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Multiple Endocrine Neoplasia (Multiple Endocrine Adenomatosis)

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A group of autosomal dominant diseases characterized by the combined occurrence of tumors involving two or more ENDOCRINE GLANDS that secrete PEPTIDE HORMONES or AMINES. These neoplasias are often benign but can be malignant. They are classified by the endocrine glands involved and the degree of aggressiveness. The two major forms are MEN1 and MEN2 with gene mutations on CHROMOSOME 11 and CHROMOSOME 10, respectively.

Also Known As:

Multiple Endocrine Adenomatosis; Adenomatosis, Multiple Endocrine; Multiple Endocrine Adenopathy; Multiple Endocrine Neoplasia Syndromes; Multiple Endocrine Neoplasms; Adenomatoses, Familial Endocrine; Adenomatoses, Multiple Endocrine; Adenopathies, Multiple Endocrine; Adenopathy, Multiple Endocrine; Endocrine Adenomatoses, Familial; Endocrine Adenomatoses, Multiple; Endocrine Adenomatosis, Familial; Endocrine Adenomatosis, Multiple; Endocrine Adenopathies, Multiple; Endocrine Adenopathy, Multiple; Endocrine Neoplasms, Multiple; Familial Endocrine Adenomatoses; Familial Endocrine Adenomatosis; Multiple Endocrine Adenomatoses; Multiple Endocrine Adenopathies; Adenomatosis, Familial Endocrine; Endocrine Neoplasia, Multiple; Neoplasia, Multiple Endocrine; Neoplasms, Multiple Endocrine

Networked: 485 relevant articles (6 outcomes, 19 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Hyperplasia
2.	Thyroid Neoplasms (Thyroid Cancer)
3.	Neoplasms (Cancer)
4.	Multiple Endocrine Neoplasia Type 1 (MEN I)
5.	Primary Hyperparathyroidism

Experts

1.	Dralle, Henning: 8 articles (04/2014 - 12/2004)
2.	Takahashi, Masahide: 8 articles (04/2006 - 09/2002)
3.	Stratakis, Constantine A: 7 articles (02/2016 - 01/2006)
4.	Takahashi, M: 6 articles (09/2008 - 02/2000)
5.	Brauckhoff, Michael: 5 articles (05/2014 - 12/2004)
6.	Machens, Andreas: 5 articles (04/2014 - 07/2005)
7.	Murakumo, Yoshiki: 5 articles (04/2006 - 05/2003)
8.	Kawai, Kumi: 5 articles (04/2006 - 09/2002)
9.	Wells, Samuel A: 4 articles (08/2013 - 09/2005)
10.	Kawai, K: 4 articles (09/2008 - 02/2000)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Multiple Endocrine Neoplasia:

