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Primary hepatic angiosarcoma (PHA) is rare, does not possess any characteristic tumor markers, is primarily observed in the elderly, and often presents with nonspecific symptoms, including discomfort or distension of the abdomen, weight loss and fatigue. Thus, PHA is difficult to diagnose, particularly if the patient presents no history of exposure to carcinogens, and its definitive diagnosis requires histological examination following surgery. Patients with PHA present poor long-term survival, and surgical resection of the tumor is currently the best treatment option for PHA. In the present report, the case of a 64-year-old man initially diagnosed with hydatid cyst, who was subsequently diagnosed with a giant PHA in the middle of the liver, is described. Further studies are required to investigate the diagnosis and treatment of PHA.

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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.

Hepatic angiosarcoma (HA) accounts for 2% of primary liver tumors. Though rare, it is exceptionally deadly. The initial presentation of HA is nonspecific and no tumor markers have been associated with it. In general, liver function is maintained until later stages of the disease, often leading to diagnosis once the disease is already advanced or metastatic. In this report, we present the case of a 54-year-old male whose vague symptoms and non-diagnostic liver biopsy delayed the diagnosis of a rapidly progressing HA.

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.

Pediatric hepatic angiosarcoma (PHAS) is extremely rare, with only five reported tumor-free survivors. Aggressive surgical resection and chemotherapy have been the management in all documented survivors of this disease, however no specific treatment guidelines are established. We present a case of PHAS with the longest reported tumor-free survival at over six years off therapy.

Highlights • Primary hepatic angiosarcomas are rare in the Western world. • At the time of diagnosis, primary hepatic angiosarcomas are often locally advanced. • Surgeons should be aggressive in the pursuit of complete resections because this is the only treatment modality that has been shown to have a survival advantage. • In selected cases, the hanging manoeuver may be employed to allow controlled parenchymal transection and minimal bleeding. • Hepatobiliary surgeons should keep the hanging manoeuver in their armamentarium when performing complex liver resections for locally advanced angiosarcomas., Introduction Angiosarcomas arising in the liver are rare tumours in the Western world. We report a case of a locally advanced primary hepatic angiosarcoma and also describe the manoeuvres used to achieve operative resection. Presentation of a case A 52-year old woman presented with vague right upper quadrant pain. Abdominal imaging revealed a heterogenous tumour in the right liver measuring 15 centimetres in maximal diameter. Although the tumour was deemed to be resectable, there was free fluid in the right paracolic gutter suggestive of rupture. Intra-operatively, the peritoneal cavity was noted to be free of metastatic disease. However, tumour was adherent to the diaphragm precluding traditional mobilization of the liver. Therefore, a modified hanging manoeuvre was performed using a nasogastric tube. This allowed controlled mobilization of the right liver, parenchymal transection and en-bloc resection of the diaphragm with good hemostasis. Histologic examination revealed a primary angiosarcoma with uninvolved margins. Discussion When they occur, primary hepatic angiosarcomas are most often locally advanced. Nevertheless, surgeons should be aggressive in the pursuit of complete resections because this is the only therapeutic modality that has been shown to have a survival advantage. Conclusion Hepatobiliary surgeons should keep the hanging manoeuver in their armamentarium when performing complex liver resections for locally advanced angiosarcomas.

Malignant vascular tumors of the liver are rare. The aim of this study was to investigate the applicability of gray scale and contrast-enhanced ultrasonography in patients with epithelioid hemangioendothelioma (EHE) of the liver and hepatic angiosarcoma (HA) and to describe the clinical presentation.We retrospectively analyzed all patients with epithelioid hemangioendothelioma or hemangiosarcoma of the liver from 1998 to 2011, who underwent ultrasound investigation. We describe the findings in gray scale and contrast-enhanced ultrasound and the clinical course of the disease of seven patients with EHE and five patients with HA.Ultrasound investigation in EHE showed mostly multiple hypoechoic irregular lesions close to the liver capsule and with a halo in some cases. Contrast enhancement revealed inhomogeneously and through all contrast phases vascularized tumors with a rim enhancement in 50%, with or without early wash out. All tumors had avascular parts. HA presented as multiple and irregular hypo-, iso- or hyperechoic lesions. After contrast enhancement, hypervascularization with individual patterns was evident in all patients. Of five, three had liquid parts. Patients with HA were significantly older (58 vs. 37 years, p = 0.014) and presented with lower thrombocyte counts (84 vs. 264, p = 0.0025) and with higher CEA levels (4.6 vs. 1.5, p = 0.03).EHE and HA are inhomogeneous tumors, explaining the high inter-individual variability and heterogeneity in ultrasound examination. The presence of multifocal lesions, heterogeneity and undefined margins may differentiate EHE or HA from hemangioma. A biopsy is essential in the diagnosis of vascular tumors.

