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Kaposiform hemangioendothelioma

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https://www.readbyqxmd.com/read/29697621/vascular-anomalies-from-a-clinicohistologic-to-a-genetic-framework
#1
Arin K Greene, Jeremy A Goss
BACKGROUND: Vascular anomalies currently are classified according to their clinical and histological characteristics. Recent advances in molecular genetics have enabled the identification of somatic mutations in most types of vascular anomalies. The purpose of this study was to collate information regarding the genetic basis of vascular anomalies. METHODS: The PubMed literature was reviewed for all citations that identified a mutation in a vascular anomaly between 1994 and 2017...
May 2018: Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/29610165/a-case-report-of-2-sirolimus-related-deaths-among-infants-with-kaposiform-hemangioendotheliomas
#2
Hanru Ying, Congzhen Qiao, Xi Yang, Xiaoxi Lin
Kaposiform hemangioendothelioma (KHE) is a rare infiltrative vascular tumor that is potentially life-threatening when presenting with Kasabach-Merritt phenomenon (KMP). KMP is clinically characterized as severe thrombocytopenia and hypofibrinogenemia and therefore is associated with a high mortality rate. There is no standard of cure for KHE currently. Potential medications, including corticosteroids, propranolol, and chemotherapy drugs such as sirolimus, are often used for alleviating KHE symptoms. Although some case reports of sirolimus treatment have shown promising results with recovered coagulant parameters, the off-target effects may cause severe problems...
April 2018: Pediatrics
https://www.readbyqxmd.com/read/29610164/-pneumocystis-jirovecii-pneumonia-during-sirolimus-therapy-for-kaposiform-hemangioendothelioma
#3
Thomas B Russell, Emily K Rinker, Claire S Dillingham, Laurence B Givner, Thomas W McLean
Sirolimus is an effective therapy for children with kaposiform hemangioendothelioma with or without the Kasabach-Merritt phenomenon. We report the case of a child with kaposiform hemangioendothelioma and the Kasabach-Merritt phenomenon who developed Pneumocystis carinii pneumonia (PCP) while on sirolimus and a prednisolone taper, after lack of adequate response to prednisolone, propranolol, and vincristine. He had a prompt positive clinical and laboratory response to sirolimus, but 4 weeks after starting it, at the age of 4 months, he developed PCP...
April 2018: Pediatrics
https://www.readbyqxmd.com/read/29561329/vascular-tumors-in-infants-case-report-and-review-of-clinical-histopathologic-and-immunohistochemical-characteristics-of-infantile-hemangioma-pyogenic-granuloma-noninvoluting-congenital-hemangioma-tufted-angioma-and-kaposiform-hemangioendothelioma
#4
Emma F Johnson, Dawn M Davis, Megha M Tollefson, Karen Fritchie, Lawrence E Gibson
Vascular tumors in infants present a diagnostic and treatment dilemma for both clinicians and pathologists. Infantile hemangioma, the most common vascular tumor in infants, can be confused for other less common vascular tumors in infants. Correct and timely diagnosis is important, as some vascular tumors can be associated with life-threatening coagulopathy. We present the cases of 5 vascular tumors that have clinical and histologic overlap: infantile hemangioma, pyogenic granuloma, noninvoluting congenital hemangioma, tufted angioma, and kaposiform hemangioendothelioma...
April 2018: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/29554025/regional-differences-in-clinical-features-of-kaposiform-hemangioendothelioma-of-the-intestinal-tract
#5
Hironori Ohshita, Koya Kawase, Daisuke Takagi, Satoshi Kondo, Shinji Saitoh
No abstract text is available yet for this article.
March 16, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29536769/clinical-and-imaging-features-of-kaposiform-hemangioendothelioma
#6
Pei-An Hu, Zheng-Rong Zhou
OBJECTIVE: 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. 7 solitary and 18 diffusive infiltrative subtypes KHE were included...
March 20, 2018: British Journal of Radiology
https://www.readbyqxmd.com/read/29391717/hemangioendothelioma-of-palate-a-case-report-with-review-of-literature
#7
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
https://www.readbyqxmd.com/read/29286963/restricted-range-of-motion-and-a-cold-upper-extremity-in-a-two-year-old-boy-kaposiform-hemangioendothelioma-of-the-bone-and-the-brachial-plexus-a-case-report
#8
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
https://www.readbyqxmd.com/read/29240034/management-of-refractory-pediatric-kaposiform-hemangioendothelioma-with-sirolimus-and-aspirin
#9
Suzanne P MacFarland, Lisa M Sullivan, Lisa J States, L Charles Bailey, Naomi J Balamuth, Richard B Womer, Timothy S Olson
Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor characterized by aggressive local invasion and a syndrome of platelet trapping known as Kasabach-Merritt phenomenon that, through deposition of platelet derived growth factors, may perpetuate the growth of the tumor. Although many cases of KHE are successfully treated with local control or low-intensity chemotherapy, some cases are often refractory even to aggressive treatment. Herein, we describe a patient with a refractory, recurrent KHE despite multiple attempts at local control and intensive chemotherapy, that ultimately was successfully treated with rationally designed and low-intensity combination therapy of sirolimus and aspirin...
December 12, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29154372/kaposiform-hemangioendothelioma-in-an-adult-with-rheumatoid-arthritis
#10
M Filotico, R Filotico
This report describes a case of kaposiform hemangioendothelioma arising in an adult man during the course of rheumatoid arthritis treated with steroids and methotrexate. The vascular proliferation began in the terminal phase of the disease, which culminated in acute renal failure and death. We discuss the possible relationship between rheumatoid arthritis, its treatment, and the onset of vascular proliferation, as well as the role of kaposiform hemangioendothelioma in aggravating the autoimmune disease and leading to its fatal outcome...
