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Epithelioid Hemangioendothelioma

Ari Rosenberg, Mark Agulnik
Epithelioid hemangioendothelioma (EHE) is an extremely rare sarcoma, as such it can pose a clinical dilemma based solely on its rarity. Also, the spectrum of disease varies greatly between an indolent disease and aggressive disease with widespread metastases. In our clinical practice, the primary focus has been to get a handle on the aggressive nature of the disease, which will then dictate how urgently one needs to treat the patient. Pathological review with immunohistochemistry and molecular characterization is paramount...
March 15, 2018: Current Treatment Options in Oncology
Ana Ortins-Pina, Mar Llamas-Velasco, Sara Turpin, Luís Soares-de-Almeida, Paulo Filipe, Heinz Kutzner
BACKGROUND: Accurate distinction of epithelioid hemangioma (EH) from its malignant mimics is paramount but it remains challenging due to its wide morphological spectrum and lack of objective molecular markers. FOSB oncogenic activation was recently identified as a key event in endothelial proliferation. We sought to investigate the FOSB staining pattern in EH with angiolymphoid hyperplasia with eosinophilia morphology (EH-AHLE) and to evaluate its value in differential diagnosis of epithelioid vascular tumors...
March 12, 2018: Journal of Cutaneous Pathology
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
Emily Raftopoulos, Michael Royer, Mildred Warren, Jin Zhao, Walter Rush
Pseudomyogenic hemangioendothelioma (PHE; epithelioid sarcoma-like hemangioendothelioma) is a rare entity, with 129 reported cases. It is a rarely metastasizing, low-grade vascular tumor that can histologically mimic epithelioid sarcoma and often presents as multiple discontinuous nodules in the extremities. PHEs characteristically and consistently show immunoreactivity for cytokeratins and endothelial markers. We present a case in the thigh of a 65-year-old-man with a 3-month history of painful lesions in the dermis and review the published literature...
February 2, 2018: American Journal of Dermatopathology
R Heera, Latha Mary Cherian, Rupali Lav, V Ravikumar
Hemangioendothelioma commonly occurs in the superficial or deep soft tissue of the extremities, lungs, liver, bone and lymph nodes, with oral cavity being a rare location. It is usually benign but can show variable grades of malignancy. According to the histological presentation, hemangioendothelioma has been classified as epithelioid, Kaposiform, hobnail (Dabska-Retiform), epithelioid sarcoma like and composite. We present a case of ulcerated swelling of palate clinically diagnosed as pyogenic granuloma which presented a diagnostically challenging histological picture...
September 2017: Journal of Oral and Maxillofacial Pathology: JOMFP
Jasmine Haller, Marjorie Parker David, Nathan E Lee, Sara C Shalin, Jerad M Gardner
CONTEXT: - Patients with rare tumors have difficulty finding reliable information about their disease. Facebook patient support groups allow patients to educate one another. OBJECTIVE: - To investigate how these patients perceive the value of pathologists, both in Facebook groups and real-world patient care. DESIGN: - Survey links were posted in 12 Facebook patient groups: 6 with an active pathologist member (angiosarcoma, epithelioid hemangioendothelioma, epithelioid sarcoma, dermatofibrosarcoma protuberans [×2], and desmoid fibromatosis), and 6 without "active" pathologist involvement (aggressive angiomyxoma, chondrosarcoma, Ewing sarcoma, leiomyosarcoma, liposarcoma, and osteosarcoma)...
January 29, 2018: Archives of Pathology & Laboratory Medicine
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
Fukuko Yamamoto, Hironori Yamagiwa, Fuminori Iwamoto, Tsutomu Kasugai
Epithelioid hemangioendotheliomas(EHEs)most commonly occur in the liver, lung, bone, and soft tissues, but rarely in the brain. Here, we describe a case of primary intracranial epithelioid hemangioendothelioma manifested by an epileptic attack. A 53-year-old woman with no previous convulsive history presented at our hospital after experiencing a seizure. Magnetic resonance imaging revealed a mass in her right frontal lobe, with edema, which was well enhanced with gadolinium. Systemic computed tomography, on the other hand, did not show any tumor involvement in other organs...
