Materiały źródłowe

• #1 Care for patients with multiple endocrine neoplasia type 1: the current evidence base – PubMed

  https://pubmed.ncbi.nlm.nih.gov/21061174/

  Multiple endocrine neoplasia type 1 (MEN1) is a rare disease caused by mutations in the MEN1 gene on chromosome 11. It is characterized by the occurrence of primary hyperparathyroidism (pHPT), duodenopancreatic neuroendocrine tumours (pNET), pituitary tumours (PIT), adrenal adenomas (ADR) and neuroendocrine tumours (NET) of the stomach, bronchus and thymus. MEN1 is a syndrome with high penetrance and high morbidity. Malignant NETs are the most important cause of MEN1-related death. Since 1997 the diagnosis can be made by genetic screening. MEN1 is a complex syndrome and the endocrine manifestations cannot be viewed upon as coinciding sporadic tumours. Differences in epidemiology and pathology between MEN1-related tumours and their sporadic counterparts show that a unique approach is needed. Therefore the care for MEN1 patients should be provided by a centre of expertise. Early genetic diagnosis and periodic screening are important pillars of care. For primary hyperparathyroidism surgery is the most important treatment modality, with a subtotal parathyroid gland resection as the procedure of choice. In neuroendocrine tumours surgery also is the most important treatment modality. Selective tumour enucleation has no place in the surgical treatment of MEN1-related pNETs; the exact procedure depends on the functionality of the tumour. In MEN1-associated pituitary and adrenal adenomas, watchful waiting and medical therapy play more important roles. In the twenty-first century new developments will impact the care for MEN1 patients. These developments should be critically evaluated in clinical research with the ultimate goal of optimizing the care for MEN1 patients on an evidence base.

• #2 Multiple endocrine neoplasia, type 1 (MEN 1) – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/men-1/symptoms-causes/syc-20353064

  Multiple endocrine neoplasia, type 1 (MEN 1) is a rare condition. It mainly causes tumors in the glands that make and release hormones. These are called the endocrine glands. The condition also can cause tumors in the small intestine and stomach. Another name for MEN 1 is Wermer’s syndrome. […] The endocrine gland tumors that form due to MEN 1 usually are not cancer. Most often, the tumors grow on the parathyroid glands, the pancreas and the pituitary gland. Some glands affected by MEN 1 also may release too many hormones. That can lead to other health concerns. […] MEN 1 can’t be cured. But regular testing can detect health concerns, and healthcare professionals can provide treatment as needed. […] Multiple endocrine neoplasia, type 1 (MEN 1) care at Mayo Clinic.

• #3 Genetic Disorder Reference Sheet: Multiple Endocrine Neoplasia Type 1 | Oncology Nursing Society

  https://onf.ons.org/publications-research/voice/news-views/07-2022/genetic-disorder-reference-sheet-multiple-endocrine

  Multiple endocrine neoplasia type 1 (MEN1) is a rare autosomal dominant genetic disease affecting about 1 in 30,000 people. Pathogenic variants in the MEN1 gene are associated with benign and malignant tumors in the parathyroid glands, pituitary gland, pancreas, duodenum, and stomach. […] Individuals with an MEN1 pathogenic variant are at increased risk for developing benign and malignant tumors (see sidebar). […] Ideally, people with a pathogenic MEN1 variant are managed by an interprofessional team that is familiar with its multiple complications. […] A primary nursing responsibility for MEN1 is providing ongoing psychosocial support and education on symptom identification and the importance of ongoing screening to detect tumors early, intervene accordingly, and improve prognosis. […] Smoking increases the risk of carcinoid tumors in MEN1. Nurses should encourage smoking cessation and help patients enroll in programs to quit.

• #4 Multiple Endocrine Neoplasia (MEN): Types & Symptoms

  https://my.clevelandclinic.org/health/diseases/23088-multiple-endocrine-neoplasia-men

  Multiple endocrine neoplasia (MEN) is a rare genetic condition characterized by multiple tumors and/or cancer that affect specific endocrine system glands and tissues. […] Multiple endocrine neoplasia (MEN) type 1: This is a genetic condition in which multiple tumors affect different aspects of your endocrine system. […] People with MEN type 1 develop tumors in multiple glands of their endocrine system. The most common affected areas include: Parathyroid glands (most common). Gastroenteropancreatic tract (a tumor can form in your pancreas or in other parts of the gastrointestinal tract, including your stomach and duodenum). Pituitary gland. […] Most tumors associated with MEN type 1 are benign (noncancerous), but some tumors can be cancerous (malignant) and can spread to other areas of your body (metastasize).

• #5 Multiple endocrine neoplasia, type 1 (MEN 1) – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/men-1/symptoms-causes/syc-20353064

  Multiple endocrine neoplasia, type 1 (MEN 1) is a rare condition. It mainly causes tumors in the glands that make and release hormones. These are called the endocrine glands. The condition also can cause tumors in the small intestine and stomach. Another name for MEN 1 is Wermer’s syndrome. […] The endocrine gland tumors that form due to MEN 1 usually are not cancer. Most often, the tumors grow on the parathyroid glands, the pancreas and the pituitary gland. Some glands affected by MEN 1 also may release too many hormones. That can lead to other health concerns. […] MEN 1 can’t be cured. But regular testing can detect health concerns, and healthcare professionals can provide treatment as needed. […] Multiple endocrine neoplasia, type 1 (MEN 1) care at Mayo Clinic.

• #6 Multiple Endocrine Neoplasia | MD Anderson Cancer Center

  https://www.mdanderson.org/cancer-types/multiple-endocrine-neoplasia.html

  Multiple endocrine neoplasia (MEN) syndromes are rare disorders of the endocrine system. They make patients more likely to develop benign (not cancer) or malignant (cancer) tumors in the endocrine glands. […] Multiple endocrine neoplasia type 1 (MEN1), also called multiple endocrine adenomatosis or Wermer’s syndrome, is found in one in 30,000 people. […] In MEN1, tumors grow in certain glands of the endocrine system. They tend to develop in more than one gland. If you have only one affected endocrine gland, you probably do not have MEN1. […] While these tumors usually are benign, they may cause problems by releasing too much hormone or growing against other parts of the body. However, about half of people with MEN1 will eventually develop cancer. […] MEN1 tends to cause tumors in the following parts of the body:

• #7 Care for patients with multiple endocrine neoplasia type 1: the current evidence base – PubMed

  https://pubmed.ncbi.nlm.nih.gov/21061174/

  Multiple endocrine neoplasia type 1 (MEN1) is a rare disease caused by mutations in the MEN1 gene on chromosome 11. It is characterized by the occurrence of primary hyperparathyroidism (pHPT), duodenopancreatic neuroendocrine tumours (pNET), pituitary tumours (PIT), adrenal adenomas (ADR) and neuroendocrine tumours (NET) of the stomach, bronchus and thymus. MEN1 is a syndrome with high penetrance and high morbidity. Malignant NETs are the most important cause of MEN1-related death. Since 1997 the diagnosis can be made by genetic screening. MEN1 is a complex syndrome and the endocrine manifestations cannot be viewed upon as coinciding sporadic tumours. Differences in epidemiology and pathology between MEN1-related tumours and their sporadic counterparts show that a unique approach is needed. Therefore the care for MEN1 patients should be provided by a centre of expertise. Early genetic diagnosis and periodic screening are important pillars of care. For primary hyperparathyroidism surgery is the most important treatment modality, with a subtotal parathyroid gland resection as the procedure of choice. In neuroendocrine tumours surgery also is the most important treatment modality. Selective tumour enucleation has no place in the surgical treatment of MEN1-related pNETs; the exact procedure depends on the functionality of the tumour. In MEN1-associated pituitary and adrenal adenomas, watchful waiting and medical therapy play more important roles. In the twenty-first century new developments will impact the care for MEN1 patients. These developments should be critically evaluated in clinical research with the ultimate goal of optimizing the care for MEN1 patients on an evidence base.

• #8 Multiple endocrine neoplasia, type 1 (MEN 1) | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/multiple-endocrine-neoplasia-type-1-men-1

  Multiple endocrine neoplasia, type 1 (MEN 1) is a rare condition. It mainly causes tumors in the glands that make and release hormones. These are called the endocrine glands. The condition also can cause tumors in the small intestine and stomach. Another name for MEN 1 is Wermer’s syndrome. […] The endocrine gland tumors that form due to MEN 1 usually are not cancer. Most often, the tumors grow on the parathyroid glands, the pancreas and the pituitary gland. Some glands affected by MEN 1 also may release too many hormones. That can lead to other health concerns. […] MEN 1 can’t be cured. But regular testing can detect health concerns, and healthcare professionals can provide treatment as needed. […] With MEN 1, tumors can grow on the parathyroid glands, the pancreas and the pituitary gland. That can lead to various conditions, all of which can be treated.

• #9 Multiple Endocrine Neoplasia Type 1 | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/25356

  MEN1 is a complex syndrome that increases morbidity and mortality by causing multiple aggressive tumors. An interprofessional team should manage these patients in a tertiary care center, including an endocrinologist, endocrine surgeon, gastroenterologist, neurosurgeon, genetic counselor, nursing staff, pharmacist, and patient navigator or advocate for achieving better outcomes.

• #10 Multiple endocrine neoplasia type 1: Clinical manifestations and diagnosis – UpToDate

  https://www.uptodate.com/contents/multiple-endocrine-neoplasia-type-1-clinical-manifestations-and-diagnosis

  The presence of MEN1 is defined clinically as the occurrence of two or more primary MEN1 tumor types, or in family members of a patient with a clinical diagnosis of MEN1, the occurrence of one of the MEN1-associated tumors (figure 1). […] The clinical presentation of patients with multiple endocrine neoplasia type 1 (MEN1) is determined by the site and spectrum of tumor development and/or consequences of hormonal hypersecretion. […] Almost all patients will develop biochemical or clinical evidence of primary hyperparathyroidism by the fifth decade. […] Furthermore, almost all patients with MEN1 undergo at least one operation related to MEN1-associated tumors in their lifetime [4].

• #11 Multiple Endocrine Neoplasia Type 1 (MEN1): Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/126438-overview

  Primary hyperparathyroidism, due to hyperplasia and/or adenoma of parathyroid glands, is the most common manifestation of multiple endocrine neoplasia type 1 (MEN1) and occurs in approximately 90% of all patients. […] Primary hyperparathyroidism in MEN1 can have a long-term asymptomatic course and is usually diagnosed by the incidental finding of elevated serum parathyroid hormone level in a patient with hypercalcemia or, in some cases, with normocalcemia. […] Common symptoms associated with hypercalcemia include polydipsia, polyuria, constipation, and generalized malaise. […] Insulinomas are the second most common functioning pancreatic neuroendocrine tumor in MEN1, developing at young age ( 35 years) in approximately 10-30% of patients. […] The rationale for an aggressive surveillance approach in MEN1 patients and asymptomatic carriers is based on the presumption that the early pre-symptomatic detection of MEN1 neoplasias may reduce the associated mortality.

• #12 Multiple Endocrine Neoplasia Type 1 | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/25356

  Primary hyperparathyroidism (PHPT) is the most common and the first clinical manifestation of MEN1. It affects approximately 90% of MEN1 patients and is commonly diagnosed during the second decade of life. […] Treatment of PHPT in MEN1 involving subtotal parathyroidectomy versus total parathyroidectomy remains controversial. […] Non-functioning pancreatic NETs are the most common gastro-entero-pancreatic tumors. […] Surgical treatment is necessary for insulinoma patients. […] The prevalence of anterior pituitary tumors in MEN1 syndrome is approximately 20% to 60%, and the anterior pituitary tumor can be an initial presentation in about 25% of MEN1 patients. […] Patient education is vital. Once the physician diagnoses MEN1, the patient’s family should see a genetic counselor to learn about the disease and how it may impact the patient and the patient’s family.

