Would you like email updates of new search results? The most common inherited cancer syndromes are hereditary breast and ovarian cancer syndrome, Lynch syndrome (also known as hereditary non-polyposis colorectal cancer), Li-Fraumeni syndrome, PTEN hamartoma tumor syndrome, familial adenomatous polyposis, Von-Hippel Lindau syndrome, and multiple endocrine neoplasia type 1 and type 2. WHAT IS MEN 2? RET proto-oncogene mutations in multiple endocrine neoplasia type 2 and medullary thyroid carcinoma. Endocr Pathol. Multiple endocrine neoplasia type 1: clinical and genetic features of the hereditary endocrine neoplasias. Overview Multiple endocrine neoplasia, type 1 (MEN 1), sometimes called Wermer's syndrome, is a rare disorder that causes tumors in the endocrine … Unable to load your collection due to an error, Unable to load your delegates due to an error. Please enable it to take advantage of the complete set of features! The probability of a de novo pathogenic variant is 5% or less in index cases with MEN 2A and 50% in index cases with MEN 2B. Multiple Endocrine Neoplasia Type 1 MEN1 syndrome is inherited in an autosomal dominant manner. Diagnosis/testing: 2007;9:101–7. MEN1 syndrome is inherited in an autosomal dominant manner. Multiple endocrine neoplasia (MEN) is the name for a group of hereditary illnesses characterized by having more than one tumor of the endocrine organs at a time. -, Bayley JP, Kunst HP, Cascón A, Sampietro ML, Gaal J, Korpershoek E, Hinojar-Gutierrez A, Timmers HJ, Hoefsloot LH, Hermsen MA, Suárez C, Hussain AK, Vriends AH, Hes FJ, Jansen JC, Tops CM, Corssmit EP, de Knijff P, Lenders JW, Cremers CW, Devilee P, Dinjens WN, de Krijger RR, Robledo M. SDHAF2 mutations in familial and sporadic paraganglioma and phaeochromocytoma. Multiple endocrine neoplasia type IIA is an autosomal dominant syndrome of multiple endocrine neoplasms, including medullary thyroid carcinoma (MTC), … All three phenotypes involve high risk for development of medullary carcinoma of the thyroid (MTC); MEN 2A and MEN 2B involve an increased risk for pheochromocytoma; MEN 2A involves an … All three phenotypes involve high risk for development of medullary carcinoma of the thyroid (MTC); MEN 2A and MEN 2B involve an increased risk for pheochromocytoma; MEN 2A involves an increased risk for parathyroid adenoma or hyperplasia. Policy statement update: genetic testing for cancer susceptibility. Endocrine tumors become evident either by overproduction of hormones by the tumor or by growth of the tumor itself. Curr Treat Options Oncol. Pregnancy management: Women with MEN 2 should be screened for pheochromocytoma prior to a planned pregnancy or as early as possible during an unplanned pregnancy. The aim of our website is to direct you as quickly as possible to our support services and to information you can trust on multiple endocrine neoplasia (MEN) syndromes types 1, 2, and 3, medullary thyroid cancer (MTC), and Phaeochromocytoma and Paraganglioma (PPGL) syndromes. GeneReviews - Multiple Endocrine Neoplasia Type 2 Medscape - Multiple Endocrine Neoplasia, Type 2. -. Monitoring for possible hypoparathyroidism in all those who have undergone thyroidectomy and parathyroid autotransplantation. FOIA More than 25 mutations in the RET gene are known to cause a form of multiple endocrine neoplasia called type 2. Pheochromocytomas detected by biochemical testing and radionuclide imaging are removed by adrenalectomy. Long-acting somatostatin analogs can control the secretory hyperfunction associated with carcinoid syndrome. Multiple endocrine neoplasia type 2B is a rare syndrome caused mainly by Met918Thr germline RET mutation, and characterised by medullary thyroid carcinoma, phaeochromocytoma, and extra-endocrine features. