Abstract 49: A single centre experience on nodular adrenocortical cushings syndrome

Background: ACTH independent cushings syndrome (CS) is commonly due to adenomas, carcinoma. Nodular adrenal cortical disease (NACD) is under appreciated and often missed. NACD is primarily divided into bilateral micronodular, bilateral macronodular and sporadic nodular which is non-functional (1). Here we describe our single centre experience of 8 cases of either bilateral micro or macronodular adrenal cortical disease. Methods: The relevant clinical, radiological and genetic test related information pertaining to these cases is presented. Classification is made based on radiological and pathological criteria (WHO 2022). Result: Out of 8 cases (5 male, 3 female), 3 cases of carneys complex (Age range:18-25), 3 of are bilateral macronodular (age range:41-66), 1 bilateral micronodular (iMAD) (Age-20) and 1 Mc cune Albright syndrome (MAS) (age-4 months) were noted. 2 out of 3 CNC has PRKAR1A mutation and 1 macronodular has ARMC5 mutation. Subclinical cushings syndrome was noted in the patients with macronodular adrenal disease. Patients with CNC and i MAD presented with frank cushings with near normal adrenal imaging. Rapid cycling of cortisol levels was noted in the subject with i MAD. Conclusion: Pathology of nodular adrenocortical disease and the underlying molecular pathways defects is slowly being understood. While macronodular disease presents with subclinical disease, has easily made out radiological features, micronodular presents with frank clinical disease and near normal adrenal imaging features.