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Cincinnati, OH, United States

Adams D.M.,University of Cincinnati | Adams D.M.,Hemangioma and Vascular Malformation Center | Hammill A.,University of Cincinnati
Seminars in Pediatric Surgery | Year: 2014

Vascular tumors are rare in children and adults. Classification of these tumors has been difficult, especially in the pediatric population, due to the rarity of these lesions, the unusual morphologic appearance, their diverse clinical behavior, and no independent stratification for pediatric tumors. In 2013, The World Health Organization updated the classification of soft tissue vascular tumors. Pediatric tumors were not independently stratified and the terminology was mostly left unchanged, but the intermediate category of tumors was divided into locally aggressive and rarely metastasizing. These tumors are treated with multimodality therapy and therefore need the guidance of an interdisciplinary team for best care. © 2014 Elsevier Inc. Source


Adams D.M.,University of Cincinnati | Adams D.M.,Hemangioma and Vascular Malformation Center
Clinics in Plastic Surgery | Year: 2011

Proper care of the patient with a vascular anomaly requires the expertise of multiple specialists. Because of the need for an interdisciplinary approach, several vascular anomalies centers have now been developed across the world. A hematologist/oncologist provides clinical acumen in establishing a correct diagnosis and guiding the medical management of these patients. These patients can have complicated coagulopathies and need medical therapy. This article emphasizes the hematologic complications and management of these patients. © 2011 Elsevier Inc. Source


Hammill A.M.,Hemangioma and Vascular Malformation Center | Hammill A.M.,University of Cincinnati | Wentzel M.,Hemangioma and Vascular Malformation Center | Gupta A.,Hemangioma and Vascular Malformation Center | And 12 more authors.
Pediatric Blood and Cancer | Year: 2011

Background: Vascular anomalies comprise a diverse group of diagnoses. While infantile hemangiomas are common, the majority of these conditions are quite rare and have not been widely studied. Some of these lesions, though benign, can impair vital structures, be deforming, or even become life-threatening. Vascular tumors such as kaposiform hemangioendotheliomas (KHE) and complicated vascular malformations have proven particularly difficult to treat. Procedure: Here we retrospectively evaluate a series of six patients with complicated, life-threatening vascular anomalies who were treated with the mTOR inhibitor sirolimus for compassionate use at two centers after failing multiple other therapies. Results: These patients showed significant improvement in clinical status with tolerable side effects. Conclusions: Sirolimus appears to be effective and safe in patients with life-threatening vascular anomalies and represents an important tool in treating these diseases. These findings are currently being further evaluated in a Phase II safety and efficacy trial. © 2011 Wiley-Liss, Inc. Source


Osborn A.J.,Hemangioma and Vascular Malformation Center | Osborn A.J.,Cincinnati Childrens Hospital and Medical Center | Dickie P.,Hemangioma and Vascular Malformation Center | Neilson D.E.,Cincinnati Childrens Hospital and Medical Center | And 4 more authors.
Human Molecular Genetics | Year: 2015

Lymphatic malformations (LMs) are developmental anomalies of the lymphatic system associated with the dysmorphogenesis of vascular channels lined by lymphatic endothelial cells (LECs). Seeking to identify intrinsic defects in affected LECs, cells were isolated from malformation tissue or fluid on the basis of CD31 and podoplanin (PDPN) expression. LECs from five unrelated LM lesions were characterized, including cells derived from one patient previously diagnosed with CLOVES. CLOVES-related LECs carried a known, activating mutation in PIK3CA (p.H1047L), confirmed by direct sequencing. Activating PIK3CA mutations (p.E542K and p.E545A) were identified in lesion-derived cells from the other four patients, also by direct sequencing. The five LM-LEC cultures shared a lymphangiogenic phenotype distinguished by PI3K/AKT activation, enhanced sprouting efficiency, elevated VEGF-C expression and COX2 expression, shorter doubling times and reduced expression of angiopoietin 2 and CXCR4. Nine additional LM-LEC populations and 12 of 15 archived LM tissue samples were shown to bear common PIK3CA variants by allele-specific PCR. The activation of a central growth/survival pathway (PI3K/AKT) represents a feasible target for the non-invasive treatment of LMs bearing in mind that background genetics may individualize lesions and influence treatments. © The Author 2014. Published by Oxford University Press. All rights reserved. Source

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