• #13 Multiple Endocrine Neoplasia Type 1 | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/25356

  Primary hyperparathyroidism (PHPT) is the most common and the first clinical manifestation of MEN1. It affects approximately 90% of MEN1 patients and is commonly diagnosed during the second decade of life. […] Treatment of PHPT in MEN1 involving subtotal parathyroidectomy versus total parathyroidectomy remains controversial. […] Non-functioning pancreatic NETs are the most common gastro-entero-pancreatic tumors. […] Surgical treatment is necessary for insulinoma patients. […] The prevalence of anterior pituitary tumors in MEN1 syndrome is approximately 20% to 60%, and the anterior pituitary tumor can be an initial presentation in about 25% of MEN1 patients. […] Patient education is vital. Once the physician diagnoses MEN1, the patient’s family should see a genetic counselor to learn about the disease and how it may impact the patient and the patient’s family.

• #14 Multiple Endocrine Neoplasia, Type 1 (MEN 1) – Endocrine and Metabolic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/endocrine-and-metabolic-disorders/multiple-endocrine-neoplasia-men-syndromes/multiple-endocrine-neoplasia-type-1-men-1

  Multiple endocrine neoplasia, type 1 (MEN 1) is an autosomal dominant syndrome characterized by hyperplasia or adenomas of the parathyroid glands, pancreatic islet cell tumors (also known as pancreatic neuroendocrine tumors), and/or pituitary gland tumors. […] Diagnosis is by hormonal and imaging tests. […] Tumors are removed surgically if they cause symptoms or for suspected malignancy based on size criteria. […] Treatment of hyperparathyroidism is primarily surgical, with subtotal parathyroidectomy; however, hyperparathyroidism frequently recurs. […] Prolactinoma is usually managed with dopamine agonists; other pituitary tumors are treated surgically. […] Patients should have genetic testing of the MEN 1 gene and clinical evaluation for other tumors of the syndrome. […] Tumors are excised when possible, but lesions are often multiple and/or difficult to find. […] Sometimes hormone excess can be managed by medications.

• #15 Multiple Endocrine Neoplasia Type 1 (MEN1): Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/126438-overview

  Primary hyperparathyroidism, due to hyperplasia and/or adenoma of parathyroid glands, is the most common manifestation of multiple endocrine neoplasia type 1 (MEN1) and occurs in approximately 90% of all patients. […] Primary hyperparathyroidism in MEN1 can have a long-term asymptomatic course and is usually diagnosed by the incidental finding of elevated serum parathyroid hormone level in a patient with hypercalcemia or, in some cases, with normocalcemia. […] Common symptoms associated with hypercalcemia include polydipsia, polyuria, constipation, and generalized malaise. […] Insulinomas are the second most common functioning pancreatic neuroendocrine tumor in MEN1, developing at young age ( 35 years) in approximately 10-30% of patients. […] The rationale for an aggressive surveillance approach in MEN1 patients and asymptomatic carriers is based on the presumption that the early pre-symptomatic detection of MEN1 neoplasias may reduce the associated mortality.

• #16 Multiple endocrine neoplasia, type 1 (MEN 1) | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/multiple-endocrine-neoplasia-type-1-men-1?content_id=CON-20309660

  Multiple endocrine neoplasia, type 1 (MEN 1) is a rare condition. It mainly causes tumors in the glands that make and release hormones. The condition also can cause tumors in the small intestine and stomach. Another name for MEN 1 is Wermer’s syndrome. […] The endocrine gland tumors that form due to MEN 1 usually are not cancer. Most often, the tumors grow on the parathyroid glands, the pancreas and the pituitary gland. Some glands affected by MEN 1 also may release too many hormones. That can lead to other health concerns. […] MEN 1 can’t be cured. But regular testing can detect health concerns, and healthcare professionals can provide treatment as needed. […] Symptoms of multiple endocrine neoplasia, type 1 (MEN 1) can include the following: Tiredness. Bone pain or broken bones. Kidney stones. Ulcers in the stomach or intestines. Stomach pain. Muscle weakness. Depression. Acid reflux. Frequent diarrhea.

• #17 Multiple Endocrine Neoplasia | MD Anderson Cancer Center

  https://www.mdanderson.org/cancer-types/multiple-endocrine-neoplasia.html

  Almost all people with MEN1 develop parathyroid gland tumors. These are usually the first glands affected by MEN1. […] MEN1 tumors may cause them to make too much parathyroid hormone (PTH). This is called hyperparathyroidism, and it leads to high levels of calcium in the blood. […] If hypercalcemia is not treated, you may develop kidney stones or kidney damage, and your bones may become thin. […] MEN1 can cause benign (non-cancerous) tumors in the front part of the pituitary gland. […] Prolactinomas can interfere with sexual function and fertility, and tumors secreting growth hormone over time can cause acromegaly (enlargement of the bones). […] Tumors also may form in the islet cells of the pancreas and the lining of the duodenum (the first portion of the small intestine), which can secrete several hormones involved with endocrine function.

• #18 Care for patients with multiple endocrine neoplasia type 1: the current evidence base – PubMed

  https://pubmed.ncbi.nlm.nih.gov/21061174/

  Multiple endocrine neoplasia type 1 (MEN1) is a rare disease caused by mutations in the MEN1 gene on chromosome 11. It is characterized by the occurrence of primary hyperparathyroidism (pHPT), duodenopancreatic neuroendocrine tumours (pNET), pituitary tumours (PIT), adrenal adenomas (ADR) and neuroendocrine tumours (NET) of the stomach, bronchus and thymus. MEN1 is a syndrome with high penetrance and high morbidity. Malignant NETs are the most important cause of MEN1-related death. Since 1997 the diagnosis can be made by genetic screening. MEN1 is a complex syndrome and the endocrine manifestations cannot be viewed upon as coinciding sporadic tumours. Differences in epidemiology and pathology between MEN1-related tumours and their sporadic counterparts show that a unique approach is needed. Therefore the care for MEN1 patients should be provided by a centre of expertise. Early genetic diagnosis and periodic screening are important pillars of care. For primary hyperparathyroidism surgery is the most important treatment modality, with a subtotal parathyroid gland resection as the procedure of choice. In neuroendocrine tumours surgery also is the most important treatment modality. Selective tumour enucleation has no place in the surgical treatment of MEN1-related pNETs; the exact procedure depends on the functionality of the tumour. In MEN1-associated pituitary and adrenal adenomas, watchful waiting and medical therapy play more important roles. In the twenty-first century new developments will impact the care for MEN1 patients. These developments should be critically evaluated in clinical research with the ultimate goal of optimizing the care for MEN1 patients on an evidence base.

• #19

  https://step1.medbullets.com/endocrine/109035/multiple-endocrine-neoplasias

  results in kidney stones […] pituitary tumors (prolactin or GH) […] pancreatic islet cell/endocrine tumors […] Zollinger-Ellison syndrome causes gastric ulcers […] insulinomas […] VIPomas part of watery diarrhea, hypokalemia, achlorhydria (WDHA) syndrome […] Symptoms dependent on which type of MEN syndrome […] MEN I flank pain secondary to kidney stones […] abdominal pain and dark stools secondary to gastric ulcers […] watery diarrhea […] Physical exam MEN I costovertebral angle (CVA) tenderness […] abdominal tenderness […] Treatment Medical proton pump inhibitor indications Zollinger-Ellison syndrome […] Surgical thyroidectomy indications medullary thyroid cancer MEN IIA or IIB […] follow with thyroid hormone supplementation […] parathyroidectomy indications parathyroid tumor MEN I […] duodenal-pancreatic surgery indications Zollinger-Ellison syndrome refractory to medical management […] adrenalectomy indications pheochromocytoma […] preoperative medications to prevent hypertensive crisis must start with alpha blockade […] follow with beta blockade.

• #20 Multiple Endocrine Neoplasia Type 1 (MEN1): Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/126438-overview

  Primary hyperparathyroidism, due to hyperplasia and/or adenoma of parathyroid glands, is the most common manifestation of multiple endocrine neoplasia type 1 (MEN1) and occurs in approximately 90% of all patients. […] Primary hyperparathyroidism in MEN1 can have a long-term asymptomatic course and is usually diagnosed by the incidental finding of elevated serum parathyroid hormone level in a patient with hypercalcemia or, in some cases, with normocalcemia. […] Common symptoms associated with hypercalcemia include polydipsia, polyuria, constipation, and generalized malaise. […] Insulinomas are the second most common functioning pancreatic neuroendocrine tumor in MEN1, developing at young age ( 35 years) in approximately 10-30% of patients. […] The rationale for an aggressive surveillance approach in MEN1 patients and asymptomatic carriers is based on the presumption that the early pre-symptomatic detection of MEN1 neoplasias may reduce the associated mortality.

• #21

  https://step1.medbullets.com/endocrine/109035/multiple-endocrine-neoplasias

  results in kidney stones […] pituitary tumors (prolactin or GH) […] pancreatic islet cell/endocrine tumors […] Zollinger-Ellison syndrome causes gastric ulcers […] insulinomas […] VIPomas part of watery diarrhea, hypokalemia, achlorhydria (WDHA) syndrome […] Symptoms dependent on which type of MEN syndrome […] MEN I flank pain secondary to kidney stones […] abdominal pain and dark stools secondary to gastric ulcers […] watery diarrhea […] Physical exam MEN I costovertebral angle (CVA) tenderness […] abdominal tenderness […] Treatment Medical proton pump inhibitor indications Zollinger-Ellison syndrome […] Surgical thyroidectomy indications medullary thyroid cancer MEN IIA or IIB […] follow with thyroid hormone supplementation […] parathyroidectomy indications parathyroid tumor MEN I […] duodenal-pancreatic surgery indications Zollinger-Ellison syndrome refractory to medical management […] adrenalectomy indications pheochromocytoma […] preoperative medications to prevent hypertensive crisis must start with alpha blockade […] follow with beta blockade.

• #22 Multiple Endocrine Neoplasia (MEN) – Free Sketchy Medical Lesson

  https://www.sketchy.com/medical-lessons/multiple-endocrine-neoplasia-men

  Multiple endocrine neoplasia (MEN) is an inherited disorder characterized by the development of tumors in multiple endocrine organs, often at a young age. MEN type 1 (MEN1) occurs due to mutations in the MEN1 gene, located on chromosome 11, and acts as a tumor suppressor gene. The primary organs affected in MEN1 are the parathyroid, pancreas, and pituitary. […] MEN type 1 is caused by mutations in the MEN1 gene, a tumor suppressor gene located on chromosome 11. This type commonly presents with tumors in the parathyroid, pancreas, and pituitary glands. These tumors usually appear before the age of 35 and are prone to recurrence after surgical intervention. Parathyroid tumors in MEN1 often involve multiple glands and lead to primary hyperparathyroidism, characterized by elevated levels of parathyroid hormone (PTH) and increased serum calcium. […] In MEN type 1, the most common pancreatic tumors are islet cell tumors, with gastrinomas being the predominant subtype. Gastrinomas lead to peptic ulcer disease due to elevated levels of gastrin. Pancreatic tumors are the leading cause of death in MEN1 patients.