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Mirzaa G, Amemiya A, editors. Carcinoid tumors are non-hormone-secreting and can manifest as a large mass after age 50 years. Each child of an individual with MEN1 syndrome has a 50% chance of inheriting the pathogenic variant. RET Gene Analysis in Multiple Endocrine Neoplasia Type 2A, Multiple Endocrine Neoplasia Type 2B, Familial Medullary Thyroid ... Marquard, J. Available, Abermil N, Guillaud-Bataille M, Burnichon N, Venisse A, Manivet P, Guignat L, Drui D, Chupin M, Josseaume C, Affres H, Plouin PF, Bertherat J, Jeunemaître X, Gimenez-Roqueplo AP. Evaluation of relatives at risk: RET molecular genetic testing should be offered to all at-risk members of kindreds in which a germline RET pathogenic variant has been identified. The disease typically involves tumors (overgrowth of tissue) in There are several different combinations of endocrine tumors that are known to occur together, and each of these patterns is categorized as one of several different MEN syndromes. American Society of Clinical Oncology. MEN2 describes a group of disorders that cause one or more glands in the body to develop tumors that produce excess hormones. Multiple endocrine neoplasia typically involves the development of tumors in two or more of the body's hormone-producing glands, called endocrine glands. V109G polymorphism is associated with sporadic MTCs negative for RET mutations, and might influence the clinical course of the patients affected by MTC. Clinical diagnostic criteria for MEN1 syndrome include the presence of two endocrine tumors that are parathyroid, pituitary, or GEP tract tumors. 2009b;71 Suppl 2:131–8. GeneReviews® [Internet]. General Discussion Multiple endocrine neoplasia type 2 (MEN2) is a rare genetic polyglandular cancer syndrome, characterized by the 100% prevalence of medullary thyroid carcinoma (MTC) and an increased risk of develop other specific tumors affecting additional glands of … Agents/circumstances to avoid: Dopamine D2 receptor antagonists and β-adrenergic receptor antagonists present a high risk for adverse reactions in individuals with pheochromocytoma. Treatment of manifestations: Treatment for MTC is surgical removal of the thyroid gland and lymph node dissection. Prolactinomas are imaged by MRI, neuroendocrine tumors (NETs) are detected by somatostatin receptor scintigraphy, and pancreatic endocrine tumors are detected by endoscopic ultrasound. The most common tumors seen in MEN1 involve the parathyroid gland , … Annual biochemical screening for those with a germline RET pathogenic variant whose initial screening results are negative for pheochromocytoma. Bethesda, MD 20894, Copyright Clinical Characteristics. Multiple endocrine neoplasia type 2 (also known as " Pheochromocytoma and amyloid producing medullary thyroid carcinoma", "PTC syndrome," and "Sipple syndrome") is a group of medical disorders associated with tumors of the endocrine system. Genetic counseling: Prenatal testing for pregnancies at increased risk is possible if the RET pathogenic variant has been identified in an affected family member. (University of Washington, Seattle, 1993). Privacy, Help Pagon, R. A. et al.) Non-endocrine tumors include facial angiofibromas, collagenomas, lipomas, meningiomas, ependymomas, and leiomyomas. -, American Society of Clinical Oncology American Society of Clinical Oncology policy statement update: genetic testing for cancer susceptibility. Exp Clin Endocrinol Diabetes. Evaluation of relatives at risk: Because early detection affects management, molecular genetic testing is offered to at-risk members of a family in which a germline MEN1 pathogenic variant has been identified. Proton pump inhibitors or H2-receptor blockers reduce gastric acid output caused by gastrinomas. -, Aiello A, Cioni K, Gobbo M, Collini P, Gullo M, Della Torre G, Passerini E, Ferrando B, Pilotti S, Pierotti MA, Pasini B. -, Al Brahim NY, Rambaldini G, Ezzat S, Asa SL. GeneReviews. American Society of Clinical Oncology. All rights reserved. Multiple endocrine neoplasia type 1 (MEN1) is a hereditary condition associated with tumors of the endocrine (hormone producing) glands. Pregnancy management: Women with primary hyperparathyroidism from any cause are at increased risk of developing preeclampsia; infants born to women with primary hyperparathyroidism should be monitored for postnatal hypocalcemia. Management: In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Mirzaa G, Amemiya A, editors. Multiple endocrine neoplasia type 2 (MEN 2) includes the following phenotypes: MEN 2A, FMTC (familial medullary thyroid carcinoma, which may be a variant of