[Ectopic ACTH secretion by neuroendocrine carcinoma of the uterine cervix].

Abstract

Cushing's syndrome due to ectopic ACTH secretion (EAS) is a consequence of deregulated ACTH secretion by neuroendocrine neoplasms with different locations, most commonly in the thorax. Neuroendocrine tumors represent less than 2% of all invasive cervical cancers. They tend to be highly aggressive, showing rapid growth and distant metastasis at the time of diagnosis. We present a 25-year-old female, with no relevant medical history, who came to our hospital with 1-monthold acne-like lesions on the face, neck, and chest, facial edema, blurred vision, and progressed with delirium, incoherent speech, and suicidal ideation. Hypertension, hypokalemia, hyperglycemia, and metabolic alkalosis were found. The condition was interpreted as a severe, rapid-onset Cushing's syndrome, with significant compromise in general condition. The syndrome and its dependence on ACTH were confirmed by biochemical tests. Computed tomography revealed multiple lesions in the liver and spine suggesting metastatic cancer. After reporting a history of postcoital bleeding, a speculoscopy was performed, and a biopsy was taken of the cervix, as well as the liver lesions. In both cases, histopathological findings revealed the presence of small cell neuroendocrine carcinoma, and chemotherapy with cisplatin and etoposide was initiated. This case represents an example of an uncommon disease that usually implies an important diagnostic and therapeutic challenge. The severity of this condition may be a consequence of the aggressiveness of the ACTH-secreting tumor and/or hypercortisolism with its multiple complications.