Markers of vascular differentiation, proliferation and tissue remodeling in juvenile nasopharyngeal angiofibromas.

Juvenile nasopharingeal angiofibroma (JNA) is a histologically benign locally aggressive tumor characterized by irregular vessels embedded in a fibrous stroma. Excessive vascularity results in bleeding complications, and the inhibition of angiogenesis is a promising strategy for managing extensive JNA tumors. To better characterize the endothelial components of JNA, we aimed to evaluate markers of vascular differentiation and proliferation, such as friend leukemia integration-1 (FLI-1) and endoglin, lymphatic markers, including podoplanin and vascular endothelial growth factor receptor 3 (VEGFR3) and its cognate ligand VEGFC, GLUT-1, a diagnostic marker that discriminates between hemangiomas and vascular malformations, and two markers of tissue remodeling, stromelysin 3 (ST3) and secreted acid protein rich in cysteine (SPARC). Antigens were assessed immunohistochemically in vessels and stromal cells of JNA archival cases (n=22). JNA endothelial cells were positive for endoglin, VEGFC and FLI-1, whereas podoplanin and VEGFR3 were negative in all cases. Both endothelial cells and fibroblasts stained for ST3 and SPARC. GLUT-1 was investigated in JNA cases, in infantile hemangiomas (n=123) and in vascular malformations (n=135) as controls. JNAs and vascular malformations were GLUT-1-negative, while hemangiomas showed positive staining. The presence of markers of endothelial differentiation and proliferation highlighted the hyper-proliferative state of JNA vessels. The absence of podoplanin and VEGFR3 underscores their blood endothelial cell characteristic. The absence of GLUT-1 discriminates JNAs from hemangiomas. ST3 and SPARC up-regulation in endothelial cells and fibroblasts may contribute to a compensatory signaling for controlling angiogenesis. Some of these markers may eventually serve as therapeutic targets. Our results may aid in the understanding of JNA pathophysiology.

Hemangioma hepático / Hepatic hemagioma

Objetivos: Descrever um caso de hamangioma hepático em recém-nascido tratado com cirurgia. Métodos: Relata-se o caso de recém-nascidos do sexo masculino, de 6 dias de vida, com extensa lesão hepática, e sua evolução. Resultados: A criança evoluiu com quadro de descompensação hemodinâmica devido a coagulopatia de consumo e insuficiência respiratória. Os exames de imagem foram inconclusivos, sendo indicado procedimento cirúrgico. Foi realizada ressecção total da massa hepática e confirmado o diagnóstico de hamangioma por estudo anatomopatológico. A criança evoluiu satisfatoriamente, recebeu alta após 15 dias e encontra-se assintomática. Conclusões: O hemangioma hepático deve ter tratamento conservador, estando a cirurgia reservada para os casos de insuficiência cardíaca intratável e/ou coagulopatia consumptiva refratária