• #23 Care for patients with multiple endocrine neoplasia type 1: the current evidence base – PubMed

  https://pubmed.ncbi.nlm.nih.gov/21061174/

  Multiple endocrine neoplasia type 1 (MEN1) is a rare disease caused by mutations in the MEN1 gene on chromosome 11. It is characterized by the occurrence of primary hyperparathyroidism (pHPT), duodenopancreatic neuroendocrine tumours (pNET), pituitary tumours (PIT), adrenal adenomas (ADR) and neuroendocrine tumours (NET) of the stomach, bronchus and thymus. MEN1 is a syndrome with high penetrance and high morbidity. Malignant NETs are the most important cause of MEN1-related death. Since 1997 the diagnosis can be made by genetic screening. MEN1 is a complex syndrome and the endocrine manifestations cannot be viewed upon as coinciding sporadic tumours. Differences in epidemiology and pathology between MEN1-related tumours and their sporadic counterparts show that a unique approach is needed. Therefore the care for MEN1 patients should be provided by a centre of expertise. Early genetic diagnosis and periodic screening are important pillars of care. For primary hyperparathyroidism surgery is the most important treatment modality, with a subtotal parathyroid gland resection as the procedure of choice. In neuroendocrine tumours surgery also is the most important treatment modality. Selective tumour enucleation has no place in the surgical treatment of MEN1-related pNETs; the exact procedure depends on the functionality of the tumour. In MEN1-associated pituitary and adrenal adenomas, watchful waiting and medical therapy play more important roles. In the twenty-first century new developments will impact the care for MEN1 patients. These developments should be critically evaluated in clinical research with the ultimate goal of optimizing the care for MEN1 patients on an evidence base.

• #24 Multiple Endocrine Neoplasia Type 1 | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/25356

  Primary hyperparathyroidism (PHPT) is the most common and the first clinical manifestation of MEN1. It affects approximately 90% of MEN1 patients and is commonly diagnosed during the second decade of life. […] Treatment of PHPT in MEN1 involving subtotal parathyroidectomy versus total parathyroidectomy remains controversial. […] Non-functioning pancreatic NETs are the most common gastro-entero-pancreatic tumors. […] Surgical treatment is necessary for insulinoma patients. […] The prevalence of anterior pituitary tumors in MEN1 syndrome is approximately 20% to 60%, and the anterior pituitary tumor can be an initial presentation in about 25% of MEN1 patients. […] Patient education is vital. Once the physician diagnoses MEN1, the patient’s family should see a genetic counselor to learn about the disease and how it may impact the patient and the patient’s family.

• #25 Multiple Endocrine Neoplasia Type 1 | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/25356

  Primary hyperparathyroidism (PHPT) is the most common and the first clinical manifestation of MEN1. It affects approximately 90% of MEN1 patients and is commonly diagnosed during the second decade of life. […] Treatment of PHPT in MEN1 involving subtotal parathyroidectomy versus total parathyroidectomy remains controversial. […] Non-functioning pancreatic NETs are the most common gastro-entero-pancreatic tumors. […] Surgical treatment is necessary for insulinoma patients. […] The prevalence of anterior pituitary tumors in MEN1 syndrome is approximately 20% to 60%, and the anterior pituitary tumor can be an initial presentation in about 25% of MEN1 patients. […] Patient education is vital. Once the physician diagnoses MEN1, the patient’s family should see a genetic counselor to learn about the disease and how it may impact the patient and the patient’s family.

• #26 Multiple Endocrine Neoplasia | MD Anderson Cancer Center

  https://www.mdanderson.org/cancer-types/multiple-endocrine-neoplasia.html

  Almost all people with MEN1 develop parathyroid gland tumors. These are usually the first glands affected by MEN1. […] MEN1 tumors may cause them to make too much parathyroid hormone (PTH). This is called hyperparathyroidism, and it leads to high levels of calcium in the blood. […] If hypercalcemia is not treated, you may develop kidney stones or kidney damage, and your bones may become thin. […] MEN1 can cause benign (non-cancerous) tumors in the front part of the pituitary gland. […] Prolactinomas can interfere with sexual function and fertility, and tumors secreting growth hormone over time can cause acromegaly (enlargement of the bones). […] Tumors also may form in the islet cells of the pancreas and the lining of the duodenum (the first portion of the small intestine), which can secrete several hormones involved with endocrine function.

• #27 Multiple Endocrine Neoplasia Syndromes (MEN) – Hormonal and Metabolic Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/hormonal-and-metabolic-disorders/multiple-endocrine-neoplasia-syndromes/multiple-endocrine-neoplasia-syndromes-men

  People with multiple endocrine neoplasia type 1 develop tumors, or excessive growth and activity, of two or more of the following glands: The parathyroid glands, The pancreas, The pituitary gland, The adrenal glands (less often affected). […] Almost all people with multiple endocrine neoplasia type 1 have tumors of the parathyroid glands. Most of the tumors are noncancerous, but they cause the glands to produce too much parathyroid hormone (primary hyperparathyroidism). […] Many (30 to 90%) of people with MEN type 1 also develop tumors of the hormone-producing cells (islet cells) of the pancreas (also called pancreatic neuroendocrine tumors). […] Some people with MEN type 1 develop pituitary gland tumors. Some of these tumors produce the hormone prolactin, causing menstrual abnormalities and often breast secretions (galactorrhea) in women who are not nursing and decreased sexual desire and erectile dysfunction (impotence) in men.

• #28 Care for patients with multiple endocrine neoplasia type 1: the current evidence base – PubMed

  https://pubmed.ncbi.nlm.nih.gov/21061174/

  Multiple endocrine neoplasia type 1 (MEN1) is a rare disease caused by mutations in the MEN1 gene on chromosome 11. It is characterized by the occurrence of primary hyperparathyroidism (pHPT), duodenopancreatic neuroendocrine tumours (pNET), pituitary tumours (PIT), adrenal adenomas (ADR) and neuroendocrine tumours (NET) of the stomach, bronchus and thymus. MEN1 is a syndrome with high penetrance and high morbidity. Malignant NETs are the most important cause of MEN1-related death. Since 1997 the diagnosis can be made by genetic screening. MEN1 is a complex syndrome and the endocrine manifestations cannot be viewed upon as coinciding sporadic tumours. Differences in epidemiology and pathology between MEN1-related tumours and their sporadic counterparts show that a unique approach is needed. Therefore the care for MEN1 patients should be provided by a centre of expertise. Early genetic diagnosis and periodic screening are important pillars of care. For primary hyperparathyroidism surgery is the most important treatment modality, with a subtotal parathyroid gland resection as the procedure of choice. In neuroendocrine tumours surgery also is the most important treatment modality. Selective tumour enucleation has no place in the surgical treatment of MEN1-related pNETs; the exact procedure depends on the functionality of the tumour. In MEN1-associated pituitary and adrenal adenomas, watchful waiting and medical therapy play more important roles. In the twenty-first century new developments will impact the care for MEN1 patients. These developments should be critically evaluated in clinical research with the ultimate goal of optimizing the care for MEN1 patients on an evidence base.

• #29 Multiple Endocrine Neoplasia Syndromes (MEN) – Hormonal and Metabolic Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/hormonal-and-metabolic-disorders/multiple-endocrine-neoplasia-syndromes/multiple-endocrine-neoplasia-syndromes-men

  People with multiple endocrine neoplasia type 1 develop tumors, or excessive growth and activity, of two or more of the following glands: The parathyroid glands, The pancreas, The pituitary gland, The adrenal glands (less often affected). […] Almost all people with multiple endocrine neoplasia type 1 have tumors of the parathyroid glands. Most of the tumors are noncancerous, but they cause the glands to produce too much parathyroid hormone (primary hyperparathyroidism). […] Many (30 to 90%) of people with MEN type 1 also develop tumors of the hormone-producing cells (islet cells) of the pancreas (also called pancreatic neuroendocrine tumors). […] Some people with MEN type 1 develop pituitary gland tumors. Some of these tumors produce the hormone prolactin, causing menstrual abnormalities and often breast secretions (galactorrhea) in women who are not nursing and decreased sexual desire and erectile dysfunction (impotence) in men.

• #30 Multiple Endocrine Neoplasia Syndromes (MEN) – Hormonal and Metabolic Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/hormonal-and-metabolic-disorders/multiple-endocrine-neoplasia-syndromes/multiple-endocrine-neoplasia-syndromes-men

  In some people with MEN type 1, tumors or excessive growth and activity of the adrenal glands develop. […] Women with MEN type 1 may be at higher risk of breast cancer. […] No overall cure is known for any of the multiple endocrine neoplasia syndromes. Doctors treat the changes in each gland individually. […] A tumor is treated by removing it surgically when possible. […] Because medullary thyroid cancer is ultimately fatal if untreated, doctors will most likely recommend preventive surgical removal of the thyroid gland if genetic testing has revealed evidence of multiple endocrine neoplasia type 2A or type 2B. […] Periodic monitoring with blood and imaging studies is important but may add to the anxiety. Counseling may be needed to help people better understand their disease and cope with this anxiety.

• #31 Care for patients with multiple endocrine neoplasia type 1: the current evidence base – PubMed

  https://pubmed.ncbi.nlm.nih.gov/21061174/

  Multiple endocrine neoplasia type 1 (MEN1) is a rare disease caused by mutations in the MEN1 gene on chromosome 11. It is characterized by the occurrence of primary hyperparathyroidism (pHPT), duodenopancreatic neuroendocrine tumours (pNET), pituitary tumours (PIT), adrenal adenomas (ADR) and neuroendocrine tumours (NET) of the stomach, bronchus and thymus. MEN1 is a syndrome with high penetrance and high morbidity. Malignant NETs are the most important cause of MEN1-related death. Since 1997 the diagnosis can be made by genetic screening. MEN1 is a complex syndrome and the endocrine manifestations cannot be viewed upon as coinciding sporadic tumours. Differences in epidemiology and pathology between MEN1-related tumours and their sporadic counterparts show that a unique approach is needed. Therefore the care for MEN1 patients should be provided by a centre of expertise. Early genetic diagnosis and periodic screening are important pillars of care. For primary hyperparathyroidism surgery is the most important treatment modality, with a subtotal parathyroid gland resection as the procedure of choice. In neuroendocrine tumours surgery also is the most important treatment modality. Selective tumour enucleation has no place in the surgical treatment of MEN1-related pNETs; the exact procedure depends on the functionality of the tumour. In MEN1-associated pituitary and adrenal adenomas, watchful waiting and medical therapy play more important roles. In the twenty-first century new developments will impact the care for MEN1 patients. These developments should be critically evaluated in clinical research with the ultimate goal of optimizing the care for MEN1 patients on an evidence base.

• #32 Multiple Endocrine Neoplasia Type 1 (MEN1): What patients should know about in 2024

  https://oncodaily.com/oncolibrary/cancer-types/multiple-endocrine-neoplasia-type-1-men1-64880

  You should see a doctor if you experience unexplained symptoms that could be linked to Multiple Endocrine Neoplasia Type 1 (MEN1). Early warning signs, such as persistent fatigue, bone pain, kidney stones, frequent stomach ulcers, or hormonal changes, may indicate that MEN1 is affecting your hormone-producing glands. […] If something feels off, trust your instincts and get checked—early detection can make a big difference in managing MEN1 effectively. […] Diagnosing Multiple Endocrine Neoplasia Type 1 (MEN1) involves a combination of genetic testing, blood tests, and imaging scans to check for hormone imbalances and tumor growth. […] Early diagnosis helps with better management and treatment of MEN1, especially for those with a family history of the condition. […] The treatment for Multiple Endocrine Neoplasia Type 1 (MEN1) depends on the type and location of tumors. Common treatment options include surgery to remove tumors, especially in the parathyroid, pancreas, or pituitary glands. […] Since MEN1 is a lifelong condition, regular monitoring and follow-up care are essential to detect and manage tumors early.

