LBSAT63 Cushing Syndrome As A Presentation Of Adrenocortical Carcinoma.Case Report

Abstract

Abstract Introduction adrenocortical carcinoma is a rare endocrine malignancy (1) Case: 44 year old afro American female with no past medical history. No regular medications. Amenorrhea for 2 years. smokes cigarettes. Irrelevant family history. She presented with itchiness after cat scratch and was found to have hypertension urgency 229/123. Review of systems was positive for weight gain and easy bruises. Physical examination alert and oriented with body mass index of 31 kg/m2 heart rate 75 beats per minute normal heart sounds and clear lungs with scratch marks on left hand. bilateral lower extremities pitting edema, supraclavicular fat pad and violet abdominal striae. No facial plethora or hirsutism or lymphadenopathy or acne. Laboratory work up was remarkable for hypokalemia and metabolic alkalosis. High plasma morning cortisol level 53.6ug/dl normal between 6.2 and 29, 24hour urine cortisol and DHEAS and low ACTH FSH and LH. Estradiol 261pg/ml 17hydroxyprogesterone 49ng/dl and prolactin 31.7ng/ml. HBA1C 6.7. Normal renin aldosterone TSH and plasma and 24hour metanephrine and normetanephrine. Normal creatinine liver function and complete blood count. CT abdomen and pelvis with contrast showed large lobular heterogeneously enhancing mass in the left adrenal gland of 9. 0×10.1×10.1cm with left kidney posterior displacement and left renal vein compression. The pancreatic tail and splenic vein were draped over the mass. The right adrenal gland and other abdominal organs were unremarkable. DEXA scan showed osteopenia. CT chest showed 5mm right lower lobe nodule and compression fracture in the 7th thoracic vertebrae. Echocardiogram showed dilated left ventricle dimension with reduced ejection fraction 45%. The diagnosis was made for ACTH independent Cushing syndrome with adrenal mass. She underwent open left adrenalectomy distal pancreatectomy splenectomy and left nephrectomy in another institution. Histopathology showed 12×7.5×7 cm stage III adrenal cortical malignancy T3N0M0. Extensive necrosis and capsular invasion but negative surgical margins. Possibility of lung metastasis. Postoperative cortisol level 6. 02ug/dl. She was kept on postoperative slow tapering hydrocortisone with plan of chemotherapy and Mitotane. Conclusion adrenocortical carcinoma has a poor prognosis. It usually metastasizes to distant organs at time of diagnosis and rarely presents as Cushing syndrome. Surgical resection is the main management of localized tumors with controlling the effects of secreted hormones. Adjuvant therapies are chemotherapy radiotherapy and Mitotane according to the tumor stage and size. Mitotane is adrenal cytotoxic agent showed increase survival of advanced stages(1).