Malignant Epithelioid Hemangioendothelioma Research Articles

Epithelioid hemangioma of the acromion causing shoulder pain: a case report.

Epithelioid hemangioma of bone is a rare benign vascular tumor. Because of the locally destructive clinical presentation and the presence of atypical histologic features with increased mitotic activity and necrosis, it is often misdiagnosed as low-grade malignant epithelioid hemangioendothelioma or high-grade malignant epithelioid angiosarcoma. Correct diagnosis through imaging studies and histopathological examination is mandatory to determine the appropriate course of treatment, as the prognosis differs from that of other malignant hemangioma tumors. A 69-year-old male who presented with intractable shoulder pain caused by epithelioid hemangioma in the acromion of the scapula was treated with tumor curettage. This paper reports a good result with a review of the relevant literature.

Evaluation of TRIM63 RNA in situ hybridization (RNA-ISH) as a potential biomarker for alveolar soft-part sarcoma (ASPS)

Alveolar soft-part sarcoma (ASPS) is a rare soft tissue tumor with a broad morphologic differential diagnosis. While histology and immunohistochemistry can be suggestive, diagnosis often requires exclusion of other entities followed by confirmatory molecular analysis for its characteristic ASPSCR1-TFE3 fusion. Current stain-based biomarkers (such as immunohistochemistry for cathepsin K and TFE3) show relatively high sensitivity but may lack specificity, often showing staining in multiple other entities under diagnostic consideration. Given the discovery of RNA in situ hybridization (RNA-ISH) for TRIM63 as a sensitive and specific marker of MiTF-family aberration renal cell carcinomas, we sought to evaluate its utility in the workup of ASPS. TRIM63 RNA-ISH demonstrated high levels (H-score greater than 200) of expression in 19/20 (95%) cases of ASPS (average H-score 330) and was weak or negative in cases of paraganglioma, clear cell sarcoma, rhabdomyosarcoma, malignant epithelioid hemangioendothelioma, as well as hepatocellular and adrenal cortical carcinomas. Staining was also identified in tumors with known subsets characterized by TFE3 alterations such as perivascular epithelioid cell neoplasm (PEComa, average H-score 228), while tumors known to exhibit overexpression of TFE3 protein without cytogenetic alterations, such as melanoma and granular cell tumor, generally showed less TRIM63 ISH staining (average H-scores 147 and 96, respectively). Quantitative assessment of TRIM63 staining by RNA-ISH is potentially a helpful biomarker for tumors with molecular TFE3 alterations such as ASPS.

Epithelioid hemangioendothelioma of the brachial artery A rare benign vascular tumor

Epithelioid hemangioendotheliomas are uncommon vascular tumors presenting with different biological behavior and clinical features. It is a pathology that should be diagnosed early because it is seen at earlier ages and can cause distant organ metastases. Due to its rarity, it is often not included in the differential diagnosis of soft tissue masses [1]. In this case, we aimed to present a malignant epithelioid hemangioendothelioma presenting with palpable swelling.

Liver Tumor Post Liver Transplantation for Hepatitis C Cirrhosis: An Unusual Cause

