Hepatic Angiosarcoma Research Articles

Diethylnitrosamine (DENA) recapitulates formation of hepatic angiosarcoma in pigs.

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.

Angiosarcoma of the Liver: Clinicopathologic Features and Morphologic Patterns.

Angiosarcoma is a rare malignant neoplasm of the liver. The various morphologic patterns seen with angiosarcomas of the liver have not been systematically studied and their recognition remains a major diagnostic challenge. In order to provide more comprehensive data on the morphologic patterns, angiosarcomas that had been diagnosed between 1996 and 2016 at a large medical referral center were reviewed. The major growth patterns were classified as sinusoidal (non-mass forming) versus mass forming. The mass-forming cases were further subdivided into epithelioid, spindled, or vasoformative. The study identified 21 patients with primary hepatic angiosarcoma: 13 men and 8 women. The ages ranged from 26 to 89 years. Seventeen angiosarcomas were mass-forming tumors, of which 9 showed predominantly vasoformative growth. Most of these vasoformative cases (6/9) were composed of small vessels, 2 cases had slit-like vascular spaces, and one case showed a mixture of small and large vessels. There were 7 mass-forming angiosarcomas without vasoformation: 3 had an epithelioid morphology and 4 were composed primarily of spindled cells. The final mass-forming tumor showed a mixture of vasoformative and nonvasoformative areas. Four of 21 cases were non-mass forming and showed either diffuse sinusoidal infiltration (N=2) or prominent peliotic changes (N=2). Finally, 3 uncommon patterns were identified. One case showed nodules of spindle cells arranged in prominent whorls in a background of loose connective tissue with abundant inflammation. A second case arose in the setting of the Blue Rubber Bleb Nevus Syndrome and showed numerous tumor nodules with an architectural pattern that resembled infantile hemangioma, some with areas of atypia consistent with malignant transformation to angiosarcoma. The third unusual pattern showed multiple nodules of thin walled large caliber vascular proliferations, some of which showed atypia that reached the level of angiosarcoma. The results from this study indicate that the majority of hepatic angiosarcomas are mass forming (two third of cases), a pattern that is recognizable on H&E when vasoformative, but can mimic carcinoma or undifferentatied sarcomas when nonvasoformative (one third of cases). The sinusoidal patterns are particularly challenging and are frequently missed on initial review. Finally, we describe several unsual patterns of angiosarcoma. Awareness of these classic and rare morphologic patterns can help make the diagnosis of angiosarcoma.

Hepatic angiosarcoma in a patient with liver cirrhosis

Hepatic angiosarcoma in a patient with liver cirrhosis

Magnetic Resonance Imaging of Uncommon Hepatic Mesenchymal Tumours: Haemangioendothelioma and Angiosarcoma.

Primary Hepatic Epithelioid Haemangioendothelioma (HEHE) and Primary Hepatic Angiosarcoma (PHA) are rare mesenchymal tumours with different malignant potential. Whereas HEHE demonstrates low to intermediate malignant potential, PHA is an aggressive malignancy with poor prognosis. The knowledge of typical imaging features of these lesions may facilitate correct diagnosis; however, the ultimate diagnosis of HEHE and PHA is based on histopathologic examination. The most typical findings helpful in diagnosing HEHE are: Presence of multiple, confluent nodules located at the liver periphery (in young to middle-aged woman), retraction of the liver capsule, marked hyperintensity on T2-weighted images, "target-sign" appearance, progressive centripetal contrast enhancement, and relatively high Apparent Diffusion Coefficient (ADC) values. More than ≥50% of nodules are hyper- or isointense on Hepatobiliary Phase (HBP) images. The imaging features suggestive of PHA are: Occurrence of metastases (lungs, spleen) at the time of diagnosis, presence of a large dominant mass with smaller satellites, heterogeneity and areas of haemorrhage in a dominant mass, progressive contrast enhancement, slightly elevated ADC values as compared to other malignant liver tumours.

Malignant hepatic vascular tumors in adults: Characteristics, diagnostic difficulties and current management.

