Pheochromocytoma might be associated with hypercortisolism and post-operative adrenal insufficiency. The aim of this study is to determine the frequency of cortisol dysregulation before and after pheochromocytoma surgery.
Single center retrospective study of consecutive pheochromocytoma patients investigated in the Endocrinology department of Cochin Hospital before and after surgery from 2008 to 2020. Demographic characteristics, medical history, treatment, clinical examination, genetic analysis, hormone levels (dexamethasone suppression test, urinary free cortisol, plasma cortisol levels, preoperatively and ACTH-stimulation test postoperatively) as well as histology reports were reviewed.
187 patients with pheochromocytoma underwent workup and surgery between 2008 and 2020. 168 patients were analyzed (19 cases excluded for insufficient data). Median age was 53 years old (1889). 70 (42%) were men, 98 (58%) women. 8 patients (4%) had bilateral pheochromocytomas. Prior to admission, 50% of patients were on antihypertensive treatment and 25% had diabetes. The majority of pheochromocytomas were incidentalomas (87 cases, 52%), 37 patients (22%) presented with classic symptoms and 12 (7%) were diagnosed during a blood pressure workup. The other 12.5% (21 patients) presented with cardiovascular manifestations such as cardiac arrest and Takotsubo cardiomyopathy whereas 11 patients (6.5%) were found positive upon genetic disease monitoring (VHL, MEN2, NF1). Preoperative workup showed abnormal dexamethasone suppression test (cortisol >50 nmol/l) in 30/168 (18%) and high urinary free cortisol in 18/168 (11%). 1 patient had adrenal insufficiency before surgery. 32 patients (19%) presented postoperative adrenal insufficiency: in 15 cases (9%), adrenal insufficiency was primary (due to bilateral adrenalectomy or previous unilateral adrenalectomy) and in 13 (7.7%) of central origin. 7/13 patients with post-operative corticotroph deficiency presented pre-operative biological hypercortisolism, while 6/13 had no abnormal preoperative investigations (4/6 having not received post-operative glucocorticoid before post-operative assessment). Histology showed coexistence of adrenocortical adenoma and pheochromocytoma in the same adrenal gland in 5 patients (3%), of whom 1 had signs of Cushing, elevated urinary free cortisol preoperatively, and adrenal insufficiency postoperatively. In 1 other case, pheochromocytoma cells stained with anti-ACTH antibody (this patient also presented signs of Cushing and elevated cortisol levels).
Possible cortisol excess is present in up to 18% of pheochromocytomas while demonstrated Cushing with histologic evidence for adrenal or ectopic Cushing and post-operative corticotroph deficiency is present in less than 5% of the cases. Post-operative glucocorticoid replacement therapy has to be discussed on an individual basis taking in account clinical and systematic hormonal pre-operative assessment.
22 May 2021 - 26 May 2021