M. E. Hasassri3, T. K. Pandian1, I. Bancos2, W. F. Young2, M. L. Richards1, D. R. Farley1, G. B. Thompson1, T. J. McKenzie1 1Mayo Clinic,Division Of Subspecialty General Surgery,Rochester, MN, USA 2Mayo Clinic,Division Of Endocrinology, Diabetes, Metabolism, And Nutrition, Department Of Internal Medicine,Rochester, MN, USA 3Mayo Clinic,Mayo Medical School,Rochester, MN, USA
Introduction:
Pheochromocytoma with synchronous adrenal cortical adenoma (PSCA) is a rare condition and may present with mixed clinical and biochemical features characteristic to each neoplasm subtype. We reviewed our experience at a large tertiary referral center to better understand the clinical and perioperative characteristics of this unique clinical entity.
Methods:
With IRB approval, all patients with a pathologic diagnosis of pheochromocytoma with a synchronous ipsilateral adrenal cortical adenoma from January 1994 through June 2015 were identified. Retrospectively extracted data included indications for adrenalectomy, diagnostic work-up (biochemical and radiographic), operative characteristics, pathologic findings, and postoperative complications.
Results:
We identified 16 cases of PSCA among 413 patients undergoing adrenalectomies for pheochromocytoma (3.9%). Mean patient age was 57 years (range: 29-78); 50% were male. Median BMI was 28.5 kg/m2 (IQR 26.8, 30.8). In 12 cases (75%), the initial primary neoplasm was found incidentally on imaging for an unrelated issue. Only 8 cases (50%) were reported on imaging to have a synchronous ipsilateral neoplasm. The preoperative presumptive diagnosis included 9 pheochromocytomas (56%), 4 cortical adenomas (25%), and 3 PSCA (19%). Paroxysmal clinical symptoms of a pheochromocytoma were documented in 13 (81%) patients. Four patients (25%) were diagnosed preoperatively with clinically relevant autonomous cortisol overproduction (3 ACTH-independent, 1 ACTH-dependent). Two patients (13%) were diagnosed preoperatively with renin-independent hyperaldosteronism and both underwent adrenal venous sampling (AVS) with one patient developing hypertensive crisis during AVS. The initial surgical approach was laparoscopic (81%), open (13%), retroperitoneoscopic (6%). One laparoscopic operation was converted to open due to bleeding. No patient required transfusion, postoperative vasopressor support, ICU care, or reoperation. There was no other major morbidity. On pathology, mean pheochromocytoma size was 2.7 cm (range: 0.8-4.8) and mean cortical adenoma size was 1.8 cm (range: 0.3-4.4).
Conclusion:
An ipsilateral cortical adenoma was found in 3.9% of 413 adrenalectomies with a final pathologic diagnosis of pheochromocytoma. Clinically important cortical hormone secretion was diagnosed in 38% of these patients; 25% had glucocorticoid secretory autonomy and 13% had hyperaldosteronism. Physicians should be aware that adrenal neoplasms with mixed findings on imaging and biochemistry may represent synchronous pheochromocytoma and a functional adrenal cortical adenoma. If cross-sectional imaging is suggestive of cortical and medullary neoplasms, biochemical evaluation for this rare co-occurrence to prevent perioperative complications from resection of an unexpected secretory cortical neoplasm should be performed. This should be done cautiously to prevent precipitation of a pheochromocytoma crisis.