Materiały źródłowe

• #1 Multiple endocrine neoplasia, type 1 (MEN 1) – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/men-1/diagnosis-treatment/drc-20446823

  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. These conditions and treatments may include: […] Pituitary tumors. These types of tumors may be treated with surgery or medicines. Rarely, radiation therapy is used. […] Hyperparathyroidism. Surgery to remove most of the parathyroid glands is the typical treatment for too much parathyroid hormone. […] Neuroendocrine tumors. These are tumors that form in specialized cells called neuroendocrine cells. With MEN 1, they’re in the pancreas or small intestine. Treatment depends on the type and size of the tumor. […] Hypoglycemic syndrome. This condition happens when tumors in the pancreas called insulinomas make too much insulin hormone. Too much insulin causes low levels of blood sugar that can be life-threatening. Treatment often involves surgery. Part of the pancreas may need to be removed too.

• #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

  MEN 1 can’t be cured. But regular testing can detect health concerns, and healthcare professionals can provide treatment as needed. […] MEN 1 is an inherited condition. That means people who have a genetic change that causes MEN 1 can pass it on to their children.

• #3 Multiple endocrine neoplasia type 1: Management – UpToDate

  https://www.uptodate.com/contents/multiple-endocrine-neoplasia-type-1-management

  Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder classically characterized by predisposition to tumors of the parathyroid glands (which occur in the large majority of patients by age 50 years), anterior pituitary, and pancreatic islet cells. Treatment of MEN1 can differ markedly from that of more common sporadic forms of the relevant endocrine tumors, and referral to centers with major experience and multidisciplinary expertise in MEN1 is strongly recommended. […] Once the biochemical diagnosis of primary hyperparathyroidism is confirmed in a patient with known or presumed MEN1, the indications for surgical intervention are similar to those in patients with sporadic primary hyperparathyroidism. These include symptomatic or marked hypercalcemia, nephrolithiasis, and evidence of bone disease, such as diminished bone density or fracture.

• #4 Multiple Endocrine Neoplasia Type 1 (MEN1) Treatment & Management: Approach Considerations, Consultations

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

  Surgical removal of the tumor is the treatment of choice for glucagonomas. […] Somatostatin analogs control symptoms in 80% of cases of VIPoma. Nevertheless, surgical cure should be attempted. […] If resectable, surgery is the treatment of choice for carcinoid tumors. […] Management is conservative for lipomas, facial angiofibromas, and collagenomas. Local excision can be performed if desired. […] Surgery is indicated for functioning tumors (eg. primary hyperaldosteronism or hypercortisolism), and nonfunctioning tumors with atypical features, size greater than 4 cm, or significant growth over a 6-month interval. […] Centers with expertise in MEN1 diagnosis and treatment are recommended for patients. Multiple consultations are generally necessary, including evaluation by specialists in endocrinology, gastroenterology, neurosurgery, general surgery, and dermatology.

• #5 Multiple Endocrine Neoplasia Type 1 – NIDDK

  https://www.niddk.nih.gov/health-information/endocrine-diseases/multiple-endocrine-neoplasia-type-1

  How do doctors treat MEN1? […] Although MEN1 cant be cured, most people with the disorder lead long and productive lives. Your doctor will monitor your health and provide treatment as needed. […] Your doctor will monitor your symptoms and screen for the signs of MEN1-related tumors on a regular basis. Commonly used screening tests include blood tests and imaging tests. […] Based on your symptoms and test results, your doctor may prescribe different medicines to manage the progress of the disorder. […] Treatment varies depending on the location and type of tumor. For example, parathyroid tumors are most often treated with surgery to remove the affected glands. […] Surgery is often successful in removing MEN1-related tumors and curing related symptoms. […] If a surgery removes an entire endocrine gland or more than three parathyroid glands, your doctor may prescribe medicines to replace the hormones that your body is no longer making.

• #6 Update on the clinical management of multiple endocrine neoplasia type 1

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9540817/

  Initiating biochemical and radiological screening in the second decade of life seems appropriate, based on currently available evidence. […] For care tailored to the needs of the individual patient and improving outcomes on an individual basis, studies are now needed to define predictors of tumour behaviour and effects of more individualized interventions.

