Síndrome de Cushing ACTH Dependiente, secreción ectópica: desarrollo de un caso

Se presenta el caso de un paciente varon de 32 anos, derivado a nuestro servicio para evaluacion de cuadro de 8 meses de evolucion caracterizado por edema y rubicundez facial, obesidad central, edema en miembros inferiores e hipertension arterial, compatible con sindrome de Cushing clinico. Aportaba estudios previos con cortisol serico 29,8 y 33 µg/dl (determinaciones realizadas con un mes de diferencia), ACTH 72,8 pg/ml, cortisol salival 2,1 µg/dl, cortisol libre urinario (CLU) 993,4 µg/24 hs. Los estudios imagenologicos (ecografia doppler renal, TAC de abdomen y pelvis c/contraste oral y e.v., TAC de craneo s/contraste y RMI de craneo c/contraste e.v.) no aportaron datos relevantes. Se confirmo bioquimicamente el sindrome de Cushing dependiente de ACTH: cortisol serico (8 hs) 34,8 µg/dl, ACTH (8 hs) 72 pg/ml, cortisol libre urinario 828 µg/24 hs. El test de Nugent no mostro freno. El test de Liddle (8 mg oral dexametasona 23 hs) produjo un descenso del cortisol plasmatico de solo 21%. La funcion tiroidea, las gonadotrofinas y la prolactina sericas eran normales. La radiografia de torax mostro mediastino ensanchado e imagen nodular parahiliar basal derecha; esto se confirmo por TAC. La formacion nodular media 20 x 13mm, era de bordes lisos y aspecto inespecifico. Se exploro quirurgicamente esta lesion, con diagnostico intraoperatorio de poblacion linfoide de pequeno tamano. Se realizo nodulectomia por toracotomia con la sospecha de lesion linfoproliferativa. El diagnostico anatomopatologico definitivo: tumor neuroendocrino bien diferenciado ... (AU) The case of a male patient aged 32, referred to our service for evaluation of 8-month history of facial redness and edema,central obesity, lower limb edema and arterial hypertension consistent with clinical Cushing syndrome is presented. He hadprevious studies showing serum cortisol 29.8 and 33 mg/dl (determinations performed one month apart), ACTH 72.8 pg/ml, salivary cortisol 2.1 µg/dl, urinary free cortisol (UFC) 993.4 µg/24 h. Imaging studies (renal ultrasound doppler, CTof the abdomen and pelvis with oral and iv contrast, skull CT without contrast and skull RMI with iv contrast) did notprovide relevant data. Serum cortisol (8 hours) 34.8 µg/dl, ACTH (8 h) 72 pg/ml, urinary free cortisol 828 µg/24 h: anACTH-dependent Cushing’s syndrome was biochemically confirmed. Nugent’s test was negative. Overnight Liddle’s test (8mg oral dexamethasone 23 h) resulted in a modest decrease (21%) in plasma cortisol. Thyroid function, serum gonadotropinsand prolactin were normal. The chest radiograph showed widened mediastinum and a right basal parahilar nodularimage; this was confirmed by CT. The nodule measured 20 x 13 mm, it had smooth edges and nonspecific appearance. Thislesion was explored surgically, with intraoperative diagnosis of small lymphoid population. Lumpectomy was performed bythoracotomy The final pathological diagnosis was well-differentiated neuroendocrine tumo...(AU)