Severe ectopic cushing syndrome caused by adenoid cystic carcinoma of a salivary gland.

ABSTRACT

OBJECTIVE: We present a rare case of Cushing syndrome due to ectopic adrenocorticotropic hormone (ACTH) secretion (EAS). To our knowledge only two similar cases have been previously reported. METHODS: This is a case report of EAS by a metastatic lingual adenoid cystic carcinoma (ACC). RESULTS: The patient was diagnosed of a Cushing syndrome caused by tumoral EAS two years after initial cancer diagnosis. Clinical presentation included asthenia, insomnia, hypertension, acne, and hyperpigmentation developing in a period of two months. Laboratory and imaging testing revealed hypokalemic metabolic alkalosis, hypercortisolemia, high ACTH, nonsuppresion by 8 mg dexamethasone, and a normal pituitary magnetic resonance imaging (MRI). With a high clinical suspicion of EAS, combined medical treatment was started but was unsuccessful. Bilateral adrenalectomy could not be performed given the patient's rapid deterioration. Immunostained tissue from the original tumor was positive for synaptophysin. CONCLUSION: This rare case of EAS illustrates the challenge that this condition may confer regarding diagnosis and management.