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Hemangioendothelioma

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https://www.readbyqxmd.com/read/28906349/intestinal-obstruction-due-to-kaposiform-hemangioendothelioma-in-a-1-month-old-infant-a-case-report
#1
Joong Kee Youn, Shin-Hoo Park, Ji-Won Han, Chaeyoun Oh, Hyun-Young Kim, Sung-Eun Jung
RATIONALE: Kaposiform hemangioendothelioma (KHE) is an aggressive vascular tumor, mainly occurring in infants and young children and previously reported cases were mainly cutaneous or visceral form. Intestinal kaposiform hemangioma was first reported in 2012. Intestinal type KHE showed better prognosis if the lesion was limited in the gastrointestinal tract and coagulopathy was not accompanied. Since the number of reported cases is small, further study for treatment options and prognosis need to be done...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28898344/-lung-epithelioid-hemangioendothelioma-report-of-one-case
#2
Manuel Quiroz, Álvaro Undurraga, Rafael Moya, Cristina Fernández, Katiuska Bezares, Virginia Linacre
Epithelioid hemangioendothelioma is a multifocal tumor that rarely metastasizes. It is difficult to diagnose, most often it is an incidental finding in young asymptomatic women. The radiologic pattern is heterogeneous. Histologic confirmation of Weibel-Palade bodies or immunohistochemistry based on specific tumor markers such as factor VIII and CD34 are the most important finding to confirm the diagnosis. We report a 21 years old woman Presenting with cough and dyspnea. A chest X ray was suggestive of tuberculosis...
May 2017: Revista Médica de Chile
https://www.readbyqxmd.com/read/28884037/new-insights-about-pulmonary-epithelioid-hemangioendothelioma-review-of-the-literature-and-two-case-reports
#3
Romeu Duarte Mesquita, Marta Sousa, Carmen Trinidad, Eugénia Pinto, Iosu Antón Badiola
Pulmonary epithelioid hemangioendothelioma (PEH) is a rare neoplasm of vascular origin. There are three different major imaging patterns identified in thoracic manifestation of epithelioid hemangioendothelioma: (1) multiple pulmonary nodules; (2) multiple pulmonary reticulonodular opacities; and (3) diffuse infiltrative pleural thickening. Radiographically, presence of bilateral multiple nodules is the most common pattern of presentation. The diagnosis is made on the basis of histopathological findings and confirmed by positive immunohistochemistry staining...
2017: Case Reports in Radiology
https://www.readbyqxmd.com/read/28881518/-refractory-kaposiform-hemangioendothelioma-with-kasabach-merritt-syndrome-clinical-analysis-of-10-cases
#4
G L Zhang, Y Gao, Y Liu, F Gu, W Su, Q Qin, J Y Chen, H H Zhang, J Yang, X Y Liu
Objective: To analyze the clinical value of sirolimus plus prednisone for the treatment of the refractory kaposiform hemangioendothelioma(RKHE) and Kasabach-Merritt syndrome(KMS). Method: Clinical retrospective analysis was carried out for 10 patients recruited in Children's Hospital Affiliated to Capital Institute of Paediatrics from January 2014 to January 2017 who were non responders to or relapsers after the treatment of propranolol, prednisone, pingyangmycin and lauromacrogol(5 cases RKHE, 5 cases RKHE plus KMS, age ranged from 6 days to 9 years); patients were treated with sirolimus at the dosage of 0...
September 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28879422/differentiating-primary-hepatic-angiosarcomas-from-hemangiomatosis-and-epithelioid-hemangioendotheliomas-using-gadoxetic-acid-enhanced-and-diffusion-weighted-mr-imaging
#5
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...
September 6, 2017: Japanese Journal of Radiology
https://www.readbyqxmd.com/read/28872546/68ga-dota-tate-pet-ct-for-molecular-imaging-of-somatostatin-receptor-expression-in-metastasizing-epithelioid-hemangioendothelioma-comparison-with-18f-fdg
#6
Thorsten Derlin, Katja Hueper, Bisharah Soudah
Epithelioid hemangioendothelioma (EHE) is a rare tumor originating from vascular endothelial or pre-endothelial cells. It has the potential to metastasize. We present the case of a 51-year-old woman with metastasizing EHE who underwent both F-FDG and Ga-DOTA-TATE PET/CT for staging of disease and for evaluation of targeted radionuclide therapy potential. On Ga-DOTA-TATE PET scans, intense tracer accumulation was observed in metastases. Besides the increased glucose metabolism in EHE, this case highlights the potential of Ga-DOTA-TATE PET/CT for restaging of EHE and the option of targeted radionuclide therapy in this entity...