1.	Calcitonin (Calcitonin, Eel)FDA LinkGeneric 07/01/2003 - "We reviewed the clinical and laboratory data in six patients (four men and two women, 39 to 76 years old)--three with sporadic MTC, one with familial MTC, and two with multiple endocrine neoplasia type 2A--who had preoperative basal serum calcitonin levels of 400 to 16,000 pg/mL (normal, 0 to 19). " 11/01/1987 - "Group 1 (n = 11) included treated patients with normal calcitonin levels; Group 2 (n = 24) included patients with elevated calcitonin levels due to sporadic and isolated MTC; Group 3 (n = 15) included patients with elevated calcitonin levels due to familial MTC or multiple endocrine neoplasia Type IIA syndrome (MEN). " 12/01/1971 - " pathogenesis of the parathyroid lesions is discussed, and it is suggested that some may be caused by the hypocalcaemic action of calcitonin, while others may be part of a syndrome of multiple endocrine adenomatosis." 11/01/2008 - "We present a retrospective series of 42 prophylactic thyroidectomies for MTC performed for one or more of the following: family history of multiple endocrine neoplasia (MEN) or MTC, elevated serum calcitonin level, or detection of a RET proto-oncogene mutation. " 08/01/1994 - "In addition, the association of multiple endocrine neoplasia syndromes with pheochromocytoma require the clinician's awareness to evaluate patients with pheochromocytoma (especially when bilateral) for abnormalities in thyroidal C-cell function with serum calcitonin determinations. "
2.	GastrinsIBA 01/01/1981 - "The results of this study do not support the view that primary HPT is often associated with a gastrin-producing tumour as part of a multiple endocrine adenomatosis (MEA type I), but indicate that the observed hypergastrinemia in HPT is almost exclusively related to hypo- or achlorhydria. " 01/01/1974 - "Gastrins, multiple endocrine adenomatosis, and the Zollinger-Ellison syndrome." 08/01/1996 - "Serum gastrin and ECL cell populations, which were elevated before treatment, remained statistically unchanged but one of the three multiple endocrine neoplasia I (MEN-I) patients developed a small carcinoid. " 01/01/1993 - "In both rodents and humans the development of gastrin-promoted gastric argyrophil enterochromaffin-like cell carcinoids requires the involvement of a genetic factor inherent to multiple endocrine neoplasia syndrome or of type A autoimmune chronic atrophic gastritis. " 03/15/2000 - "The large majority of gastric neuroendocrine tumors is made by benign, gastrin-dependent, well-differentiated ECL cell growths arising in a background of chronic atrophic gastritis (type I) or, more rarely, associated with type I multiple endocrine neoplasia (MEN I) and Zollinger-Ellison (ZE) syndromes (type II). "
3.	Pancreatic Polypeptide (Pancreatic Polypeptide (PP))IBA 02/05/1982 - "Multiple endocrine adenomatosis type I. Occurrence in an octogenarian with high levels of circulating pancreatic polypeptide." 12/01/1983 - "Pancreatic polypeptide update: its roles in detection of the trait for multiple endocrine adenopathy syndrome, type I and pancreatic polypeptide-secreting tumors." 08/01/1981 - "An apparently complete remission of metastases in the liver from a resected pure pancreatic polypeptide apudoma occurred following intra-arterial and intravenous administration of streptozocin to a patient in a family with multiple endocrine adenopathy, type 1. Plasma concentrations of human pancreatic polypeptide constitute an excellent marker system for the detection and assessment of tumor status before and after surgical and streptozocin therapy." 01/01/1980 - "Elevated basal and responsive (after a meal) plasma concentrations of a relatively new candidate-hormone, human pancreatic polypeptide (hPP), were associated with pancreatic apudoma tumors in three asymptomatic patients with multiple endocrine adenopathies, type I. Two of these patients had excision of the tumors that resulted in normal plasma hPP concentrations postoperatively. " 06/01/2002 - "Ten cases of normal pancreatic tissue, two cases of nesidioblastosis, five insulin-producing tumours and one case of multiple endocrine neoplasia 1 syndrome, containing an insulin-, a somatostatin- and several glucagon-producing tumours, as well as tiny foci of endocrine dysplasia with different predominance of the secreted hormones (mainly glucagon and pancreatic polypeptide) were included in the study. "
