RESUMO
OBJECTIVE: Lymphatic malformations are characterized by the overgrowth of lymphatic vessels during development. Activation of PI3K/AKT and MAPK/ERK signaling pathways occur in isolated lymphatic malformation and in those associated with syndromes such as CLOVES and Klippel-Trenaunay. We aimed to assess the activation of these pathways in sporadic oral lymphatic malformations. STUDY DESIGN: A convenience sample of 14 formalin-fixed paraffin-embedded samples of oral lymphatic malformations underwent immunohistochemical reactions for the phosphorylated forms of AKT1 (pAKT-Ser473) and ERK1/2 (pERK1/2-Thr202/Tyr204), which are markers of PI3K/AKT and MAPK/ERK pathways activation, respectively. RESULTS: Positive staining for pAKT1 and pERK1/2 was observed in the endothelial cells in all samples of oral lymphatic malformations evaluated. CONCLUSIONS: Our results suggest that activation of PI3K/AKT and MAPK/ERK signaling pathways participates in the pathogenesis of oral lymphatic malformations.
Assuntos
Vasos Linfáticos/anormalidades , Sistema de Sinalização das MAP Quinases , Fosfatidilinositol 3-Quinases , Células Endoteliais/metabolismo , Humanos , Boca , Fosfatidilinositol 3-Quinases/metabolismo , Proteínas Proto-Oncogênicas c-akt/metabolismoRESUMO
PURPOSE: To review a representative case series and share a stepwise approach to the diagnosis and treatment of periorbital venolymphatic malformations. METHODS: A case series of 9 patients with representative periorbital venolymphatic malformations. All patients managed at the University of California, Los Angeles Orbital Center for a vascular malformation over the last 5 years were reviewed. Cases representative of the varied pathologic findings and techniques were selected. The clinical, radiologic, and intraoperative findings are presented and a survey of techniques composed. RESULTS: Venolymphatic malformations can have protean manifestations. Characteristics vital to the understanding and management of these lesions are the classification and vascular composition of the lesion, internal flow and drainage patterns, anatomic localization and association with distant components. Options for treatment include sclerotherapy, biologic therapy, embolization, surgical excision, or some combination thereof. CONCLUSIONS: A comprehensive understanding of each patient's unique lesion is critical to a rational treatment approach. Teamwork and creativity are key to effectively managing these lesions.
Assuntos
Fatores Biológicos/uso terapêutico , Embolização Terapêutica/métodos , Vasos Linfáticos/anormalidades , Procedimentos Cirúrgicos Oftalmológicos/métodos , Órbita/irrigação sanguínea , Escleroterapia/métodos , Malformações Vasculares/terapia , Adulto , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Malformações Vasculares/diagnóstico , Adulto JovemRESUMO
OBJETIVO: Avaliar três casos de linfangioma cervical por ressonância magnética e correlacionar com os achados da ultrassonografia. MATERIAIS E MÉTODOS: Três pacientes com idade gestacional entre 24 e 35 semanas, com suspeita de higromas císticos cervicais fetais na ultrassonografia obstétrica de rotina, foram submetidas a ressonância magnética e, posteriormente, a nova ultrassonografia para correlação dos achados. Em ambos os métodos de imagem foram avaliadas as dimensões, a localização, o conteúdo e a extensão das lesões. RESULTADOS: Tanto a ultrassonografia quanto a ressonância magnética avaliaram de modo semelhante a localização, o tamanho e o conteúdo dos tumores. As três lesões localizavam-se na região cervical posterior e lateral. Quanto ao conteúdo, duas eram predominantemente císticas com finos septos em seu interior e uma era heterogênea. A extensão e invasão das estruturas adjacentes foram mais bem caracterizadas na ressonância magnética do que na ultrassonografia, demonstrando de forma adequada o acometimento do pavilhão auditivo do feto em um caso e do mediastino superior em outro. CONCLUSÃO: A ressonância magnética fetal pode ser um complemento útil da ultrassonografia em fetos portadores de linfangiomas, avaliando de forma mais precisa a extensão e invasão de estruturas vizinhas, permitindo melhor planejamento cirúrgico pós-natal.
OBJECTIVE: To evaluate three cases of cervical lymphangioma with magnetic resonance imaging and correlating with sonographic findings. MATERIALS AND METHODS: Three pregnant women between the 24th and 35th gestational weeks, with sonographic findings suggestive of fetal cystic hygroma, were submitted to magnetic resonance and subsequently to a new ultrasonography for correlation of imaging findings. Tumors size, location, content and extent were evaluated both at magnetic resonance imaging and ultrasonography. RESULTS: Findings regarding tumor location, size and content were similar for both methods. All the lesions were found in the posterior and lateral cervical space. As regards the tumors content, two of the lesions were predominantly cystic, with thin septations, and the other was heterogeneous. Lesions extent and adjacent structures invasion were better characterized by magnetic resonance imaging, with appropriate demonstration of invasion of the pinna in one case and invasion of the superior mediastinum in another. CONCLUSION: Fetal magnetic resonance imaging can be a useful adjuvant to obstetric ultrasonography in cases of lymphangioma because of its higher accuracy in the determination of these tumors extent and adjacent structures invasion, allowing a better postnatal surgical planning.