Cervical Small Cell Neuroendocrine Carcinoma With Ectopic ACTH Secretion and Acute Pancreatitis

Abstract

Ectopic Cushing’s Syndrome from a primary carcinoma of the cervix is an exceedingly rare phenomenon. The objective of this review is to describe our clinical case and summarize the clinical characteristics, investigations, and outcomes of all published cases. We describe a previously healthy 39-year-old female presenting with abnormal uterine bleeding, leading to a diagnosis of stage IIIC1 small cell neuroendocrine carcinoma of the cervix, HPV-associated. She was treated with neoadjuvant chemotherapy, radical hysterectomy, adjuvant chemotherapy, and pelvic radiation. Unfortunately, she developed progressive disease with multifocal hepatic metastases and lesions in the para-aortic lymph nodes. Eighteen months after her initial diagnosis, she presented with severe Cushing’s Syndrome and acute pancreatitis, and died from a splenic artery pseudoaneurysm. Literature review identified 10 published cases of ectopic ACTH secretion in the setting of carcinoma of the cervix. The mean age of presentation was 40. The majority (70%) occurred in the small cell neuroendocrine variant. In 80% of patients, Cushingoid features developed concomitantly or shortly after the discovery of metastatic disease; Cushingoid features included new-onset hypertension (30% of patients), hyperglycemia (50%), and hypokalemia (50%). All 10 patients died of the disease with a median survival of 2.5 months (range: 1 - 10 months) from the time of presentation with Cushing’s Syndrome. Carcinoma of the cervix is a rare cause of ectopic Cushing’s Syndrome. Nevertheless, it should be considered as a source of ectopic ACTH secretion in all women, especially those presenting with gynecologic complaints. Despite surgical resection and systemic therapy, prognosis is poor.