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Kaposiform hemangioendothelioma

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https://www.readbyqxmd.com/read/28027747/imaging-findings-of-kaposiform-hemangioendothelioma-in-children
#1
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
#2
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...
December 18, 2016: Angiogenesis
https://www.readbyqxmd.com/read/27983885/multifocal-kaposiform-hemangioendothelioma-causing-massive-fetal-chylous-ascites
#3
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...
December 16, 2016: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/27981218/sirolimus-for-treatment-of-kaposiform-hemangioendothelioma-associated-with-kasabach-merritt-phenomenon
#4
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
#5
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
#6
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
#7
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
#8
Anirban Das, Deepak Bansal, Debajyoti Chatterjee, Ashim Das
No abstract text is available yet for this article.
November 4, 2016: 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
#9
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
#10
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
#11
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])...
October 10, 2016: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27721772/response-to-paclitaxel-in-an-adult-patient-with-advanced-kaposiform-hemangioendothelioma
#12
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
#13
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
https://www.readbyqxmd.com/read/27607323/the-clinical-spectrum-of-kaposiform-hemangioendothelioma-and-tufted-angioma
#14
REVIEW
Stacy E Croteau, Deepti Gupta
Kasposiform hemoangioendothelioma (KHE) and tufted angioma (TA) are classifed as vascular tumors with locally aggressive and benign growth potential, respectively, within the classification schema proposed by the International Society for the Study of Vascular Anomalies. A unique feature of these vascular tumors is the risk of Kasabach-Merritt phenomenon (KMP), a severe thrombocytopenia with mild to moderate coagulopathy resulting from intralesional platelet trapping. As with many vascular anomalies, accurate description of clinical course, responses to therapy, and long-term outcomes have been hindered by lesion misidentification, imprecise nomenclature, and lack of prospective, randomized clinical trials to assess therapeutic efficacy...
September 2016: Seminars in Cutaneous Medicine and Surgery
https://www.readbyqxmd.com/read/27510687/recurrent-multifocal-cutaneous-kaposiform-hemangioendothelioma-a-rare-vascular-tumor-of-infancy-and-childhood
#15
Bhagyalakshmi Atla, P V Sudhakar, Nagarjun Rao, Uma Prasad
Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor of childhood although cases occurring in adulthood are also described. The features overlap with juvenile capillary hemangioma and Kaposi sarcoma. We report a rare case of recurrent, multifocal (nose and chin) cutaneous KHE initially occurring in a 3-year-old female child, uncomplicated by Kasabach-Merritt syndrome. Recurrences occurred over the next 6 years and resulted in complete distortion of the nose, requiring plastic repair.
July 2016: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/27476652/gna14-somatic-mutation-causes-congenital-and-sporadic-vascular-tumors-by-mapk-activation
#16
Young H Lim, Antonella Bacchiocchi, Jingyao Qiu, Robert Straub, Anna Bruckner, Lionel Bercovitch, Deepak Narayan, Jennifer McNiff, Christine Ko, Leslie Robinson-Bostom, Richard Antaya, Ruth Halaban, Keith A Choate
Vascular tumors are among the most common neoplasms in infants and children; 5%-10% of newborns present with or develop lesions within the first 3 months of life. Most are benign infantile hemangiomas that typically regress by 5 years of age; other vascular tumors include congenital tufted angiomas (TAs), kaposiform hemangioendotheliomas (KHEs), and childhood lobular capillary hemangiomas (LCHs). Some of these lesions can become locally invasive and unresponsive to pharmacologic intervention, leading to significant complications...
August 4, 2016: American Journal of Human Genetics
https://www.readbyqxmd.com/read/27438697/symptomatic-congenital-hemangioma-and-congenital-hemangiomatosis-associated-with-a-somatic-activating-mutation-in-gna11
#17
Tracy Funk, Young Lim, Ann M Kulungowski, Lori Prok, Timothy M Crombleholme, Keith Choate, Anna L Bruckner
IMPORTANCE: Congenital hemangiomas are uncommon benign vascular tumors that present fully formed at birth. They are rarely associated with transient hematologic abnormalities, which are typically less severe than the Kasabach-Merritt phenomenon associated with kaposiform hemangioendotheliomas. Congenital hemangiomas are typically solitary and have not been reported to occur in a multifocal, generalized pattern. OBJECTIVE: To describe a male infant born with an unusual, large vascular mass complicated by anemia, thrombocytopenia, and disseminated intravascular coagulopathy, as well as innumerable small vascular papules in a generalized cutaneous distribution...
September 1, 2016: JAMA Dermatology
https://www.readbyqxmd.com/read/27430695/-kaposiform-hemangioendothelioma-a-clinicopathologic-analysis-of-23-cases
#18
Y Shu, H F Tong
No abstract text is available yet for this article.
July 8, 2016: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/27408442/kaposiform-hemangioendothelioma-in-a-3%C3%A2-months-old-infant
#19
Ramya Uppuluri, Vimal Kumar, Deenadayalan Munirathnam, Hemalatha Doss, Sreejith Ramachandrakurup, Divya Subburaj, Revathi Raj
Kaposiform hemangioendothelioma is a rare, highly vascular and aggressive soft tissue tumor frequently associated with Kasabach-Merritt phenomenon, usually seen in early infancy. Early diagnosis by means of MRI and tissue biopsy portends a better outcome. Treatment includes surgical excision when feasible and medical management with steroids, propranolol, vincristine and supportive treatment for coagulopathy. We report a 3 months old female infant who was diagnosed, treated successfully and is now in complete remission...
June 2016: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/27342010/neonatal-kaposiform-hemangioendothelioma-of-the-spleen-associated-with-kasabach-merritt-phenomenon
#20
Samuel A Shabtaie, Bo Wang, Mark Owyong, Catalina Ruiz-Mesa, Fernando F Corrales-Medina, Claudia P Rojas, Juan C Infante, Holly L Neville, Eduardo A Perez, Juan E Sola, Anthony R Hogan
Kaposiform hemangioendothelioma is a rare locally aggressive vascular tumor that usually manifests during early childhood. Typically the lesion presents with skin, soft tissue and bone involvement and is characterized histologically by ill-defined nodularity and the presence of spindle cells with resemblance to Kaposi's sarcoma. We report a rare neonatal case of a splenic kaposiform hemangioendothelioma associated with Kasabach-Merritt phenomenon that was diagnosed with radiographic imaging. Because of the rapid onset of thrombocytopenia and anemia, the patient required urgent splenectomy with subsequent resolution of the blood dyscrasias...
June 2016: Journal of Pediatric Surgery
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