• #33 Multiple Endocrine Neoplasia Type 1 | St. Jude Care & Treatment

  https://www.stjude.org/care-treatment/treatment/genetic-syndromes/multiple-endocrine-neoplasia-type-1.html

  It is also important that parents work with a genetic counselor to find experienced health care providers for their child. […] Although most of the tumors related to MEN are not cancerous, people with MEN1 should be monitored for the development of these tumors throughout their lives. Some non-cancerous tumors cause problems. […] The following lifelong screenings are recommended for people with MEN1: Yearly physical exams by a health care provider who is familiar with multiple endocrine neoplasia type 1; Blood tests to screen for abnormally high levels of various hormones and calcium in the blood. These should be started at age 5 and repeated yearly; MRI of the head starting at age 5 and repeated every 3-5 years; MRI or CT scan of the abdomen beginning at age 10. […] People of any age with MEN1 have a higher risk of tumors than people without MEN1. They should monitor their health and adopt healthy habits throughout life. It is important to continue to have regular physical checkups and screenings. That way, any tumor can be found early at the most treatable stage.

• #34 Multiple Endocrine Neoplasia Type 1 (MEN1) Follow-up: Further Outpatient Care, Prognosis

  https://emedicine.medscape.com/article/126438-followup

  Current clinical guidelines suggest that patients with multiple endocrine neoplasia type 1 undergo annual screening for pancreatic neuroendocrine tumors (pNETs), using plasma hormonal measurements and imaging studies. Screening can permit timely interventions to prevent morbidity and mortality related to metastasis. […] Current guidelines also suggest biochemical screening of pituitary tumors, with levels of plasma prolactin (PRL) and insulinlike growth factor1 (IGF-1) measured annually and head magnetic resonance imaging (MRI) performed every 3-5 years. […] Untreated multiple endocrine neoplasia type 1 (MEN1) patients have a decreased life expectancy, with a 50% probability of death by age 50 years. The cause of death is usually associated with a malignant tumor or sequelae of the disease.

• #35 Multiple Endocrine Neoplasia Type 1 | St. Jude Care & Treatment

  https://www.stjude.org/care-treatment/treatment/genetic-syndromes/multiple-endocrine-neoplasia-type-1.html

  It is also important that parents work with a genetic counselor to find experienced health care providers for their child. […] Although most of the tumors related to MEN are not cancerous, people with MEN1 should be monitored for the development of these tumors throughout their lives. Some non-cancerous tumors cause problems. […] The following lifelong screenings are recommended for people with MEN1: Yearly physical exams by a health care provider who is familiar with multiple endocrine neoplasia type 1; Blood tests to screen for abnormally high levels of various hormones and calcium in the blood. These should be started at age 5 and repeated yearly; MRI of the head starting at age 5 and repeated every 3-5 years; MRI or CT scan of the abdomen beginning at age 10. […] People of any age with MEN1 have a higher risk of tumors than people without MEN1. They should monitor their health and adopt healthy habits throughout life. It is important to continue to have regular physical checkups and screenings. That way, any tumor can be found early at the most treatable stage.

• #36 Multiple Endocrine Neoplasia Type 1 | St. Jude Care & Treatment

  https://www.stjude.org/care-treatment/treatment/genetic-syndromes/multiple-endocrine-neoplasia-type-1.html

  It is also important that parents work with a genetic counselor to find experienced health care providers for their child. […] Although most of the tumors related to MEN are not cancerous, people with MEN1 should be monitored for the development of these tumors throughout their lives. Some non-cancerous tumors cause problems. […] The following lifelong screenings are recommended for people with MEN1: Yearly physical exams by a health care provider who is familiar with multiple endocrine neoplasia type 1; Blood tests to screen for abnormally high levels of various hormones and calcium in the blood. These should be started at age 5 and repeated yearly; MRI of the head starting at age 5 and repeated every 3-5 years; MRI or CT scan of the abdomen beginning at age 10. […] People of any age with MEN1 have a higher risk of tumors than people without MEN1. They should monitor their health and adopt healthy habits throughout life. It is important to continue to have regular physical checkups and screenings. That way, any tumor can be found early at the most treatable stage.

• #37 Multiple endocrine neoplasia type 1 | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-1-38

  As many of the organs at highest risk of tumour development in MEN1 syndrome such as duodenum, pancreas and lungs (bronchial carcinoids) are not suitable for preventive ablative surgery, routine surveillance of asymptomatic MEN1 at-risk individuals by biochemical analysis and imaging procedures (beginning in early childhood and continuing for life) is recommended. […] Early detection and treatment of the potential malignant neuroendocrine tumours should reduce the morbidity and mortality of MEN1 syndrome. Such screenings can detect the onset of the disease about ten years before symptoms develop and thus provide an opportunity for earlier treatment. […] According to the International Guidelines for Diagnosis and Therapy of the MENs syndromes the minimal surveillance program for individuals known to have MEN1 syndrome or to have a family-specific mutation of the MEN1 gene should include: biochemical evaluation of serum concentration of prolactin from age 5; biochemical screening of fasting total serum calcium concentration (corrected for albumin) from age 8; biochemical screening of fasting serum gastrin concentration from age 20; magnetic resonance imaging (MRI) of the head from age 5 and every 3-5 years; abdominal CT or MRI from age 20 and every 3-5 years.

• #38 Care for patients with multiple endocrine neoplasia type 1: the current evidence base – PubMed

  https://pubmed.ncbi.nlm.nih.gov/21061174/

  Multiple endocrine neoplasia type 1 (MEN1) is a rare disease caused by mutations in the MEN1 gene on chromosome 11. It is characterized by the occurrence of primary hyperparathyroidism (pHPT), duodenopancreatic neuroendocrine tumours (pNET), pituitary tumours (PIT), adrenal adenomas (ADR) and neuroendocrine tumours (NET) of the stomach, bronchus and thymus. MEN1 is a syndrome with high penetrance and high morbidity. Malignant NETs are the most important cause of MEN1-related death. Since 1997 the diagnosis can be made by genetic screening. MEN1 is a complex syndrome and the endocrine manifestations cannot be viewed upon as coinciding sporadic tumours. Differences in epidemiology and pathology between MEN1-related tumours and their sporadic counterparts show that a unique approach is needed. Therefore the care for MEN1 patients should be provided by a centre of expertise. Early genetic diagnosis and periodic screening are important pillars of care. For primary hyperparathyroidism surgery is the most important treatment modality, with a subtotal parathyroid gland resection as the procedure of choice. In neuroendocrine tumours surgery also is the most important treatment modality. Selective tumour enucleation has no place in the surgical treatment of MEN1-related pNETs; the exact procedure depends on the functionality of the tumour. In MEN1-associated pituitary and adrenal adenomas, watchful waiting and medical therapy play more important roles. In the twenty-first century new developments will impact the care for MEN1 patients. These developments should be critically evaluated in clinical research with the ultimate goal of optimizing the care for MEN1 patients on an evidence base.

• #39 Genetic Disorder Reference Sheet: Multiple Endocrine Neoplasia Type 1 | Oncology Nursing Society

  https://onf.ons.org/publications-research/voice/news-views/07-2022/genetic-disorder-reference-sheet-multiple-endocrine

  The identification of a pathogenic MEN1 variant in a patient has implications for family members because first-degree relatives have a 50% risk of developing the syndrome. Patients with two or more symptoms should be referred to a genetics professional for MEN1 testing, which is recommended around age 3.

• #40 Multiple endocrine neoplasia, type 1 (MEN 1) | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/multiple-endocrine-neoplasia-type-1-men-1?content_id=CON-20309660

  Symptoms are caused by the release of too many hormones in the body. […] With MEN 1, tumors can grow on the parathyroid glands, the pancreas and the pituitary gland. That can lead to various conditions, all of which can be treated. […] Genetic testing may help find out whether someone has a genetic change that causes MEN 1. If so, that person’s children are at risk of having the same genetic change and getting MEN 1. Parents and siblings also are at risk of having the genetic change that causes MEN 1. […] If no related genetic changes are found in family members, then family members don’t need more screening tests. But genetic testing cannot find all the genetic changes that can cause MEN 1. If genetic testing doesn’t confirm MEN 1, but it’s likely that a person has it, more testing is needed. That person, as well as family members, still needs follow-up healthcare checkups with blood tests and imaging tests.

• #41 Genetic Disorder Reference Sheet: Multiple Endocrine Neoplasia Type 1 | Oncology Nursing Society

  https://onf.ons.org/publications-research/voice/news-views/07-2022/genetic-disorder-reference-sheet-multiple-endocrine

  Multiple endocrine neoplasia type 1 (MEN1) is a rare autosomal dominant genetic disease affecting about 1 in 30,000 people. Pathogenic variants in the MEN1 gene are associated with benign and malignant tumors in the parathyroid glands, pituitary gland, pancreas, duodenum, and stomach. […] Individuals with an MEN1 pathogenic variant are at increased risk for developing benign and malignant tumors (see sidebar). […] Ideally, people with a pathogenic MEN1 variant are managed by an interprofessional team that is familiar with its multiple complications. […] A primary nursing responsibility for MEN1 is providing ongoing psychosocial support and education on symptom identification and the importance of ongoing screening to detect tumors early, intervene accordingly, and improve prognosis. […] Smoking increases the risk of carcinoid tumors in MEN1. Nurses should encourage smoking cessation and help patients enroll in programs to quit.

• #42 AACE Management of Multiple Endocrine Neoplasia Type 1 Guideline Summary

  https://www.guidelinecentral.com/guideline/4530663/

  This consensus statement provides a comprehensive framework for managing patients with MEN1 and associated tumors. MEN1 is a rare hereditary condition characterized by the development of tumors in multiple endocrine glands, including the parathyroids, pancreatic islets, and anterior pituitary gland, among other tissues. […] Guidance includes the use of high-resolution ultrasound, CT, MRI, and nuclear medicine techniques such as somatostatin receptor PET scans, as well as guidance on surgical management for the different types of tumors associated with MEN1. Since MEN1 is a chronic condition with potential for recurrent tumors, this consensus statement emphasizes the importance of lifelong monitoring. This could involve regular physical examinations, biochemical assessments, and repeat imaging as appropriate. Emphasis is placed on the importance of a multidisciplinary team involving endocrinologists, surgeons, genetic counselors, radiologists, pathologists, oncologists, and primary care physicians to handle the various aspects of MEN1 management.

• #43

  https://link.springer.com/article/10.1007/s10689-025-00440-4

  In addition, the lifelong risk of developing NEN requires special considerations to avoid excessive surgeries and to minimize damage to the patients function and well-being. This progress report will outline current insights into surveillance and management of the major clinical manifestation of MEN1 syndrome in children and adults with MEN1 diagnosis. […] The goal of screening is two-fold: (1) Early detection of neoplastic processes, mainly regarding NEN of the duodenum, pancreas, lungs, and pituitary adenomas; and (2) Identification of endocrine alterations that require intervention (in PHPT) or of those alterations that may stem from the neoplastic process (PitAd, functional DP-NEN). […] The first line of treatment for PHPT in MEN1 is surgical intervention. The classic surgical approach for patients with MEN1 dictates open bilateral exploration with either subtotal (ST, 3-3.5 glands) removal or total parathyroidectomy with partial gland auto-transplantation (TP/AT).

• #44 Multiple Endocrine Neoplasia, Type 1 (MEN 1) – Endocrine and Metabolic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/endocrine-and-metabolic-disorders/multiple-endocrine-neoplasia-men-syndromes/multiple-endocrine-neoplasia-type-1-men-1

  Multiple endocrine neoplasia, type 1 (MEN 1) is an autosomal dominant syndrome characterized by hyperplasia or adenomas of the parathyroid glands, pancreatic islet cell tumors (also known as pancreatic neuroendocrine tumors), and/or pituitary gland tumors. […] Diagnosis is by hormonal and imaging tests. […] Tumors are removed surgically if they cause symptoms or for suspected malignancy based on size criteria. […] Treatment of hyperparathyroidism is primarily surgical, with subtotal parathyroidectomy; however, hyperparathyroidism frequently recurs. […] Prolactinoma is usually managed with dopamine agonists; other pituitary tumors are treated surgically. […] Patients should have genetic testing of the MEN 1 gene and clinical evaluation for other tumors of the syndrome. […] Tumors are excised when possible, but lesions are often multiple and/or difficult to find. […] Sometimes hormone excess can be managed by medications.

