the difficult differential diagnosis between HAS and hemangioendothelioma (HE), the study was deliberately restricted to the period 1986 to 2004 to allow comparison of clinical and biochemical behavior of HAS and HE liver recipients transplanted during the same time period. Results. Clinical signs, symptoms, and biochemical parameters differed significantly. Pre-LT diagnosis of HAS was made in only 5 of 16 (31%) biopsied patients. HE (7 patients) and hepatocellular cancer (2 patients) were confounding diagnoses leading to LT. Extrahepatic disease was present at time of LT in 4 (19%) patients. Giant invalidating tumor (5 HAS, 1 with Budd-Chiari syndrome [BCS], and 10 supposed epithelioid hemangioendothelioma, 1 with BCS), acute BCS of unknown origin (2 patients), chronic liver failure (4 patients), and solitary hepatocellular cancer (1 patient) were the main indications for LT. Overall survival was 7.2±2.6 months; no patient survived after 23 months. Recurrence was diagnosed after 5.0±2.6 months. Seventeen (77.2%) patients died of tumor recurrence, and the remaining 5 patients died of early infections. Conclusions. HAS is an absolute contraindication to LT due to the poor outcome. When dealing with the difficult differential diagnosis between HAS and HE, futile LT can be avoided by taking into consideration their distinct clinical and biochemical behaviors as well as a 6-month wait-list observation period. This time period enables the evaluation of HAS disease progression without compromising prognosis of HE patients, thereby allowing to avoid organ wastage. © 2013 Lippincott Williams & Wilkins.

A 69-year old man came to our hospital complaining of abdominal pain. Contrast-enhanced computed tomography (CT) showed a 65-mm ruptured mass in Couinaud segment 5 of the liver. The mass was treated with emergent transcatheter arterial embolization (TAE), followed by partial hepatectomy. Microscopically, the mass was determined to be an angiosarcoma. Six months previously, enhanced CT had shown a 15-mm mass diagnosed as a cavernous hemangioma in the same region of the liver. Even when the enhancement pattern of a small hepatic mass resembles that of hemangioma, the mass should be reassessed within several months to exclude a diagnosis of hepatic angiosarcoma.

Nail dystrophy, oral leukoplakia and abnormal skin pigmentation are the defining features of dyskeratosis congenita. Dyskeratosis congenita is a disorder of poor telomere maintenance and is known to increase the risk of developing multiple types of malignancy. However, there are few reports of liver tumors arising in dyskeratosis congenita patients. We herein report the second case of hepatic angiosarcoma arising from dyskeratosis congenita: a 23-year-old man was introduced to our hospital due to the detection of multiple tumors in the liver. A histological analysis showed angiosarcoma that stained positive for antibodies to both CD31 and blood coagulation factor VIII.

We present dual-phase computed tomographic (CT) and angiographic findings of a ruptured hepatic angiosarcoma. These tumors can be divided into two types: those with and those without gross central necrosis with hemorrhage. In our case, the tumor had gross central necrosis, and CT and angiographic findings showed a small number of areas with a centripetal enhancement pattern and the rest of the tumor with avascular areas. We found that dual-phase CT and angiographic findings are able to distinguish angiosarcoma, which mimics a hemangioma, as these lesions show avascular areas that reflect a mass with gross central necrosis.

Liver neoplasms, whether they are benign or malignant, are usually fed by the hepatic artery. We experienced a case of hepatic angiosarcoma supplied by both hepatic artery and portal vein. Since there are currently no specific laboratory tests to diagnose hepatic angiosarcoma, it is important to detect suspect cases from imaging features. This unique hemodynamic property was detected on computed tomography (CT) during hepatic arteriography and CT during hepatic arterioportography. If any imaging examinations indicate the liver tumor to be fed by the portal vein, hepatic angiosarcoma should be suspected.

Liver neoplasms, whether they are benign or malignant, are usually fed by the hepatic artery. We experienced a case of hepatic angiosarcoma supplied by both hepatic artery and portal vein. Since there are currently no specific laboratory tests to diagnose hepatic angiosarcoma, it is important to detect suspect cases from imaging features. This unique hemodynamic property was detected on computed tomography (CT) during hepatic arteriography and CT during hepatic arterioporto­ graphy. If any imaging examinations indicate the liver tumor to be fed by the portal vein, hepatic angiosarcoma should be suspected.

Primary hepatic angiosarcoma (PHA) is a rare malignancy that carries a poor prognosis. Of 1500 patients who underwent hepatectomy for primary hepatic tumors between 1994 and 2013 at our center, two patients were pathologically diagnosed with PHA. Clinical characteristics, treatment modalities, and outcomes of the two patients were collected and analyzed. Both patients underwent hepatectomy and had a postoperative survival time of 8 and 16 mo, respectively. A search of PubMed yielded eight references reporting 35 cases of PHA published between 2004 and 2013. On the basis of the presented cases and review of the literature, we endorse complete surgical resection as the mainstay definitive treatment of PHA, with adjuvant postoperative chemotherapy potentially improving survival. Palliative chemotherapy is an option in advanced hepatic angiosarcoma.

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.

Primary hepatic angiosarcoma is a rare, aggressive tumor; composed of spindle or pleomorphic cells that line, or grow into, the lumina of pre-existing vascular spaces like sinusoids and terminal hepatic venules; with only about 200 cases diagnosed annually worldwide but it is the most common primary malignant mesenchymal tumor of the liver in adults and accounts for 2% of all primary hepatic malignancies. HAS occurs in association with known chemical carcinogens, but 75% of the tumors have no known etiology. Patients present with vague symptoms like abdominal pain, weight loss, fatigue or an abdominal mass. Hepatic angiosarcoma is usually multicentric and involves both lobes, entire liver may also found to be involved. CD31 is the most reliable marker. These tumors lack specific features on imaging, so, pathological diagnosis is necessary. There are no established treatment guidelines because of low frequency and aggressive nature of tumor, chemotherapy is only palliative, liver resection is indicated for solitary mass and liver transplant is contraindicated. The aim of this article is to comprehensively review all the available literature and to present detailed information and an update on primary hepatic angiosarcoma.

Background: Hepatic angiosarcomas are rare tumors most often associated with exposure to vinyl chloride or other carcinogens. Only a few cases have been published without such a history. Case Report: We report the case of a 73-year-old woman who was admitted to our medical department with unclear upper abdominal pain, thrombocytopenia and anemia. Both computed tomography and magnet resonance imaging revealed a giant hemangioma in the right liver with multiple small hemangiomas. To cure the problem of thrombocytopenia due to sequestration of blood cells in the hemangioma, we decided to resect the large tumor. Intraoperatively, however, the diagnosis of angiosarcoma with multiple metastases was made. The patient died 6 weeks after surgery. Conclusion: Problems in diagnosing angiosarcoma include the brief duration of antecedent symptoms, difficulties in radiological diagnosis, and patients without a history of professional exposure to carcinogens.