Malignant vascular tumors of the liver include rare primary hepatic mesenchymal tumors developed in the background of a normal liver parenchyma. Most of them are detected incidentally by the increased use of performing imaging techniques. Their diagnosis is challenging, involving clinical and imaging criteria, with final confirmation by histology and immunohistochemistry. Surgery represents the mainstay of treatment. Liver transplantation (LT) has improved substantially the prognosis of hepatic epithelioid hemangioendothelioma (HEHE), with 5-year patient survival rates of up to 81%, based on the European Liver Intestine Transplantation Association-European Liver Transplant Registry study. Unfortunately, the results of surgery and LT are dismal in cases of hepatic angiosarcoma (HAS). Due to the disappointing results of very short survival periods of approximately 6-7 mo after LT, because of tumor recurrence and rapid progression of the disease, HAS is considered an absolute contraindication to LT. Recurrences after surgical resection are high in cases of HEHE and invariably present in cases of HAS. The discovery of reliable prognostic markers and the elaboration of prognostic scores following LT are needed to provide the best therapeutic choice for each patient. Studies on a few patients have demonstrated the stabilization of the disease in a proportion of patients with hepatic vascular tumors using novel targeted antiangiogenic agents, cytokines or immunotherapy. These new approaches, alone or in combination with other therapeutic modalities, such as surgery and classical chemotherapy, need further investigation to assess their role in prolonging patient survival. Personalized therapeutic algorithms according to the histopathological features, behavior, molecular biology and genetics of the tumors should be elaborated in the near future for the management of patients diagnosed with primary malignant vascular tumors of the liver.

Molecular biology of sarcoma

Soft tissue sarcomas are a large group of heterogenous neoplasms, many of them are highly aggressive. Most of the cases are sporadic, without any well-defined pathogenetic factor. Potential risk factors are ionizing radiation, lymphatic oedema (secondary angiosarcoma of the breast), viral infections (HHV8 and Kaposi sarcoma), exposure to chemical factors (vinyl chloride and hepatic angiosarcoma). Genetic susceptibility plays a role in a minority of cases. However, mutations in TP53, ATM and ATR genes are associated with enhanced susceptibility to radiation. Li-Fraumeni syndrome (autosomal dominant TP53 mutation) predisposes to development of malignancies, one third of them are sarcomas. Genetic alterations observed in sarcomas could be divided into three major groups characterized by: (1) chromosome translocations; (2) simple karyotype and mutations; (3) variably complex karyotypes. A large part of sarcomas belong to the first group and the specific chromosal translocations could be utilized in the diagnostic process. A smaller number of sarcomas could be assigned to the second group, e.g. desmoid fibromatosis (CTNNB1 or APC mutations) and GIST (KIT, PDGFRA, or less frequently BRAF, SDH, NF1). A large number of sarcomas are characterized by complex and variable karyotypes. Gene copy number alterations are frequent in this group, e.g. in well-differentiated liposarcoma there is an amplification of MDM2, CDK4 and HMGA2 genes or sarcoma-specific chromosomal break regions present in the CHOP gene in myxoid liposarcoma and FKHR in alveolar rhabdomyosarcoma.

CT and MRI studies of hepatic angiosarcoma

To analyse the computed tomography (CT) and magnetic resonance imaging (MRI) manifestations of hepatic angiosarcoma. Nineteen patients with hepatic angiosarcoma underwent preoperative abdominal unenhanced and contrast-enhanced CT (11 cases) or (eight cases) MRI. 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. 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.

Surgical therapy of primary hepatic angiosarcoma

BackgroundPrimary 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.MethodsAll 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).ResultsNine 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.ConclusionsResection 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.

ROS1-GOPC/FIG: a novel gene fusion in hepatic angiosarcoma.