• #45

  https://link.springer.com/article/10.1007/s10689-025-00440-4

  The recurrence rate of PHPT in MEN1 is as high as 53% after an average ten-year follow-up, compared with up to 5% recurrence in the general population after surgery. […] The management of patients with functional neuroendocrine neoplasms is bi-dimensional and requires taking into account both the oncological implications of the tumor and the endocrine derangements. […] The endocrine manifestations are unique for each hormone oversecretion-tumor: gastrin oversecretion, leading to Zollinger-Ellison syndrome (ZES), can be readily controlled using proton-pump inhibitors (PPIs) while insulin oversecretion leading to hypoglycemia episodes might be more challenging to control. […] The diagnosis and management of insulinoma follow the current guidelines for sporadic disease. Once there is a biochemical validation for insulinoma, it should be located and resected to avoid additional hypoglycemic episodes.

• #46 Multiple Endocrine Neoplasia Type 1 (MEN1) | Doctor

  https://patient.info/doctor/multiple-endocrine-neoplasia-type-1-men1

  The surgical approach to pancreatic endocrine tumours in MEN1 is controversial. Surgical cure is best achieved by removing the pancreas and duodenum with adjacent lymph nodes. There is still a high rate of recurrence but the overall mortality remains low. […] Parathyroidectomy, subtotal or complete, is practised for MEN1 but long-term follow-up reveals a high rate of recurrence in MEN1 despite surgical intervention.

• #47 Multiple Endocrine Neoplasia Type 1 (09.08.2024)

  https://di.aerzteblatt.de/int/archive/article/240614

  The treatment of MEN1-associated pituitary adenoma is similar to that of sporadic pituitary adenoma. It is determined based on the size of the tumor and its functional activity. […] MEN1-associated primary hyperparathyroidism (pHPT) is diagnosed based on laboratory tests showing hypercalcemia and elevated parathyroid hormone levels. Surgery is always indicated, given that long-term hypercalcemia can result in secondary damage such as osteoporosis, depression, kidney stones or gastric ulcers. […] The extent to which the parathyroid gland tissue should be removed is subject to ongoing controversy. […] In patients with MEN1-associated bronchial NENs, segmental lung resection or lobectomy with lymphadenectomy is indicated in the following cases: functioning tumors, symptomatic tumors and tumor size 2 cm.

• #48 Multiple Endocrine Neoplasia, Type 1 (MEN 1) – Endocrine and Metabolic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/endocrine-and-metabolic-disorders/multiple-endocrine-neoplasia-men-syndromes/multiple-endocrine-neoplasia-type-1-men-1

  Multiple endocrine neoplasia, type 1 (MEN 1) is an autosomal dominant syndrome characterized by hyperplasia or adenomas of the parathyroid glands, pancreatic islet cell tumors (also known as pancreatic neuroendocrine tumors), and/or pituitary gland tumors. […] Diagnosis is by hormonal and imaging tests. […] Tumors are removed surgically if they cause symptoms or for suspected malignancy based on size criteria. […] Treatment of hyperparathyroidism is primarily surgical, with subtotal parathyroidectomy; however, hyperparathyroidism frequently recurs. […] Prolactinoma is usually managed with dopamine agonists; other pituitary tumors are treated surgically. […] Patients should have genetic testing of the MEN 1 gene and clinical evaluation for other tumors of the syndrome. […] Tumors are excised when possible, but lesions are often multiple and/or difficult to find. […] Sometimes hormone excess can be managed by medications.

• #49 Multiple Endocrine Neoplasia Type 1 (09.08.2024)

  https://di.aerzteblatt.de/int/archive/article/240614

  The treatment of MEN1-associated pituitary adenoma is similar to that of sporadic pituitary adenoma. It is determined based on the size of the tumor and its functional activity. […] MEN1-associated primary hyperparathyroidism (pHPT) is diagnosed based on laboratory tests showing hypercalcemia and elevated parathyroid hormone levels. Surgery is always indicated, given that long-term hypercalcemia can result in secondary damage such as osteoporosis, depression, kidney stones or gastric ulcers. […] The extent to which the parathyroid gland tissue should be removed is subject to ongoing controversy. […] In patients with MEN1-associated bronchial NENs, segmental lung resection or lobectomy with lymphadenectomy is indicated in the following cases: functioning tumors, symptomatic tumors and tumor size 2 cm.

• #50 Multiple endocrine neoplasia, type 1 (MEN 1) | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/multiple-endocrine-neoplasia-type-1-men-1

  Genetic testing may help find out whether someone has a genetic change that causes MEN 1. If so, that person’s children are at risk of having the same genetic change and getting MEN 1. Parents and siblings also are at risk of having the genetic change that causes MEN 1. […] Surgery to remove most of the parathyroid glands is the typical treatment for too much parathyroid hormone. […] Healthcare professionals may prescribe medicine or try to remove the tumors with surgery. […] If the tumors make hormones or they’re large and thought to be cancerous, healthcare professionals recommend removing them. […] Surgeons remove these tumors when they haven’t spread to other areas. Healthcare professionals may use chemotherapy, radiation therapy or hormone-based therapy for advanced carcinoid tumors.

• #51 Multiple endocrine neoplasia type 1 | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-1-38

  As many of the organs at highest risk of tumour development in MEN1 syndrome such as duodenum, pancreas and lungs (bronchial carcinoids) are not suitable for preventive ablative surgery, routine surveillance of asymptomatic MEN1 at-risk individuals by biochemical analysis and imaging procedures (beginning in early childhood and continuing for life) is recommended. […] Early detection and treatment of the potential malignant neuroendocrine tumours should reduce the morbidity and mortality of MEN1 syndrome. Such screenings can detect the onset of the disease about ten years before symptoms develop and thus provide an opportunity for earlier treatment. […] According to the International Guidelines for Diagnosis and Therapy of the MENs syndromes the minimal surveillance program for individuals known to have MEN1 syndrome or to have a family-specific mutation of the MEN1 gene should include: biochemical evaluation of serum concentration of prolactin from age 5; biochemical screening of fasting total serum calcium concentration (corrected for albumin) from age 8; biochemical screening of fasting serum gastrin concentration from age 20; magnetic resonance imaging (MRI) of the head from age 5 and every 3-5 years; abdominal CT or MRI from age 20 and every 3-5 years.

• #52 Multiple endocrine neoplasia type 1 – Genomics Education Programme

  https://www.genomicseducation.hee.nhs.uk/documents/multiple-endocrine-neoplasia-type-1/

  Multiple endocrine neoplasia type 1 (MEN1) is an inherited condition that leads to growth of mainly benign (non-cancerous) tumours of the endocrine glands. […] Individuals with a variant in the MEN1 gene should undergo regular surveillance to screen for MEN1-associated tumours and their hormonal effects. […] Early diagnosis, treatment and management improves outcome and quality of life for those affected. […] Regular surveillance is needed to screen for MEN1-associated tumours and their hormonal effects. […] Screening may include a medical review for assessment of symptoms, biochemical screening to check the level of PTH /calcium and other hormones, and imaging of the pituitary/pancreatic gland. […] Parathyroid tumours can be treated by surgical removal of the parathyroid glands, of which there are four. […] Treatment of pituitary tumours may require medical treatment or surgery, and in some cases radiotherapy. […] The treatment of pancreatic tumours will depend on the size, type, number and location of the tumours.

• #53 Multiple Endocrine Neoplasia Type 1 | St. Jude Care & Treatment

  https://www.stjude.org/care-treatment/treatment/genetic-syndromes/multiple-endocrine-neoplasia-type-1.html

  It is also important that parents work with a genetic counselor to find experienced health care providers for their child. […] Although most of the tumors related to MEN are not cancerous, people with MEN1 should be monitored for the development of these tumors throughout their lives. Some non-cancerous tumors cause problems. […] The following lifelong screenings are recommended for people with MEN1: Yearly physical exams by a health care provider who is familiar with multiple endocrine neoplasia type 1; Blood tests to screen for abnormally high levels of various hormones and calcium in the blood. These should be started at age 5 and repeated yearly; MRI of the head starting at age 5 and repeated every 3-5 years; MRI or CT scan of the abdomen beginning at age 10. […] People of any age with MEN1 have a higher risk of tumors than people without MEN1. They should monitor their health and adopt healthy habits throughout life. It is important to continue to have regular physical checkups and screenings. That way, any tumor can be found early at the most treatable stage.

• #54 Multiple endocrine neoplasia type 1 | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-1-38

  As many of the organs at highest risk of tumour development in MEN1 syndrome such as duodenum, pancreas and lungs (bronchial carcinoids) are not suitable for preventive ablative surgery, routine surveillance of asymptomatic MEN1 at-risk individuals by biochemical analysis and imaging procedures (beginning in early childhood and continuing for life) is recommended. […] Early detection and treatment of the potential malignant neuroendocrine tumours should reduce the morbidity and mortality of MEN1 syndrome. Such screenings can detect the onset of the disease about ten years before symptoms develop and thus provide an opportunity for earlier treatment. […] According to the International Guidelines for Diagnosis and Therapy of the MENs syndromes the minimal surveillance program for individuals known to have MEN1 syndrome or to have a family-specific mutation of the MEN1 gene should include: biochemical evaluation of serum concentration of prolactin from age 5; biochemical screening of fasting total serum calcium concentration (corrected for albumin) from age 8; biochemical screening of fasting serum gastrin concentration from age 20; magnetic resonance imaging (MRI) of the head from age 5 and every 3-5 years; abdominal CT or MRI from age 20 and every 3-5 years.

• #55

  https://www.singhealth.com.sg/patient-care/conditions-treatments/multiple-endocrine-neoplasia-type-1

  Multiple Endocrine Neoplasia Type 1 is a hereditary tumour/cancer syndrome. […] Multiple Endocrine Neoplasia Type 1 (MEN1) is a hereditary condition associated with an increased risk of tumours of the endocrine (hormoneproducing) glands. […] MEN1 is caused by a fault (i.e., mutation) in the MEN1 gene. […] The majority of individuals with MEN1 inherit the condition from a parent. […] Genetic testing is offered to individuals where a hereditary cause of their personal and/or family history of tumours/cancer is suspected. […] Screening helps to detect (and in some cases treat) tumours at an early and manageable stage. […] In some cases, risk-reducing surgery may be offered to help reduce the risk of tumours developing. […] Your managing doctor(s) will discuss screening recommendations with you in greater detail. The onset and frequency of screening would be personalised according to your needs, personal and family history. […] Understanding if you have MEN1 provides you with options for managing your increased risk of tumours/cancer.

• #56 Multiple endocrine neoplasia type 1 | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-1-38

  As many of the organs at highest risk of tumour development in MEN1 syndrome such as duodenum, pancreas and lungs (bronchial carcinoids) are not suitable for preventive ablative surgery, routine surveillance of asymptomatic MEN1 at-risk individuals by biochemical analysis and imaging procedures (beginning in early childhood and continuing for life) is recommended. […] Early detection and treatment of the potential malignant neuroendocrine tumours should reduce the morbidity and mortality of MEN1 syndrome. Such screenings can detect the onset of the disease about ten years before symptoms develop and thus provide an opportunity for earlier treatment. […] According to the International Guidelines for Diagnosis and Therapy of the MENs syndromes the minimal surveillance program for individuals known to have MEN1 syndrome or to have a family-specific mutation of the MEN1 gene should include: biochemical evaluation of serum concentration of prolactin from age 5; biochemical screening of fasting total serum calcium concentration (corrected for albumin) from age 8; biochemical screening of fasting serum gastrin concentration from age 20; magnetic resonance imaging (MRI) of the head from age 5 and every 3-5 years; abdominal CT or MRI from age 20 and every 3-5 years.

