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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
Pei-An Hu, Zheng-Rong Zhou
PURPOSES: Kaposiform hemangioendothelioma (KHE) is a unique locally aggressive vascular tumor with poor prognosis. The aim of this study is to assess the clinical and imaging features of KHE, and to compare the differences between solitary and diffusive infiltrative subtype further. METHODS: The clinical and radiological findings of a cohort of 25 cases with histologically proven KHE, between June 2011 and June 2016, were reviewed retrospectively. Seven solitary and 18 diffusive infiltrative subtypes KHE were included...
March 14, 2018: British Journal of Radiology
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
David Gerardus Pieter van IJzendoorn, Stefan Sleijfer, Hans Gelderblom, Ferry A L M Eskens, Geert Jlh van Leenders, Karoly Szuhai, Judith V M G Bovee
PURPOSE: Pseudomyogenic hemangioendothelioma (PHE) is an extremely rare locally aggressive neoplasm with endothelial differentiation, which often presents with multiple lesions. These tumors have characteristic SERPINE1-FOSB fusions. We report a 17 years old patient with advanced unresectable PHE with a durable complete remission to the multi-tyrosine kinase inhibitor telatinib. The aim of this study was to generate an in vitro model for PHE, to study the functional consequences of SERPINE1-FOSB in endothelial cells, and its interaction with telatinib, to biologically substantiate the complete response to telatinib...
March 6, 2018: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
María José Moyano-Rodríguez, Florencio Quero-Valenzuela
No abstract text is available yet for this article.
March 1, 2018: Archivos de Bronconeumología
Eugenio Rimondi, Andreas F Mavrogenis, Costantino Errani, Teresa Calabrò, Alberto Bazzocchi, Giancarlo Facchini, Salvatore Donatiello, Paolo Spinnato, Daniel Vanel, Ugo Albisinni, Patrizia Pelotti
OBJECTIVE: To describe the clinical and ultrasonography (US) findings of soft tissue hemangiomas, and to compare with the results of histologic diagnosis after US-guided biopsy. METHOD AND MATERIALS: We retrospectively studied the files of 97 patients (48 female, 49 male; mean age, 34 years; range 4-84 years) with soft tissue hemangiomas diagnosed from 2004 to 2011. Mean follow-up was 9 years (range 7-13 years). Clinical presentation included intermittent mild pain associated with a soft tissue swelling/palpable mass in all patients, chronic pain and increased local heat in 29 patients, local swelling and decreased range of motion of the adjacent joint in 45 patients, and all the above symptoms in 23 patients...
February 23, 2018: La Radiologia Medica
Yuxin Wang, Huan Han, Yiqi Du
A 27-year-old man referred to our department with a 1-month history of intermittent melena and dizziness. Gastroscopy and colonoscopy did not identify the site of bleeding. Laboratory data showed hemoglobin 6.6g/dL. Video capsule endoscopy (CE, Anhon,China) and antegrade double-ballon enteroscopy (DBE, EN450P5, Fujinon, Japan) were carried out and a space occupying lesion in the proximal jejunum was identified (Fig.1). Ink tattooing via DBE was performed to mark the site of the tumor.Then the patient underwent jejunectomy...
February 17, 2018: Digestive Endoscopy: Official Journal of the Japan Gastroenterological Endoscopy Society
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
Prasenjit Das, Pragya Sharma, Tripti Nakra, Shouriyo Ghosh, Rajni Yadav, Brijnandan Gupta, Gaurav Khanna, K S Madhusudhan, Rajesh Panwar, M K Anoop, Ragini Kilambi, Anand N Singh, Nihar R Dash, Sujoy Pal, Siddhartha Datta Gupta
CONTEXT: Cysts arising from the hepatobiliary tree are a group of heterogeneous lesions with regard to pathogenesis, clinical presentation, and radiological finding. They can be intrahepatic or extrahepatic, developmental, secondary to infective/inflammatory etiologies, as well as neoplastic. This study was conducted to determine the spectrum of hepatobiliary cysts in surgically intervened cases, with regard to their prevalence, histological spectrum, and clinicoradiological correlation, wherever possible...
October 2017: Indian Journal of Pathology & Microbiology
Sachi Patel, Sonia Kamath, Nick M Shillingford, Chadi Zeinati, Vernon Tolo, Minnelly Luu
CASE: We report a case of kaposiform hemangioendothelioma (KHE) of the scapula in a 2-year-old boy with motor and sensory abnormalities of the left upper extremity, suggesting brachial plexus involvement. The locally invasive nature prevented resection; sirolimus therapy resulted in improvement of the motor and sensory impairment, as well as decreased tumor size on imaging. CONCLUSION: Osseous infiltration of KHE is known to occur, but its primary presentation in bone without skin involvement is rare and diagnostically challenging...
October 2017: JBJS Case Connector
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
X N Fu, L B Liu, X L Liu, P J Zong
No abstract text is available yet for this article.
December 7, 2017: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
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