Supplementary Material for: Cushing’s syndrome due to a renal neuroendocrine tumor: Case Report

Introduction: Cushing’s Syndrome (CS) due to ectopic adrenocorticotrophic hormone (ACTH) is rare and usually due to neuroendocrine neoplasia (NEN). Primary renal NEN is exceptionally rare but may be a cause of rapidly progressive CS.
Case presentation: A 51-year-old man presented with profound hypokalemia, cellulitis and new-onset type 2 diabetes and hypertension with a one-month of muscle weakness, labile mood, and insomnia. CS due to ectopic ACTH production was confirmed. Biochemical control was achieved using a ‘block and replace’ regimen with dual blockade with ketoconazole and metyrapone and hydrocortisone replacement in addition to mineralocorticoid receptor blockade using spironolactone. CT and ultrasound demonstrated a 24mm right renal lesion with features concerning for renal cell carcinoma. Right laparoscopic nephrectomy was performed. Histology demonstrated a WHO grade one NEN with ACTH staining.
Conclusion: In CS where the source of ectopic ACTH production is unable to be identified, a renal source should be considered. Diagnosis may be difficult as there are no reliable radiological characteristics to distinguish renal NENs from renal cell carcinomas, so a high degree of suspicion is required.