4.	Glucagon (Glukagon)FDA Link 04/01/1977 - "Fasting plasma glucagon was elevated in patients with islet cell adenomatosis and in patients with islet cell carcinoma some of whom had multiple endocrine adenomatosis. " 01/01/1981 - "A paradoxical response of plasma gastrin level in the patients with the Zollinger-Ellison syndrome and a marked decrease of plasma gastrin level in the patients with gastric ulcer, duodenal ulcer, excluded gastric antrum, multiple endocrine adenomatosis, pernicious anemia and chronic renal failure were demonstrated by glucagon infusion. " 01/01/1986 - "He was diagnosed as suffering from multiple endocrine adenomatosis (MEA) type 1. Screening of his family members revealed that his mother had high levels of gastrin, glucagon and parathormone and his father had a high level of gastrin. " 06/01/2002 - "Ten cases of normal pancreatic tissue, two cases of nesidioblastosis, five insulin-producing tumours and one case of multiple endocrine neoplasia 1 syndrome, containing an insulin-, a somatostatin- and several glucagon-producing tumours, as well as tiny foci of endocrine dysplasia with different predominance of the secreted hormones (mainly glucagon and pancreatic polypeptide) were included in the study. " 05/01/1988 - "One hundred four endocrine tumors found in the body and tail of the pancreas of a patient with the Zollinger-Ellison syndrome (ZES) and multiple endocrine neoplasia (MEN-I) were investigated by immunohistochemistry for insulin, glucagon, somatostatin, pancreatic polypeptide (PP), and gastrin. "
5.	Streptozocin (Streptozotocin)FDA Link 08/01/1981 - "An apparently complete remission of metastases in the liver from a resected pure pancreatic polypeptide apudoma occurred following intra-arterial and intravenous administration of streptozocin to a patient in a family with multiple endocrine adenopathy, type 1. Plasma concentrations of human pancreatic polypeptide constitute an excellent marker system for the detection and assessment of tumor status before and after surgical and streptozocin therapy."
6.	Propranolol (Inderal)FDA LinkGeneric 07/01/1990 - "Adrenal medullary hyperplasia was suspected in one patient, whose first cousin had multiple endocrine neoplasia type IIa, and in two others, all of whom have experienced relief from symptoms during propranolol or atenolol administration. "
7.	Atenolol (Tenormin)FDA LinkGeneric 07/01/1990 - "Adrenal medullary hyperplasia was suspected in one patient, whose first cousin had multiple endocrine neoplasia type IIa, and in two others, all of whom have experienced relief from symptoms during propranolol or atenolol administration. "
8.	familial medullary Thyroid cancerIBA 03/31/1990 - "The study contributed to the diagnosis of multiple endocrine neoplasia type IIa, whereas the condition had been diagnosed for 6 years as familial medullary thyroid carcinoma, without phaechromocytoma. " 01/01/2013 - "Hereditary MTC appears as part of the multiple endocrine neoplasia syndrome type 2A or 2B, or familial medullary thyroid cancer. " 01/01/2012 - "Some may occur spontaneously or can be associated with Multiple Endocrine Neoplasia syndromes, or Familial Medullary Thyroid Cancer syndrome. " 03/01/2009 - "Activating mutations in the Ret proto-oncogene are responsible for occurrence of multiple endocrine neoplasia (MEN) type 2A and 2B, and familial medullary thyroid carcinoma (FMTC). " 09/01/2008 - "It may occur either sporadically (75%) or in familial forms (25%) in familial medullary thyroid carcinoma and multiple endocrine neoplasia types 2A and 2B. "
9.	HormonesIBA 06/01/2005 - "Multiple endocrine neoplasia (MEN) syndromes represent familial disorders characterized by endocrine cell growth and hormone production dysregulation. " 01/01/2005 - "Each multiple endocrine neoplasia (MEN) syndrome expresses striking features of hormone oversecretion from its own characteristic group of tissues. " 02/01/1989 - "A case of multiple endocrine neoplasia (MEN) type 1; the immunohistochemical and ultrastructural studies of its tumors and the analysis of hormones in tumor extracts." 01/01/2012 - "Previous discussions about surgical management have focused mainly on syndromes of hormone excess and, in particular, the management of multiple endocrine neoplasia syndrome type 1-related Zollinger-Ellison syndrome. " 10/01/2005 - "The various syndromes -- polyglandular autoimmune syndrome, autoimmune hypophysitis, multiple pituitary hormone deficiency, multiple endocrine neoplasia, and Carney complex -- are discussed. "
10.	DNA (Deoxyribonucleic Acid)IBA 01/01/2000 - "Familial form can be isolated or expression of a multiple endocrine neoplasia type II. Mutations of the RET proto-oncogene have been identified in the germline DNA of patients with familial MTC syndromes. " 12/01/1999 - "DNA testing is of great importance in families with multiple endocrine neoplasia (MEN) type 2A to identify non-mutant carrying family members and asymptomatic mutation carriers, and also to confirm the diagnosis in patients who already show clinical or biochemical signs of disease. " 10/07/1999 - "[Identification and screening of a family with type 2A multiple endocrine neoplasia with DNA analysis]." 01/01/1999 - "DNA extracted from the peripheral blood leukocytes of 36 members of five families with multiple endocrine neoplasia (MEN) type 2A was analyzed for mutations of exons 10, 11, 13, 14, and 16 of the RET proto-oncogene by nonisotopic polymerase chain reaction-single-strand conformation polymorphism analysis and automated DNA sequencing. " 01/01/1990 - "[Diagnosis of multiple endocrine neoplasms type IIa using DNA analysis]."

