endocrine and metabolic disorders
Multiple Endocrine Neoplasia, Type 1 (MEN 1)
Treatment of MEN 1
Surgical excision when possible Pharmacologic management of endocrinopathies Treatment ofhyperparathyroidismis primarily surgical, with subtotal parathyroidectomy; however, hyperparathyroidism frequently recurs.Octreotideandcinacalcetmay help control recurrent or persistent postoperative hypercalcemia. Prolactinomais usually managed withdopamineagonists; other pituitary tumors are treated surgically. are more difficult to manage because the lesions are often small and difficult to find, multiple lesions are common, and surgery often is not curative. Small (< 2 cm) nonfunctioning pancreatic islet cell tumors may be managed by active surveillance; risk of hepatic metastases is significantly higher for tumors > 3 cm ( Treatment references ). The treatment ofgastrin-secreting gastroenteropancreatic neuroendocrine tumors (GEP-NETs)is complex. When possible, the tumor is located and removed. Larger tumors have a higher incidence of metastasis, so gastrinomas should be removed when they are smaller than a threshold, typically between 2 and 3 cm. If localization and/or removal is impossible, a proton pump inhibitor frequently provides long-term control of symptomatic peptic ulcer disease. In patients withinsulinomas,if a single tumor cannot be found, distal subtotal pancreatectomy with enucleation of any palpable tumors in the head of the pancreas is recommended.Diazoxideor a somatostatin analogue (octreotide,lanreotide) may help treat hypoglycemia.Streptozocinor other cytotoxic drugs may relieve symptoms by reducing tumor burden. Somatostatin analogs also can block hormone secretion from other nongastrin-secreting pancreatic tumors, including carcinoid tumors, and are well tolerated. Palliative treatments for metastatic pancreatic tumors include hepatic debulking surgery and hepatic artery chemoembolization.Streptozocin,doxorubicin, and other cytotoxic drugs may relieve symptoms by reducing tumor burden. Because MEN 1 tumors do not occur simultaneously, patients tend to have a high fear of disease occurrence for additional tumors in themselves or for disease beginning in their relatives. Studies indicate that this leads to lower quality of life (as measured by emotional and physical well-being), suggesting that medical care should also address and treat the psychological burden of this condition ( Treatment references ). 1.Nell S, Verkooijen HM, Pieterman CRC, et al: Management of MEN1 related nonfunctioning pancreatic NETs: A shifting paradigm. Results from the Dutch MEN1 Study Group.Ann Surg267(6):1155-1160, 2018. 2.Leeuwaarde R Pieterman CRC, Bleiker EMA, et al: High fear of disease occurrence is associated with low quality of life in patients with multiple endocrine neoplasia type 1: Results from the Dutch MEN1 Study Group.J Clin Endocrinol Metab103(6): 2354–2361, 2018. 