MEN 2A), and MEN 2B. Clipboard, Search History, and several other advanced features are temporarily unavailable. Multiple endocrine neoplasia (MEN) type 1 is a rare genetic disorder characterized by multiple tumors arising from cells of specific neuroendocrine tissues. Medullary thyroid carcinoma in children with multiple endocrine neoplasia types 2A and 2B. Diagnosis/testing: Each child of an individual with MEN1 syndrome has a 50% chance of inheriting the pathogenic variant. Copyright © 1993-2021, University of Washington, Seattle. All three phenotypes involve high risk for development of medullary carcinoma of the thyroid (MTC); MEN 2A and MEN 2B … Multiple endocrine neoplasia type 2 is historically composed of three clinical subtypes, all of which are associated with germline mutations in the RET proto-oncogene. Policy statement update: genetic testing for cancer susceptibility. * Previously listed as test code 93660. Many different types of tumors are associated with multiple endocrine neoplasia. GeneReviews is a registered trademark of the University of Washington, Seattle. A number sign (#) is used with this entry because of evidence that multiple endocrine neoplasia type IV (MEN4) is caused by heterozygous mutation in the CDKN1B gene (600778) on chromosome 12p13. 2008 Dec 31 [updated 2018 Apr 26]. Molecular genetic testing to identify a heterozygous germline RET pathogenic variant is indicated in all individuals with a diagnosis of primary C-cell hyperplasia or MTC or a clinical diagnosis of MEN 2. NCI CPTC Antibody Characterization Program. J Clin Endocrinol Metab. 1999;54:397-438; discussion 438-9. Hyde SM, Rich TA, Waguespack SG, Perrier ND, Hu MI. Elisei R, Bottici V, Cappagli V, Ramone T, Tacito A, Ciampi R, Romei C. Ann Endocrinol (Paris). Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1). The major forms of multiple endocrine neoplasia are called type 1, type 2, and type 4. Growth hormone-secreting tumors causing acromegaly are treated by transsphenoidal surgery; medical therapy for growth hormone-secreting tumors includes somatostatin analogs, octreotide, and lanreotide. Multiple endocrine neoplasia type 2 (MEN2) is an inherited disorder in which … Bethesda, MD 20894, Copyright Kinase inhibitors may be used in metastatic MTC. Prenatal diagnosis for pregnancies at increased risk is possible if the pathogenic variant in a family is known. Neoplasia. 2000;108:128–32. Prevention of primary manifestations: Thymectomy may prevent thymic carcinoid in males, particularly in smokers. 2003. Horm Res. 2005;137:574–6. Approximately 10% of cases are caused by a de novo pathogenic variant. 2016 Fall;62(9 Suppl 3):140-149. 2004 Aug;5(4):315-25. doi: 10.1007/s11864-004-0022-6. External beam radiation therapy or intensity-modulated radiation therapy can be considered for advanced locoregional disease. J Clin Oncol. FOIA In MEN2A, Codon 634 in exon 11 is the most commonly altered codon. -, Agarwal SK, Mateo CM, Marx SJ. [Multiple Endocrine Neoplasia I (Wermers Syndrome), Forms of Clinical Manifestation, 5 Case Studies]. Multiple Endocrine Neoplasia, Type IIB. Prevention of secondary complications: Measure PTH and/or serum calcium to assess for hypoparathyroidism following subtotal or total parathyroidectomy. Multiple endocrine neoplasia type 1 is a rare genetic syndrome, characterized by the co-occurrence, in the same individual or in related individuals of the same family, of hyperparathyroidism, duodenopancraetic neuroendocrine tumors, pituitary adenomas, adrenocortical tumors, and neuroendocrine tumors (carcinoids) in the thymus, the bronchi, or the stomach. Well-differentiated endocrine tumors of the gastro-entero-pancreatic (GEP) tract can manifest as Zollinger-Ellison syndrome (gastrinoma); hypoglycemia (insulinoma); hyperglycemia, anorexia, glossitis, anemia, diarrhea, venous thrombosis, and skin rash (glucagonoma); and watery diarrhea, hypokalemia, and achlorhydria syndrome (vasoactive intestinal peptide [VIP]-secreting tumor). Multiple Endocrine Neoplasia type 1 (MEN1) is a rare hereditary endocrine cancer syndrome characterized primarily by tumors of the parathyroid glands (95% of cases), endocrine gastroenteropancreatic (GEP) tract (30-80% of cases), and anterior pituitary (15-90% of cases). & Eng, C. Multiple Endocrine Neoplasia Type 2. in GeneReviews(®) (eds. 2009a;94:1826–34. [Neural crest and multiple endocrinopathies]. These types are distinguished by the genes involved, the types of hormones made, and the characteristic signs and symptoms. 