Question: A 60-year-old African American man with a history of hepatitis C virus (HCV) cirrhosis underwent orthotopic liver transplantation in 2009. The explant showed chronic HCV with mild activity and cirrhosis (grade 2, stage 4) without evidence of malignancy. The post-transplant course was complicated by recurrent HCV treated 5 years later with simeprevir and sofosbuvir with a sustained viral response. However, the patient developed fibrosing cholestatic hepatitis with rapid progression to fibrosis and portal hypertension demonstrated by small varices and ascites. A liver biopsy in August 2010 revealed mild to moderated inflammatory activity and incipient bridging fibrosis grades 2–3, stages 2–3. Due to worsening renal function and new-onset proteinuria, the dose of the calcineurin inhibitor was reduced and mycophenolate mofetil was added. He was lost to follow-up from 2014 to 2016. Magnetic resonance imaging in February 2017 for screening showed numerous masses in the liver with arterial enhancement and incomplete but progressive washout, some with an enhancing capsule (Figure A–C). At the time, his laboratory tests showed excellent graft function. What is the diagnosis and strategy for treatment? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. Given the history of HCV infection and transplantation, there was concern for hepatocellular carcinoma. An ultrasound-guided core biopsy showed a vascular tumor positive for CD34 and negative for pancytokeratin and CK7, consistent with epithelioid hemangioendothelioma (HEHE) (Figure D-F). Low-power image on with hematoxylin and eosin staining (Figure D) shows upper core with normal liver, and lower core with replacement by vascular lesion (black arrow) with hemorrhage (thick black arrows). Figure E shows high power image with multiple diminutive vascular channels replacing liver parenchyma (black arrowheads), and Figure F with vascular channels replacing liver parenchyma on CD34 stain (black arrows). Figures A-C demonstrate HEHE lesions as target shaped lesion on a T1-weighted volumetric interpolated breath-hold examination sequence (Figure A), with largest lesion in a postcontrast sequence (Figure B), and multiple lesions seen on venous phase sequence (Figure C). Retransplantation was considered; however, given the indolent nature of the lesions, he was referred to oncology and interventional radiology for treatment. He did not make those appointments, and the latest blood tests in August 2020 showed stable graft function with no decompensation. He developed dementia and has not been able to follow-up with further imaging. HEHE was first described in 1984 and is a rare indolent malignant tumor with unclear etiology with vague presentations including abdominal discomfort, nausea, and vomitting.1Ishak K.G. Sesterhenn I.A. Goodman M.Z.D. et al.Epithelioid hemangioendothelioma of the liver: A clinicopathologic and follow-up study of 32 cases.Hum Pathol. 1984; 15: 839-852Crossref PubMed Scopus (382) Google Scholar HEHE rarely involves the vasculature, causing Budd–Chiari Syndrome, veno-occlusive disease, and portal hypertension. At diagnosis, extrahepatic involvement is reported in 36.6% of patients involving lungs, regional lymph nodes, and peritoneum.2Mehrabi A. Kashfi A. Fonouni H. et al.Primary malignant hepatic epithelioid hemangioendothelioma: a comprehensive review of the literature with emphasis on the surgical therapy.Cancer. 2006; 107: 2108-2121Crossref PubMed Scopus (343) Google Scholar Although the etiology is unknown, reported associations include oral contraceptive use, alcohol consumption, liver trauma, sarcoidosis, Crohn’s disease, viral hepatitis, and vinyl chloride or asbestos exposure.2Mehrabi A. Kashfi A. Fonouni H. et al.Primary malignant hepatic epithelioid hemangioendothelioma: a comprehensive review of the literature with emphasis on the surgical therapy.Cancer. 2006; 107: 2108-2121Crossref PubMed Scopus (343) Google Scholar Treatment options include a combination of liver resection, transarterial chemoembolization, liver transplantation, and the possible addition of chemotherapy.2Mehrabi A. Kashfi A. Fonouni H. et al.Primary malignant hepatic epithelioid hemangioendothelioma: a comprehensive review of the literature with emphasis on the surgical therapy.Cancer. 2006; 107: 2108-2121Crossref PubMed Scopus (343) Google Scholar,3Brahmbhatt M. Prenner S. Bittermann T. Liver transplantation for hepatic epithelioid hemangioendothelioma is facilitated by exception points with acceptable long-term outcomes.Transplantation. 2020; 104: 1187-1192Crossref PubMed Scopus (13) Google Scholar HEHE should be considered in the differential diagnosis of de novo liver masses after liver transplantation.

Hepatic vascular malignancies in children are associated with increased rates of surgical resection and improved overall survival compared with adults.

