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Kaposiform hemangioendothelioma

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https://www.readbyqxmd.com/read/28486787/sirolimus-for-the-treatment-of-progressive-kaposiform-hemangioendothelioma-a-multicenter-retrospective-study
#1
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...
May 9, 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
#2
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
https://www.readbyqxmd.com/read/28382716/identical-presentation-of-scapular-osteolysis-in-two-patients-with-thoracic-kaposiform-hemangioendothelioma
#3
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
https://www.readbyqxmd.com/read/28318049/kaposiform-hemangioendothelioma-presenting-as-hydrops-fetalis
#4
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
https://www.readbyqxmd.com/read/28302004/a-new-perspective-for-infantile-hepatic-hemangioma-in-the-age-of-propranolol-experience-at-baskent-university
#5
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
https://www.readbyqxmd.com/read/28297758/-role-of-master-transcriptional-factor-prox-1-in-lymphatic-endothelial-differentiation-of-kaposiform-hemangioendothelioma
#6
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
https://www.readbyqxmd.com/read/28099595/exome-sequence-analysis-of-kaposiform-hemangioendothelioma-identification-of-putative-driver-mutations
#7
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
https://www.readbyqxmd.com/read/28027747/imaging-findings-of-kaposiform-hemangioendothelioma-in-children
#8
Young Jin Ryu, Young Hun Choi, Jung-Eun Cheon, Woo Sun Kim, In-One Kim, Ji Eun Park, Yu Jin Kim
PURPOSE: Kaposiform hemangioendothelioma (KHE) is a rare, aggressive vascular tumor that typically occurs during infancy or early childhood. Though several case reports have discussed the imaging findings of KHE, larger comprehensive studies are lacking. The purpose of this study was to evaluate the imaging findings of KHE in children. MATERIALS AND METHODS: A total of twelve cases of pathologically proven KHE were collected by searching our institution's pathology database for children diagnosed between January 2004 and April 2016 (6 male, 6 female; median age: 3 months; age range 7days - 18 years)...
January 2017: European Journal of Radiology
https://www.readbyqxmd.com/read/27990590/angiopoietins-as-serum-biomarkers-for-lymphatic-anomalies
#9
Timothy D Le Cras, Paula S Mobberley-Schuman, Mary Broering, Lin Fei, Cameron C Trenor, Denise M Adams
Vascular anomalies can cause significant morbidity and mortality. Advances in diagnosis will be improved if noninvasive biomarkers can be identified, as obtaining a tissue biopsy can worsen the disease and precipitate complications. The goal of this study was to identify biomarkers for vascular anomaly patients to aid diagnosis and potentially give insights into pathogenesis. Blood was collected at baseline and then 6 and 12 months after treatment with the mTOR inhibitor sirolimus. Patients groups included generalized lymphatic anomaly (GLA), kaposiform lymphangiomatosis (KLA) and kaposiform hemangioendothelioma (KHE) with or without the Kasabach-Merritt phenomenon (KMP) coagulopathy...
February 2017: Angiogenesis
https://www.readbyqxmd.com/read/27983885/multifocal-kaposiform-hemangioendothelioma-causing-massive-fetal-chylous-ascites
#10
Hyo Jung An, Eun Jung Jung, Mi Seon Kang, Hwa Jin Cho, Kyung Han Nam, Jung Mi Byun, Dae Hoon Jeong, Young Nam Kim
Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor that usually occurs in soft tissues of the extremity and rarely in the retroperitoneum. We report a unique case of isolated massive fetal ascites attributed to KHE, involving the retroperitoneum and multiple visceral organs, along with the Kasabach-Merritt phenomenon. We suspect that retroperitoneal KHE might have caused massive fetal ascites because of its high potential to invade the lymphatic vessels aggressively in the retroperitoneal space, which possibly permits intestinal lymph leakage into the peritoneal cavities...
April 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/27981218/sirolimus-for-treatment-of-kaposiform-hemangioendothelioma-associated-with-kasabach-merritt-phenomenon
#11
Alaa M Alaqeel, Nuha A Alfurayh, Alanoud A Alhedyani, Saad M Alajlan
No abstract text is available yet for this article.
November 2016: JAAD Case Reports
https://www.readbyqxmd.com/read/27882874/an-intermediate-vascular-tumour-between-kaposiform-hemangioendothelioma-and-tufted-angioma-with-regression-of-the-skin-lesion
#12
Teruhiko Makino, Waka Ishida, Takeru Hamashima, Megumi Mizawa, Masakiyo Sasahara, Tadamichi Shimizu
No abstract text is available yet for this article.
November 23, 2016: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/27851352/1717-kaposiform-hemangioendothelioma-an-infant-s-suspicious-heart
#13
Shaista Shaik, Caitlin Agrawal, Chetan Bhupali
No abstract text is available yet for this article.