Therapies and Procedures

1.	Parathyroidectomy 06/01/2012 - "We excluded patients who had undergone prior neck surgery or had known multiple endocrine neoplasia 1 or 2. A total of 1,231 patients underwent a parathyroidectomy, 155 of whom met the study's inclusion/exclusion criteria (12.6%). " 12/01/2004 - "From these findings, she was diagnosed with multiple endocrine neoplasia (MEN) type 1. Subsequently, a parathyroidectomy was performed successfully, a parathyroid gland was transplanted to her right forearm, and her serum Ca(2+) level was controlled at 8.5-9.0 mg/dl. " 08/01/2002 - "We analyzed 115 unselected patients with pHPT without a family history or multiple endocrine neoplasia but who had undergone parathyroidectomy. " 12/01/1996 - "One hundred ten consecutive parathyroidectomy patients with no previous neck surgery or history of multiple endocrine neoplasia had intraoperative iPTH assays performed before and after excision of any suspected abnormal parathyroid gland(s). " 06/01/1987 - "Resolution of hypergastrinaemia after parathyroidectomy in multiple endocrine neoplasia syndrome type I (MEN type I)."
2.	Radiotherapy 10/01/1988 - "A retrospective study of 202 patients with medullary thyroid carcinoma (MTC) diagnosed between 1943 and 1987 was done to compare the prognosis of patients with sporadic disease and those with the familial form of multiple endocrine neoplasia type II and to study the effect of radiotherapy. " 01/01/1989 - "A retrospective study of 202 patients with medullary thyroid carcinoma (MTC) diagnosed between 1943 and 1987 was done to compare the prognosis of patients with sporadic disease and those with the familial form of multiple endocrine neoplasia type II (MEN-II) and to study the effect of radiotherapy. "
3.	Thyroidectomy 06/01/2014 - "There have been few reports of prophylactic thyroidectomy using the minimally invasive video-assisted thyroidectomy (MIVAT) approach in children with multiple endocrine neoplasia 2 (MEN2). " 01/01/2013 - "Ultrasonography should not guide the timing of thyroidectomy in pediatric patients diagnosed with multiple endocrine neoplasia syndrome 2A through genetic screening." 08/01/2011 - "A 56-year-old woman with multiple endocrine neoplasia 2B syndrome, post subtotal thyroidectomy for MTC in childhood, presented with extensive metastatic spread of MTC to the lungs and liver, 47 years after the original diagnosis. " 12/15/2010 - "When specific germline mutations in RET are identified early, prophylactic thyroidectomy can be timed to remove at-risk tissue in patients with multiple endocrine neoplasia 2 (MEN2) syndromes who would otherwise develop MTC. " 05/01/2010 - "Multiple endocrine neoplasia [corrected] syndrome type 2B in early childhood: long-term benefit of prophylactic thyroidectomy."
4.	Adrenalectomy 01/01/2014 - "Laparoscopic adrenalectomy has gained favour as a preferred surgical approach in the multiple endocrine neoplasia (MEN) type 2 patients. " 01/01/2002 - "Cortical-sparing laparoscopic adrenalectomy in a patient with multiple endocrine neoplasia type IIA." 09/01/1999 - "To evaluate the complications of the adrenocortical supplementation that is needed after bilateral adrenalectomy for phaeochromocytoma in patients with multiple endocrine neoplasia (MEN) type 2 syndrome. " 09/01/1999 - "Complications after bilateral adrenalectomy for phaeochromocytoma in multiple endocrine neoplasia type 2--a plea to conserve adrenal function." 06/01/1999 - "To describe our surgical technique for, and results of, subtotal adrenalectomy for phaeochromocytoma in multiple endocrine neoplasia (MEN) type 2. Retrospective study. "
5.	Pancreatectomy 01/01/2013 - "[Pancreatectomy for multiple endocrine neoplasia with distant metastases and magistral arteries invasion]." 07/01/2006 - "Pancreatectomy in multiple endocrine neoplasia type 1-related gastrinomas and pancreatic endocrine neoplasias." 06/01/2002 - "The records of patients (multiple endocrine neoplasia excluded) with pathologically proved insulinoma who were screened at our institution or referred to us after a failed blind distal pancreatectomy were reviewed. " 07/01/2013 - "The types of resections for pancreatic NETs vary from enucleation to pancreatectomy with or without regional lymph node dissection, based on the type of excessive hormone, tumor size, distance from the main pancreatic duct, and the presence of type 1 multiple endocrine neoplasia. "