Hepatic vascular malignancies (HVMs) are rare malignancies, with no standardized treatment regimens. The most common HVMs, angiosarcoma and malignant epithelioid hemangioendothelioma (EHE), are often grouped together in the literature complicating our ability to achieve reliable survival data and treatment strategies. To compare the disease characteristics of HVMs, with a subanalysis on pediatric patients. The 2016 National Cancer Database was queried for patients with HVMs using international classification of diseases-oncology-3 (ICD-O-3) codes yielding 699 patients. Descriptive statistics, chi-square, Kaplan-Meier, and log-rank analyses were performed. We found 478 patients (68%) with angiosarcoma and 221 (32%) with EHE. The median (Q1, Q3) age for angiosarcoma patients was 65years (56, 75) versus 54years (37, 65) in EHE patients (P<.001). The rate of resection was lower in patients with angiosarcoma than EHE (13% vs 32%, P<.001). The mean 1-, 3-, and 5-year overall survival for angiosarcoma patients was 17%, 8%, and 6%, respectively, versus 80%, 65%, and 62% in EHE patients (P<.0001). A subgroup analysis was performed on pediatric patients demonstrating six with angiosarcoma and 10 with EHE. The mean 1-, 3-, and 5-year overall survival for pediatric angiosarcoma patients was 67%, 50%, and 50%, respectively, and 90%, 90%, and 90% for pediatric EHE patients. In the largest study of HVMs to date, we found angiosarcoma has significantly worse overall survival than EHE. Pediatric patients appear to have improved survival and higher rates of resection. Larger studies of HVMs are needed to clearly differentiate tumor types, standardize care, and improve survivorship.

P1262 If anything can go wrong, it will: management of a pericardial effusion in a young patient

Abstract Case Report A 25 year-old male patient with an unremarkable past medical history presented to the Emergency Department due to a hemodynamically stable wide complex tachycardia at 260bpm with no response to adenosine, which was successfully treated with synchronized electrical cardioversion. Post cardioversion EKG revealed a pre excitation pattern and the transthoracic echocardiogram showed a large pericardial effusion with diastolic right atrial collapse. The patient was admitted in the Cardiology Department for emergent pericardiocentesis and further investigation. A total of 800 mL of bloody pericardial fluid was removed, compatible with an exudate, but fluid culture and cytology were inconclusive. Due to a rapid reaccumulation of pericardial fluid, the patient underwent a pericardial window, after which he developed an intractable pleuritic thoracalgia, refractory to the combination of ibuprofen, colchicine and oral steroid, despite the resolution of the pericardial effusion. He was also submitted to an electrophysiologic study with successful ablation of a right sided accessory pathway. Analysis of the pericardial fluid and biopsy were inconclusive once again, including a low level of adenosine deaminase and negative acid-fast bacilli test and nucleic acid amplification test for Mycobacterium tuberculosis. The patient was afebrile and denied any constitutional symptoms or relevant epidemiological context. The remainder etiological study was unremarkable, with the exception of fluctuating antibody titers for adenovirus, Borrelia burgdorferi and Mycoplasma pneumoniae, which were interpreted as a result of cross-reactivity. Nevertheless, a course of 21 days of doxycycline was tried without any significant improvement. Thoracic-abdominal-pelvic CT and PET scan were normal. Direct inoculation in guinea pig led to positive results for Mycobacterium tuberculosis and antituberculous therapy was started. Despite pathogen directed antibiotic treatment, there was no clinical improvement and the pericardial effusion gradually relapsed, evolving to cardiac tamponade requiring emergent drainage. Histologic examination of pericardial fragments finally revealed massive infiltration by an undifferentiated malignant tumor compatible with a malignant epithelioid hemangioendothelioma. Due to the patient’s poor performance status and rapidly deteriorating clinical course, it was decided not to start chemotherapy. The patient eventually died, 6 months after his admission to the ED and 2 weeks after the neoplasm’s diagnosis. Conclusion In regions with a high Tuberculosis incidence, there should be a high degree of suspicion for tuberculous pericarditis, especially in cases of recurrent pericardial effusion. Immunosuppressed individuals, such as oncologic patients, are at an increased risk for tuberculosis. The management of relapsing pericardial effusion remains a diagnostic challenge as described in this clinical case.

Endothelial cell malignancies in infants, children and adolescents: Treatment results of three Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry.

