Caso inusual de síndrome de cushing
2014
Objetivo: discutir las dificultades en el diagnostico y manejo terapeutico de un caso poco frecuente de Sindrome de Cushing (SC). Caso Clinico: se reporta el caso de un paciente femenino, de 24 anos, con hipercortisolismo y patron bioquimico sugestivo de SC dependiente de corticotropina (ACTH). Estudios imagenologicos mostraron agrandamiento de la glandula hipofisis, extension supraselar y una lesion lateral izquierda, sugestiva de microadenoma. Sometida a cirugia transesfenoidal en dos oportunidades, sin obtenerse la remision del cuadro. Tomografia computarizada (TC) de torax demostro la presencia de nodulo pulmonar unico izquierdo, hipercaptante en el gammagrama con octreotido. Fue removido quirurgicamente en forma satisfactoria produciendose un abrupto descenso en los niveles de ACTH y cortisol pero nuevamente se incrementaron 24 horas despues, permaneciendo elevados desde entonces. La inmunohistoquimica fue positiva para ACTH y cromogranina; el estudio anatomapatologico reporto tumor carcinoide tipico sin invasion a ganglios linfaticos; sin embargo, la evolucion clinica sugirio enfermedad residual. Se inicio tratamiento con analogos de somatostatina (octreotido), el cual se ha mantenido por once meses, obteniendose mejoria significativa del cuadro clinico y control parcial del hipercortisolismo. TC de torax y gammagrama con octreotido recientemente practicados, revelaron pequeno foco, el cual correspondio a adenopatia mediastinal derecha. Se plantea la hipotesis de que el tumor producia simultaneamente ACTH y hormona liberadora de corticotropina (CRH, por sus siglas en ingles), explicandose de esta manera la hiperplasia hipofisaria. Conclusion: En pacientes con SC dependiente de ACTH hay que tener presente la existencia de incidentalomas que pueden confundir el diagnostico. Considerar que la hiperplasia hipofisaria puede ser secundaria a una fuente ectopica productora de CRH y aunque en este caso no pudo realizarse inmunohistoquimica para CRH en las celulas tumorales, es posible suponer que dichas celulas secretaban conjuntamente CRH y ACTH lo cual explica la hiperplasia hipofisaria. Objective: to discuss the diagnostic and therapeutic difficulties of a rare case of Cushing´s syndrome. Case Report: we describe a case of a 24-year-old female patient who developed symptoms compatible with hypercortisolism; the biochemical pattern was suggestive of ACTH dependent Cushin´s Syndrome. Imaging studies showed pituitary enlargement with suprasellar extension and a lesion suggestive microadenoma on the left side. Transsphenoidal surgery was performed in two occasions without remission. Subsequent explorations showed the presence of a single left lung nodule, positive to octreoscan. It was successfully operated and, a sharp decline on ACTH and cortisol levels was seen immediately after operation, increasing again 24 hours later and, remained elevated since then. Inmunohistochemestry studies were positive for ACTH and chromogranin and although the pathology was compatible with typical carcinoid, and no lymph node invasion was seen, the clinical evolution suggested the presence of residual disease. Treatment with somatostatin analogues (Octreotide) was started and has being maintained for eleven months, up to the present. A significant improvement of the clinical picture and partial control of the hypercortisolism has being obtained. Chest CT and octreoscan recently performed, revealed small focus, which corresponded to a right mediastinal adenopathy. We hypothesize that the tumor was simulta-neously producing ACTH and CRH, explaining in this way the pituitary hyperplasia. Conclusion: In patients with ACTH dependent Cushing the existence of incidentalomas could confuse the diagnosis and should be ruled out. Pituitary hyperplasia might be secondary to an ectopic CRH source. Although in our case immunohistochemostry for CRH was not performed, we assume that tumor cells secreted both, CRH and ACTH, explaining the pituitary hyperplasia.
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