Información del artículo
Dos hermanas (14 y 18 años) estudiadas por hipercortisolismo en nuestro servicio en el último año presentaron datos analíticos y bioquímicos de síndrome de Cushing independiente del ACTH, aun cuando los estudios de localización realizados no pudieron poner de manifiesto un agrandamiento uni o bilateral de las glándulas adrenales. En sus antecedentes figuraba una tía materna con hallazgos “similares”. La clínica de hipercortisolismo sólo fue evidente en la mayor de las hermanas, mientras que la hiperfunción adrenal fue demostrada tras un estudio de secreción espontánea en 24 h en la más pequeña.
Fueron remitidas a cirugía siendo practicada en ambas una adrenalectomía bilateral. El estudio anatomopatológico reveló la presencia de múltiples nódulos entre 1 y 3 mm, de coloración oscura y atrofia perinodular confirmando la existencia de displasia micronodular pigmentaria. No se objetivaron datos de presencia de síndrome de Carney en ellas.
Presentamos la clínica, los hallazgos analíticos e histológicos de nuestras pacientes afectadas de esta inusual enfermedad y una revisión de la bibliografía científica.
Palabras clave:
Hipercortisolismo
Síndrome de Cushing
Displasia micronodular pigmentaria
Síndrome de Carney
Two sisters (14 and 18 years old) presented with hypercortisolism in our Department over the last year. They showed biochemical tests of ACTHindependent Cushing's syndrome without radiological adrenal gland enlargement. A maternal aunt also presented similar findings. Hypercortisolism was more evident in the elder one; her sister only showed elevated 24 hours spontaneous cortisol secretion.
Both patients underwent adrenalectomy. Pathological examination of the adrenal gland revealed dark nodules that ranged from 1 to 3 mm and perinodular atrophy, lesions specific for pigmented micronodular dysplasia. No other clinical data suggestive of the Carney complex were observed.
We present the clinical and analytical data of these two patients with an unusual pathology.
Key words:
Hipercortisolism
Cushing's syndrome
Pigmented micronodular dysplasia
Carney complex
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