• #7 Multiple Endocrine Neoplasia Type 1 (MEN1) Treatment & Management: Approach Considerations, Consultations

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

  Surgery is the definitive treatment for the control of hypercalcemia. Subtotal (3.5 glands) or total parathyroidectomy with forearm autotransplantation is performed with an open bilateral neck exploration. The recommended timing and type of surgery is controversial. Recurrent hypercalcemia is common. Reimplantation after total parathyroidectomy has a high incidence of graft failure and subsequent permanent hypoparathyrodism. […] Surgery is fundamental in patients with hypercalcemia due to primary hyperparathyroidism with Zollinger-Ellison syndrome (ZES), since restoring a normal calcium level contributes to reducing gastric acid output and the consequent risk of peptic ulcers. […] Multiple percutaneous parathyroid ethanol ablation (PEA) treatment has been shown to safely and effectively control hyperparathyroidsim with a low rate of hypocalcemia and permanent complications, when performed by an experienced radiologist.

• #8 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. […] At present, total parathyroidectomy is the proposed effective treatment for PHPT in symptomatic hypercalcaemic MEN1 patients. […] The treatment for a non-metastatic gastrinoma is surgical resection. The treatment for multiple and disseminate gastrinomas consists of therapy with a human somatostatin analogue (octreotide), administration of proton pump inhibitors or H2-receptor blockers to reduce gastric acid output, chemotherapy with 5-fluoroaracil and streptozotocin, and surgical excision of all resectable tumours (however, the success rate seems very low).

• #9 Update on the clinical management of multiple endocrine neoplasia type 1

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9540817/

  This review provides an overview of novel insights in the clinical management of patients with Multiple Endocrine Neoplasia Type 1, focusing on the last decade since the last update of the MEN1 guidelines. […] As for primary hyperparathyroidism: subtotal parathyroidectomy is the initial procedure of choice. Current debate centres around the timing of initial parathyroidectomy as well as the controversial topic of unilateral clearance in young patients. […] The treatment of pHPT is surgical, and the aim of initial parathyroidectomy is achieving eucalcemia for as long as possible, while preventing hypoparathyroidism and facilitating potential reoperative surgery. Since the publication of the guidelines and publication of additional studies, most experts agree that the preferred initial operation in MEN1 is a bilateral cervical exploration, identifying all four parathyroid glands and performing a subtotal parathyroidectomy with concomitant cervical thymectomy.

• #10 4 Innovative Multiple Endocrine Neoplasia Treatment Options | MD Anderson Cancer Center

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

  Because we see more patients with multiple endocrine neoplasia than most programs, we have a higher level of experience and skill in treating these rare and complex diseases. This can give you a better chance for successful treatment. […] Surgery is often the main therapy for MEN. Like all surgeries, surgery to treat multiple endocrine neoplasia is most successful when performed by a specialist with a great deal of experience in the particular procedure. […] Many times, the affected gland can be surgically removed to treat symptoms caused by MEN. Hyperparathyroidism caused by MEN1 is typically treated with surgical removal of three-and-a-half of the four parathyroid glands, although sometimes all four glands are removed, with a portion of the parathyroid gland inserted into the forearm.

• #11

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

  Multiple endocrine neoplasia type 1 (MEN1) syndrome is an autosomal dominant disorder caused by a germline pathogenic variant in the MEN1 tumor suppressor gene. Management of PHPT differs from that of patients with sporadic disease, as the surgical approach in MEN1-related PHPT includes near-total or total parathyroidectomy because of multigland hyperplasia in most patients and the consequent high risk of recurrence. NEN management also differs from patients with sporadic disease due to multiple synchronous and metasynchronous neoplasms. […] 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).

• #12 Multiple Endocrine Neoplasia type 1 (MEN1) – Familial Isolated Pituitary Adenoma (FIPA)

  https://www.qmul.ac.uk/fipa-patients/pituitary-disorders/multiple-endocrine-neoplasia-type-1/

  In MEN1 patients, all four parathyroid glands tend to be overactive, causing hyperparathyroidism. […] The usual treatment is an operation to remove most or all of the parathyroid glands. One option is to remove the three largest glands and all but a small part of the fourth. Another is to remove all four glands and at the same time transplant a small part of one gland into the forearm. […] After parathyroid surgery, regular monitoring of blood calcium is needed because often the small piece of remaining parathyroid tissue grows larger and causes recurrent hyperparathyroidism. […] People whose parathyroid glands have been completely removed must take daily supplements of calcium and vitamin D or another related treatment to prevent hypocalcaemia, or low blood calcium. […] The mainstay of treatment is powerful medicines called acid pump inhibitors that block stomach acid release.

