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Hepatic epithelioid hemangioendothelioma

Fabian Ganss, Rainer Theurer, Niels Zorger
No abstract text is available yet for this article.
February 7, 2018: RöFo: Fortschritte Auf Dem Gebiete der Röntgenstrahlen und der Nuklearmedizin
Lynette L Studer, Dale M Selby
Epithelioid hemangioendothelioma is a rare vascular tumor, composed of epithelioid and histiocytoid vascular endothelial cells in myxoid or fibrotic stroma, which can arise in multiple locations throughout the body. In the liver, this neoplasm usually presents on imaging as an incidental finding of multifocal, heterogeneously enhancing nodules in both lobes or presents clinically with nonspecific abdominal symptoms. Histologically, the tumor has been mistaken for metastatic carcinoma, angiosarcoma, hepatocellular carcinoma, and cholangiocarcinoma...
February 2018: Archives of Pathology & Laboratory Medicine
Sarah Walcott-Sapp, Ephraim Tang, Sanjay Kakar, Jian Shen, Paul Hansen
Hepatic small vessel neoplasm (HSVN) is a recently described vascular neoplasm of the adult liver. The neoplastic cells are positive for markers of vascular lineage (CD31, CD34, FLI-1). The distinctive morphology and infiltrative borders separate HSVN from benign vascular tumors such as cavernous hemangioma, while lack of atypical morphologic features, low to absent mitotic activity and low proliferation index distinguish it from malignant vascular tumors such as epithelioid hemangioendothelioma and angiosarcoma...
January 20, 2018: Human Pathology
Ioannis T Konstantinidis, Carolijn Nota, Zeljka Jutric, Philip Ituarte, Warren Chow, Peiguo Chu, Gagandeep Singh, Susanne G Warner, Laleh G Melstrom, Yuman Fong
BACKGROUND AND OBJECTIVES: Primary liver sarcomas (PLS) are rare. Published series are limited by small numbers of patients. METHODS: We reviewed the National Cancer Database (2004-2014) for patients who underwent surgical resection of PLS. RESULTS: Of 237 patients identified, the majority were female (60.8%), with median age of 52 years. Histologies were: epithelioid hemangioendothelioma (n = 67), angiosarcoma (n = 64), leiomyosarcoma (n = 33), embryonal rhabdomyosarcoma (n = 31), carcinosarcoma (n = 16), giant cell sarcoma (n = 14), spindle cell sarcoma (n = 12)...
January 22, 2018: Journal of Surgical Oncology
Eric C Ehman, Michael S Torbenson, Michael L Wells, Brian T Welch, Scott M Thompson, Ishan Garg, Sudhakar K Venkatesh
A number of benign and malignant neoplasms may arise from the vascular elements within the liver parenchyma. Lesions discussed in this article include angiosarcoma, epithelioid hemangioendothelioma, solitary fibrous tumor (hemangiopericytoma), infantile, and cavernous hemangiomas. Despite a common theme of vascular origin, the pathologic and imaging appearance of these entities can be heterogeneous. Angiosarcomas are bizarrely enhancing, highly aggressive tumors, which often present with metastatic disease...
November 20, 2017: Abdominal Radiology
Daichi Nakamura, Yasushi Adachi, Yasuaki Nakashima, Noriko Sakaida, Hiroyuki Narahara, Ming Shi, Michihiko Tsubono, Susumu Ikehara
Epithelioid hemangioendothelioma (EHAE) is a vascular tumor which, due to its rarity, is often misdiagnosed as other hepatic tumors based on radiological characteristics. We herein report a case of EHAE in the liver and the mesentery of the small intestine. A 64-year-old asymptomatic woman was admitted to the hospital due to a hepatic tumor identified using computed tomography (CT). An enhanced CT scan revealed multiple tumors in the liver and a tumor in the mesentery. One of the hepatic tumors and the mesenteric tumor were resected and histologically examined...
November 2017: Molecular and Clinical Oncology
Z B Wang, X J An, J F Deng, J H Liu, H Y Shi
Objective: To investigate the expression of ERG, Fli-1, CD34, CD31 and factor Ⅷ-related antigen(FⅧRAg) in hepatic malignant vascular tumors. Methods: A retrospective analysis was conducted on 63 cases of primary hepatic malignant vascular tumors and 31 cases of hepatic other malignant spindle cell tumors collected during January 1986 to January 2014. EnVision method was used to detect the expression of ERG, Fli-1, CD34, CD31, FⅧRAg. Results: Sixty-three cases of malignant vascular tumors, including 24 cases of angiosarcoma, 38 cases of epithelioid hemangioendothelioma and 1 case of hepatic Kaposi's sarcoma...
