Ectopic Adrenocorticotropic Hormone Production: A Case of Neuroendocrine Cervical Small Cell Carcinoma Presenting as Cushing Syndrome

ABSTRACT Objective: To present a case of Cushing syndrome caused by a rare, ectopic, adrenocorticotropic hormone (ACTH)-secreting neuroendocrine small cell carcinoma of the cervix. Methods: A differential diagnosis was considered in a young female with ectopic ACTH-dependent Cushing syndrome. Results: A 35-year-old female presented with multiple complications of Cushing syndrome including hypertension, diabetes mellitus, weight gain, and profound lower-extremity weakness. Serum ACTH was elevated and dexamethasone suppression testing with both 1 mg and 8 mg failed to suppress cortisol. Pituitary magnetic resonance imaging and computed tomography of the adrenals were negative. Positron emission tomography and computed tomography revealed a 5.4 × 5.3-cm hypermetabolic pelvic mass with metastases to the lungs. Cervical biopsy confirmed small cell carcinoma of the cervix. The patient underwent1 round of chemotherapy however later passed away due to septic shock and multiple organ failure. Conclusion: Neuroendocrine small cell carcinoma of the cervix is a rare presentation of Cushing syndrome and should be considered in the differential diagnosis of a young female presenting with ACTH-dependent hypercortisolism. Abbreviations: ACTH = adrenocorticotropic hormone;CT = computed tomography