Background. Hepatic angiosarcoma is a rare and aggressive tumor that often presents at an advanced stage with nonspecific symptoms.Objective. To report a case of primary hepatic angiosarcoma in an otherwise healthy man with normal liver function tests two months prior to presenting with a short period of jaundice that progressed to fulminant hepatic failure.Methods. Case report and review of literature.Conclusion. This case illustrates the rapidity of progression to death after the onset of symptoms in a patient with hepatic angiosarcoma. Research on early diagnostic strategies and newer therapies are needed to improve prognosis in this rare and poorly understood malignancy with limited treatment options.

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.

Background Primary hepatic angiosarcoma is a non-epithelial malignancy derived from sinusoidal endothelial cells, accounting for approximately 1.8% of primary hepatic malignancies. Diagnosis of primary hepatic angiosarcoma is complicated by difficulties in the qualitative radiological assessment of these tumors. Prognosis is very poor due to local recurrence and distant metastasis after liver resection or liver transplantation (LT). Case presentation This case report describes two patients with primary hepatic angiosarcoma who were diagnosed by histopathological examination of the explanted liver after LT. One patient had undergone living donor LT, and the other had undergone deceased donor LT. Neither showed evidence of malignancy on the pre-operative imaging tests. Conclusions Hepatic angiosarcoma has a very high relapse rate after LT. Pre-transplant liver biopsy may be necessary to distinguish diffuse hepatic angiosarcoma from tumors of other origin in patients with cryptogenic liver failure.

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.

Hepatic angiosarcoma is an extremely rare diagnosis in children, with fewer than 50 pediatric cases reported in the literature worldwide. This aggressive vascular sarcoma carries a very dismal prognosis and is known to be resistant to radiation, chemotherapy, and other vascular-targeted agents. Complete surgical resection is felt to provide the best chance for long-term survival. In patients with tumors not amenable to resection, a liver transplant can be considered. However, very few such transplants have been reported, given that they remain controversial due to high cancer recurrence and mortality post-transplant. Herein, we report the unique case of a 2-year-old child with localized hepatic angiosarcoma not amendable to resection who successfully underwent a liver transplant and received chemotherapy with six cycles of doxorubicin, docetaxel, and ifosfamide.

Hepatic angiosarcomas are uncommon, highly aggressive tumors, rarely seen in children. A 3-month-old female infant was admitted to hospital for evaluation of multiple petechiae on her body. She had hepatosplenomegaly and scattered petechiae over her entire body. Laboratory tests indicated thrombocytopenia and positive cytomegalovirus (CMV) polymerase chain reaction. Ganciclovir was started, and the platelet count increased. After 4 months the patient was readmitted to hospital for drowsy mental status and eventually died from severe bleeding. Needle biopsy of the liver was performed after receiving written consent from the parents. Pathological findings of the liver lesion included features consistent with hepatic angiosarcoma. There have been no previous reports of hepatic angiosarcoma in Korean infants. Here, we report an infant with hepatosplenomegaly and thrombocytopenia who was diagnosed with hepatic angiosarcoma mimicking congenital CMV infection.

We present a case of hepatic angiosarcoma that presented with disseminated intravascular coagulopathy to highlight the difficulty in diagnosing this disease due its aggressive clinical course, the overlapping features of various coagulopathies, and the nonspecific appearance of angiosarcomas on imaging.

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.

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.

A 78-year-old male was admitted to our hospital for treatment of multiple hepatic tumors, which were suspected as hepatic epithelioid hemangioendothelioma (HEHE) by percutaneous tumor needle biopsy. With a diagnosis of HEHE, the patient underwent hepatic resection. In pathological findings, the tumor was composed of numerous endothelial cells without epithelioid cells, and was diagnosed as hepatic angiosarcoma (HAS). The patient received percutaneous radiofrequency ablation for recurrent HAS at 5 and 25 months postoperatively, and remains well with recurrence as of 28 months after the primary hepatic resection. In spite of improvement in radiological imaging, preoperative differential diagnosis between HAS and other malignant vascular tumors of the liver is still difficult. We herein report a case of HAS mimicking HEHE, treated successfully.

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.

症例は46歳，男性．上腹部痛を主訴に当院消化器科を受診した．画像検査上膵頭部に4cm大の内部が不均一でhypovascularな腫瘤が存在し肝内にはいくつかの小結節性病変を認めた．膵腫瘤，肝内の多発結節はサイズが徐々に増大し，入院後10日，膵頭部腫瘤が破裂出血したため，緊急に止血ドレナージ術を行った．術後6日には肝内多発結節のサイズが増大，S3のものが破裂出血しており，一時的な止血救命を目的にTAEを行った．術後10日（入院後22日）肝不全のため死亡．剖検病理所見では肝血管肉腫と診断された．診断に苦慮し，膵頭部腫瘤を契機に発見されるという非常に稀な肝血管肉腫を経験した．若干の文献的考察を加え報告する．