Young, William F.

Abstract Disclosure: A.Y. Chang: None. D.Z. Erickson: None. W.F. Young: None. J.C. Andrews: None. E. Kapoor: None. Background: While mild androgen excess is frequently encountered in endocrine practices, severe symptoms, rapid onset or markedly abnormal biochemical testing lead to further evaluation for rare ovarian androgen-producing tumors, ovarian hyperthecosis or androgen-secreting adrenal tumors. Combined ovarian and adrenal vein sampling (COAVS) might be necessary to identify the source of androgen hypersecretion and guide treatment. The utility of COAVS has been studied in limited case series, combining case reports and different centers. There is no consensus regarding the testosterone gradient that localizes the androgen source or the added value of COAVS when a pathologic source of androgen excess is suspected. The additional clinical benefit of adrenal vein sampling (AVS) when combined with ovarian vein sampling (OVS) has also not been examined. Objectives: We sought to determine: a) the optimal total testosterone (TT) gradient for identifying unilateral vs bilateral source of hyperandrogenism and b) the utility of AVS in addition to OVS. Methods: We performed a retrospective chart review of all adult women who had COAVS at Mayo Clinic, Rochester from 1996 to 2023. Pathologic sources of androgen excess were defined as ovarian hyperthecosis (OH) as well as ovarian and adrenal tumors. We calculated the ratio of dominant OV or AV divided by the nondominant OV or AV. Results: 22 COAVS procedures from 7 premenopausal and 15 postmenopausal women were included in the study: 12 (55%) women had adrenal abnormalities seen on computed cross sectional imaging and 5 (23%) had unilateral ovarian imaging abnormalities. Of the 22 participants in the case series, successful 4-vessel catheterization was completed in 20 (1 right ovarian vein could not be cannulated, and 1 had bilateral oophorectomy). Nine (41%) women had unilateral and 10 (45%) bilateral ovarian androgen hypersecretion. Eleven (50%) women had pathologic causes for elevated TT (5 ovarian tumors, 6 cases of OH), 5 lacked follow-up pathology and 5 had benign pathology. AVS did not diagnose an adrenal source of androgen hypersecretion. TT concentrations were significantly greater in patients with unilateral compared to those with bilateral ovarian androgen hypersecretion (median 262.5 ng/dL [245- 401.5 IQR] vs 104 ng/dL [82-190], P = 0.01). In addition, TT concentrations were higher in those women with a pathologic source of TT hypersecretion compared to those with benign ovaries (349 ng/dL [251-538] vs 115 ng/dL [93-195], P = 0.03). All cases of unilateral ovarian disease had a dominant OV gradient of TT ≥ 2.1 compared to the nondominant OV. Conclusion: In a series of successful COAVS in women with androgen hypersecretion, an ovarian side-to-side gradient of ≥ 2.1 diagnosed all sources of unilateral ovarian pathology. AVS did not diagnose any pathologic source of adrenal androgen excess in this patient cohort. Presentation: 6/3/2024

Abstract Objective: The detection and management of concomitant pheochromocytoma (PHEO) and primary aldosteronism (PA) is not well understood. Our objectives were to investigate varying presentations and outcomes of cases with coexisting PHEO and PA to provide an approach to its diagnosis and management. Design: Retrospective case series from 2000–2020 at a single institution tertiary center; additional review of previously known cases before 2000 and from the medical literature. Patients and Measurements: Adult patients with concomitant PHEO and PA. Clinical, biochemical, radiologic, and histologic parameters were reviewed. Results: Fifteen patients (53% men, median age 53 years) were diagnosed with concomitant PHEO and PA. The majority presented with hypertension (13, 87%) and hypokalemia (13, 87%), but only 6 (40%) presented with symptoms suggestive of catecholamine excess. All patients with preoperative work-up for catecholamine excess (14, 93%) were found to have elevated plasma or urinary metanephrines/catecholamines above the upper limit of normal. Adrenal vein sampling (AVS) was performed in 9 (60%) patients, where 5 (56%) were diagnosed with bilateral PA, and 4 (44%) with unilateral PA. All patients underwent either unilateral (12, 80%) or bilateral (3, 20%) adrenalectomy to treat their PHEO and/or PA. Postoperative catecholamines and/or catecholamine breakdown products normalized or improved in 13 (87%) patients and were not measured in 2. Recurrence of PHEO was not observed. Six (40%) displayed persistent PA postoperatively, where 4 required long-term mineralocorticoid blockade. Conclusions: Concomitant PHEO and PA is a rare but likely under-reported condition. Hypertension with or without hypokalemia should prompt evaluation for PA, while any indeterminate adrenal mass should be worked up for PHEO. Coexisting disease warrants consideration of AVS to determine the laterality of PA to ensure appropriate management.