Endothelial cell malignancies are extremely rare in childhood. New identification of genetic abnormalities (WWTR1:CAMTA1 translocation) helps to recognize potential therapeutic targets. Little is known about treatment and outcome of these patients. Clinical course, treatment, and outcome in patients with endothelial cell malignancies treated within the Cooperative Weichteilsarkom Studiengruppe (CWS) trials CWS-91, -96, -2002P, and the Soft-Tissue Sarcoma Registry (SoTiSaR) were analyzed (1991-2019). Patients had angiosarcoma (AS) (n=12), malignant epithelioid hemangioendothelioma (EHE) (n=16), and kaposiform hemangioendothelioma (KHE) (n=13). The median age was 5.39years (range, 0.8-17.34); 33 patients had localized disease (LD), and 8 patients had metastatic disease. Therapy consisted of chemotherapy (CHT) (AS n=8, EHE n=9, KHE n=5), interferon or new agent therapy (EHE n=5, 2 KHE n=2), microscopically or macroscopically complete resection (AS n=3, EHE n=6, KHE n=3), and radiotherapy (AS n=6, EHE n=2, KHE n=1). Two patients (KHE) had watch-and-wait strategy resulting in stable disease. Complete remission (CR) was achieved in AS (10/12; 83%), EHE (10/16; 63%), and KHE (5/13; 38%). The five-year EFS and OS for patients with AS was 64% (± 29 CI 95%) and 80% (± 25, CI 95%), with EHE 62% (± 24, CI 95%) and 78% (± 23, CI 95%), with KHE 33% (± 34, CI 95%) and 92% (± 15, CI 95%), respectively. Complete resection was a significant prognostic factor for AS, LD for EHE. Endothelial cell malignancies in childhood have a fair outcome with multimodal treatment. New treatment options are needed for metastic disease.

Limited biopsies of soft tissue tumors: the contemporary role of immunohistochemistry and molecular diagnostics.

Diagnosing soft tissue tumors is challenging, even on ample incisional biopsies or resection specimens. There are more than 100 distinct types of soft tissue neoplasms, including more than 80 benign and intermediate mesenchymal tumors and around 40 soft tissue sarcomas. Accurate diagnosis relies first upon recognition of characteristic histologic and cytologic features, including architecture, stromal characteristics, vascular patterns, and dominant cytology; these features may not be represented or apparent in limited core needle biopsy or fine needle aspiration specimens. Once a differential diagnosis is established, application of immunohistochemistry and cytogenetic or molecular diagnostic assays (especially fluorescence in situ hybridization) is used in an attempt to reach a specific diagnosis. In recent years, the diagnostic armamentarium for soft tissue tumors has expanded dramatically, following the discovery of molecular alterations that underlie the pathogenesis of soft tissue tumors. These include new diagnostic immunohistochemical markers that serve as useful surrogates for molecular genetic alterations. Availability of such markers has improved our ability to render accurate and specific diagnoses based on limited biopsy samples. In this review, examples of recently developed markers for the diagnosis of selected soft tissue tumor types will be discussed, including solitary fibrous tumor (STAT6), malignant peripheral nerve sheath tumor (H3K27me3), epithelioid hemangioendothelioma (CAMTA1), dedifferentiated liposarcoma (MDM2), and CIC-DUX4 sarcoma (WT1 and ETV4).

Malignant Hepatic Epithelioid Hemangioendothelioma with Villaret Syndrome

Malignant hepatic epithelioid hemangioendothelioma is a rare, vascular tumor of the liver with peak incidence in the middle age with a female preponderance and of unknown etiology. Majority of the tumors are asymptomatic. The gold standard for diagnosis of the tumor is liver histology showing presence of epithelioid tumor cells on a background of a hyaline stroma. Immunohistochemical positivity for CD31, CD34, Factor VIII, and Podoplanin (D2-40) is confirmatory. The treatment of choice is liver transplantation.

Thalassemia major between liver and heart: Where we are now

The aim of the study was to assess the current state in terms of liver and heart iron overload as well as of liver and heart related morbidity and mortality in a large cohort of thalassemia patients. Myocardial iron loading was present in 28.9% patients, which was severe in 3.2%. Liver iron was normal in 9.3% and severe in 15%. The rate of cardiac deaths started to decrease between 2000 and 2003 and dropped significantly afterwards. The prescription of combination therapy soon after the hospital admission for decompensated heart failure was associated with a decrease in the short-term mortality. In 111 adult patients who underwent liver elastometry, 14 HCVRNA positive subjects and 2 HCVRNA negative, had stiffness values suggestive of cirrhosis. No cases of hepatocarcinoma were reported. Liver "iron free foci" occurred in a HCV negative patient and the occurrence of a malignant epithelioid hemangioendothelioma led to liver transplantation in another. The study suggests that a subset of patients continues to develop progressive hemosiderosis that may lead to cardiac disease and death. Beyond its key role in preventing myocardial iron overload, liver iron chelation is essential for hampering the onset of hepatic tumors, which may not be limited to hepatocarcinoma.