November 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Lauren F Alexander, Peter Harri, Brent Little, Courtney C Moreno, Pardeep K Mittal
Primary hepatic malignancies are less common than metastatic diseases, but a recognition of these lesions is important for diagnosis and treatment planning. Magnetic resonance imaging (MRI) provides the most imaging information to diagnose lesions noninvasively and to narrow differential diagnoses. This paper reviews the imaging findings of chronic liver disease and primary hepatic malignancies, including hepatocellular carcinoma (HCC), intrahepatic cholangiocarcinoma (CCA), epithelioid hemangioendothelioma, hepatic angiosarcoma, and primary hepatic lymphoma...
August 4, 2017: Curēus
Jung Wook Seo, Seong Hyun Kim, Ah Yeong Kim, Woo Kyoung Jeong, Ji Young Woo, Won Jeong Park
OBJECTIVES: To assess the value of gadoxetic acid-enhanced and diffusion-weighted (DW) magnetic resonance (MR) imaging for differentiating primary hepatic angiosarcomas from hemangiomatosis and epithelioid hemangioendotheliomas (EHEs). METHODS: We reviewed MR images of seven patients with pathologically determined hepatic angiosarcomas, 11 patients with hemangiomatosis, and five patients with EHEs. Two radiologists assessed morphologic features, signal intensity (SI), enhancement patterns, and the presence of diffusion restriction by consensus and compared between angiosarcoma vs hemangiomatosis and angiosarcoma vs EHEs...
November 2017: Japanese Journal of Radiology
Rajesh Thampy, Khaled M Elsayes, Christine O Menias, Perry J Pickhardt, Hyunseon C Kang, Sandeep P Deshmukh, Kareem Ahmed, Brinda Rao Korivi
Tumours arising from mesenchymal tissue components such as vascular, fibrous and adipose tissue can manifest in the liver. Although histopathology is often necessary for definitive diagnosis, many of these lesions exhibit characteristic imaging features. The radiologist plays an important role in suggesting the diagnosis, which can direct appropriate immunohistochemical staining at histology. The aim of this review is to present clinical and imaging findings of a spectrum of mesenchymal liver tumours such as haemangioma, epithelioid haemangioendothelioma, lipoma, PEComa, angiosarcoma, inflammatory myofibroblastic tumour, solitary fibrous tumour, leiomyoma, leiomyosarcoma, Kaposi sarcoma, mesenchymal hamartoma, undifferentiated embryonal sarcoma, rhabdomyosarcoma and hepatic metastases...
November 2017: British Journal of Radiology
Wen-Wu Ling, Yan Luo, Ling Lin, Lin Ma, Ting-Ting Qiu, Lu-Lu Yang, Qiang Lu
OBJECTIVE: To investigate the ultrasonographic features of hepatic epithelioid hemangioendothelioma (HEHE) on B-mode and contrast-enhanced ultrasound (CEUS). METHODS: From January 2012 to September 2016, 17 patients with surgery or biopsy confirmed HEHE were retrospectively analyzed for their clinical history, ultrasonographic data of B-mode ultrasound and CEUS characteristics. RESULTS: The mean age of the 17 patients was (42.88±13.95) yr...
July 2017: Sichuan da Xue Xue Bao. Yi Xue Ban, Journal of Sichuan University. Medical Science Edition
Shamim Ahmed Shamim, Sarthak Tripathy, Anirban Mukherjee, Chandrasekhar Bal, Shambo Guha Roy
Hepatic epithelioid hemangioendothelioma is a rare variant of mesenchymal tumor. Surgical resection or partial hepatectomy is the treatment of choice in the case of localized disease. However, in metastatic cases, chemotherapeutic drugs targeting the tyrosine kinase are being used. We hereby present 18-F-fludeoxyglucose positron emission tomography-computed tomography findings in a case of a 35-year old woman with metastatic HEHE showing significant response to Sorafenib therapy after 6 months.
July 2017: Indian Journal of Nuclear Medicine: IJNM: the Official Journal of the Society of Nuclear Medicine, India
Vladislav Treska, Ondrej Daum, Martin Svajdler, Vaclav Liska, Jiri Ferda, Jan Baxa
BACKGROUND/AIM: Hepatic epithelioid haemangio-endothelioma (HEHE) is a very rare malignant tumor of vascular origin and uncertain biological behaviour that is difficult to diagnose using preoperative radiology diagnostic techniques. PATIENTS AND METHODS: The authors present here two patients with HEHE of different extent. The first patient had a generalised form of disease, with involvement of the liver, lungs and bones and was treated with a combination of bevacizumab and capecitabine...
