Multiple endocrine neoplasia type 4

Multiple endocrine neoplasia type 4

Described in 2006, this is an exceptionally rare autosomal dominant syndrome consisting of MEN1 -associated tumours (parathyroid and pituitary) in association with tumours of the kidney , adrenal glands and reproductive organs. P-NETs have also been described. The disease is thought to be due to mutations in CDNK1B . Treatment is the same as for MEN /uni00A0 1. Alesina PF , Hommeltenberg S, Meier B et al . Posterior retroperitoneoscopic adrenalectomy for clinical and subclinical Cushing’s syndrome. World J Surg 2010; 34 : 1391–7. American Thyroid Association Guidelines Task Force, Wells SA, Asa SL, Dralle H et al. Revised American Thyroid Association guidelines for the management of medullary thyroid carcinoma. Thyroid 2015; 25 : 567–610. Andrew A, Kramer B, Rawdon BB. The origin of gut and pancreatic neuroendocrine (APUD) cells—the last word? J Pathol 1998; 186 : 117–18. Bornstein SR, Allolio B, Arlt W et al . Diagnosis and treatment of primary adrenal insu ffi ciency: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab 2016; 101 : 364–89. Burton TJ, Mackenzie IS, Balan K et al . Evaluation of the sensitivity and specificity of (11)C-metomidate positron emission tomography (PET)-CT for lateralizing aldosterone secretion by Conn’s adenomas. J Clin Endocrinol Metab 2012; 97 : 100–9. Dekkers T , Prejbisz A, Kool LJS et al . Adrenal vein sampling versus CT scan to determine treatment in primary aldosteronism: an outcome-based randomised diagnostic trial. Lancet Diabetes Endocrinol 2016; 4 : 739–46. Fassnacht M, Arlt W , Bancos I et al . Management of adrenal incidentalomas: European Society of Endocrinology clinical practice guideline in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol 2016; 175 : G1–34. - Fassnacht M, Dekkers O, Else T et al . European Society of MEN2 Endocrinology clinical practice guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol 2018; 179 : G1–46. Fave GD, Delle Fave G, O’Toole D et al . ENETS consensus guidelines update for gastroduodenal neuroendocrine neoplasms. Neuroendocrinology 2016; 103 : 119–24. Gagner M, Lacroix A, Bolté E. Laparoscopic adrenalectomy in Cushing’s syndrome and pheochromocytoma. N Engl J Med 1992; 327 : 1033. Nanba K, Omata K, Else T et al . Targeted molecular characterization of aldosterone-producing adenomas in white Americans. J Clin Endocrinol Metab 2018; 103 : 3869–76. Omata K, Satoh F , Morimoto R et al . Cellular and genetic causes of idiopathic hyperaldosteronism. Hypertension 2018; 72 : 874–80. Osswald A, Quinkler M, Di Dalmazi G et al . Long-term outcome of primary bilateral macronodular adrenocortical hyperplasia after unilateral adrenalectomy . J Clin Endocrinol Metab 2019; 104 : 2985– 93. O’Toole D, Kianmanesh R, Caplin M. ENETS 2016 consensus guidelines for the management of patients with digestive neuroendocrine tumors: an update. Neuroendocrinology 2016; 103 : 117–18. Patel N, Egan R, Scott-Coombes D, Stechman M. Adrenalectomy in the UK: results from the British Association Endocrine and thyroid surgeons UKRETS database. Eur J Surg Oncol 2017; 43 : 2398. Ra ff H, Sharma ST , Nieman LK. Physiological basis for the etiology , diagnosis, and treatment of adrenal disorders: Cushing’s syndrome, adrenal insu ffi ciency , and congenital adrenal hyperplasia. Compr Physiol 2014; 4 : 739–69. Thiesmeyer JW , Ullmann TM, Stamatiou AT et al . Association of adrenal venous sampling with outcomes in primary aldosteronism for unilateral adenomas. JAMA Surg 2021; 156 : 165–71.