Malignant Epithelioid Hemangioendothelioma of the Adrenal Gland Treated by Laparoscopic Excision

Epithelioid hemangioendothelioma is a rare vascular tumor mostly seen in liver, lung, and bone. Involvement of the adrenal gland has not been reported previously. Here, we report the first case of adrenal malignant epithelioidhemangioendothelioma in an 81-year-old man who was successfully treated by laparoscopic excision.

Penile epithelioid hemangioma: A case report

Penile epithelioid hemangioma is a very rare benign vascular neoplasm of unclear etiology. To the best of our knowledge, only 28 cases in the literature have been reported to date. We report a case of epithelioid hemangioma of the penis in a 41-year-old man with a painful penile mass. The presented case exemplifies the importance of establishing a correct diagnosis through appropriate histologic analysis, as epithelioid hemangiomas are often misdiagnosed as two highly malignant tumors, epithelioid hemangioendothelioma and epithelioid angiosarcoma. Establishing benignancy is crucial in order to avoid inappropriate and overly aggressive treatment.

Malignant pleuropulmonary epithelioid hemangioendothelioma – Unusual presentation of an aggressive angiogenic neoplasm

Epithelioid hemangioendothelioma (EHE) of the lung and pleura are rare tumors. Primary pleural EHE are associated with aggressive behavior and poor clinical outcome. Recent advances in EHE include the development of immunohistochemical markers of vascular differentiation, namely Fli-1, and the identification of a specific chromosomal translocation (t(1;3)(p36;q25)). We present a 19 year old male patient with a rapidly progressive malignant neoplasm that presented as a multinodular lung parenchymal process, with associated pleural effusion and thickening. Pathologic examination revealed a pleuropulmonary neoplasm with cytologic features including round to oval epithelioid cells with frequent cytoplasmic vacuoles. Immunohistochemistry confirmed vascular differentiation of tumor cells (CD34, CD31 and Fli-1 positive). While the cytologic features were suggestive of EHE, the aggressive nature of the neoplasm, with disseminated pleuropulmonary involvement, raised the question of whether the neoplasm should be classified as EHE or epithelioid angiosarcoma. Here, we review the clinicopathologic characteristics of pleuropulmonary EHE and the overlap between malignant EHE and angiosarcoma.

Malignant vascular tumors—an update

Although benign hemangiomas are among the most common diagnoses amid connective tissue tumors, sarcomas showing endothelial differentiation (ie, angiosarcoma and epithelioid hemangioendothelioma) represent under 1% of all sarcoma diagnoses, and thus it is likely that fewer than 500 people in the United States are affected each year. Differential diagnosis of malignant vascular tumors can be often quite challenging, either at the low end of the spectrum, distinguishing an epithelioid hemangioendothelioma from an epithelioid hemangioma, or at the high-grade end of the spectrum, between an angiosarcoma and a malignant epithelioid hemangioendothelioma. Within this differential diagnosis both clinico-radiological features (ie, size and multifocality) and immunohistochemical markers (ie, expression of endothelial markers) are often similar and cannot distinguish between benign and malignant vascular lesions. Molecular ancillary tests have long been needed for a more objective diagnosis and classification of malignant vascular tumors, particularly within the epithelioid phenotype. As significant advances have been recently made in understanding the genetic signatures of vascular tumors, this review will take the opportunity to provide a detailed update on these findings. Specifically, this article will focus on the following aspects: (1) pathological and molecular features of epithelioid hemangioendothelioma, including the more common WWTR1–CAMTA1 fusion, as well as the recently described YAP1–TFE3 fusion, identified in a morphological variant of epithelioid hemangioendothelioma; (2) discuss the heterogeneity of angiosarcoma clinical, morphological and genetic spectrum, with particular emphasis of MYC and FLT4 gene amplification in radiation-induced angiosarcoma; and (3) provide a practical guide in the differential diagnosis of epithelioid vascular tumors using molecular testing.