• #13 Update on the clinical management of multiple endocrine neoplasia type 1

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9540817/

  In recent years it has been debated if for young people with MEN1-related pHPT, a stepwise approach to parathyroid surgery should be offered in the form of unilateral clearance (resection of all parathyroids and cervical thymus on one side) as initial operation. […] Presently, surgery is the only curative treatment for MEN1-related dpNETs. However duodenopancreatic surgery is associated with high short and long-term morbidity. […] Current consensus is that surgical resection in NFPanNETs is indicated for those 2cm or progressing during surveillance. […] For MEN1-related gastrinomas, the if, when and how of a surgical intervention are still controversial and there is an excellent review on the contemporary surgical management by the Marburg team. […] Localized insulinomas are an indication for surgical resection. Regarding the extent of resection (enucleation vs. resection) recent data show excellent outcomes of enucleations in patients with solitary insulinomas.

• #14 Multiple Endocrine Neoplasia Type 1 (09.08.2024)

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

  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. […] Surgical treatment is based on the location, size, growth dynamics, and functional activity of the tumors. […] 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.

• #15 Multiple endocrine neoplasia type 1: Management – UpToDate

  https://www.uptodate.com/contents/multiple-endocrine-neoplasia-type-1-management

  Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder classically characterized by predisposition to tumors of the parathyroid glands (which occur in the large majority of patients by age 50 years), anterior pituitary, and pancreatic islet cells. Treatment of MEN1 can differ markedly from that of more common sporadic forms of the relevant endocrine tumors, and referral to centers with major experience and multidisciplinary expertise in MEN1 is strongly recommended. […] Once the biochemical diagnosis of primary hyperparathyroidism is confirmed in a patient with known or presumed MEN1, the indications for surgical intervention are similar to those in patients with sporadic primary hyperparathyroidism. These include symptomatic or marked hypercalcemia, nephrolithiasis, and evidence of bone disease, such as diminished bone density or fracture.

• #16 Multiple endocrine neoplasia type 1: Management – UpToDate

  https://www.uptodate.com/contents/multiple-endocrine-neoplasia-type-1-management

  An additional indication for parathyroidectomy in patients with MEN1 is the presence of severe peptic ulcer disease or other symptoms caused by a gastrinoma (the Zollinger-Ellison syndrome) that are difficult to control with medications. Hypercalcemia typically worsens hypergastrinemia, and parathyroidectomy may markedly reduce gastrin secretion in patients with a gastrinoma.

• #17 Multiple Endocrine Neoplasia Type 1 (MEN1) Treatment & Management: Approach Considerations, Consultations

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

  Surgery is the definitive treatment for the control of hypercalcemia. Subtotal (3.5 glands) or total parathyroidectomy with forearm autotransplantation is performed with an open bilateral neck exploration. The recommended timing and type of surgery is controversial. Recurrent hypercalcemia is common. Reimplantation after total parathyroidectomy has a high incidence of graft failure and subsequent permanent hypoparathyrodism. […] Surgery is fundamental in patients with hypercalcemia due to primary hyperparathyroidism with Zollinger-Ellison syndrome (ZES), since restoring a normal calcium level contributes to reducing gastric acid output and the consequent risk of peptic ulcers. […] Multiple percutaneous parathyroid ethanol ablation (PEA) treatment has been shown to safely and effectively control hyperparathyroidsim with a low rate of hypocalcemia and permanent complications, when performed by an experienced radiologist.

• #18 Multiple Endocrine Neoplasia Type 1 (MEN1) Treatment & Management: Approach Considerations, Consultations

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

  PEA is not a definitive therapy and cannot replace primary surgical therapy. However, it can be considered as an alternative approach who requires re-operation. […] Calcimimetics (eg, cinacalcet), a class of calcium-sensing receptor agonists, can be used to reduce parathyroid hormone release by parathyroid cells and to control cell growth. […] Inhibition of acid hypersecretion is achieved with proton pump inhibitors (eg, omeprazole, lansoprazole, pantoprazole). […] Nonmetastatic gastrinomas located in the pancreas are rare but can be surgically excised. […] Surgical cure of multiple duodenal gastrinomas is difficult and is not associated with a high disease-free state. […] Surgical removal of the tumor is the treatment of choice for insulinomas. […] Unresectable tumors can be treated with diazoxide or octreotide.

