Neoplasia Endócrína Múltipla tipo 2b - Relato de três casos e revisão de Literatura
DOI:
https://doi.org/10.32635/2176-9745.RBC.1999v45n2.2776Palavras-chave:
Carcinoma Medular de Tireóide, Feocromocitoma, Neoplasia Endócrina MúltiplaResumo
Neoplasia Endocrina Múltipla tipo 2B (NEM-2B) é uma síndrome incomum, herdada de forma autossômica dominante e com penetrância variável. Múltiplos órgãos são envolvidos, mas com uma tríade predominante de carcinoma medular de tireóide, feocromocitoma e ganglioneuromas do trato gastrointestinal. A maioria dos pacientes tem fácies característica, composta por eversão e alargamento dos lábios e eversão das pálpebras por neuromas mucosos. Os autores relatam três casos de NEM-2B, sendo dois do sexo masculino, com idade média de 15,9 anos. A manifestação clínica inicial em todos os casos foi crescimento de massa cervical em topografia de tireóide e hábito marfanóide. Foram submetidos a tireoidectomia total, com esvaziamento cervical bilateral por carcinoma medular de tireóide. Um paciente apresentou feocromocitoma quatro anos após o diagnóstico de tumor de tireóide, sendo submetido a laparotomia com excisão do tumor. Um paciente evoluiu a óbito 2,5 anos após o diagnóstico e os outros dois encontram-se livres de doença, com uma média de acompanhamento ambulatorial de um ano, apesar do teste de calcitonina positivo em um dos casos.
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