Emmanuelle Debillon1, Fritz-Line Velayoudom-Cephise1, Sylvie Salenave1, Philippe Caron1, Philippe Chaffanjon1, Tristan Wagner1, Maximilien Massoutier1, Benoit Lambert1, Marine Benoit1, Jacques Young1, Antoine Tabarin1, Olivier Chabre1. 1. Service d'Endocrinologie-Diabétologie-Nutrition (E.D., M.B., O.C.), Service de Chirugie Endocrine et Thoracique (P.Ch.), and Centre d'Investigation (M.M.), Clinique, Centre Hospitalier Universitaire de Grenoble, F-38043 Grenoble, France; Service d'Endocrinologie (F.V.-C., A.T.) and Service de Chirurgie Digestive et Endocrinienne (T.W.), Centre Hospitalier Universitaire de Bordeaux, F-33600 Pessac, France; Service d'Endocrinologie et des Maladies de la Reproduction and Centre de Référence des Maladies Endocriniennes Rares de la Croissance (S.S., J.Y.), Assistance Publique-Hôpitaux de Paris, Hôpital de Bicêtre, and Service de Chirurgie Digestive et Endocrinienne (B.L.), Hôpital Bicêtre F-94275 Le Kremlin-Bicêtre, France; and Service d'Endocrinologie et Maladies Métaboliques (P.Ca.), Centre Hospitalier Universitaire Larrey, F-31059 Toulouse, France.
Abstract
CONTEXT: Bilateral adrenalectomy is the reference treatment for Cushing's syndrome (CS) related to primary bilateral macronodular adrenal hyperplasia (PBMAH). It is, however, responsible for definitive adrenal insufficiency. OBJECTIVE: The objective of the study was to evaluate the clinical interest of unilateral adrenalectomy (UA) of the larger gland for the treatment of CS related to PBMAH. DESIGN, SETTING, PATIENTS, AND INTERVENTION: This was a retrospective study in four tertiary French centers including all 15 patients with PBMAH and CS who underwent UA of the larger gland between 2001 and 2015. MAIN OUTCOME MEASURES: Urinary free cortisol, plasma cortisol, ACTH, body mass index, blood pressure, plasma glucose, and lipids were registered pre- and postoperatively and on follow-up. Median follow-up was 60 months (interquartile range 39-105), including 8 of 15 patients followed up for at least 5 years. RESULTS: A normal or low urinary free cortisol was obtained in 15 of 15 patients (100%) postoperatively. Six patients (40%) became adrenal insufficient, of whom three of six recovered a quantitatively normal cortisol secretion on follow-up. Decrease of both body mass index and blood pressure were observed at 1 year, and decrease of blood pressure was persistent 5 years postoperatively. Diabetes was cured in four of six patients. Two patients experienced a recurrence of hypercortisolism, and one was treated with mitotane, whereas the other underwent a second adrenal surgery 9 years after initial UA. CONCLUSION: UA induced remission of hypercortisolism in all patients, with sustained significant clinical improvement. The rates of both definitive adrenal insufficiency and 5-year recurrence were low. UA appears an interesting alternative to bilateral adrenalectomy as a first-line treatment in PBMAH responsible for overt CS.
CONTEXT: Bilateral adrenalectomy is the reference treatment for Cushing's syndrome (CS) related to primary bilateral macronodular adrenal hyperplasia (PBMAH). It is, however, responsible for definitive adrenal insufficiency. OBJECTIVE: The objective of the study was to evaluate the clinical interest of unilateral adrenalectomy (UA) of the larger gland for the treatment of CS related to PBMAH. DESIGN, SETTING, PATIENTS, AND INTERVENTION: This was a retrospective study in four tertiary French centers including all 15 patients with PBMAH and CS who underwent UA of the larger gland between 2001 and 2015. MAIN OUTCOME MEASURES: Urinary free cortisol, plasma cortisol, ACTH, body mass index, blood pressure, plasma glucose, and lipids were registered pre- and postoperatively and on follow-up. Median follow-up was 60 months (interquartile range 39-105), including 8 of 15 patients followed up for at least 5 years. RESULTS: A normal or low urinary free cortisol was obtained in 15 of 15 patients (100%) postoperatively. Six patients (40%) became adrenal insufficient, of whom three of six recovered a quantitatively normal cortisol secretion on follow-up. Decrease of both body mass index and blood pressure were observed at 1 year, and decrease of blood pressure was persistent 5 years postoperatively. Diabetes was cured in four of six patients. Two patients experienced a recurrence of hypercortisolism, and one was treated with mitotane, whereas the other underwent a second adrenal surgery 9 years after initial UA. CONCLUSION:UA induced remission of hypercortisolism in all patients, with sustained significant clinical improvement. The rates of both definitive adrenal insufficiency and 5-year recurrence were low. UA appears an interesting alternative to bilateral adrenalectomy as a first-line treatment in PBMAH responsible for overt CS.
Authors: Thomas Szabo Yamashita; Omair A Shariq; Trenton R Foster; Melanie L Lyden; Benzon M Dy; William F Young; Irina Bancos; Travis J McKenzie Journal: World J Surg Date: 2022-10-07 Impact factor: 3.282