• #19 Orphanet: Multiple endocrine neoplasia type 1

  https://www.orpha.net/en/disease/detail/652

  Management and treatment: Annual life-long screening is recommended for all the carriers of a MEN1 mutations and individuals from affected families without an identified MEN1 mutations or who have not undergone the genetic test. […] Six-monthly screening is recommended for affected patients (CT, MRI, blood biochemistry). Treatment is prevalently by surgery based on approaches for each specific tumor. Some pharmacological therapies are available for treatment of hormone over-secretion and related symptoms; calcimimetic can be used to treat PHPT in patients in whom surgery had either failed or was contraindicated. […] Prior to surgery, bone anti-resorptive agents are used to reduce hypercalcemia and limit bone resorption. Dopamine agonists are used for prolactinoma. Somatostatin analogs for GH-secreting tumors and for controlling the secretory hyperfunction associated with carcinoid syndrome. Proton pump inhibitors or H2-receptor blockers reduce gastric acid output caused by gastrinomas.

• #20 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

  Treatment of MEN 1 is primarily surgical, with subtotal parathyroidectomy; however, hyperparathyroidism frequently recurs. Octreotide and cinacalcet may help control recurrent or persistent postoperative hypercalcemia. […] Prolactinoma is usually managed with dopamine agonists; other pituitary tumors are treated surgically. […] Islet cell tumors are more difficult to manage because the lesions are often small and difficult to find, multiple lesions are common, and surgery often is not curative. […] The treatment of gastrin-secreting gastroenteropancreatic neuroendocrine tumors (GEP-NETs) is complex. When possible, the tumor is located and removed. […] In patients with insulinomas, if a single tumor cannot be found, distal subtotal pancreatectomy with enucleation of any palpable tumors in the head of the pancreas is recommended. Diazoxide or a somatostatin analogue (octreotide, lanreotide) may help treat hypoglycemia.

• #21 A New Medical Therapy for Multiple Endocrine Neoplasia Type 1? – touchENDOCRINOLOGY

  https://touchendocrinology.com/endocrine-oncology/journal-articles/a-new-medical-therapy-for-multiple-endocrine-neoplasia-type-1/

  Pancreatic neuroendocrine tumours (pNETs) are a major manifestation of multiple endocrine neoplasia type 1 (MEN1), and the most significant cause of morbidity and mortality in this disorder. […] There is some evidence that the early use of somatostatin analogues can retard progression, especially of small non-functioning tumours, but there are no other prophylactic therapies for patients, and the treatment of metastatic disease is similar to that for sporadic pNETs. […] In clinical terms, the parathyroid glands are most often affected in relation to clinical disease, while duodenal gastrin-secreting tumours are common and most often treated medically in the first instance. […] However, it is pancreatic NETs (pNETs), which are often multifocal, that are the major cause of morbidity and indeed mortality, as a substantial minority undergo malignant transformation with metastatic spread.

• #22 A New Medical Therapy for Multiple Endocrine Neoplasia Type 1? – touchENDOCRINOLOGY

  https://touchendocrinology.com/endocrine-oncology/journal-articles/a-new-medical-therapy-for-multiple-endocrine-neoplasia-type-1/

  In such cases, treatment is generally based on the standard protocols for other pNETs, which would include surgery, somatostatin receptor ligands, peptide receptor radionuclide therapy and chemotherapy, as well as molecular targeted agents such as sunitinib and everolimus. […] Currently, pNETs are the leading cause of MEN1-related death. […] In terms of medical therapy that could be initiated at an early stage to avoid metastatic spread, somatostatin analogues represent the first therapeutic option in patients affected with low-grade (G1-G2) NETs. […] According to the current guidelines, somatostatin analogues should be considered in this setting for functioning endocrine syndromes not controlled by symptomatic therapies, and for metastatic unresectable pNETs or those with residual disease following partial resection.

• #23 Update on the clinical management of multiple endocrine neoplasia type 1

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9540817/

  In recent years it has been debated if for young people with MEN1-related pHPT, a stepwise approach to parathyroid surgery should be offered in the form of unilateral clearance (resection of all parathyroids and cervical thymus on one side) as initial operation. […] Presently, surgery is the only curative treatment for MEN1-related dpNETs. However duodenopancreatic surgery is associated with high short and long-term morbidity. […] Current consensus is that surgical resection in NFPanNETs is indicated for those 2cm or progressing during surveillance. […] For MEN1-related gastrinomas, the if, when and how of a surgical intervention are still controversial and there is an excellent review on the contemporary surgical management by the Marburg team. […] Localized insulinomas are an indication for surgical resection. Regarding the extent of resection (enucleation vs. resection) recent data show excellent outcomes of enucleations in patients with solitary insulinomas.

