A rare case of ectopic adrenocorticotropic hormone syndrome in small cell carcinoma of the vagina: A case report

OBJECTIVE. To describe ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) to increase awareness of this condition when treating patients with gynecological small cell carcinoma (SmCC). MATERIALS AND METHODS. This is a review of a 61-year-old woman with primary vaginal SmCC and an atypical presentation of Cushing syndrome. This case describes the molecular rationale, caveats in clinical presentation, pathological diagnosis, and management options for patients with this rare syndrome. RESULTS. After treatment with primary chemotherapy, the patient presented acutely with delirium and metabolic disturbances. This was associated with elevated ACTH and gastrin levels without any tumor staining for ACTH. The patient was initially managed with ketoconazole to control the EAS. Complications developed related to her Cushing syndrome, and she succumbed to complications of her disease. CONCLUSIONS. Vaginal SmCC complicated by EAS is a rare paraneoplastic syndrome, and this case history outlines the management options for patients with this condition and reviews the pertinent literature.