The surgical treatment of severe ectopic adrenocorticotrophic hormone syndrome / 中华泌尿外科杂志

ABSTRACT

Objective To investigate the surgical treatment of severe ectopic adrenocorticotrophic hormone(ACTH) syndrome.Methods The clinical data of 12 cases of severe ecotopic ACTH syndrome from January 1996 to December 2016 were retrospectively analyzed.The cases include 7 males and 5 females with a mean age 38 years(range 11 to 64 years).12 cases demonstrated typical Cushing syndrome(CS),accompanied by hypertension, diabetes, hypokalemia and severe osteoporosis.9 cases were complicated with thoracolumbar compression fractures.8 cases presented with pulmonary infection and fever during operation.7 cases had cardiac dysfunction.Laboratory tests showed elevated serum cortisol(695.0 ～ 1 661.5 nmol/L,mean 1 055.7 nmol/L), high urinary free cortisol excretion (807.3 ～ 28 240.0 nmol/24 h, mean 5 270.5 nmol/24h)and high ACTH plasma levels(16.5 ～ 273.9 pmol/L, mean 80.4 pmol/L).Source of ectopic ATCH were not identified and CT showed bilateral enlargement of adrenal in 12 patients.Results We performed emergency bilateral or unilateral adrenalectomy in 12 cases.Among them, one-stage bilateral adrenalectomy were given to 6 cases, staged bilateral adrenalectomy was performed in 3 cases, and unilateral adrenalectomy were given to 3 cases.The patients were treated with hormone replacement therapy after bilateral adrenalectomy.The excised adrenal gland showed diffuse thickening and multiple nodular.Pathological diagnosis were adrenal cortical hyperplasia.They have been followed up for 1 to 8years(median 2.5 years), 9 cases survived, 2 cases died of diabetes and severe pulmonary infection, and 1 case was lost to follow-up.Conclusions Severe ectopic ACTH syndrome is difficult to treat.Emergency adrenalectomy is effective for the management of severe ectopic ACTH syndrome especially for those patients with severe Cushing syndrome but primary tumor can not be located.