• #24 Multiple Endocrine Neoplasia Type 1 (09.08.2024)

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

  The extent to which the parathyroid gland tissue should be removed is subject to ongoing controversy. […] In the case of resectable neuroendocrine neoplasms (NEN) of the thymus, complete thymic resection is indicated, typically with lymphadenectomy. […] 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. […] Adequate treatment of duodenopancreatic neuroendocrine neoplasms (dpNENs) is also based on tumor size, observed growth dynamics and functional activity. […] Nonfunctioning (NF) pancreatic neuroendocrine neoplasms (pNENs) are with 70-80% the most common manifestation of dpNENs. […] For nonmalignant small NF-pNENs, a pancreatic parenchyma-sparing resection with lymph node sampling is recommended.

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

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

  Hypoglycemic syndrome. This condition happens when tumors in the pancreas called insulinomas make too much insulin hormone. Too much insulin causes low levels of blood sugar that can be life-threatening. Treatment often involves surgery. Part of the pancreas may need to be removed too. […] Zollinger-Ellison syndrome (ZES). ZES can result in tumors called gastrinomas that make too much stomach acid. This leads to ulcers and diarrhea. Healthcare professionals may prescribe medicine or try to remove the tumors with surgery. […] Other pancreatic neuroendocrine tumors. These tumors sometimes make other hormones that can cause health issues. Treatment of these types of tumors may involve medicine or surgery. Another treatment called ablation can be done to destroy irregular tissue that may be present.

• #26 Multiple endocrine neoplasia, type 1 (MEN 1) – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/men-1/diagnosis-treatment/drc-20446823

  Zollinger-Ellison syndrome (ZES). ZES can result in tumors called gastrinomas that make too much stomach acid. This leads to ulcers and diarrhea. Healthcare professionals may prescribe medicine or try to remove the tumors with surgery. […] Other pancreatic neuroendocrine tumors. These tumors sometimes make other hormones that can cause health issues. Treatment of these types of tumors may involve medicine or surgery. Another treatment called ablation can be done to destroy irregular tissue that may be present. […] Metastatic neuroendocrine tumors. Tumors that spread are called metastatic tumors. Sometimes with MEN 1, tumors spread to the lymph nodes or liver. They may be treated with surgery. Surgery options include liver surgery or different types of ablation. […] Adrenal tumors. Most of these tumors can be watched with tests over time and not treated. But if the tumors make hormones or they’re large and thought to be cancerous, healthcare professionals recommend removing them. Often, the tumors can be removed with surgery that involves small incisions. This is known as minimally invasive surgery.

• #27 Multiple endocrine neoplasia 1 (MEN1) | Macmillan Cancer Support

  https://www.macmillan.org.uk/cancer-information-and-support/worried-about-cancer/pre-cancerous-and-genetic-conditions/multiple-endocrine-neoplasia-1-men1

  A team of specialists meet to talk about the best treatment for you. They are called a multidisciplinary team (MDT). […] Your treatment will depend on the type of tumour and where it is. You may need surgery to remove tumours, or drugs to control symptoms. […] The main treatment for parathyroid tumours is surgery. Some people may have monitoring with blood tests to check calcium levels, or medicines to control the blood calcium levels. […] If you have a gastrinoma, your doctors will usually give you tablets that reduce the amount of acid made by the stomach. This helps prevent more serious problems, such as stomach ulcers. Some people may have other treatments such as surgery, hormone treatment or chemotherapy. […] You may be offered treatment with drugs to reduce insulin levels. Or you may have surgery to remove the affected area of the pancreas.

• #28 Multiple Endocrine Neoplasia Type 1 (MEN1) Treatment & Management: Approach Considerations, Consultations

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

  PEA is not a definitive therapy and cannot replace primary surgical therapy. However, it can be considered as an alternative approach who requires re-operation. […] Calcimimetics (eg, cinacalcet), a class of calcium-sensing receptor agonists, can be used to reduce parathyroid hormone release by parathyroid cells and to control cell growth. […] Inhibition of acid hypersecretion is achieved with proton pump inhibitors (eg, omeprazole, lansoprazole, pantoprazole). […] Nonmetastatic gastrinomas located in the pancreas are rare but can be surgically excised. […] Surgical cure of multiple duodenal gastrinomas is difficult and is not associated with a high disease-free state. […] Surgical removal of the tumor is the treatment of choice for insulinomas. […] Unresectable tumors can be treated with diazoxide or octreotide.

