ArticleEuropean journal of pediatric surgery reports2023
Successful Sirolimus Treatment for Recurrent Pericardial Effusion in a Large Cervicomediastinal Provisionally Unclassified Vascular Anomaly: A Case Report.
Article in European journal of pediatric surgery reports, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Provisionally unclassified vascular anomalies (PUVA) are a group of diseases with unique characteristics that make them unclassifiable within vascular tumors or malformations. We describe a PUVA as the cause of recurrent pericardial effusion and its response to sirolimus. A 6-year-old girl was referred with a cervicothoracic vascular anomaly, a violaceous, and irregular lesion in the neck and upper chest, diagnosed as "hemangioma". She had pericardial effusion at the neonatal age that required pericardiocentesis, propranolol, and corticosteroids. She remained stable for 5 years, when she presented with a severe pericardial effusion. A magnetic resonance visualized a diffuse vascular image in the cervical and thoracic region with mediastinal extension. The pathological study showed a vascular proliferation in the dermis and hypodermis with positive staining for Wilms' Tumor 1 Protein (WT1) and negative for Glut-1. Genetic testing found a variant in
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