SUN-399 The Utility of Adrenal and Ovarian Venous Sampling in a Progesterone-Producing Adrenal Tumor

Background: A clinical case presenting secondary amenorrhea accompanied by an adrenal adenoma and hyperprogesteronemia is described in this study. Clinical Case: A 33-year-old woman was admitted on November 23, 2017 because of a menstrual disorder that had persisted for 15 years. First menarche had occurred at 12 years old; however, the patient had been suffering from delayed menstruation since 2002, her last menstruation occurred in October, 2014, and she had no children after marriage. A physical examination indicated she was a young, thin woman (weight:55 kg, height:165 cm) with blood pressure varying between 95-120/60-80 mmHg. There was no evidence of Cushing syndrome or virilization. A serum steroid test revealed elevated progesterone and 17α-hydroxyprogesterone (17 α -OHP) levels. This lead to the suspicion of congenital adrenal hyperplasia (CAH) as the cause of the symptoms, and, accordingly, 5mg of prednisone acetate per day and estradiol as well as dydrogesterone replacement were prescribed. However, menstruation still did not occur. The patient was then admitted to the Department of Endocrinology, where a medium-dose dexamethasone suppression test revealed her plasma progesterone, 17 α -OHP and cortisol were not suppressed while ACTH was suppressed under 5 pg/ml by administration of 3 mg dexamethasone. A pelvic ultrasound showed that the patient had a normal uterus and ovaries with several follicles. Subsequent computed tomography (CT) of the adrenal glands revealed a 2.6 cm mass with inhomogeneous enhancement in the right adrenal gland. Combined adrenal and ovarian venous sampling was performed. The sampling indicated overproduction of progesterone, 17 α -OHP and cortisol in the right adrenal gland compared to the left gland. The patient underwent laparoscopic resection of the right adrenal tumor, which was suspected of producing the excess progesterone, 17 α -OHP and cortisol. A well-encapsulated tumor measuring 2.8 cm×3 cm was removed. Pathology was reviewed by an experienced endocrine pathologist, which suggested adrenocortical adenoma. Progesterone and 17 α -OHP levels normalized immediately after resection of the adrenal tumor. The patient’s menstruation reoccurred 26 days after resection, and she was pregnant 3 months postoperatively. Conclusions: Progesterone-producing tumors are easily overlooked in the differential diagnosis of amenorrhea since it is very rare in routine clinical practice. However, diagnosis of progesterone-producing tumors should be considered with the presence of an adrenal mass; furthermore, ovarian and adrenal venous catheterization and sampling may be highly valuable in the diagnosis of patients presenting small adrenal tumors.