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Inicio Endocrinología y Nutrición Guía clínica del diagnóstico y tratamiento del craneofaringioma y otras lesio...
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Vol. 54. Núm. 1.
Páginas 13-22 (enero 2007)
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Vol. 54. Núm. 1.
Páginas 13-22 (enero 2007)
Documentos de los Grupos de Trabajo
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Guía clínica del diagnóstico y tratamiento del craneofaringioma y otras lesiones paraselares
Clinical practice guideline for the diagnosis and treatment of craniopharyngioma and other parasellar lesions
Visitas
84918
Concepción Páramo Fernández, Antonio Picó Alfonso, Carlos del Pozo Picó, César Varela da Costa, Tomás Lucas Morante, Miguel Català Bauset, Alberto Gilsanz Peral, Irene Halperin Ravinovich, Basilio Moreno Esteban, Gabriel Obiols Alfonso, Elena Torres Vela, Frederic Tortosa Henzi, Susan Webb Youdale, Ana Zugasti Murillo, Carles Villabona Artero
Autor para correspondencia
13861cva@comb.es

Correspondencia: Dr. C. Villabona Artero. Servei d’Endocrinologia i Nutrició. Hospital Universitari de Bellvitge. Feixa Llarga, s/n. 08907 L’Hospitalet de Llobregat. Barcelona. España.
Grupo de Trabajo de Neuroendocrinología de la Sociedad Española de Endocrinología y Nutrición
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Información del artículo

El craneofaringioma es un tumor, con frecuencia quístico, habitualmente supraselar, que deriva de restos de células embrionarias de la bolsa de Rathke. Si bien es un tumor benigno, tiene un comportamiento agresivo con frecuentes secuelas neurológicas y endocrinas. Presenta dos picos de aparición: en la edad infantil y en adultos añosos. La clínica depende de la localización, el tamaño, el potencial de crecimiento y la edad de presentación. Clínicamente suele aparecer como una combinación de signos y síntomas de hipertensión intracraneal, alteraciones visuales, deficiencias hormonales y disfunción hipotalámica. Si la lesión es intraselar la clínica puede remedar a la de un adenoma hipofisario.

Las técnicas de neuroimagen, especialmente la resonancia magnética, permiten caracterizar la lesión. La apariencia varía dependiendo de la proporción del componente sólido y quístico, de las posibles calcificaciones y de la composición de un eventual quiste. Antes del abordaje terapéutico debe efectuarse una completa evaluación endocrinológica y oftalmológica.

Las opciones terapéuticas incluyen cirugía, radioterapia y una combinación de ambas. La extensión óptima de la cirugía es motivo de controversia. Actualmente se prefiere una aproximación más conservadora que combina una cirugía menos agresiva con radioterapia.

La radioterapia sin cirugía únicamente es aplicable a los pacientes con tumores muy pequeños. Otras aproximaciones incluyen: aspiración intermitente mediante punción esterotáxica, colocación de un reservorio, esclerosis de las paredes del quiste mediante fármacos, o irradiación interna con radioisótopos.

Las lesiones paraselares son lesiones de muy baja prevalencia y pueden ser, entre otros tumores, aneurismas, quistes o granulomas. Las técnicas de neuroimagen, tanto la tomografía computarizada como la resonancia magnética, son útiles para precisar las características de la lesión.

Palabras clave:
Craneofaringioma
Lesión paraselar
Tumores hipotálamo-hipofisarios

Craniopharyngiomas are often cystic tumors, usually suprasellar, resulting from embryonic cell remnants of Rathke's pouch. Although benign, these tumors can be aggressive and frequently have neurological and endocrinological sequelae. Craniopharyngiomas usually develop in children or in the elderly. Symptoms depend on localization, size, potential for growth, and age of onset. Clinically, craniopharyngiomas usually manifest with a combination of symptoms and signs of intracranial hypertension, visual alterations, hormone deficiencies, and hypothalamic dysfunction. Intracellular lesions can mimic pituitary adenoma. Neuroimaging techniques, especially magnetic resonance imaging, allow these lesions to be characterized. Their appearance varies depending on the proportion of solid and cystic components, on the possible calcifications, and on the composition of an eventual cyst. Complete endocrinological and ophthalmological evaluation should be performed before establishing the therapeutic approach. The therapeutic options include surgery, radiotherapy, and a combination of both. The optimal extension of surgery is controversial. Currently, a conservative approach combining less aggressive surgery with radiotherapy is preferred. Radiotherapy without surgery is only applicable in patients with very small tumors. Other approaches include intermittent aspiration by stereotactic puncture, placement of a reservoir, cystic wall sclerosis through drugs, or internal radiation with radioisotopes.

Parasellar lesions have a very low prevalence and can consist of cystic aneurysms or granulomas, among other tumors. Neuroimaging techniques, both computed tomography and MRI, are useful for characterizing the lesion.

Key words:
Craniopharyngioma
Parasellar lesion
Hypothalamic-pituitary tumors
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Copyright © 2007. Sociedad Española de Endocrinología y Nutrición
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