January 2018: No Shinkei Geka. Neurological Surgery
Nicholas A Horan, Dominick J DiMaio
Pseudomyogenic hemangioendothelioma (PMHE) is a rare vascular tumor that was added to the World Health Organization classification of soft tissue tumors. These tumors have a unique clinical presentation and microscopic appearance as compared to other vascular tumors in the differential diagnosis. Unlike its microscopic mimicker epithelioid sarcoma, PMHE rarely metastasizes and long-term survival in affected patients is excellent. In this report, we present a patient with PMHE and review the current literature on clinical presentation and histologic differentiation of this unique tumor, comparing findings to its mimickers...
December 2017: Cutis; Cutaneous Medicine for the Practitioner
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
F F Cheo, K Sittampalam
Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma is a rare, low grade vascular (endothelial) neoplasm typically presenting as multicentric, superficial to deep nodules in extremities with a slight tendency of affecting young adult males. We report a case of pseudomyogenic hemangioendothelioma in a 15-year-old boy presenting initially with a 1 cm right thigh painless cutaneous lump. The lump was excised with the clinical impression of a sebaceous cyst. On microscopy, a poorly circumscribed, mild to moderately atypical spindle cell lesion in fascicular and storiform patterns with strikingly myoid-like eosinophilic cytoplasm was identified...
December 2017: Malaysian Journal of Pathology
Luying Gao, Yahong Wang, Yuxin Jiang, Xingjian Lai, Ming Wang, Jianchu Li
RATIONALE: Intravascular epithelioid hemangioendothelioma (EHE) is a rare endothelial tumor with an intermediate grade of malignancy. We present a case of one woman affected by EHE of the femoral vein. Contrast-enhanced ultrasonography played a role in diagnosing EHE and helped differentiate it from thrombosis. To our knowledge, this is the first reported contrast-enhanced ultrasonography of intravascular EHE in the imaging literature. PATIENT CONCERNS: A 46-year-old woman presented to our hospital due to pain and swelling in her right lower limb since 5 years prior to her presentation...
December 2017: Medicine (Baltimore)
Jalal A Khan, Robert G Maki, Vinod Ravi
Angiosarcoma, epithelioid hemangioendothelioma, and Kaposi sarcoma are classified according to the line of differentiation that these neoplastic cells most closely resemble: the endothelial cell. Although these malignant vascular sarcomas demonstrate immunohistochemical and ultrastructural features typical of this lineage, they vary dramatically in presentation and behavior, reflecting oncologic mechanisms unique to each. Antineoplastic therapies offer significant benefit, but because of the rarity of these cancers, novel therapies are slow to develop, and treatment options for these cancers remain limited...
January 10, 2018: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
Mariusz Łochowski, Marek Rębowski, Dorota Jesionek-Kupnicka, Józef Kozak
No abstract text is available yet for this article.
September 2017: Kardiochirurgia i Torakochirurgia Polska, Polish Journal of Cardio-Thoracic Surgery
Carlos Mayoral-Guisado, Weimar Toro-Zambrano, María López-Macías, Antonio Ruíz-Guerrero, Alejandro Rubio-Fernández, Mario Díaz-Delgado
Pseudomyogenic hemangioendothelioma, also called epithelioid sarcoma-like hemangioendothelioma, is a rare, vascular neoplasm usually with indolent behaviour. It was introduced in the latest World Health Organization (WHO) Classification of Tumours of Soft Tissue. We report a case of a 45 year-old patient presenting with a localized, palpable and slightly painful lesion in the left arm. Histologically it consisted of fascicles of spindle and epithelioid cells with ample eosinophilic cytoplasm, without nuclear pleomorphism or significant mitotic activity...
January 2017: Revista Española de Patología
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
Zhipeng Zheng, Hanying Wang, Hanliang Jiang, Enguo Chen, Jun Zhang, Xinyou Xie
RATIONALE: Pulmonary epithelioid hemangioendothelioma (P-EHE) is a rare tumor, with no established standard treatment. Overexpression of vascular endothelial growth factor receptor 2 (VEGFR-2) has been reported in some P-EHE patients. Apatinib, a new small molecule tyrosine kinase inhibitor that specifically targets VEGFR-2, has therapeutic benefits in some advanced tumors. However, its efficacy in P-EHE cases has not been reported. PATIENT CONCERNS: Herein, we presented a 44-year-old man with recurrent hemoptysis for approximately 9 years...
November 2017: Medicine (Baltimore)
M Zhao, L L Yu, Q L Tang, X L He
No abstract text is available yet for this article.
November 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
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