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Inicio Endocrinología y Nutrición Metodología diagnóstica en la identificación del Cushing ectópico
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Vol. 54. Núm. 8.
Páginas 432-437 (Octubre 2007)
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Vol. 54. Núm. 8.
Páginas 432-437 (Octubre 2007)
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Acceso a texto completo
Metodología diagnóstica en la identificación del Cushing ectópico
Diagnostic methods in ectopic cushing's syndrome
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Cristina Lamas Oliveira??
Autor para correspondencia
lamas@mixmail.com

Correspondencia: Dra. C. Lamas Oliveira. Sección de Endocrinología y Nutrición. Hospital Universitario de Albacete. Hermanos Falcó, 37. 02006 Albacete. España.
, José Joaquín Alfaro Martínez, Pedro Pinés Corrales, Silvia Aznar Rodríguez, Antonio Hernández López
Sección de Endocrinología y Nutrición. Hospital Universitario de Albacete. Albacete. España
Información del artículo

La distinción entre las distintas formas de síndrome de Cushing dependiente de corticotropina (ACTH), es decir, los adenomas hipofisarios y los tumores ectópicos, puede resultar difícil en determinados casos. Las pruebas diagnósticas que nos orientan en esa situación son la supresión con dosis altas de dexametasona, la respuesta a metopirona, al estímulo con corticoliberina (CRH) o desmopresina, la determinación de otras hormonas o marcadores tumorales, las pruebas de imagen y el cateterismo de senos petrosos inferiores, pero todas ellas ofrecen sensibilidades y especificidades subóptimas, por lo que solemos recurrir a la combinación de varias pruebas. Una vez confirmado el origen ectópico de la secreción de ACTH, debemos localizar el tumor que la origina. Para ello hay que conocer los tipos tumorales implicados con mayor frecuencia en el síndrome de secreción ectópica de ACTH/CRH (carcinoides, carcinomas pulmonares de células pequeñas, tumores neuroendocrinos, carcinomas medulares de tiroides, feocromocitomas y otros) y utilizar las distintas técnicas de imagen disponibles. La tomografía computarizada (TC) y la resonancia magnética (RM) son las más utilizadas y las de mayor rentabilidad diagnóstica. Las pruebas de imagen “funcionales”, la gammagrafía con octreotida y la tomografía poremisión de positrones, pueden ofrecer resultados positivos cuando la TC y la RM no encuentran la lesión que causa el síndrome. Aun después de emplear todas estas técnicas, y otras menos habituales por resultar menos rentables, queda un 15-20% de pacientes con tumores no localizados. Su seguimiento a largo plazo hace que algunos tumores pasen a ser clínicamente evidentes y que el porcentaje de tumores ocultos vaya reduciéndose a medida que pasan los años.

Palabras clave:
Síndrome de Cushing
Síndrome de ACTH ectópica
Hormonas ectópicas

The differential diagnosis of adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome, that is, the distinction between pituitary adenomas and ectopic ACTH-secreting tumors, can be difficult. The diagnostic tools in this setting are the high-dose dexamethasone suppression test, the metyrapone test, the corticotropin-releasing hormone stimulation test (with or without desmopressin), determination of levels of other hormones or tumoral markers, imaging techniques, and inferior bilateral petrosal sinus sampling. However, the sensitivity and specificity of all these tests are suboptimal, and a combination of two or more tests is the best diagnostic approach.

Once the ectopic origin of ACTH secretion has been confirmed, the tumor must be located. The most common tumors leading to ectopic ACTH secretion are carcinoid tumors, small-cell lung carcinomas, neuroendocrine tumors, medullary thyroid cancer, and pheochromocytomas. Among imaging techniques, computed tomography (CT) and magnetic resonance imaging (MRI) are widely used and provide the best results.

Functional imaging techniques (somatostatin receptor scintigraphy and positron emission tomography) can locate the tumor causing Cushing's syndrome in patients with negative CT and MRI scans. Even after all these techniques, as well as other less frequent and useful procedures have been used, the tumor remains occult in 15-20% of patients. Some of these tumors become clinically evident during long-term follow-up and thus the percentage of ocult tumors decreases with time.

Key words:
Cushing's syndrome
Ectopic ACTH syndrome
Ectopic hormones
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Copyright © 2007. Sociedad Española de Endocrinología y Nutrición
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