Hepatic angiosarcoma (HAS) is a rare and highly lethal malignancy with few effective systemic treatments. Relatively little is known about the genetic abnormalities that drive this disease. As a result, there has been minimal progress towards applying targeted therapies to the treatment of HAS. We describe the first reported case of a patient with HAS that harbored a fusion of ROS1 with GOPC/FIG. Similar to other rearrangements involving ROS1, the resulting fusion protein is believed to act as a major driver of carcinogenesis and may be subject to inhibition by drugs that target ROS1 such as crizotinib. We then queried the MSK-IMPACT clinical sequencing cohort and cBioportal datasets, demonstrating the previously unknown prevalence of ROS1-GOPC fusions in soft tissue sarcomas and hepatobiliary cancers. Amplification of these genes was also found to correlate with reduced overall survival. This is followed by a review of the role played by ROS1 rearrangements in cancer, as well as the evidence supporting the use of targeted therapies against the resulting fusion protein. We suggest that testing for ROS1 fusion and, if positive, treatment with a targeted therapy could be considered at the time of diagnosis for patients with angiosarcoma. This report also highlights the need for further investigation into the molecular pathophysiology of this deadly disease.

Hepatic Angiosarcoma: A Rare Cause of Acute Liver Failure Case Report and Review of the Literature

Hepatic Angiosarcoma is a rare, high grade, aggressive mesenchymal tumor. It is the most common malignant mesenchymal tumor of the liver, but is nonetheless rare, accounting for 2% of all primary liver malignancies, with a male predominance. Some hepatic Angiosarcoma were associated with exposure to anabolic steroids, radiation, thorium dioxide, arsenic, vinyl chloride and use of oral contraceptives, but most cases are now considered idiopathic. Given that hepatic angiosarcoma clinical, laboratory and radiologic presentation are non specific, radical resection of the tumor is being currently the best treatment option. Its diagnosis is difficult and often delayed, and requires histopathological examination. The prognosis is extremely poor with a high mortality rate and few therapeutic options. We report a case of a woman who presented with acute liver failure, which is a very unusual presentation of HAS, diagnosed with hepatic angiosarcoma through a liver biopsy. She had a rapidly worsening clinical course and died within four months of diagnosis.

Primary Hepatic Angiosarcoma Revealed by a Hemorrhagic Parietal Metastasis about a Case in Lomé

Angiosarcoma is a malignant tumor developed at the expense of endothelial cells. Primary hepatic localization is rare. Chronic toxic exposure is classically described as responsible for the development of this tumor. The diagnosis is based on the pathological examination. The authors report a case of hepatic angiosarcoma in a 28-year-old woman who received for a thoraco-abdominal hemorrhagic parietal secondary lesion. A thoracic abdominal and parietal ultrasound was performed followed by thoraco-abdomino-pelvic CT. The diagnosis was confirmed by histology. Palliative treatment combining surgery and chemotherapy has been established with a poor prognosis.

Melena as a first sign of metastatic hepatic angiosarcoma: A case report

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.

CAMTA-1 Expression in 24 Cases of Hepatic Epithelioid Hemangioendothelioma in a Single Institute: Diagnostic Utility for Differential Diagnosis from Hepatic Angiosarcoma

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. IHC was performed using anti-CAMTA1 antibody in 34 tumors (24 hepatic EHEs and 10 angiosarcomas). In CAMTA1-negative EHEs, TFE3 IHC was performed. 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. 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.

Surgical indications for focal nodular hyperplasia of the liver: Single-center experience of 48 adult cases.

Backgrounds/AimsFocal nodular hyperplasia (FNH) is one of the most common benign tumors of the liver. There is still a lack of evidence on surgical indications for FNH. This study intended to analyze the surgical indications for FNH.MethodsWe analyzed 48 cases of FNH diagnosed after hepatic resection.ResultsCommon reasons leading to surgical resection were diagnostic uncertainty (n=31), and persistent symptoms (n=8). None of our patients had a past history of contraceptive use. Percutaneous biopsy was performed in 14 patients and FNH was diagnosed in nine patients, and hepatic adenoma, hepatocellular carcinoma, plasmacytoma, angiosarcoma, and atypical hepatocellular proliferation in one patient each. Minor hepatectomy (n=37) was performed more frequently than major hepatectomy (n=11). Open hepatectomy (n=29) was performed more frequently than laparoscopic hepatectomy (n=19), but laparoscopic and minimally-invasive surgery was frequently performed during the late phase of the study period. Postoperative surgical complications occurred in two patients (4.1%).ConclusionsFNH can be diagnosed by imaging studies, but surgical treatment may be considered in cases of diagnostic uncertainty or persistent symptoms.