July 2017: In Vivo
Mehdi Afrit, Meher Nasri, Soumaya Labidi, Nesrine Mejri, Houda El Benna, Hamouda Boussen
A new case of epithelioid hemangioendothelioma is reported to have occurred to a 67-year-old patient who consulted for right-sided chest pain. The work-up showed multiple right pulmonary lesions associated with bilateral moderate pleural effusion and left-sided pleural thickening and three hypodense nodules in the right lobe of the liver, peritoneal thickening, ascites, and multiple vertebral lytic lesions. The diagnosis of an epithelioid hemangioendothelioma was concluded through a histological examination of a computed tomography scan guided biopsy of the liver...
May 2017: Cancer Biology & Medicine
Fang-Ying Kuo, Hsuan-Ying Huang, Chao-Long Chen, Hock-Liew Eng, Chao-Cheng Huang
A recurrent YAP1-TFE3 gene fusion has been identified in WWTR1-CAMTA1-negative epithelioid hemangioendotheliomas arising in soft tissue, bone, and lung, but not in liver. We present the first case of TFE3-rearranged hepatic epithelioid hemangioendothelioma in a 39-year-old Taiwanese woman. Computed tomography scan revealed multifocal, ill-defined nodules involving both hepatic lobes. She then underwent deceased donor liver transplantation. Histologically, the tumors in the liver explant showed a biphasic growth pattern...
September 2017: APMIS: Acta Pathologica, Microbiologica, et Immunologica Scandinavica
Asahiro Morishita, Hisakazu Iwama, Hirohito Yoneyama, Teppei Sakamoto, Koji Fujita, Takako Nomura, Joji Tani, Hisaaki Miyoshi, Shintaro Fujihara, Noriko Nishiyama, Hideki Kobara, Hirohito Mori, Naoki Yamamoto, Keiichi Okano, Yasuyuki Suzuki, Emi Ibuki, Reiji Haba, Takashi Himoto, Tsutomu Masaki
A 72-year-old female was referred for further evaluation of epigastralgia. Abdominal contrast computed tomography revealed numerous tumors in the two lobes of the liver. Liver biopsy and immunohistochemical staining revealed that the tumor cells were positive for factor VIII-associated antigen, platelet endothelial cell adhesion molecule 1 and human hematopoietic progenitor cell antigen, concordant with a diagnosis of hepatic epithelioid hemangioendothelioma (HEH). To elucidate the etiology of HEH, particularly the microRNA (miRNA) profiles, tissue samples obtained from normal and tumor tissues were analyzed using a miRNA array system...
March 2017: Oncology Letters
Jeong Hyun Lee, Woo Kyoung Jeong, Young Kon Kim, Won Jae Lee, Sang Yun Ha, Kyoung Won Kim, Jihun Kim
PURPOSE: To examine the characteristic features of hepatic epithelioid hemangioendothelioma (HEH) on magnetic resonance imaging (MRI) using Gd-EOB-DTPA. MATERIAL AND METHODS: Twelve patients (mean age, 50 years; male:female = 6:6) who were pathologically confirmed to have HEH in two tertiary institutions were retrospectively investigated. For qualitative analysis, the MRI features of HEH including core pattern were characterized, and lesions were divided into core and non-core groups...
September 2017: Abdominal Radiology
Hanchanale Pavan, Joy Varghese, Kavya Harika, Mukul Vij, Venkataraman Jayanthi, Mohamed Rela
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...
March 2017: Journal of Clinical and Experimental Hepatology
Nihan Haberal Reyhan
Liver transplant is now an acceptable and effective treatment for specific nonhepatocellular malignancies. Worldwide, hilar cholangiocarcinoma accounts for 3% of all primary gastrointestinal malignancies and for 10% of primary hepatobiliary malignancies. For patients who have early-stage, unresectable cholangiocarcinoma, liver transplant preceded by neoadjuvant radiotherapy can result in tumor-free margins, accomplish a radical resection, and treat the underlying primary sclerosing cholangitis when present...
March 2017: Experimental and Clinical Transplantation
Quirino Lai, Estelle Feys, Vincent Karam, Rene Adam, Jurgen Klempnauer, Martin Oliverius, Vincenzo Mazzaferro, Andreas Pascher, Piotr Remiszewski, Helena Isoniemi, Jacques Pirenne, Aksel Foss, Bo G Ericzon, Sasa Markovic, Jan P Lerut
BACKGROUND: Hepatic epithelioid hemangioendothelioma (HEHE) is a rare vascular tumor which has an intermediate aggressive behavior. Although the value of liver transplantation (LT) is well established, its place in the management of HEHE is still unclear. The aim of this study is to confirm, based on a very large patient cohort, the value of LT in the management of HEHE and to identify risk factors for post-LT recurrence. METHODS: The outcome of 149 transplant recipients with HEHE recorded in the European Liver Transplant Registry during the period November 1984 to May 2014 was analyzed...
March 2017: Transplantation
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