Young Male with Rare Tumor of Liver

Purpose: A 38-year-old Asian male presented with 4 months' history of non-productive cough, severe back pain, loss of appetite, and weight loss. Physical examination revealed non-tender hepatomegaly and mild spinal tenderness at lower back. Laboratory studies included the following: normal complete blood count, aspartate aminotransferase 58 IU/L (normal 10-40), alanine aminotransferase 136 IU/L (normal 0-40), alkaline phosphatase 211 IU/L (normal 53-128), gamma-glutamyl transpeptidase 167 IU/L (normal 0-50), albumin 3.5 mg/dl, bilirubin 0.6mg/dl, normal alpha-fetoprotein, CA 19-9, and carcinoembrinogen antigen. Hepatitis A, B, and C serology was negative. Computerized tomography with contrast enhancement of the chest and abdomen revealed bilateral reticular infiltrates in the lung and 4.5-cm hypoechoic mass in the right lobe of the liver with several round hepatic calcifications. MRI of the spine showed abnormal signal from T8 to L2, suspicious for metastasis. CT-guided liver biopsy was done, which revealed presence of patchy areas of perivascular, large, atypical epitheloid and spindle cells in the pool of chondromyxoid stroma, with mild-to-moderate nuclear atypia and hyperchromasia. Immunohistochemistry displayed reactivity to cytokeratin's AE1/AE3, S-100, and vimentin, suggesting epithelioid hemangioendothelioma (EHE). Chemo-radiation therapy was contemplated, but due to complications secondary to chemotherapy, the patient expired. EHE is a rare, well-differentiated endothelial tumor representing 1% of all vascular tumors. Primary malignant EHE of the liver is a rare tumor with an incidence of less than 0.1 per 100,000, and arise in absence of any underlying chronic liver disease. It is more frequent in males (3:1) during the second and third decades of life. Liver involvement can present as right-upper quadrant pain or hepatomegaly, with laboratory data usually revealing elevated alkaline phosphatase. Computed tomography findings suggestive of EHE include presence of a large mass located mainly in periphery, with peripheral enhancement. The definitive diagnosis requires histopathological and immunohistochemical evidence of endothelial differentiation. Treatment is usually surgical resection or liver transplant. However, it can be associated with early hematogenous metastasis, as happened in our patient. In multicentric or metastatic lesions, adjuvant chemoradiation therapy can be given. Prognosis is variable; however, involvement of liver is a poor prognostic sign. Although EHE is classified in WHO classification as low-grade to borderline malignant tumor, it has variable malignant potential and can present with metastasis at initial presentation.

Computed Tomography and Magnetic Resonance Imaging Findings of a Malignant Hepatic Epithelioid Hemangioendothelioma: A Rare Case of Solitary Small Nodular Form.

We report the case of a rare solitary small nodular form of malignant hepatic epithelioid hemangioendothelioma in a patient followed by computed tomography and gadoxetic acid (Gd-EOB-DTPA)–enhanced magnetic resonance imaging with histological analysis. This case showed early peripheral septal and nodular enhancement and delayed centripetal enhancing pattern with capsular retraction, mimicking peripheral cholangiocarcinoma, inflammatory pseudotumor, or metastases. The histological and immunohistochemical findings were diagnostic of a malignant hepatic epithelioid hemangioendothelioma.

Malignant Epithelioid Hemangioendothelioma: A Case Report

Malignant epithelioid hemangioendothelioma (EH) is a rare tumor of vascular origin. We report a case of a woman who was found to have multiple hepatic masses in the right lobe of the liver on radiologic investigations, initially misdiagnosed as a metastatic carcinoma. The diagnosis of EH was made on histopathological study and confirmed by immunohistochemistry, which showed diffuse response for CD34 marker and no response to tissue CEA, HMB-45 or S-100 protein. Partial hepatectomy was made with good results.