September 1, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28855107/multicentric-visceral-epithelioid-hemangioendothelioma-with-extremity-dermal-deposits-unusual-late-recurrence-on-the-nasal-bridge-and-tfe3-gene-rearrangement
#7
Khin Thway, Thomas Mentzel, Conal M Perrett, Eduardo Calonje
Epithelioid hemangioendothelioma (EHE) is a malignant neoplasm with vascular differentiation that most frequently occurs within soft tissues, bone, lung and liver. It is histologically typified by epithelioid or spindle cells present singly or in cords or clusters, many with cytoplasmic vacuoles that can contain intraluminal erythrocytes (in keeping with primitive vascular differentiation), within myxohyaline or sclerotic matrix. Up to 50% present with synchronous lesions as multifocal disease. The WWTR1-CAMTA1 fusion has been demonstrated in EHEs at a variety of sites and is considered to represent its genetic hallmark...
August 27, 2017: Human Pathology
https://www.readbyqxmd.com/read/28845553/survivin-a-novel-marker-and-potential-therapeutic-target-for-human-angiosarcoma
#8
Masayuki Tsuneki, Takao Kinjo, Taisuke Mori, Akihiko Yoshida, Kayo Kuyama, Aoi Ohira, Takuya Miyagi, Kenzo Takahashi, Akira Kawai, Hirokazu Chuman, Naoya Yamazaki, Mikio Masuzawa, Hirofumi Arakawa
Human angiosarcoma is a rare malignant vascular tumor associated with extremely poor clinical outcome and generally arising in the skin of head and neck. However, little is known about the molecular pathogeneses and useful immunohistochemical markers of angiosarcoma. To investigate the mechanisms of angiosarcoma progression, we collected 85 cases of human angiosarcoma specimens with clinical records and used ISO-HAS-B patient derived angiosarcoma cells. As control subjects, 54 cases of hemangioma and 34 of pyogenic granuloma were collected...
August 28, 2017: Cancer Science
https://www.readbyqxmd.com/read/28843050/sirolimus-therapy-in-the-treatment-of-pseudomyogenic-hemangioendothelioma
#9
Krisztina Mita Gabor, Zoltan Sapi, Lilla Gyorgyi Tiszlavicz, Anita Fige, Csaba Bereczki, Katalin Bartyik
Pseudomyogenic hemangioendothelioma (PMH) is a rare, mostly indolent vascular tumor. Extensive cases are treated with amputation as chemotherapy seems to be ineffective. Recently, promising results were published using mammalian target of rapamycin (mTOR) inhibitors in tumors of vascular origin. Here, we present a case of a child with advanced PMH relapsing after surgery and chemotherapy. Sirolimus achieved significant clinical improvement and stabilization of the lesions without any remarkable toxicity. This case contributes to the growing evidence regarding the efficacy of mTOR inhibitors, such as sirolimus, in multifocal PMH...
August 26, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28840142/-18-f-fdg-pet-ct-in-pleural-epithelioid-hemangioendothelioma
#10
Domenico Albano, Giovanni Bosio, Andrea Tironi, Francesco Bertagna
Pleural epithelioid hemangioendothelioma (EHE) is a rare malignancy of vascular-endothelial origin with non-specific symptoms and an unpredictable outcome. Diagnosis of this condition by imaging modalities is challenging, and no standard therapeutic approaches have been established in this regard. In this paper, we described the case of a patient with a low-grade fever, coughing and chest pain who underwent (18)F-FDG PET/CT after a positive thorax CT showing multiple bilateral calcified pulmonary nodules and extensive right-sided pleural effusion...