• #29 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. […] At present, total parathyroidectomy is the proposed effective treatment for PHPT in symptomatic hypercalcaemic MEN1 patients. […] The treatment for a non-metastatic gastrinoma is surgical resection. The treatment for multiple and disseminate gastrinomas consists of therapy with a human somatostatin analogue (octreotide), administration of proton pump inhibitors or H2-receptor blockers to reduce gastric acid output, chemotherapy with 5-fluoroaracil and streptozotocin, and surgical excision of all resectable tumours (however, the success rate seems very low).

• #30 Multiple Endocrine Neoplasia type 1 (MEN1) – Familial Isolated Pituitary Adenoma (FIPA)

  https://www.qmul.ac.uk/fipa-patients/pituitary-disorders/multiple-endocrine-neoplasia-type-1/

  In MEN1 patients, all four parathyroid glands tend to be overactive, causing hyperparathyroidism. […] The usual treatment is an operation to remove most or all of the parathyroid glands. One option is to remove the three largest glands and all but a small part of the fourth. Another is to remove all four glands and at the same time transplant a small part of one gland into the forearm. […] After parathyroid surgery, regular monitoring of blood calcium is needed because often the small piece of remaining parathyroid tissue grows larger and causes recurrent hyperparathyroidism. […] People whose parathyroid glands have been completely removed must take daily supplements of calcium and vitamin D or another related treatment to prevent hypocalcaemia, or low blood calcium. […] The mainstay of treatment is powerful medicines called acid pump inhibitors that block stomach acid release.

• #31 Multiple Endocrine Neoplasia Type 1 (09.08.2024)

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

  As an alternative to surgery for small NF-pNENs, radiofrequency ablation has recently been declared a successful treatment, especially for elderly patients. […] Functioning dpNENs include gastrinomas, insulinomas, vasoactive intestinal peptide (VIP)-secreting tumors (VIPomas), and glucagonomas. […] 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. […] If a gastrinoma is confirmed by clinical and biochemical findings, some experts recommend drug therapy with proton pump inhibitors (PPIs) alone. […] When deciding on a treatment, the preference of the fully informed patient should always be taken into account. […] 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. […] 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. Screening programs in specialized centers can help to detect tumor manifestations in time and provide organ-sparing treatment.

• #32 Multiple endocrine neoplasia, type 1 (MEN 1) – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/men-1/diagnosis-treatment/drc-20446823

  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. These conditions and treatments may include: […] Pituitary tumors. These types of tumors may be treated with surgery or medicines. Rarely, radiation therapy is used. […] Hyperparathyroidism. Surgery to remove most of the parathyroid glands is the typical treatment for too much parathyroid hormone. […] Neuroendocrine tumors. These are tumors that form in specialized cells called neuroendocrine cells. With MEN 1, they’re in the pancreas or small intestine. Treatment depends on the type and size of the tumor. […] Hypoglycemic syndrome. This condition happens when tumors in the pancreas called insulinomas make too much insulin hormone. Too much insulin causes low levels of blood sugar that can be life-threatening. Treatment often involves surgery. Part of the pancreas may need to be removed too.

• #33 Multiple endocrine neoplasia 1 (MEN1) | Macmillan Cancer Support

  https://www.macmillan.org.uk/cancer-information-and-support/worried-about-cancer/pre-cancerous-and-genetic-conditions/multiple-endocrine-neoplasia-1-men1

  A team of specialists meet to talk about the best treatment for you. They are called a multidisciplinary team (MDT). […] Your treatment will depend on the type of tumour and where it is. You may need surgery to remove tumours, or drugs to control symptoms. […] The main treatment for parathyroid tumours is surgery. Some people may have monitoring with blood tests to check calcium levels, or medicines to control the blood calcium levels. […] If you have a gastrinoma, your doctors will usually give you tablets that reduce the amount of acid made by the stomach. This helps prevent more serious problems, such as stomach ulcers. Some people may have other treatments such as surgery, hormone treatment or chemotherapy. […] You may be offered treatment with drugs to reduce insulin levels. Or you may have surgery to remove the affected area of the pancreas.