Primary malignant mesenchymal tumors of the liver and the inferior vena cava: a rare tumor entity

Background: Malignant mesenchymal tumors (MMT) of the liver and the inferior vena cava are a rare tumor entity. Primary hepatic angiosarcoma (PHA) and hepatic epithelioid hemangioendothelioma (HEH) are of endothelial origin, whereas leiomyosarcoma (LMS) arise from smooth muscle cells. While HEH seems to be a low-grade malignant tumor, PHA is assumed to be an aggressive tumor. In the absence of established treatment guidelines radical surgical resection is considered the best treatment. Methods: All resections of primary MMT of the liver (January 2007 until January 2017) were identified from a database of all liver resections performed at our department. We analyzed the completeness of resection (R0/R1), disease-free (DFS) and overall survivals (OS). Results: 15 resections were performed for MMT. Of these, 7 resections were performed for PHA, 4 for HEH and 4 for LMS. 8 out of 15 (53%) patients are tumor free until last follow-up: 3 patients with LMS relapsed, while 3 patients with HEH were tumor-free during follow-up, as well as 2 patients with PHA, who had received neoadjuvant chemotherapy (Ifosamid/Vincristin/Doxorubicin or Paclitaxel). 3 patients with PHA were multifocal when operated and relapsed within three months. One patient with HEH died independently from tumor. The median OS was 108 months and the median DFS was 45 months. Conclusion: Until now the efficiency of chemotherapy could not be proved. In accordance with the literature the best treatment for MMT is the radical surgical resection.

Hepatic angiosarcoma

Hepatic Angiosarcoma is a rare malignancy of endothelial cell origin that is generally idiopathic and presents non-specific clinical manifestations. Hemoperitoneum can occur in 17-27% of cases and is a result of tumor rupture, which has a devastating prognosis. We present a 79-year-old female patient with history of diffuse right upper quadrant abdominal pain associated with syncope episodes and unintentional weight loss. Physical examination was unremarkable except for painful palpation of the right upper quadrant. Laboratory exams indicated chronic disease anemia, a 2.7 INR, decreased albumin, increased C-Reactive Protein and GGT, and normal AST, ALT, Alkaline Phosphatase and Bilirubin. Viral hepatitis serologies were negative. Abdominal ultrasonography revealed hepatomegaly and solid liver lesions of heterogeneous echogenicity and imprecise limits. Three-phase abdominal CT showed multiple liver masses of heterogeneous pattern of enhancement suggestive of atypical hemangioma associated with ascites. Chest CT revealed bilateral pulmonary nodules suggestive of metastasis. During hospital stay, the patient developed a massive hemoperitoneum that required emergency laparotomy. In this circumstance, liver and omentum biopsies were performed and the pathology reports ultimately indicated the possibility of hepatic angiosarcoma. The patient developed refractory hemoperitoneum and hemorrhagic shock and ultimately passed away, 48 days after hospital admission. Definitive diagnosis was only available posteriorly, with immunohistochemistry positivity for ERG, CD34 and Factor VIII-related antigen on both omentum and lung samples. This case study provides valuable clinical discussion and emphasizes how a transdisciplinary approach is essential to correctly diagnose and manage such complex cases.

Malignant Vascular Tumors of the Liver in Adults.

Hepatic angiosarcoma and epithelioid hemangioendothelioma (EHE) might be clinically considered a spectrum since, although more frequently presenting indolent behavior, EHE occasionally evolves to high-grade neoplasms. However, in most circumstances, pathological and immunohistochemical patterns define this differential diagnosis. More recently, molecular pathways for angiosarcoma and for EHE from other organs and from soft tissue have been proved different, paving the way for future morpho-molecular assessment of their hepatic counterpart. The frequency of liver involvement by Kaposi sarcoma in HIV-infected patients is lower nowadays. Histological findings and immunostaining for HHV-8 Ag are characteristic. Hepatic small vessel neoplasms have been recently recognized as important mimickers of angiosarcoma. The criteria for this differential diagnosis and the clinical behavior, up to now considered favorable, must be further studied.