8600 Rockville Pike Multiple endocrine neoplasia type 1 (MEN1; OMIM 131100) is an autosomal dominant disorder distinguished by tumors of the parathyroid glands, pancreatic islet cells, and anterior pituitary gland (reviewed by Pannett and Thakker Endocrine-Related Cancer 6:449-473, 1999). Adrenocortical tumors can be associated with primary hypercortisolism or hyperaldosteronism. Multiple endocrine neoplasia type 2 (MEN 2) includes the following phenotypes: MEN 2A, FMTC (familial medullary thyroid carcinoma, which may be a variant of MEN 2A), and MEN 2B. Multiple endocrine neoplasia type 1 (MEN1), also called multiple endocrine adenomatosis or Wermer's syndrome, is found in one in 30,000 people. Multiple endocrine neoplasia type 1 (MEN1) syndrome includes varying combinations of more than 20 endocrine and non-endocrine tumors. Horm Res. Multiple Endocrine Neoplasia Type 2 - GeneReviews® - NCBI Bookshelf. Offspring of affected individuals have a 50% chance of inheriting the pathogenic variant. -, Agarwal SK, Ozawa A, Mateo CM, Marx SJ. GeneReviews is a registered trademark of the University of Washington, Seattle. 2004;1014:189–98. 2019 Jun;80(3):187-190. doi: 10.1016/j.ando.2019.04.014. Skinner MA, DeBenedetti MK, Moley JF, Norton JA, Wells SA Jr. J Pediatr Surg. Prevention and treatment information (HHS). Copyright © 1993-2021, University of Washington, Seattle. -, Agarwal SK, Lee Burns A, Sukhodolets KE, Kennedy PA, Obungu VH, Hickman AB, Mullendore ME, Whitten I, Skarulis MC, Simonds WF, Mateo C, Crabtree JS, Scacheri PC, Ji Y, Novotny EA, Garrett-Beal L, Ward JM, Libutti SK, Richard Alexander H, Cerrato A, Parisi MJ, Santa Anna-A S, Oliver B, Chandrasekharappa SC, Collins FS, Spiegel AM, Marx SJ. Treatment of manifestations: Hyperparathyroidism is treated with subtotal parathyroidectomy and cryopreservation of parathyroid tissue or total parathyroidectomy and autotransplantation of parathyroid tissue; calcimimetics are used to treat primary hyperparathyroidism in those for whom surgery is contraindicated or has failed; prior to surgery, bone antiresorptive agents are used to reduce hypercalcemia and limit bone resorption. Rare germline mutations in cyclin-dependent kinase inhibitor genes in MEN1 and related states. Multiple endocrine neoplasia, type 1 (MEN1) is caused by mutations in the … Additional features in MEN 2B include mucosal neuromas of the lips and tongue, distinctive facies with enlarged lips, ganglioneuromatosis of the gastrointestinal tract, and a marfanoid habitus. Please enable it to take advantage of the complete set of features! Located in different parts of the body, these glands control the production of hormones that direct many body processes, including growth, digestion, and sexual function. Prevention of primary manifestations: Prophylactic thyroidectomy for individuals with an identified germline RET pathogenic variant. These conditions usually run in families and can be passed from one generation to the next. National Library of Medicine 1997;47(4-6):168-78. doi: 10.1159/000185461. 2003;21:2397–406. Distribution of menin-occupied regions in chromatin specifies a broad role of menin in transcriptional regulation. Accessibility All rights reserved. Would you like email updates of new search results? There are two types: MEN2A and MEN2B. Pasini A, Michiels FM, Chappuis-Flament S, Geneste O, Rossel M, Fournier L, Feunteun J, Lenoir G, Schuffenecker I, Billaud M. C R Seances Soc Biol Fil. It can affect people of any age, ethnic group or gender. Prevention of secondary complications: Prior to any surgery in an individual with MEN 2A or MEN 2B, the presence of a functioning pheochromocytoma should be excluded by appropriate biochemical screening. Epub 2019 Apr 11. 