• #57 Multiple Endocrine Neoplasia Type 1 | SpringerLink

  https://link.springer.com/10.1007/978-3-319-89497-3_8

  The Multiple Endocrine Neoplasia (MEN1) is an autosomal dominant disease due to mutation in the MEN1 gene. MEN1 is a life-threatening disease, because duodeno-pancreatic and thymic tumors may turn into aggressive neuroendocrine cancers without early detection. […] Consequently, the patients are identified as at risk for developing other MEN1- lesions and should be offered a program of combined clinical, biochemical, and radiological screening, according to clinical practice guidelines. […] Furthermore, the identification of MEN1 mutation in relatives is considered as a predictive test for developing MEN1 lesions. […] Nevertheless, the pitfalls of genetic testing and the difficulties in the interpretation of MEN1 variants are the new challenges in 2018.

• #58 Genetic Disorder Reference Sheet: Multiple Endocrine Neoplasia Type 1 | Oncology Nursing Society

  https://onf.ons.org/publications-research/voice/news-views/07-2022/genetic-disorder-reference-sheet-multiple-endocrine

  The identification of a pathogenic MEN1 variant in a patient has implications for family members because first-degree relatives have a 50% risk of developing the syndrome. Patients with two or more symptoms should be referred to a genetics professional for MEN1 testing, which is recommended around age 3.

• #59 Multiple endocrine neoplasia 1: providing care for the family – PubMed

  https://pubmed.ncbi.nlm.nih.gov/20880824/

  Multiple endocrine neoplasia 1 (MEN1) is a hereditary syndrome associated with a number of endocrine and nonendocrine tumors. […] Nurses need to be familiar with hallmark signs of this syndrome to facilitate an early diagnosis and appropriately refer families for genetic assessment and, ultimately, develop a long-term plan for early detection and intervention for all family members at risk for MEN1. […] Multiple Endocrine Neoplasia Type 1 / nursing.

• #60 Genetic Disorder Reference Sheet: Multiple Endocrine Neoplasia Type 1 | Oncology Nursing Society

  https://onf.ons.org/publications-research/voice/news-views/07-2022/genetic-disorder-reference-sheet-multiple-endocrine

  Multiple endocrine neoplasia type 1 (MEN1) is a rare autosomal dominant genetic disease affecting about 1 in 30,000 people. Pathogenic variants in the MEN1 gene are associated with benign and malignant tumors in the parathyroid glands, pituitary gland, pancreas, duodenum, and stomach. […] Individuals with an MEN1 pathogenic variant are at increased risk for developing benign and malignant tumors (see sidebar). […] Ideally, people with a pathogenic MEN1 variant are managed by an interprofessional team that is familiar with its multiple complications. […] A primary nursing responsibility for MEN1 is providing ongoing psychosocial support and education on symptom identification and the importance of ongoing screening to detect tumors early, intervene accordingly, and improve prognosis. […] Smoking increases the risk of carcinoid tumors in MEN1. Nurses should encourage smoking cessation and help patients enroll in programs to quit.

• #61 Multiple endocrine neoplasia 1: providing care for the family – PubMed

  https://pubmed.ncbi.nlm.nih.gov/20880824/

  Multiple endocrine neoplasia 1 (MEN1) is a hereditary syndrome associated with a number of endocrine and nonendocrine tumors. […] Nurses need to be familiar with hallmark signs of this syndrome to facilitate an early diagnosis and appropriately refer families for genetic assessment and, ultimately, develop a long-term plan for early detection and intervention for all family members at risk for MEN1. […] Multiple Endocrine Neoplasia Type 1 / nursing.

• #62 Genetic Disorder Reference Sheet: Multiple Endocrine Neoplasia Type 1 | Oncology Nursing Society

  https://onf.ons.org/publications-research/voice/news-views/07-2022/genetic-disorder-reference-sheet-multiple-endocrine

  The identification of a pathogenic MEN1 variant in a patient has implications for family members because first-degree relatives have a 50% risk of developing the syndrome. Patients with two or more symptoms should be referred to a genetics professional for MEN1 testing, which is recommended around age 3.

• #63 Quality of life in Italian patients with Multiple endocrine neoplasia type 1 (MEN 1): results of an extensive survey | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-01650-y

  The IES-R test revealed the presence of symptoms of post-traumatic stress in 75% of our MEN1 patients: the most common symptom was intrusiveness, followed by avoidance and hyperarousal. […] The HADS test showed that the cases of major anxiety and depression were 27.64% and 7.90%, respectively. […] Our results confirmed that MEN1 patients should be followed up over time regarding their psychological status, and they should be given specific assessments for anxiety and/or depression evaluation. […] The SF-36 questionnaire showed that our MEN1 series was principally composed by people who have minimal difficulties in working or performing other daily activities due to emotional or physical problems, have good physical health, and believe that it is similar to that of their peers. […] Despite the presence of a rare pathology affecting patients throughout their lifetimes, results from the study indicated that our group of patients was moderately optimistic (50%), correlating with a QoL in the norm.

• #64 Genetic Disorder Reference Sheet: Multiple Endocrine Neoplasia Type 1 | Oncology Nursing Society

  https://onf.ons.org/publications-research/voice/news-views/07-2022/genetic-disorder-reference-sheet-multiple-endocrine

  Multiple endocrine neoplasia type 1 (MEN1) is a rare autosomal dominant genetic disease affecting about 1 in 30,000 people. Pathogenic variants in the MEN1 gene are associated with benign and malignant tumors in the parathyroid glands, pituitary gland, pancreas, duodenum, and stomach. […] Individuals with an MEN1 pathogenic variant are at increased risk for developing benign and malignant tumors (see sidebar). […] Ideally, people with a pathogenic MEN1 variant are managed by an interprofessional team that is familiar with its multiple complications. […] A primary nursing responsibility for MEN1 is providing ongoing psychosocial support and education on symptom identification and the importance of ongoing screening to detect tumors early, intervene accordingly, and improve prognosis. […] Smoking increases the risk of carcinoid tumors in MEN1. Nurses should encourage smoking cessation and help patients enroll in programs to quit.

• #65 Quality of life in Italian patients with Multiple endocrine neoplasia type 1 (MEN 1): results of an extensive survey | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-01650-y

  The IES-R test revealed the presence of symptoms of post-traumatic stress in 75% of our MEN1 patients: the most common symptom was intrusiveness, followed by avoidance and hyperarousal. […] The HADS test showed that the cases of major anxiety and depression were 27.64% and 7.90%, respectively. […] Our results confirmed that MEN1 patients should be followed up over time regarding their psychological status, and they should be given specific assessments for anxiety and/or depression evaluation. […] The SF-36 questionnaire showed that our MEN1 series was principally composed by people who have minimal difficulties in working or performing other daily activities due to emotional or physical problems, have good physical health, and believe that it is similar to that of their peers. […] Despite the presence of a rare pathology affecting patients throughout their lifetimes, results from the study indicated that our group of patients was moderately optimistic (50%), correlating with a QoL in the norm.

• #66 Multiple endocrine neoplasia 1: providing care for the family – PubMed

  https://pubmed.ncbi.nlm.nih.gov/20880824/

  Multiple endocrine neoplasia 1 (MEN1) is a hereditary syndrome associated with a number of endocrine and nonendocrine tumors. […] Nurses need to be familiar with hallmark signs of this syndrome to facilitate an early diagnosis and appropriately refer families for genetic assessment and, ultimately, develop a long-term plan for early detection and intervention for all family members at risk for MEN1. […] Multiple Endocrine Neoplasia Type 1 / nursing.

• #67 Multiple Endocrine Neoplasia Type 1 | St. Jude Care & Treatment

  https://www.stjude.org/care-treatment/treatment/genetic-syndromes/multiple-endocrine-neoplasia-type-1.html

  The St. Jude Cancer Predisposition Program is dedicated to diagnosing and screening children with a genetic predisposition for cancer. Our program offers the best clinical care possible. We engage in cutting-edge research to gain more information about genetic disorders and cancer, and to improve the care and treatment for patients who are affected by these conditions. […] Our genetic counselors, clinicians, genetics nurse practitioners, and research assistants work together to provide patients with: A complete health history; A record of cancer that happened over the past 3 generations (family tree or pedigree) to see how diseases were passed down; Physical exams to check for physical signs of a genetic disorder or disease; Confidential genetic counseling and testing; Cancer screenings for those with genetic disease to detect and treat cancer as soon as it occurs; Recommended cancer treatments and ways to reduce cancer risk; Genetic testing for immediate (1st degree) relatives (including siblings and parents).

• #68 Multiple endocrine neoplasia 1: providing care for the family – PubMed

  https://pubmed.ncbi.nlm.nih.gov/20880824/

  Multiple endocrine neoplasia 1 (MEN1) is a hereditary syndrome associated with a number of endocrine and nonendocrine tumors. […] Nurses need to be familiar with hallmark signs of this syndrome to facilitate an early diagnosis and appropriately refer families for genetic assessment and, ultimately, develop a long-term plan for early detection and intervention for all family members at risk for MEN1. […] Multiple Endocrine Neoplasia Type 1 / nursing.

• #69 Multiple Endocrine Neoplasia Type 1 | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/25356

  MEN1 is a complex syndrome that increases morbidity and mortality by causing multiple aggressive tumors. An interprofessional team should manage these patients in a tertiary care center, including an endocrinologist, endocrine surgeon, gastroenterologist, neurosurgeon, genetic counselor, nursing staff, pharmacist, and patient navigator or advocate for achieving better outcomes.

• #70 Multiple Endocrine Neoplasia Type 1 | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/25356

  MEN1 is a complex syndrome that increases morbidity and mortality by causing multiple aggressive tumors. An interprofessional team should manage these patients in a tertiary care center, including an endocrinologist, endocrine surgeon, gastroenterologist, neurosurgeon, genetic counselor, nursing staff, pharmacist, and patient navigator or advocate for achieving better outcomes.

• #71 Multiple Endocrine Neoplasia (MEN): Types & Symptoms

  https://my.clevelandclinic.org/health/diseases/23088-multiple-endocrine-neoplasia-men

  Endocrine glands that are affected by tumors usually release excessive amounts of hormones into your bloodstream, which can result in a variety of symptoms and health issues. […] Since MEN type 1 was discovered, healthcare providers have identified more than 20 different endocrine and non-endocrine tumors in individuals with MEN type 1. […] The treatment of multiple endocrine neoplasia (MEN) depends entirely on what endocrine glands and organs are affected and usually requires a team of healthcare providers, including: Endocrinologists. Surgeons. Oncologists (cancer specialists). Pediatricians, if applicable. […] Treatment may include: Medications to treat symptoms and to counteract the effects of excess hormones. Surgery to remove tumors or entire affected glands, such as the thyroid. Replacement hormones if an endocrine gland is surgically removed. Cancer treatment, such as chemotherapy and radiation therapy, cancer has metastasized (spread to other areas of your body).