Hepatic Angiosarcoma: A Multi-institutional, International Experience with 44 Cases.

Hepatic angiosarcoma is a rare primary liver tumor. The aim of this current study was to evaluate the presentation and treatment outcomes in a modern cohort. This was a retrospective, multi-institutional, observational study of patients with histopathologic diagnoses of primary hepatic angiosarcoma from four institutions. Clinicopathologic characteristics, treatments, and patient outcomes were examined. Forty-four patients with hepatic angiosarcoma were identified. Patients were predominantly Caucasian and presented at a median age of 63.7years; 81.4% of patients had bilobar disease and 37.2% had metastatic disease at the time of presentation. Only 10 patients underwent surgical resection. Median overall survival for the entire cohort was 5.8months (interquartile range 1.9-16.4), and 1-, 3-, and 5-year actual survival was 30.0%, 8.1%, and 5.6%, respectively. There were only two 5-year survivors, both of whom presented with localized disease and underwent curative resection. The prognosis for hepatic angiosarcoma remains quite poor. Surgical resection for localized disease results in the best outcomes. Unfortunately, current imaging modalities are often non- diagnostic, and most patients are unresectable at the time of presentation.

Management of disseminated intravascular coagulation in a patient with hepatic angiosarcoma

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.

Angiosarcoma: A Rare Cause of Acute Liver Failure

We are presenting a case of acute liver failure from angiosarcoma. The patient underwent liver transplant and her explanted liver showed angiosarcoma. She passed away from failure of her transplanted liver with autopsy showing angiosarcoma in her new liver. Hepatic angiosarcoma is difficult to diagnose, is a contraindication to liver transplantation, and carries a poor prognosis with or without liver transplantation. Our patient was a 56-year-old African American female found to have elevated liver function tests as part of bronchitis evaluation. She had no history of alcohol or drug use, or prior history of liver disease. Her acute liver injury rapidly progressed. Liver biopsy at outside hospital showed severe acute hepatitis with confluent necrosis and infiltrate of atypical reactive cells. On our assessment, her Model for End-Stage Liver Disease (MELD) score was greater than 40 and she developed altered mental status. Testing was negative for viral hepatitis. Alpha feto protein level was normal. Abdominal magnetic resonance imaging and CT chest showed a cirrhotic liver with heterogenous parenchyma suggestive of fibrotic changes and no abdominal or thoracic masses. She received orthotopic deceased-donor liver transplantation. Her post-operative course was complicated by liver function test abnormalities and intermittent altered mental status. Three weeks after liver transplant, a CT angiogram showed areas of necrosis and infarct in her liver despite patent hepatic vasculature. Pathology of her explanted liver revealed angiosarcoma with extensive vascular involvement with massive hepatic necrosis. A subsequent CT angiogram of her abdomen revealed worsening hepatic infarcts with diminutive right and left hepatic arteries. She was transitioned to comfort care and passed away. Autopsy revealed angiosarcoma in her liver, with diffuse hepatic necrosis and numerous infarcts.2309_A Figure 1 No Caption available.2309_B Figure 2 No Caption available.2309_C Figure 3 No Caption available.Despite extensive hepatic involvement of angiosarcoma in this patient's explanted liver and autopsy, pre and post transplant imaging were not interpreted as being concerning for malignancy, and pre transplant liver biopsy had nonspecific features without concern for malignancy. Hepatic angiosarcoma is rare, and it rarely results in fulminant liver failure. This atypical presentation poses a clinical dilemna. Hepatic angiosarcoma should be considered in the differential diagnosis in the setting of hepatic necrosis and worsening hepatic infarcts pre- and post-liver transplant.