• #34 Multiple Endocrine Neoplasia Type 1 (MEN1) Treatment & Management: Approach Considerations, Consultations

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

  PEA is not a definitive therapy and cannot replace primary surgical therapy. However, it can be considered as an alternative approach who requires re-operation. […] Calcimimetics (eg, cinacalcet), a class of calcium-sensing receptor agonists, can be used to reduce parathyroid hormone release by parathyroid cells and to control cell growth. […] Inhibition of acid hypersecretion is achieved with proton pump inhibitors (eg, omeprazole, lansoprazole, pantoprazole). […] Nonmetastatic gastrinomas located in the pancreas are rare but can be surgically excised. […] Surgical cure of multiple duodenal gastrinomas is difficult and is not associated with a high disease-free state. […] Surgical removal of the tumor is the treatment of choice for insulinomas. […] Unresectable tumors can be treated with diazoxide or octreotide.

• #35 Multiple endocrine neoplasia type 1 | Endocrine Conditions

  https://www.yourhormones.info/endocrine-conditions/multiple-endocrine-neoplasia-type-1/

  How is multiple endocrine neoplasia type 1 treated? […] Exact treatment depends on the type of tumours present. If no abnormalities are detected then there is no need for any specific treatment. The three different kinds of tumours are treated in different ways: 1. Primary hyperparathyroidism is usually managed surgically although the timing of intervention remains controversial. Occasionally a medical treatment may be offered for the short or longer term. Usually an operation will involve removal of 3 or all 4 parathyroid glands and patients will then require life-long supplements of activated vitamin D and possibly calcium to maintain a normal healthy blood calcium level. 2. Pituitary tumours prolactinomas are usually managed with drugs called dopamine agonists such as cabergoline or bromocriptine. Surgery may occasionally be needed for large tumours that are unresponsive to medical treatment. Patients with acromegaly or Cushings disease nearly always require pituitary surgery to remove the tumour. Drugs such as somatostatin analogues may be used prior to surgery in acromegaly, or after if a patient is not cured, and radiotherapy may be necessary also if there is residual tumour after surgery (see the articles on acromegaly, Cushings disease and prolactinoma, for more information). Non-functioning pituitary tumours may undergo a period of observation depending upon their size and whether a patient’s vision is affected. Surgery and or radiotherapy are treatment options. 3. Pancreatic NETs gastrinomas are managed initially with medication (proton pump inhibitors), which reduces gastric acid secretion and therefore reduces ulceration. However, some patients have very severe disease and may still require surgery for symptom control. Pancreatic surgery to remove the tumour is the mainstay of treatment for insulinoma. Occasionally, a drug, diazoxide can be used while a patient is awaiting a definitive surgical procedure; long-term drug treatment is not usually used in the management of an insulinoma. In non-functioning pancreatic NETs, currently, the recommendation is to pursue surgery if tumours are larger than 2 cm in size, to consider surgery if tumours are 1-2 cm, and probably to opt for surveillance if tumours are 1 cm. Pancreatic surgery may include enucleation of the lesion only, removing a part of the pancreas, or part of the pancreas, stomach and duodenum (Whipples procedure), or in very extreme circumstances the whole pancreas, although this is never undertaken lightly due to the long-term complications for the patient. Adrenal nodules can often simply be monitored by imaging unless they behave suspiciously; as there is a small increased risk of adrenal cancer in MEN1. Non-pancreatic NETs in MEN1 are often treated surgically although other treatment modalities may be considered. Skin lesions are often treated as per patient preference.

• #36 Update on the clinical management of multiple endocrine neoplasia type 1

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9540817/

  In recent years it has been debated if for young people with MEN1-related pHPT, a stepwise approach to parathyroid surgery should be offered in the form of unilateral clearance (resection of all parathyroids and cervical thymus on one side) as initial operation. […] Presently, surgery is the only curative treatment for MEN1-related dpNETs. However duodenopancreatic surgery is associated with high short and long-term morbidity. […] Current consensus is that surgical resection in NFPanNETs is indicated for those 2cm or progressing during surveillance. […] For MEN1-related gastrinomas, the if, when and how of a surgical intervention are still controversial and there is an excellent review on the contemporary surgical management by the Marburg team. […] Localized insulinomas are an indication for surgical resection. Regarding the extent of resection (enucleation vs. resection) recent data show excellent outcomes of enucleations in patients with solitary insulinomas.

