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Vol. 58. Issue 5.
Pages 456-463 (1 May 2003)
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Vol. 58. Issue 5.
Pages 456-463 (1 May 2003)
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Avances en el diagnóstico de los tumores otorrinolaringológicos
Advances in the diagnosis of ent tumors in childhood
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F. Muñoz Borgea, J. González Alonsoa, H. Galera Ruiza,
Corresponding author
hgalera@hvm.sas.junta-andalucia.es

Correspondencia: Departamento de Anatomía Patológica. Hospital Universitario Virgen Macarena.
, F. Delgado Morenob, H. Galera Davidsonc
a Servicio de ORL Hospital Universitario Virgen Macarena. Sevilla. España.
b Sección de ORL (Materno-Infantil). Hospital Universitario Virgen del Rocío. Sevilla. España
c Departamento de Anatomía Patológica. Hospital Universitario Virgen Macarena. Sevilla.
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En el presente trabajo se revisa la patología tumoral otorrinolaringológica propia de la infancia. Se incide sólo en aquellos aspectos más sobresalientes, pues la variedad de entidades en estudio es restringida. En el 50 % de los papilomas puede demostrarse la infección por virus del papiloma humano (tipos 6 y 11) mediante técnicas de biología molecular, siendo menor la capacidad demostrativa de la inmunohistoquímica. En el angiofibroma nasal se ha puesto en evidencia la naturaleza miofibroblástica y su incidencia es 25 veces más frecuente en poblaciones de pacientes con poliposis adenomatosa familiar del colon. En el carcinoma nasofaríngeo ocurre sobreexpresión de p53 en los estadios iniciales y la sobreexpresión de c-myc se correlaciona con peor pronóstico. Recientemente se ha demostrado que el neuroblastoma olfatorio no expresa la proteína producto del gen MIC2 (anticuerpo 12E7), por lo que no se confirma la hipótesis de que puede ser miembro de la familia del tumor de Ewing (tumores neuroectodérmicos periféricos), aunque sí es un tumor neural primitivo. El rabdomiosarcoma de cabeza y cuello con mejor pronóstico es el orbitario, y los estudios citogenéticos han señalado la translocación cromosómica t(2;13) en el 50% de estos tumores infantiles cuando son de tipo alveolar, mientras que la trisomía del cromosoma 2 o del 20 es más peculiar del tipo embrionario. Por otro lado, cualquier asunto clasificatorio de los linfomas del área otorrinolaringológica, actualmente tiene que basarse en la clasificación REAL (Revised European-American Classification of Lymphoid Neoplasms).

Entre los tipos histológicos de carcinoma de tiroides, el papilar y el medular son los que más relieve poseen en la edad infantil y en la génesis de ambos, las alteraciones del protooncogén ret desempeñan un papel importante.

Palabras clave:
Angiofibroma
Neuroblastoma
Carcinoma nasofaríngeo
Rabdomiosarcoma
Linfomas otorrinolaringológicos(org)
Oncogénret Carcinoma medular

In the present study we review ENT tumor pathology in childhood. Only the most salient aspects are emphasized and the variety of entities reviewed was restricted. Molecular biology techniques reveal infection by human papilloma virus (types 6 and 11) in 50 % of papillomas, while immunohistochemical techniques are less effective in papilloma virus detection. The myofibroblastic nature of nasal angiofibroma has been demonstrated and its incidence is 25 times more frequent in patients with familial polyposis of the colon. Overexpression of p53 occurs in the initial stages of nasopharyngeal carcinoma, while overexpression of c-myc is correlated with an unfavorable prognosis. Recently, olfactory neuroblastoma has been shown not to express the protein product of the MIC-2 gene (antibody 12E7), thus the hypothesis that it could be a member of the Ewing tumor family (neuroectodermal peripheral tumors) has not been confirmed, although it is a primitive neural tumor. The head and neck rhabdomyosarcoma with the best prognosis is that located in the orbit, and cytogenetic studies have shown chromosomic translocation t(2;13) in 50 % of these childhood tumors when they are of the alveolar-type, while trisomy of chromosome 2 or 20 is more characteristic of the embryonic-type. Currently, any classifying features of ENT lymphomas must be based on the Revised European-American Classification of Lymphoid Neoplasms (REAL).

Papillary and medullary carcinomas are the most common histological types of thyroid carcinoma in childhood. Alterations in ret/PTC play a significant role in the pathogenesis of both.

Key words:
Angiofibroma
Neuroblastoma
Nasopharyngeal carcinoma
Rhabdomyosarcoma
ENT Lymphomas
Oncogen
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