Revised text to state that multiple endocrine neoplasia type 1 (MEN1), multiple endocrine neoplasia type 2 (MEN2), multiple endocrine neoplasia type 4 (MEN4), familial pheochromocytoma (PHEO) and paraganglioma (PGL) syndrome, Carney-Stratakis syndrome, and familial nonmedullary thyroid cancer are covered in this summary.
Added text to state that MEN4 is a rare syndrome with clinical features that overlap with the other MEN syndromes; the most common features are primary hyperparathyroidism and pituitary adenomas. MEN4 is caused by germline pathogenic variants in the CDKN1B gene.
Added text to state that parathyroid and pituitary tumors associated with MEN4 are managed surgically, in accordance with treatment for other familial syndromes such as MEN1.
Added text to state that MEN2B is sometimes referred to as MEN3 and that MEN4 was described as a novel syndrome in humans in 2011, with the major characteristics including primary hyperparathyroidism and pituitary adenomas; MEN syndrome–associated tumors usually manifest themselves by overproduction of hormones, tumor growth, or both.
Added text to state that a study has shown that the incidence of duodenopancreatic neuroendocrine tumors may be as great as twofold higher in young patients with pathogenic variants in exon 2 of MEN1; these individuals are also more likely to have more aggressive disease and distant metastases (cited Christakis et al. as reference 18).
Added text to state that preoperative knowledge of a germline pathogenic variant significantly affects variables associated with a cortical-sparing adrenalectomy. Preserving the cortex is important in patients with a known pathogenic variant because they are at risk of developing a contralateral tumor. Cortical sparing reduces the possibility of future adrenal insufficiency with contralateral adrenalectomy. This consideration must be weighed against the high risk of malignancy in SDHB carriers. In one study cohort of 108 patients, 33% of patients with a germline pathogenic variant did not have a family history of an inherited syndrome, and 36% of the patients with SDHB germline pathogenic variants had no family history and no previous history of PGL/PHEO on presentation (cited Nockel et al. as reference 86).
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