• #72 Care for patients with multiple endocrine neoplasia type 1: the current evidence base – PubMed

  https://pubmed.ncbi.nlm.nih.gov/21061174/

  Multiple endocrine neoplasia type 1 (MEN1) is a rare disease caused by mutations in the MEN1 gene on chromosome 11. It is characterized by the occurrence of primary hyperparathyroidism (pHPT), duodenopancreatic neuroendocrine tumours (pNET), pituitary tumours (PIT), adrenal adenomas (ADR) and neuroendocrine tumours (NET) of the stomach, bronchus and thymus. MEN1 is a syndrome with high penetrance and high morbidity. Malignant NETs are the most important cause of MEN1-related death. Since 1997 the diagnosis can be made by genetic screening. MEN1 is a complex syndrome and the endocrine manifestations cannot be viewed upon as coinciding sporadic tumours. Differences in epidemiology and pathology between MEN1-related tumours and their sporadic counterparts show that a unique approach is needed. Therefore the care for MEN1 patients should be provided by a centre of expertise. Early genetic diagnosis and periodic screening are important pillars of care. For primary hyperparathyroidism surgery is the most important treatment modality, with a subtotal parathyroid gland resection as the procedure of choice. In neuroendocrine tumours surgery also is the most important treatment modality. Selective tumour enucleation has no place in the surgical treatment of MEN1-related pNETs; the exact procedure depends on the functionality of the tumour. In MEN1-associated pituitary and adrenal adenomas, watchful waiting and medical therapy play more important roles. In the twenty-first century new developments will impact the care for MEN1 patients. These developments should be critically evaluated in clinical research with the ultimate goal of optimizing the care for MEN1 patients on an evidence base.

• #73 Multiple Endocrine Neoplasia Type 1 (09.08.2024)

  https://di.aerzteblatt.de/int/archive/article/240614

  Multiple endocrine neoplasia type 1 (MEN1) is a rare genetic disease of autosomal dominant inheritance, with an estimated prevalence of 320/100 000. Its main feature is neuroendocrine neoplasia in the parathyroid glands, the endocrine pancreas, the duodenum, and the pituitary gland. […] The early diagnosis and individualized treatment of MEN1-associated tumors can prevent the development of life-threatening hormonal syndromes and prolong the expected life span of MEN1 patients from 55 to 70 years, as well as improving their quality of life. […] Carriers of disease-causing mutations in the MEN1 gene should be cared for in specialized interdisciplinary centers, so that any appreciable tumor growth or hormonal activity can be detected early and organ-sparing treatment can be provided. […] Given the complexity of the disease, specific diagnosis and treatment should be the responsibility of a multidisciplinary team (covering endocrinology, gastroenterology, surgery, radiology, nuclear medicine, and pathology).

• #74 Quality of life in Italian patients with Multiple endocrine neoplasia type 1 (MEN 1): results of an extensive survey | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-01650-y

  We hypothesized that the relatively high percentage of optimistic approach could be due to the fact that, thanks to the dedicated Referral Center and the availability of personalized and constant follow-up, usually carried out by the same clinicians, patients feel involved in the decision-making process regarding their care, and consider their team well-informed on the management of their syndrome, giving them confidence that they will receive the best possible treatment.

• #75 Multiple Endocrine Neoplasia | MD Anderson Cancer Center

  https://www.mdanderson.org/cancer-types/multiple-endocrine-neoplasia.html

  The role of surgery in the treatment of other pancreatic tumors depends on each individual case. […] These tumors usually are benign. […] We recommend genetic counseling for anyone with MEN or a family history of the disease. […] Multiple endocrine neoplasia (MEN) syndromes are treated in MD Anderson’s Endocrine Center, one of the nation’s most active programs for diagnosis and treatment of these complex and rare diseases. […] Your care is personalized by a team of renowned experts from many specialties. Working closely with each other and with you, they customize your treatment plan and deliver the most advanced therapies with the least impact on your body. […] If surgery is needed, our surgeons have vast experience in proven procedures to treat multiple endocrine neoplasia. […] As one of the leaders in endocrine disorders, we are constantly researching new ways to treat MEN-related diseases.

• #76 Quality of life in Italian patients with Multiple endocrine neoplasia type 1 (MEN 1): results of an extensive survey | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-01650-y

  MEN1 is a complex, rare, syndrome inherited in an autosomal dominant tract and characterized by the development of multiple neuroendocrine tumors, requiring lifelong surveillance and multiple medical and surgical therapies throughout the patients life. […] The results of the study showed that our patients, despite having a complex multi-tumor syndrome, were moderately optimistic (50%), and this corresponds with a normal Quality of Life. […] The possibility of having access to a clinical Referral Center for their complex rare disease, together with the support of a dedicated patient association, has been demonstrated to be the ideal model for the management of post-diagnosis shock, and contributes to the preservation of a good Health-Related Quality of Life for MEN1 patients. […] The occurrence of multiple tumors, together with consequent surgery, accounts for a reduction of quality of life for MEN1 patients.

• #77 Quality of life in Italian patients with Multiple endocrine neoplasia type 1 (MEN 1): results of an extensive survey | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-01650-y

  MEN1 is a complex, rare, syndrome inherited in an autosomal dominant tract and characterized by the development of multiple neuroendocrine tumors, requiring lifelong surveillance and multiple medical and surgical therapies throughout the patients life. […] The results of the study showed that our patients, despite having a complex multi-tumor syndrome, were moderately optimistic (50%), and this corresponds with a normal Quality of Life. […] The possibility of having access to a clinical Referral Center for their complex rare disease, together with the support of a dedicated patient association, has been demonstrated to be the ideal model for the management of post-diagnosis shock, and contributes to the preservation of a good Health-Related Quality of Life for MEN1 patients. […] The occurrence of multiple tumors, together with consequent surgery, accounts for a reduction of quality of life for MEN1 patients.

• #78 Quality of life in Italian patients with Multiple endocrine neoplasia type 1 (MEN 1): results of an extensive survey | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-01650-y

  The diagnosis of a tumor is a trauma for patients and their families, mainly due to the uncertainty of its outcome. […] For years, Health-Related Quality of Life (HRQoL) remained only a marginal aspect of MEN1, with clinicians focusing only on solving diagnostic and therapeutic issues of the disease. […] An increase of our knowledge about emotional, psychological, relational, and social aspects of MEN1-related QoL in affected and/or diagnosed patients will help us provide them with the best care, reducing the negative influence of a pessimistic approach to the management of the disease. […] The present study aimed to perform a comprehensive survey about the individual perception of disease and HRQoL, in terms of both physical status and psychological, emotional, social, and economic impacts.

• #79 Quality of life in Italian patients with Multiple endocrine neoplasia type 1 (MEN 1): results of an extensive survey | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-01650-y

  MEN1 is a complex, rare, syndrome inherited in an autosomal dominant tract and characterized by the development of multiple neuroendocrine tumors, requiring lifelong surveillance and multiple medical and surgical therapies throughout the patients life. […] The results of the study showed that our patients, despite having a complex multi-tumor syndrome, were moderately optimistic (50%), and this corresponds with a normal Quality of Life. […] The possibility of having access to a clinical Referral Center for their complex rare disease, together with the support of a dedicated patient association, has been demonstrated to be the ideal model for the management of post-diagnosis shock, and contributes to the preservation of a good Health-Related Quality of Life for MEN1 patients. […] The occurrence of multiple tumors, together with consequent surgery, accounts for a reduction of quality of life for MEN1 patients.

• #80 Quality of life in Italian patients with Multiple endocrine neoplasia type 1 (MEN 1): results of an extensive survey | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-01650-y

  MEN1 is a complex, rare, syndrome inherited in an autosomal dominant tract and characterized by the development of multiple neuroendocrine tumors, requiring lifelong surveillance and multiple medical and surgical therapies throughout the patients life. […] The results of the study showed that our patients, despite having a complex multi-tumor syndrome, were moderately optimistic (50%), and this corresponds with a normal Quality of Life. […] The possibility of having access to a clinical Referral Center for their complex rare disease, together with the support of a dedicated patient association, has been demonstrated to be the ideal model for the management of post-diagnosis shock, and contributes to the preservation of a good Health-Related Quality of Life for MEN1 patients. […] The occurrence of multiple tumors, together with consequent surgery, accounts for a reduction of quality of life for MEN1 patients.

• #81 Quality of life in Italian patients with Multiple endocrine neoplasia type 1 (MEN 1): results of an extensive survey | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-01650-y

  The IES-R test revealed the presence of symptoms of post-traumatic stress in 75% of our MEN1 patients: the most common symptom was intrusiveness, followed by avoidance and hyperarousal. […] The HADS test showed that the cases of major anxiety and depression were 27.64% and 7.90%, respectively. […] Our results confirmed that MEN1 patients should be followed up over time regarding their psychological status, and they should be given specific assessments for anxiety and/or depression evaluation. […] The SF-36 questionnaire showed that our MEN1 series was principally composed by people who have minimal difficulties in working or performing other daily activities due to emotional or physical problems, have good physical health, and believe that it is similar to that of their peers. […] Despite the presence of a rare pathology affecting patients throughout their lifetimes, results from the study indicated that our group of patients was moderately optimistic (50%), correlating with a QoL in the norm.

• #82 Balancing the Benefits and Harms of Multiple Endocrine Neoplasia Type 1 (MEN1) Tumor ScreeningBalancing the Benefits and Harms of Multiple Endocrine Neoplasia Type 1 (MEN1) Tumor Screening, Cancer Screening

  https://www.ebsco.com/blogs/health-notes/balancing-benefits-and-harms-multiple-endocrine-neoplasia-type-1-men1-tumor

  Nearly all patients with MEN1 develop tumors, so screening is an important aspect of managing disease progression. […] Due to its high penetrance, screening for MEN1-related tumors is extremely important for improving prognosis and preventing mortality. Current screening guidelines recommend clinical, biochemical, and radiological screening for MEN1-related tumors every one-three years starting as young as five years old. […] Screening for MEN-1 related tumors is important, but is it effective enough to warrant the high physical, mental, and financial burden? […] To address the uncertainties surrounding MEN1 screening, larger studies and new biomarkers that can predict tumor behavior and disease course are needed; in the meantime, employing a joint decision-making approach can help patients feel that they have more control and alleviate some of their anxiety. […] Screening for MEN1-related tumors can be overwhelming, but including patients in the decision-making process may help lessen the mental, physical, and financial burden.

• #83 Quality of life in Italian patients with Multiple endocrine neoplasia type 1 (MEN 1): results of an extensive survey | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-01650-y

  MEN1 is a complex, rare, syndrome inherited in an autosomal dominant tract and characterized by the development of multiple neuroendocrine tumors, requiring lifelong surveillance and multiple medical and surgical therapies throughout the patients life. […] The results of the study showed that our patients, despite having a complex multi-tumor syndrome, were moderately optimistic (50%), and this corresponds with a normal Quality of Life. […] The possibility of having access to a clinical Referral Center for their complex rare disease, together with the support of a dedicated patient association, has been demonstrated to be the ideal model for the management of post-diagnosis shock, and contributes to the preservation of a good Health-Related Quality of Life for MEN1 patients. […] The occurrence of multiple tumors, together with consequent surgery, accounts for a reduction of quality of life for MEN1 patients.