75% is sporadic and 25% is the dominant component of the hereditary multiple endocrine neoplasia (MEN) type 2 syndromes. Clipboard, Search History, and several other advanced features are temporarily unavailable. Pituitary tumors include prolactinoma (the most common), which manifests as oligomenorrhea/amenorrhea and galactorrhea in females and sexual dysfunction in males. Multiple endocrine neoplasia syndromes are rare, inherited disorders in which several endocrine glands develop noncancerous (benign) or cancerous (malignant) tumors or grow excessively without forming tumors. Multiple Endocrine Neoplasia (MEN) Type 2. The diagnosis of MEN 2 is established in a proband who fulfills existing clinical diagnostic criteria. Available, Agarwal SK, Impey S, McWeeney S, Scacheri PC, Collins FS, Goodman RH, Spiegel AM, Marx SJ. -. Marx SJ, Agarwal SK, Kester MB, Heppner C, Kim YS, Skarulis MC, James LA, Goldsmith PK, Saggar SK, Park SY, Spiegel AM, Burns AL, Debelenko LV, Zhuang Z, Lubensky IA, Liotta LA, Emmert-Buck MR, Guru SC, Manickam P, Crabtree J, Erdos MR, Collins FS, Chandrasekharappa SC. These tumors can be noncancerous or cancerous. Surgery is indicated for insulinoma and most other pancreatic tumors. Unable to load your collection due to an error, Unable to load your delegates due to an error. Multiple endocrine neoplasia (MEN) is a group of disorders that affect the endocrine system, which is made up of glands that produce, store, and release hormones into the bloodstream. Drbalová K, Herdová K, Krejčí P, Nývltová M, Solař S, Vedralová L, Záruba P, Netuka D, Bavor P. Vnitr Lek. Two that have been identified as affecting several endocrine glands are MEN-1 and MEN-2 (Multiple Endocrine Neoplasia, types 1 and 2). 1996;190(5-6):557-67. 2012 Jun 21 [updated 2020 Apr 16]. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Mirzaa G, Amemiya A, editors. Giusti F, Marini F, Brandi ML, Adam MP, Ardinger HH, Pagon RA, et al. Approximately 10% of cases are caused by a de novo pathogenic variant. Recent Prog Horm Res. -, Bartsch DK, Hasse C, Schug C, Barth P, Rothmund M, Höppner W. A RET double mutation in the germline of a kindred with FMTC. There are two GeneReviews for RET gene: Hirschsprung Disease GeneReview and Multiple Endocrine Neoplasia Type 2 GeneReview. Surveillance: Annual measurement of serum calcitonin concentration to detect residual or recurrent MTC after thyroidectomy, even if thyroidectomy was performed prior to biochemical evidence of disease. Subtypes MEN1 and MEN2 are distinguished by clinical features and molecular testing. Biochemical testing detects an increased serum concentration of parathyroid hormone and calcium in primary hyperparathyroidism, increased serum concentrations of prolactin from a prolactinoma, and increased serum concentrations of gastrin, insulin, and VIP from tumors of the GEP tract. Complex endocrinopathies in MEN-1: diagnostic dilemmas in endocrine oncology. Multiple endocrine neoplasia (MEN) syndromes are characterized by tumors involving multiple endocrine glands. Clinical characteristics: 2003. This site needs JavaScript to work properly. MEN1 was originally known as Wermer syndrome. Parathyroid tumors are the main MEN1-associated endocrinopathy; onset in 90% of individuals is between ages 20 and 25 years with hypercalcemia evident by age 50 years; hypercalcemia causes lethargy, depression, confusion, anorexia, constipation, nausea, vomiting, diuresis, dehydration, hypercalciuria, kidney stones, increased bone resorption/fracture risk, hypertension, and shortened QT interval. MTC typically occurs in early childhood in MEN 2B, early adulthood in MEN 2A, and middle age in FMTC. Multiple endocrine neoplasia type 1 (MEN1) is a rare genetic disorder that mainly affects the endocrine glands. Available, Thakker RV, Newey PJ, Walls GV, Bilezikian J, Dralle H, Ebeling PR, Melmed S, Sakurai A, Tonelli F, Brandi ML. National Library of Medicine Ann N Y Acad Sci. For a phenotypic description and a discussion of genetic heterogeneity of multiple endocrine neoplasia, see MEN1 (131100). Molecular genetic testing of MEN1, the only gene in which pathogenic variants are known to cause MEN1 syndrome, detects a heterozygous MEN1 pathogenic variant in approximately 80%-90% of probands with familial MEN1 syndrome and in approximately 65% of simplex cases (i.e., a single