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Kaposiform hemangioendothelioma

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
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
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
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
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
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)
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
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)
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
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
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
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
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
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
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
Verónica Alonso Arroyo, Juan Carlos Lopez-Gutierrez, Rox Martinez, Israel Fernandez-Pineda
Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor that does not metastasize. We report two unique cases of KHE involving the right hemithorax and the upper ipsilateral extremity. Kasabach-Merrit phenomenon and osteolytic lesions in the scapula were observed in both cases.
April 6, 2017: Pediatric Dermatology
Elena Sobrino-Fernández, Minia Campos-Domínguez, Rebeca Gregorio-Hernández, Jorge Huerta-Aragonés, Cristina Beléndez-Bieler, Ángel Lancharro-Zapata, María Luisa Franco-Fernández, Belén Bernardo-Atienza, Manuel Sánchez-Luna
We describe the case of a 33-week preterm infant who developed nonimmune hydrops fetalis secondary to a kaposiform hemangioendothelioma (KHE). The tumor was successfully treated with vincristine, prednisone, ticlopidine, and aspirin. KHE can be an unusual cause of hydrops fetalis; in such cases, diagnosis can be challenging since generalized edema can obscure KHE.
March 20, 2017: Pediatric Dermatology
Faik Sarıalioğlu, Nalan Yazıcı, Ayşe Erbay, Fatih Boyvat, Şenay Demir, Figen Özçay, Nihal Uslu
Propranolol was first used in 2008 to treat hemangioma; its efficacy and safety have since changed the classical treatment indications. Infantile hepatic hemangioma presents as a spectrum of clinical conditions varying from simple asymptomatic lesions to lethal complications. Tufted hemangioma and Kaposiform hemangioendothelioma are congenital vascular tumors that lead to Kasabach-Merritt syndrome. Hemangiomas, like pure arteriovenous malformations, can cause hyperdynamic heart failure, and diffuse nodular-type hemangiomas can present with hypothyroidism...
March 2017: Experimental and Clinical Transplantation
Z Y Ke, S J Yang
Objective: To analyze the clinical and pathological features of Kaposiform hemangioendothelioma (KHE), and to investigate the role of master transcriptional factor Prox-1 in the regulation of lymphatic differentiation. Methods: Nine cases of KHE (during the period from October 2009 to June 2016) were collected with clinical and pathological data. H&E stained section review and immunohistochemietry using the Dako EnVision method were performed. Results: There were 6 female and 3 male patients with age ranging from 2 months to 8 years (median 3 years and 4 months)...
March 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Sho Egashira, Masatoshi Jinnin, Miho Harada, Shinichi Masuguchi, Satoshi Fukushima, Hironobu Ihn
BACKGROUND: Kaposiform hemangioendothelioma is a rare, intermediate, malignant tumor. The tumor's etiology remains unknown and there are no specific treatments. OBJECTIVE: In this study, we performed exome sequencing using DNA from a Kaposiform hemangioendothelioma patient, and found putative candidates for the responsible mutations. METHOD: The genomic DNA for exome sequencing was obtained from the tumor tissue and matched normal tissue from the same individual...
November 2016: Anais Brasileiros de Dermatologia
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