Endocrinology Fellow Stony Brook Medicine Stony Brook, New York, United States
Introduction : Ectopic ACTH secretion is a rare cause of hypercortisolism primarily treated with surgical resection.Octreotide, a somatostatin analogue,is usedin the medical management of Cushing Disease.Though it is considereda potential therapy for ectopic ACTH secretion,data supporting its use is lacking. In this case, we describethe successfulreduction in serum cortisol with subcutaneous octreotidein a patient with an ACTH-secreting pulmonary carcinoid tumor.
Case(s) Description : A 72-year-old femalewith lung carcinoidandmetastasis to the liver was found to have severe hypercortisolismby her outside endocrinologist.Upon her initialoutpatientworkup, labs demonstrated anACTH 600-700pg/mL, serum cortisol >100 mcg/dL, and urine free cortisol >7,000 mcg/24 hours. Given her extensive metastatic disease, surgical resection was not an option.Her endocrinologist initiated ketoconazole 400mgtwice daily with an unknown response. One month later, she presented to our hospital with septic shock and acute hypoxic respiratory failure requiring vasopressors and mechanical ventilation.Upon admission to the intensive care unit, she was noted to have significanttransaminitis(ALT 471 IU/L, AST 519 IU/L),and herserum cortisol was elevated to 192.4 mcg/dL.Due to her acuteliver disease, ketoconazolewas discontinued and she was started on an etomidate infusion.She was also started on P</span>neumocystis jirovecii prophylaxis withatovaquone 750 mg twice daily.After six consecutive days of the etomidate infusion, her serum cortisol level decreased to19.2 mcg/dL. Given planned extubation, the etomidate infusionwastransitioned to octreotide 50 mcg subcutaneously every twelvehours, intended toserve asabridge therapywhile awaiting the arrival of osilodrostat from an outside pharmacy.Unfortunately, the patient expired 72 hoursafter initiation ofoctreotide due to septic shock from Pseudomonas aeruginosa.However, during that time (while receiving octreotide),her serum cortisol levels remained stable, around 20 mcg/dL.
Discussion : In this case,octreotide was demonstrated to be just as effective as etomidate at maintaining suppression of cortisol levels.To our knowledge, there have been no clinical trials examining octreotide use in ectopic ACTH secretion, only case reports, and the Endocrine Society 2015 Clinical Practice Guidelines do not fully endorse its use.While off-label, octreotide may offer a therapeutic alternative to those with ectopic ACTH secretiondeemed not a surgical candidate.