2017: Asia Oceania Journal of Nuclear Medicine & Biology
https://www.readbyqxmd.com/read/28838853/successful-treatment-of-a-hepatic-hemangioendothelioma-infant-presenting-with-hypothyroidism-and-tetralogy-of-fallot
#11
Akari Takai, Tomoko Iehara, Mitsuru Miyachi, Yoshiki Okumura, Tatsuji Hasegawa, Sachiko Tokuda, Kazuyuki Ikeda, Masaaki Yamagishi, Tatsuro Tajiri, Hajime Hosoi
No abstract text is available yet for this article.
August 12, 2017: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/28834832/an-epithelioid-hemangioendothelioma-of-the-head-and-neck
#12
Sertac Duzer, Abdulvahap Akyigit, Ozgen Arslan Solmaz, Oner Sakallioglu, Ahmet Kilicarslan, Cahit Polat
An epithelioid hemangioendothelioma (EHE) is a rare vascular tumor of the head and neck region, which carries a risk of recurrence and metastasis. Its etiology is still unclear. It frequently involves the lungs, liver, and bones. Rarely, it may manifest in other parts of the body. Pathologic immunohistochemical investigations are essential to make the definite diagnosis. As it is uncommon, there is no standardized protocol for the treatment of EHE. Herein, the authors report a rare patient with of an EHE localized in the head and neck region...
August 22, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28832074/programmed-death-ligand-1-pd-l1-expression-in-malignant-mesenchymal-tumors
#13
Kemal Kösemehmetoğlu, Ece Özoğul, Berrin Babaoğlu, Gaye Güler Tezel, Gökhan Gedikoğlu
OBJECTIVE: Programmed death ligand 1 (PD-L1) found on tumor cells has recently been reported to have a key role in the development and dissemination of many tumors, such as lung and breast carcinomas. In this study, we retrospectively analyzed PD-L1 expression among different types of sarcomas. MATERIAL AND METHOD: Tissue microarrays of 3-4 mm diameter were composed from paraffin blocks of 222 various sarcomas. Slides prepared from microarrays were stained for PD-L1 antibody (Cell Signaling, E1L3N®) using Leica Bond Autostainer...
2017: Türk Patoloji Dergisi
https://www.readbyqxmd.com/read/28815311/-acute-complications-of-vascular-anomalies-in-childhood
#14
REVIEW
M Fließer, A Teichler, P H Höger
Congenital and acquired vascular anomalies are common in childhood. In addition to predominantly harmless vascular skin alterations there are others which must be immediately treated due to the potentially threatening complications. As examples three anomalies and the typical complications are presented. Hemangiomas in infancy can make urgent treatment necessary because they can be complicated by obstruction or painful ulceration. Kaposiform hemangioendothelioma can lead to a life-threatening consumption coagulopathy...
August 16, 2017: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/28802504/cutaneous-malignant-vascular-neoplasms
#15
REVIEW
Wonwoo Shon, Steven D Billings
Accurate diagnosis of cutaneous malignant vascular tumors, including angiosarcoma and epithelioid hemangioendothelioma, is critical for determination of appropriate clinical management and prognosis. Although there have been significant advances in understanding genetic aspects of cutaneous vascular biology, differential diagnosis of malignant vascular tumor involving skin and superficial soft tissue is a frequent source of difficulty. This brief overview highlights the clinicopathologic features of primary and secondary cutaneous angiosarcoma and epithelioid hemangioendothelioma and also provides a short summary of newer molecular data...
September 2017: Clinics in Laboratory Medicine
https://www.readbyqxmd.com/read/28777494/clinico-radiological-features-and-next-generation-sequencing-of-pulmonary-epithelioid-hemangioendothelioma-a-case-report-and-review-of-literature
#16
Xiaoyong Mao, Zibin Liang, Fidelis Chibhabha, Wei Ou, Ning Li, Pengfei Xu, Siyu Wang
Epithelioid hemangioendothelioma is a very rare, vascular, low-grade malignant tumor found in the lungs, liver, bone, and other soft tissues. Most patients with pulmonary epithelioid hemangioendothelioma (PEH) are asymptomatic but usually present with multiple bilateral nodular lesions in the lungs. Currently, surgical lung biopsy, histology, and immunohistochemical methods are essential for diagnosis. However, there is no standard therapy for the treatment for PEH. Our paper describes the clinico-radiologic features and genomics of PEH based on next-generation sequencing (NGS) in a 43-year-old male we encountered...