• #84 Care for patients with multiple endocrine neoplasia type 1: the current evidence base – PubMed

  https://pubmed.ncbi.nlm.nih.gov/21061174/

  Multiple endocrine neoplasia type 1 (MEN1) is a rare disease caused by mutations in the MEN1 gene on chromosome 11. It is characterized by the occurrence of primary hyperparathyroidism (pHPT), duodenopancreatic neuroendocrine tumours (pNET), pituitary tumours (PIT), adrenal adenomas (ADR) and neuroendocrine tumours (NET) of the stomach, bronchus and thymus. MEN1 is a syndrome with high penetrance and high morbidity. Malignant NETs are the most important cause of MEN1-related death. Since 1997 the diagnosis can be made by genetic screening. MEN1 is a complex syndrome and the endocrine manifestations cannot be viewed upon as coinciding sporadic tumours. Differences in epidemiology and pathology between MEN1-related tumours and their sporadic counterparts show that a unique approach is needed. Therefore the care for MEN1 patients should be provided by a centre of expertise. Early genetic diagnosis and periodic screening are important pillars of care. For primary hyperparathyroidism surgery is the most important treatment modality, with a subtotal parathyroid gland resection as the procedure of choice. In neuroendocrine tumours surgery also is the most important treatment modality. Selective tumour enucleation has no place in the surgical treatment of MEN1-related pNETs; the exact procedure depends on the functionality of the tumour. In MEN1-associated pituitary and adrenal adenomas, watchful waiting and medical therapy play more important roles. In the twenty-first century new developments will impact the care for MEN1 patients. These developments should be critically evaluated in clinical research with the ultimate goal of optimizing the care for MEN1 patients on an evidence base.

• #85 Balancing the Benefits and Harms of Multiple Endocrine Neoplasia Type 1 (MEN1) Tumor ScreeningBalancing the Benefits and Harms of Multiple Endocrine Neoplasia Type 1 (MEN1) Tumor Screening, Cancer Screening

  https://www.ebsco.com/blogs/health-notes/balancing-benefits-and-harms-multiple-endocrine-neoplasia-type-1-men1-tumor

  Nearly all patients with MEN1 develop tumors, so screening is an important aspect of managing disease progression. […] Due to its high penetrance, screening for MEN1-related tumors is extremely important for improving prognosis and preventing mortality. Current screening guidelines recommend clinical, biochemical, and radiological screening for MEN1-related tumors every one-three years starting as young as five years old. […] Screening for MEN-1 related tumors is important, but is it effective enough to warrant the high physical, mental, and financial burden? […] To address the uncertainties surrounding MEN1 screening, larger studies and new biomarkers that can predict tumor behavior and disease course are needed; in the meantime, employing a joint decision-making approach can help patients feel that they have more control and alleviate some of their anxiety. […] Screening for MEN1-related tumors can be overwhelming, but including patients in the decision-making process may help lessen the mental, physical, and financial burden.

• #86 Multiple Endocrine Neoplasia Type 1 (09.08.2024)

  https://di.aerzteblatt.de/int/archive/article/240614

  Multiple endocrine neoplasia type 1 (MEN1) is a rare genetic disease of autosomal dominant inheritance, with an estimated prevalence of 320/100 000. Its main feature is neuroendocrine neoplasia in the parathyroid glands, the endocrine pancreas, the duodenum, and the pituitary gland. […] The early diagnosis and individualized treatment of MEN1-associated tumors can prevent the development of life-threatening hormonal syndromes and prolong the expected life span of MEN1 patients from 55 to 70 years, as well as improving their quality of life. […] Carriers of disease-causing mutations in the MEN1 gene should be cared for in specialized interdisciplinary centers, so that any appreciable tumor growth or hormonal activity can be detected early and organ-sparing treatment can be provided. […] Given the complexity of the disease, specific diagnosis and treatment should be the responsibility of a multidisciplinary team (covering endocrinology, gastroenterology, surgery, radiology, nuclear medicine, and pathology).

• #87 AACE Management of Multiple Endocrine Neoplasia Type 1 Guideline Summary

  https://www.guidelinecentral.com/guideline/4530663/

  This document presents the findings of the American Association of Clinical Endocrinology (AACE) on the diagnosis, management, and surveillance of patients with multiple endocrine neoplasia type 1 (MEN1) and associated tumors. The task force included a diverse group of experts in endocrinology, oncology, genetics, surgery, and patient representation. A comprehensive literature review was conducted to address key issues related to the evaluation, surveillance, and treatment of MEN1-related tumors.

• #88 2025 Consensus Statement on Management of Multiple Endocrine Neoplasia, Type 1 | American Association of Clinical Endocrinology

  https://pro.aace.com/clinical-guidance/2025-consensus-statement-management-multiple-endocrine-neoplasia-type-1

  This AACE consensus statement was developed by a multidisciplinary task force, which included medical professionals in endocrinology, endocrine surgery, surgical oncology, genetics, and clinical research as well as a patient representative, who conducted a targeted review of the literature to provide practical patient-centered clinical guidance for practitioners who care for individuals with MEN1. […] The role of surgical and non-surgical management strategies for patients with different types of MEN1-related tumors. […] The importance of patient values and preferences and risks/benefits of different screening modalities when determining appropriate screening frequencies for patients. […] Current gaps in the literature and future considerations for MEN1 research.

• #89 Multiple Endocrine Neoplasia Type 1 (09.08.2024)

  https://di.aerzteblatt.de/int/archive/article/240614

  The effectiveness of somatostatin analogues (SSAs) with regard to the disease course in patients with MEN1-associated pNENs has been evaluated in a limited number of studies. […] With regular screening examinations and early treatment, an improvement in the mean life expectancy of patients with MEN1 compared to historic cohorts before 1990 is noted. […] Despite the increase in life expectancy, the health-related quality of life of patients with MEN1 is found reduced compared to the normal population. […] The identification of the gene that causes MEN1 has deepened our understanding of the disease and led to advances in the diagnosis and treatment of the condition.

• #90 Care for patients with multiple endocrine neoplasia type 1: the current evidence base – PubMed

  https://pubmed.ncbi.nlm.nih.gov/21061174/

  Multiple endocrine neoplasia type 1 (MEN1) is a rare disease caused by mutations in the MEN1 gene on chromosome 11. It is characterized by the occurrence of primary hyperparathyroidism (pHPT), duodenopancreatic neuroendocrine tumours (pNET), pituitary tumours (PIT), adrenal adenomas (ADR) and neuroendocrine tumours (NET) of the stomach, bronchus and thymus. MEN1 is a syndrome with high penetrance and high morbidity. Malignant NETs are the most important cause of MEN1-related death. Since 1997 the diagnosis can be made by genetic screening. MEN1 is a complex syndrome and the endocrine manifestations cannot be viewed upon as coinciding sporadic tumours. Differences in epidemiology and pathology between MEN1-related tumours and their sporadic counterparts show that a unique approach is needed. Therefore the care for MEN1 patients should be provided by a centre of expertise. Early genetic diagnosis and periodic screening are important pillars of care. For primary hyperparathyroidism surgery is the most important treatment modality, with a subtotal parathyroid gland resection as the procedure of choice. In neuroendocrine tumours surgery also is the most important treatment modality. Selective tumour enucleation has no place in the surgical treatment of MEN1-related pNETs; the exact procedure depends on the functionality of the tumour. In MEN1-associated pituitary and adrenal adenomas, watchful waiting and medical therapy play more important roles. In the twenty-first century new developments will impact the care for MEN1 patients. These developments should be critically evaluated in clinical research with the ultimate goal of optimizing the care for MEN1 patients on an evidence base.

• #91 Multiple Endocrine Neoplasia Type 1 | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/25356

  MEN1 is a complex syndrome that increases morbidity and mortality by causing multiple aggressive tumors. An interprofessional team should manage these patients in a tertiary care center, including an endocrinologist, endocrine surgeon, gastroenterologist, neurosurgeon, genetic counselor, nursing staff, pharmacist, and patient navigator or advocate for achieving better outcomes.

• #92 Multiple endocrine neoplasia type 1 | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-1-38

  Multiple Endocrine Neoplasia type 1 (MEN1) is a rare autosomal dominant hereditary cancer syndrome presented mostly by tumours of the parathyroids, endocrine pancreas and anterior pituitary, and characterised by a very high penetrance and an equal sex distribution. […] Treatment consists of surgery and/or drug therapy, often in association with radiotherapy or chemotherapy. Currently, DNA testing allows the early identification of germline mutations in asymptomatic gene carriers, to whom routine surveillance (regular biochemical and/or radiological screenings to detect the development of MEN1-associated tumours and lesions) is recommended. […] The combination of clinical and genetic investigations, together with the improving of molecular genetics knowledge of the syndrome, helps in the clinical management of patients.

• #93 Genetic Disorder Reference Sheet: Multiple Endocrine Neoplasia Type 1 | Oncology Nursing Society

  https://onf.ons.org/publications-research/voice/news-views/07-2022/genetic-disorder-reference-sheet-multiple-endocrine

  Multiple endocrine neoplasia type 1 (MEN1) is a rare autosomal dominant genetic disease affecting about 1 in 30,000 people. Pathogenic variants in the MEN1 gene are associated with benign and malignant tumors in the parathyroid glands, pituitary gland, pancreas, duodenum, and stomach. […] Individuals with an MEN1 pathogenic variant are at increased risk for developing benign and malignant tumors (see sidebar). […] Ideally, people with a pathogenic MEN1 variant are managed by an interprofessional team that is familiar with its multiple complications. […] A primary nursing responsibility for MEN1 is providing ongoing psychosocial support and education on symptom identification and the importance of ongoing screening to detect tumors early, intervene accordingly, and improve prognosis. […] Smoking increases the risk of carcinoid tumors in MEN1. Nurses should encourage smoking cessation and help patients enroll in programs to quit.

• #94 Multiple endocrine neoplasia 1: providing care for the family – PubMed

  https://pubmed.ncbi.nlm.nih.gov/20880824/

  Multiple endocrine neoplasia 1 (MEN1) is a hereditary syndrome associated with a number of endocrine and nonendocrine tumors. […] Nurses need to be familiar with hallmark signs of this syndrome to facilitate an early diagnosis and appropriately refer families for genetic assessment and, ultimately, develop a long-term plan for early detection and intervention for all family members at risk for MEN1. […] Multiple Endocrine Neoplasia Type 1 / nursing.

• #95 Genetic Disorder Reference Sheet: Multiple Endocrine Neoplasia Type 1 | Oncology Nursing Society

  https://onf.ons.org/publications-research/voice/news-views/07-2022/genetic-disorder-reference-sheet-multiple-endocrine

  Multiple endocrine neoplasia type 1 (MEN1) is a rare autosomal dominant genetic disease affecting about 1 in 30,000 people. Pathogenic variants in the MEN1 gene are associated with benign and malignant tumors in the parathyroid glands, pituitary gland, pancreas, duodenum, and stomach. […] Individuals with an MEN1 pathogenic variant are at increased risk for developing benign and malignant tumors (see sidebar). […] Ideally, people with a pathogenic MEN1 variant are managed by an interprofessional team that is familiar with its multiple complications. […] A primary nursing responsibility for MEN1 is providing ongoing psychosocial support and education on symptom identification and the importance of ongoing screening to detect tumors early, intervene accordingly, and improve prognosis. […] Smoking increases the risk of carcinoid tumors in MEN1. Nurses should encourage smoking cessation and help patients enroll in programs to quit.

• #96 Multiple Endocrine Neoplasia Type 1 (09.08.2024)

  https://di.aerzteblatt.de/int/archive/article/240614

  Multiple endocrine neoplasia type 1 (MEN1) is a rare genetic disease of autosomal dominant inheritance, with an estimated prevalence of 320/100 000. Its main feature is neuroendocrine neoplasia in the parathyroid glands, the endocrine pancreas, the duodenum, and the pituitary gland. […] The early diagnosis and individualized treatment of MEN1-associated tumors can prevent the development of life-threatening hormonal syndromes and prolong the expected life span of MEN1 patients from 55 to 70 years, as well as improving their quality of life. […] Carriers of disease-causing mutations in the MEN1 gene should be cared for in specialized interdisciplinary centers, so that any appreciable tumor growth or hormonal activity can be detected early and organ-sparing treatment can be provided. […] Given the complexity of the disease, specific diagnosis and treatment should be the responsibility of a multidisciplinary team (covering endocrinology, gastroenterology, surgery, radiology, nuclear medicine, and pathology).

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