December 2016: Critical Care Medicine
https://www.readbyqxmd.com/read/27829366/anesthestic-management-of-kassabach-meritt-phenomenon-in-an-adult-case-report
#14
Abdelghafour Elkoundi, Mehdi Samali, Noureddine Kartite, Mohammed Tbouda, Mustapha Bensghir, Charki Haimeur
BACKGROUND: Kasabach-Merritt phenomenon (KMP) is characterized by a vascular tumor with profound thrombocytopenia and consumptive coagulopathy that may presents significant challenges for anesthesiologist. CASE PRESENTATION: An 87-year-old man presented with kaposiform hemangioendothelioma involving the right leg in critical condition due to massive bleeding. Hematology investigations indicated the presence of KMP. Association of this type of tumor with KMP in adults has never been reported...
November 9, 2016: BMC Anesthesiology
https://www.readbyqxmd.com/read/27820128/kaposiform-hemangioendothelioma-multifocal-involvement-chylothorax-and-kasabach-merritt-phenomenon
#15
Anirban Das, Deepak Bansal, Debajyoti Chatterjee, Ashim Das
No abstract text is available yet for this article.
March 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27803613/adult-onset-kaposiform-hemangioendothelioma-of-the-tongue-case-report-and-review-of-the-literature
#16
P Vashi, E Abboud, C Bier-Laning, D Gupta
We present here a very rare clinical case of a 38-year-old man with Kaposiform hemangioendothelioma (khe) of the tongue who presented to our institution with a growth under the left side of the tongue with no pain or discomfort. There were no enlarged lymph nodes and no significant neurologic findings. Diagnostic histopathology confirmed the lesion to be khe. The tumour was removed surgically, and the surgical specimen confirmed the diagnosis. Follow-up at 3 months shows no clinical evidence of recurrence.
October 2016: Current Oncology
https://www.readbyqxmd.com/read/27796340/interferon-alpha-therapy-for-refractory-kaposiform-hemangioendothelioma-a-single-center-experience
#17
Hai Wei Wu, Xuan Wang, Ling Zhang, Hai Guang Zhao, Yan An Wang, Li Xin Su, Xin Dong Fan, Jia Wei Zheng
Kaposiform hemangioendothelioma (KHE) is a relatively rare vascular tumor with an aggressive and infiltrating nature. Previous studies have revealed an exclusive relationship between KHE and Kasabach-Merritt Phenomenon (KMP), which is associated with high morbidity and mortality. No universally accepted treatment modality exists for refractory KHE with or without KMP. The aim of this study was to evaluate the safety and efficacy of interferon-alpha (IFN-α) therapy for treatment of refractory KHE. Twelve consecutive patients with KHE were treated with subcutaneous injections of IFN-α after other treatments had failed...
October 31, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27723921/sirolimus-in-the-treatment-of-vascular-anomalies
#18
Paloma Triana, Mariela Dore, Vanesa Nuñez Cerezo, Manuel Cervantes, Alejandra Vilanova Sánchez, Miriam Miguel Ferrero, Mercedes Díaz González, Juan Carlos Lopez-Gutierrez
Aim of the Study mTOR inhibitors are showing promising results in the management of vascular anomalies. Although current controlled trials remain to be completed, many individual experiences are being published. We present our series of children with complex vascular anomalies treated with sirolimus. Patients and Methods A retrospective review of 41 patients treated with sirolimus between January 2011 and December 2015 was performed: 15% (n = 6) had vascular tumors (4 kaposiform hemangioendotheliomas, 1 PTEN) and 85% (n = 35) had malformations (13 generalized lymphatic anomalies/Gorham-Stout diseases [GSD], 1 kaposiform lymphangiomatosis [KLA], 11 large lymphatic malformations (LMs) in critical areas, 2 lymphedemas, 4 venous malformations, and 4 aggressive arteriovenous malformations [AVM])...
February 2017: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27721772/response-to-paclitaxel-in-an-adult-patient-with-advanced-kaposiform-hemangioendothelioma
#19
José Maurício Mota, Mariana Scaranti, Leonardo G Fonseca, Diego Araújo Tolói, Veridiana Pires de Camargo, Rodrigo Ramella Munhoz, Olavo Feher, Paulo M Hoff
BACKGROUND: Kaposiform hemangioendothelioma (KHE) is a rare neoplasm of vascular origin that typically arises from the skin or soft tissues as a solitary tumor. The optimal therapy for this disease is still unknown. We report the case of an adult patient presenting with metastatic KHE of the spleen, who had a partial response after treatment with paclitaxel. CASE PRESENTATION: A 36-year-old man presented in November 2012 with a nontraumatic rupture of the spleen...
May 2016: Case Reports in Oncology
https://www.readbyqxmd.com/read/27701822/long-term-outcome-for-kaposiform-hemangioendothelioma-a-report-of-two-cases
#20
Beverly A Schaefer, Dehua Wang, Arnold C Merrow, Belinda H Dickie, Denise M Adams
Kaposiform hemangioendothelioma (KHE) is a rare aggressive vascular tumor of skin and deep soft tissues that typically presents in infancy and may be associated with a potentially life-threatening coagulopathy known as Kasabach-Merrit phenomenon (KMP). Recent advances in medical therapy have successfully treated many patients. However, our knowledge regarding the natural history of these lesions and optimum surveillance strategies remains rudimentary. We report two young women who had KHE with KMP treated in infancy and presented in adolescence with comorbidities related to their KHE tumor...
February 2017: Pediatric Blood & Cancer
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