August 4, 2017: Thoracic Cancer
https://www.readbyqxmd.com/read/28766950/imaging-features-of-rare-mesenychmal-liver-tumors-beyond-hemangiomas
#17
Rajesh Thampy, Khaled M Elsayes, Christine O Menias, Perry J Pickhardt, Hyunseon C Kang, Sandeep Deshmukh, Kareem Ahmed, Brinda Rao Korivi
Tumors 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 tumors such as hemangioma, epithelioid hemangioendothelioma, lipoma, PEComa, angiosarcoma, inflammatory myofibroblastic tumor, solitary fibrous tumor, leiomyoma, leiomyosarcoma, Kaposi sarcoma, mesenchymal hamartoma, undifferentiated embryonal sarcoma, rhabdomyosarcoma and hepatic metastases...
August 2, 2017: British Journal of Radiology
https://www.readbyqxmd.com/read/28765663/-highly-aggresive-pulmonary-epithelioid-hemangioendothelioma-a-case-report
#18
H Arasanz Esteban, B Hernández Marín, S Pérez Ricarte, A Lacalle Emborujo, L Pérez Ricarte, M Martínez Aguillo
Pulmonary epithelioid hemangioendothelioma is an uncommon neoplastic disease of endothelial origin, with an incidence inferior to one in a million. It usually behaves as a low-grade malignancy; nevertheless, a few cases have been described in which this tumour manifests a highly aggressive behavior, spreading throughout the organism. We report the case of a 77-year-old woman who was diagnosed with metastatic pulmonary epithelioid hemangioendothelioma with severe symptoms, requiring urgent treatment to palliate them...
July 28, 2017: Anales del Sistema Sanitario de Navarra
https://www.readbyqxmd.com/read/28762232/morphofunctional-basis-of-the-different-types-of-angiogenesis-and-formation-of-postnatal-angiogenesis-related-secondary-structures
#19
REVIEW
L Díaz-Flores, R Gutiérrez, M P García-Suárez, F J Sáez, E Gutiérrez, F Valladares, J L Carrasco, L Díaz-Flores, J F Madrid
We review the morpho-functional basis of the different types of angiogenesis and report our observations, including the formation of angiogenesis-related secondary structures. First of all, we consider the following issues: a) conceptual differences between angiogenesis and vasculogenesis, b) incidence of angiogenesis in pre- and postnatal life, c) regions of vascular tree with angiogenic capacity, d) cells (endothelial cells, pericytes, CD34+ adventitial stromal cells of the microvasculature and inflammatory cells) and extracellular matrix components involved in angiogenesis, e) events associated with angiogenesis, f) different types of angiogenesis, including sprouting and intussusceptive angiogenesis, and other angiogenic or vascularization forms arising from endothelial precursor cells (postnatal vasculogenesis), vasculogenesis mimicry, vessel co-option and piecemeal angiogenesis...
August 1, 2017: Histology and Histopathology
https://www.readbyqxmd.com/read/28761800/pancreatic-kaposiform-hemangioendothelioma-not-responding-to-sirolimus
#20
Paloma Junco Triana, Mariela Dore, Vanesa Cerezo Nuñez, Javier Gomez Jimenez, Miriam Ferrero Miguel, Mercedes González Díaz, Joan Novo Ricardo, Ane Andres, Manuel Lopez Santamaria, Juan Carlos Lopez-Gutierrez
Background  Kaposiform hemangioendothelioma (KHE) is a vascular tumor frequently associated with Kasabach-Merritt phenomenon (KMP), characterized by severe thrombocytopenia and consumptive coagulopathy. Visceral involvement in KHE is rare. In our recent experience, sirolimus has shown to be an effective treatment in cutaneous KHE, becoming indeed the treatment of choice in KMP. We report a case of pancreatic KHE associated with KMP and refractory to sirolimus. Case Report  A 4-month-old infant is referred for obstructive jaundice (10 mg/dL conjugated bilirubin) secondary to vascular pancreatic tumor...
January 2017: European Journal of Pediatric Surgery Reports
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