Resumen
Introducción: los incidentalomas adrenales son tumores que suelen ser detectados en estudios de imágenes indicados sin sospecha de enfermedad adrenal. El feocromocitoma es un tumor neuroendocrino que puede presentarse esporádicamente o asociado a síndromes genéticos, habiendo sido descrito en 0.1-5% de pacientes con neurofibromatosis tipo 1, que es una enfermedad progresiva multisistémica de herencia autosómica dominante que afecta a 1/2600-3000 individuos.
Caso clínico: se reporta el caso de un paciente de 50 años quien ingresó al departamento de Emergencia por apendicitis aguda y cuya tomografía mostró la presencia de masas adrenales bilaterales. Luego de la intervención quirúrgica fue hospitalizado para estudio. El paciente negó cefalea, diaforesis, hipertensión arterial, palpitaciones y pérdida de peso; en el examen físico se evidenció presencia de múltiples lentígines, manchas café con leche > 15 mm, lesiones nodulares fijas y pedunculadas, compatibles con neurofibromas focales, sin signos sugestivos de hipo o hipercortisolismo. En la analítica se identificaron metanefrinas totales y ácido vanilil-mandélico en orina encontrándose en valores muy elevados, siendo sometido a adrenalectomía laparoscópica en 2 tiempos, luego de una adecuada preparación que incluyó bloqueo alfa y beta adrenérgicos.
Conclusiones: en los pacientes con masas adrenales es necesario reconocer la importancia de una evaluación clínica integral para guiar un adecuado estudio diagnóstico, así como la realización de un óptimo estudio preoperatorio que incluye las pruebas hormonales para descartar funcionalidad.
Abstract
Background: Adrenal incidentalomas are tumors that are usually detected in imaging studies indicated without suspicion of adrenal disease. Pheochromocytoma is a neuroendocrine tumor that can occur sporadically or associated with genetic syndromes, having been described in 0.1-5% of patients with type 1 neurofibromatosis, which is a progressive multisystemic disease of autosomal dominant inheritance that affects 1 / 2600-3000 individuals .
Clinical case: We present the case of a 50-year-old patient who was admitted to the Emergency Department for acute appendicitis and whose CT scan showed the presence of bilateral adrenal masses is reported. After the surgical intervention, he was hospitalized for study. The patient denied headache, sweating, hypertension, palpitations, and weight loss; Physical examination revealed the presence of multiple lentigines, café-au-lait spots > 15 mm, fixed and pedunculated nodular lesions, compatible with focal neurofibromas, without signs suggestive of hypo or hypercortisolism. In the analysis, total metanephrines and vanillyl-mandelic acid were identified in urine, they were found in very high values, being subjected to laparoscopic adrenalectomy in 2 stages, after adequate preparation that included alpha and beta adrenergic blockade.
Conclusions: In patients with adrenal masses, it is necessary to recognize the importance of a comprehensive clinical evaluation to guide an adequate diagnostic study, as well as the performance of an optimal preoperative study that includes hormonal tests to rule out functionality.
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