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Tiroidectomía profiláctica en el síndrome de neoplasia endocrina múltiple / Prophylactic thyroidectomy in multiple endocrine neoplasia syndrome
Cir. pediátr ; 22(4): 189-192, oct. 2009. ilus, tab
Artigo em Espanhol | IBECS | ID: ibc-107217
Biblioteca responsável: ES1.1
Localização: BNCS
RESUMEN
La neoplasia endocrina múltiple (MEN) 2A se caracteriza por la asociación de carcinoma medular de tiroides (CMT), feocromocitomae hiperparatiroidismo, ser hereditaria y tener una transferencia autosómica dominante. La identificación del protooncogen RET en 1993 ha cambiado el pronóstico de esta enfermedad. El objetivo del presente trabajo es realizar un estudio retrospectivo de los pacientes diagnosticados de síndrome MEN2a en nuestro centro en los últimos 7 años para establecer la edad más apropiada para la cirugía. Presentamos 10 casos diagnosticados de MEN2a entre 1,5 y 11años; estos se han intervenido con una edad media a la cirugía de 6,4años.En el preoperatorio se realiza ecografía cervical, determinación decalcitonina, catecolaminas y metanefrinas en orina. El tratamiento quirúrgico consiste en todos los casos en tiroidectomía total y en casos seleccionados (mayores de 5 años) vaciamiento (..) (AU)
ABSTRACT
Multiple endocrine neoplasia (MEN) 2a consists on medullary thyroid carcinoma, pheochromocytoma and hyperparathyroidism. The identification of the RET proto-oncogene in 1993 has changed the prognosis of the disease. We have retrospectively studied the patients diagnosed of MEN 2a in our centre for the last 7 years in order to establish the most adequate age to undergo surgery. We present ten patients diagnosed with MEN 2a, whose ages ranged from 1’5 to 11 years old. Mean age at time of operation 6,4 years An ultrasound study, calcitonin determinations and cathecholamines and urinary metanephrine levels were obtained before surgery. The surgical treatment is based on total total thyroidectomy, inselected cases lymph node resection in the central zone lf the neck. The most frequent RET mutation is the one affecting codon 634(exon 10), which was found in children. Both of them had an alterationin codon 611 (exon11). No complications appeared after surgery and hospital discharge took place in the 2nd-4th day after surgery. Pathological findings were medullary thyroid microcarcinoma (..) (AU)
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Coleções: Bases de dados nacionais / Espanha Base de dados: IBECS Assunto principal: Tireoidectomia / Neoplasias da Glândula Tireoide / Neoplasia Endócrina Múltipla Tipo 2a Aspecto clínico: Prognóstico Limite: Criança / Feminino / Humanos / Masculino Idioma: Espanhol Revista: Cir. pediátr Ano de publicação: 2009 Tipo de documento: Artigo Instituição/País de afiliação: Hospital Materno-Infantil La Fe/España