Endocrine Abstracts

Introduction: Adrenocortical Carcinoma (ACC) is a rare endocrine neoplasm with poor prognosis, and an estimated incidence of approximately 2.5/1,000,000 patients. The functional state of these tumors varies from non-secretory (20%) to non-secretory. The latter category is subdivided into glucocorticoid-producing (45%) or glucocorticoid-androgen producing (45%) or androgen-producing only (10%). We report a case of a 35-year-old lady who initially presented with symptoms of Cushing’s Syndrome and underwent adrenalectomy, with post-operative histology compatible with adrenocortical Carcinoma.

Case presentation: Health problems started 5 years ago, when she was diagnosed with Type 2 Diabetes Mellitus, on treatment with oral agents, at that time she underwent sleeve gastrectomy resulting to improved glycemic control and weight loss for the next 4 years. After this time, she started gaining weight again and her glycemic control deteriorated. A few weeks later, she was admitted to the hospital with complaints of diarrhea. Imaging with a Computed tomography of the abdomen and pelvis showed an incidental 30 mm left medial limb adrenal nodule with central focus of calcification with absolute washout of 76% is consistent with adrenal adenoma. A smaller 10 mm left adrenal nodule is also compatible with a lipid rich adrenal adenoma. Biochemical assessment showed failure to suppress adrenal function (overnight dexamethasone suppression test: pre-test cortisol 9 am 695 nmol/l, post-test cortisol 9 am 587 nmol/l; paired ACTH was suppressed to <1.5 ng/l pre-test; low-dose dexamethasone suppression test, post-test 227 nmol/l). The remaining biochemical screen showed raised 24-hour urinary free cortisol of 699 nmol/l (range 0-125 nmol/l/24 h), whereas levels of metanephrines, dehydroepiandrosterone, renin and aldosterone were not elevated. At this point, she started on Metyrapone 250 mg 8-hourly as well as hydrocortisone replacement dose, which resulted in an improvement of her glycemic control. The clinical and biochemical findings were discussed in the adrenal multidisciplinary meeting, which advised elective robotic adrenalectomy was made. The surgery was un-eventful with good pos-surgical recovery. Post-surgical histology report was compatible with a multinodular adrenocortical neoplasm, the largest with potentially aggressive behavior (Modified Weiss score: 3/9). Immunohistochemistry showed that the lesion is diffusely positive for inhibin and patchy positive for synaptophysin. Staining for calretinin and chromogranin is negative. MIB1 rate is <1%. The follow-up CT scan showed no evidence of recurrence.

Discussion: The radiological features of the lesion were not suggestive of ACC. The Surgical decision was made based on secretory nature of the nodule. It might be advisable to revise the radiological diagnostic criteria in future with emerging new radiological techniques.