Rare vascular neoplasm as a reason for hypertension: case report

A 47-year-old caucasian female was referred to our ED with spontaneously Epistaxis (Nosebleed) in August 2012. The initial BP was 200 mm Hg. After using Nitrospray this was 158mmHg. She has no significant medical problems other than being allergic to penicillin and ambroxol. Physical exam (Vital signs, neuro, cardiovascular, Respiratory, abdominal exam) were in norm. Carotid Doppler, Chest X-ray, ECG, Echocardiogram were without any pathological changes. Lab studies, which included serum aminotranspherases, alkalaine phosphatase, bilirubin (total and direct), BUN and creatinine, were all in the normal range. Only INR was 0.89, and AFP was 6.8klU/l (Ref. <5.8), otherwise, patient history and physical examination did not contribute to a conclusive working diagnosis. Ultrasonography reported about multiple hypoechoic masses with a hypoechoic halo or rim and with diffusely heterogeneous regions. The lesion seemed to be hepatic in origin. Intra- and extrahepatic billiary ducts and the portal vein were normal in diameter. The gallbladder showed normal wall thickness without stone, sludge or space-occupying lesions. Kidneys, ureters, and the bladder were normal in ultrasonography. The patient underwent computed tomography (CT) scanning of the abdomen – this reported about disseminated metastatic disease without primary tumour. The magnetic resonance imaging (MRI Angio) shows us a lot of cystic lesions in the liver and kidney. So we were discussing about a metastatic disease or even a porphyria. To determine the characterisation of focal liver lesions we decided us for application of contrast-enhanced ultrasound. CEUS correctly identified malignant liver lesions unknown origin. So transabdominal punkture was done. Histologically, the tumors were comprised of spindel and epithelioid cells with lightly fatty liver cells. The stroma was fibrous, with myxohyaline areas. Immunohistochemically, they were positive for at least one endothelial marker (CD34, and CD31). Proliferation with Ki67 was approximately 1%. Diagnosis: Epithelioid hemangioendothelioma of the liver. Malignant epithelioid hemangioendothelioma is a rare hepatic tumor of vascular origin, which occurs at sites such as soft tissues, liver, or lung, and has a highly unpredictable malignant potential. It is an intermediate entity between well-differentiated hemangioma and angiosarcoma and most commonly found in young to middle aged women. I am presenting a case with this rare disease in which the tumor was detected fortuitously and the definitive diagnosis was based on histological evidence. Neoplastic cells expressed the CD31 or CD34. Treatment was surgical resection - like liver transplant. Conservative treatment for this disease is not recommended. Our cases highlight the importance of a ultrasonography for detecting liver lesions and value of a histological diagnosis to avoid it being mistaken for another entity. This Patient was sent for liver transplantation.

Malignant hepatic epithelioid hemangioendothelioma with high‐output heart failure: Successful management of heart failure with transcatheter arterial chemoembolization

A 73-year-old woman was admitted to hospital because of progressive dyspnea on exertion. Computed tomography revealed a large hepatic tumor, which was proved to be a hepatic epithelioid hemangioendothelioma (EHE). Echocardiography demonstrated high cardiac output, for which the tumor was considered to be the leading cause. A transcatheter arterial chemoembolization (TACE) was performed sequentially at 1-month intervals to reduce the size of the hepatic tumor, and this temporarily improved the patient's cardiac condition and quality of life. In this case, we successfully used TACE in the treatment of hepatic EHE with high-output heart failure. TACE is a reasonable choice of treatment both for managing malignant hepatic tumors and resolving low systemic vascular resistance by embolization of the abnormal neoangiogenic vessels. Nevertheless, clinicians should be aware of the potential adverse effect of hepatic decompensation induced by TACE, especially when the tumor involvement is widespread and poorly preserved hepatic function is encountered.

Malignant epithelioid hemangioendothelioma progressing after chemotherapy and Interferon treatment: A case presentation and a brief review of the literature

Epithelioid hemangioendothelioma is a rare, low-grade malignant vascular tumour. It is frequently seen in the liver, but can occur in the lungs, bones, and other soft tissues. Although survival time might be reasonable in cases that can undergo liver transplantation, there is no consensus on the treatment of metastatic patients. We report a 24-year-old female patient with rapidly progressing malignant epithelioid hemangioendothelioma that presented with acute abdominal distension. The patient was refractory to anthracycline and Interferon treatment and died 6.5 months after the diagnosis.