occurrence of MEN1 syndrome in the family). Clinical characteristics: Multiple endocrine neoplasia type 2 (MEN 2) includes the following phenotypes: MEN 2A, FMTC (familial medullary thyroid carcinoma, which may be a variant of MEN 2A), and MEN 2B. The endocrine system is the network of glands that secrete hormones into the bloodstream to reach their target organs along the entire body. Multiple endocrine neoplasia type 2 (MEN 2) includes the following phenotypes: MEN 2A, FMTC (familial medullary thyroid carcinoma, which may be a variant of MEN 2A), and MEN 2B. Surgical removal of adrenocortical tumors that exceed 3.0 cm in diameter can prevent malignancy. 1996 Jan;31(1):177-81; discussion 181-2. doi: 10.1016/s0022-3468(96)90343-7. Ocular Features: Corneal nerves are medullated and appear prominent. Surgery. J Clin Endocrinol Metab. ACTH-secreting pituitary tumors associated with Cushing syndrome are surgically removed; nonsecreting pituitary adenomas are treated by transsphenoidal surgery. Surveillance: Serum concentrations of calcium from age eight years, gastrin from age 20 years, and prolactin from age five years; abdominal CT or MRI from age 20 years and head MRI from age five years. Careers. Careers. GeneReviews, 1999 Sep 27 [updated 2019 Aug 15]. Management: Multiple endocrine neoplasia (MEN) syndromes are infrequent inherited disorders in which more than one endocrine glands develop noncancerous (benign) or cancerous (malignant) tumors or grow excessively without forming tumors. This site needs JavaScript to work properly. Molecular pathology of the MEN1 gene. Data are scarce on the natural history of multiple endocrine neoplasia … Multiple Endocrine Neoplasia Type 1. 8600 Rockville Pike Privacy, Help All MEN 2 phenotypes are inherited in an autosomal dominant manner. Measure urinary catecholamines prior to surgery to diagnose and treat a pheochromocytoma to avoid blood pressure peaks during surgery. Lancet Oncol. 2010;11:366–72. The tumors may be benign or malignant (cancer). The MEN1 gene and pituitary tumours. Statement on genetic testing for cancer susceptibility. History Cutaneous tumors in multiple endocrine neoplasia type 1 (MEN1) are of long duration and generally grow slowly or not at all. Clinical utility of genetic diagnosis for sporadic and hereditary medullary thyroid carcinoma. Identification of a heterozygous germline RET pathogenic variant on molecular genetic testing establishes the diagnosis if clinical features are inconclusive. MEN2 includes the additional subtypes MEN2A, MEN2B, and familial medullary thyroid carcinoma (FMTC). American Society of Clinical Oncology. There are 3 famous and well-known forms of MEN syndromes (MEN 1, MEN 2A, and MEN 2B) and a newly documented one (MEN4). Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant familial tumor syndrome (also termed Wermer syndrome) in which persons develop tumors of the parathyroid glands, the enteropancreatic neuroendocrine system, the anterior pituitary gland, and the skin. Clinical characteristics: Consider fasting serum PTH concentration and yearly chest CT. Multiple endocrine neoplasia syndromes are caused … The familial medullary thyroid carcinoma-associated RET E768D mutation in a multiple endocrine neoplasia type 2A case. Prevention and treatment information (HHS). It is caused by mutations in the MEN1 gene, which is a tumor suppressor gene. GeneReviews. Genetic counseling: Search For A Disorder. -, American Society of Clinical Oncology. Multiple endocrine neoplasia. These conditions are related to alterations in specific genes , and they increase the lifetime risk that affected people will develop tumors in one or more of their endocrine glands. Primary hyperparathyroidism is treated with surgery to remove one or more parathyroid glands, or more rarely, with medications to reduce parathyroid hormone secretion. Accessibility Policy statement update: genetic testing for cancer susceptibility. Prolactinomas are treated with dopamine agonists (cabergoline being the drug of choice). 2. TMEM127 screening in a large cohort of patients with pheochromocytoma and/or paraganglioma. 2007;18:37–41. 2012;97:E805–9.

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