September 2017: Pathologica
https://www.readbyqxmd.com/read/29151787/infantile-hemangiomas-a-7-year-experience-of-a-single-center
#11
Mădălina Bota, Gheorghe Popa, Cristina Lucia Blag, Daniel-Corneliu Leucuta, Alexandru Tătaru
Objectives: The aim of the study was to describe the historical and clinical characteristics of hemangiomas in a series of cases of our clinic. Methods: This is a retrospective study of 36 patients with infantile hemangiomas consulted in our clinic. Results: We had 14 multiple hemangiomas, and 1 kaposiform hemangioendothelioma. Almost two-thirds involved the cephalic extremity, and 76% of the cases were treated. Pregnancy risk factors included prematurity, low-birth weight and respiratory distress syndrome...
2017: Clujul Medical (1957)
https://www.readbyqxmd.com/read/29050802/episode-of-kasabach-merritt-phenomenon-following-japanese-encephalitis-vaccination-case-report
#12
Xiaojie Yue, Xiong Zhao, Yefeng Dai, Qiang Shu
Kasabach-Merritt phenomenon (KMP) is a rare potentially life-threatening consumptive coagulopathy characterized by thrombocytopenia and hypofibrinogenemia occurring associated with the vascular tumors kaposiform hemangioendothelioma (KHE) and tufted angioma (TA). A 10-month old male infant, diagnosed with KHE on his left leg, underwent a rapid increase of the lesion and severe thrombocytopenia, one day after the first dose of inactivated Japanese encephalitis (JE) vaccination. The episode of KMP was treated successfully by steroid...
October 16, 2017: Vaccine
https://www.readbyqxmd.com/read/28906349/intestinal-obstruction-due-to-kaposiform-hemangioendothelioma-in-a-1-month-old-infant-a-case-report
#13
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/28881518/-refractory-kaposiform-hemangioendothelioma-with-kasabach-merritt-syndrome-clinical-analysis-of-10-cases
#14
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/28815311/-acute-complications-of-vascular-anomalies-in-childhood
#15
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...
October 2017: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/28761800/pancreatic-kaposiform-hemangioendothelioma-not-responding-to-sirolimus
#16
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
https://www.readbyqxmd.com/read/28579853/kasabach-merritt-phenomenon-classic-presentation-and-management-options
#17
REVIEW
Priya Mahajan, Judith Margolin, Ionela Iacobas
Kasabach-Merritt phenomenon (KMP) is a rare consumptive coagulopathy associated with specific vascular tumors, kaposiform hemangioendothelioma, and tufted angioma. Kasabach-Merritt phenomenon, characterized by profound thrombocytopenia, hypofibrinogenemia, elevated fibrin split products, and rapid tumor growth, can be life-threatening. Severe symptomatic anemia may also be present. With prompt diagnosis and management, KMP can resolve and vascular tumors have been shown to regress. This review highlights the clinical presentation, histopathology, management, and treatment of KMP associated with kaposiform hemangioendothelioma, and less frequently tufted angioma...
2017: Clinical Medicine Insights. Blood Disorders
https://www.readbyqxmd.com/read/28552538/the-utility-of-glut1-as-a-diagnostic-marker-in-cutaneous-vascular-anomalies-a-review-of-literature-and-recommendations-for-daily-practice
#18
REVIEW
Lieke J van Vugt, Carine J M van der Vleuten, Uta Flucke, Willeke A M Blokx
OBJECTIVE: To assess the utility of GLUT1 as an immunohistochemical marker in the diagnostics of cutaneous vascular anomalies. METHODS: A systematic literature search was conducted for studies on GLUT1 staining patterns in cutaneous vascular lesions. Data was grouped according to the latest ISSVA classification for vascular anomalies. RESULTS: Vascular tumors: GLUT1 staining was positive in 368/386 (95%) of infantile hemangiomas. Congenital hemangiomas (16 cases) and kaposiform hemangioendotheliomas (62 cases) were all negative for GLUT1...
June 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28486787/sirolimus-for-the-treatment-of-progressive-kaposiform-hemangioendothelioma-a-multicenter-retrospective-study
#19
MULTICENTER STUDY
Yi Ji, Siyuan Chen, Bo Xiang, Kai Li, Zhicheng Xu, Wei Yao, Guoyan Lu, Xingtao Liu, Chuncao Xia, Qi Wang, Yanan Li, Chuan Wang, Kaiying Yang, Gang Yang, Xueyang Tang, Ting Xu, Hao Wu
Kaposiform hemangioendothelioma (KHE) is an aggressive disease with high morbidity and mortality. The aim of this study was to retrospectively evaluate the efficacy and safety of sirolimus for the treatment of progressive KHE. A multicenter, retrospective cohort study was conducted in patients with progressive KHE treated with sirolimus. A total of 52 patients were analyzed. Thirty-seven (71%) patients exhibited Kasabach-Merritt phenomenon (KMP) and were significantly younger than the patients without KMP [95% confidence interval (CI), 14...
August 15, 2017: International Journal of Cancer. Journal International du Cancer
https://www.readbyqxmd.com/read/28426496/successful-management-of-pancreatic-kaposiform-hemangioendothelioma-with-sirolimus-case-report-and-literature-review
#20
Chuan Wang, Yanan Li, Bo Xiang, Fuyu Li, Siyuan Chen, Li Li, Yi Ji
No abstract text is available yet for this article.
May 2017: Pancreas
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