• #37 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

  Treatment of MEN 1 is primarily surgical, with subtotal parathyroidectomy; however, hyperparathyroidism frequently recurs. Octreotide and cinacalcet may help control recurrent or persistent postoperative hypercalcemia. […] Prolactinoma is usually managed with dopamine agonists; other pituitary tumors are treated surgically. […] Islet cell tumors are more difficult to manage because the lesions are often small and difficult to find, multiple lesions are common, and surgery often is not curative. […] The treatment of gastrin-secreting gastroenteropancreatic neuroendocrine tumors (GEP-NETs) is complex. When possible, the tumor is located and removed. […] In patients with insulinomas, if a single tumor cannot be found, distal subtotal pancreatectomy with enucleation of any palpable tumors in the head of the pancreas is recommended. Diazoxide or a somatostatin analogue (octreotide, lanreotide) may help treat hypoglycemia.

• #38 Multiple Endocrine Neoplasia Type 1 (MEN1) Treatment & Management: Approach Considerations, Consultations

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

  Surgical removal of the tumor is the treatment of choice for glucagonomas. […] Somatostatin analogs control symptoms in 80% of cases of VIPoma. Nevertheless, surgical cure should be attempted. […] If resectable, surgery is the treatment of choice for carcinoid tumors. […] Management is conservative for lipomas, facial angiofibromas, and collagenomas. Local excision can be performed if desired. […] Surgery is indicated for functioning tumors (eg. primary hyperaldosteronism or hypercortisolism), and nonfunctioning tumors with atypical features, size greater than 4 cm, or significant growth over a 6-month interval. […] Centers with expertise in MEN1 diagnosis and treatment are recommended for patients. Multiple consultations are generally necessary, including evaluation by specialists in endocrinology, gastroenterology, neurosurgery, general surgery, and dermatology.

• #39 Frontiers | Multiple Endocrine Neoplasia Type 1 (MEN1): An Update and the Significance of Early Genetic and Clinical Diagnosis

  https://www.frontiersin.org/journals/endocrinology/articles/10.3389/fendo.2019.00339/full

  The recommended surgical approach for the treatment of MEN1-associated PHPT is subtotal parathyroidectomy with removal of 3.5 parathyroid glands. […] The use of surgical resection is also limited in advanced metastatic disease, with treatment involving several approaches that may include medical therapy (with everolimus, tyrosine kinase inhibitors such as sunitinib, or chemotherapy), peptide radioreceptor therapy with lutetium-177-labeled somatostatin analog, chemotherapy, or liver directed therapies (such as embolization, chemoembolization, or radioembolization). […] The treatment approach for both glucagonomas and VIPomas is similar to that for insulinomas as described above.

• #40 Multiple Endocrine Neoplasia in Childhood: An Update on Diagnosis, Screening, Management and Treatment

  https://www.mdpi.com/2673-396X/3/1/7

  In adults, surgical options include partial parathyroidectomy, subtotal parathyroidectomy (removal of 3.5 glands), or total parathyroidectomy with or without autotransplantation (transplantation of the remaining normal parathyroid tissue into the forearm). […] Post-surgical complications, such as permanent hypoparathyroidism and laryngeal nerve damage, can occur and are important to consider with respect to age and timing of surgery. […] Medical therapy is the preferred treatment for gastrinomas. H+-K+-adenosine triphosphatase inhibitors (i.e., omeprazole or lansoprazole) have been proven to be most effective for medical therapy. […] Surgical therapy is the treatment of choice for insulinomas, glucagonomas, and VIPomas. […] Insulinomas require removal of a single tumor enucleation or partial pancreatectomy, and these surgical procedures have been quite successful, leading to minimal morbidity in children. […] Surgical treatment for PHPT is similar to treatment in those with MEN1 as discussed earlier.

• #41 Multiple Endocrine Neoplasia Type 1 (09.08.2024)

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

  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. […] Surgical treatment is based on the location, size, growth dynamics, and functional activity of the tumors. […] 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.

• #42 Update on the clinical management of multiple endocrine neoplasia type 1

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9540817/

  The option of chemoprevention for PanNETs in MEN1 is interesting and a recent small observational cohort study compared lanreotide with a standard of care active surveillance. […] Prophylactic thymectomy during initial parathyroidectomy is advocated to reduce the risk of subsequent thNETs, although the risk is not abolished. […] Current guidelines recommend that patients with MEN1 are screened for thoracic NETs by thoracic imaging every 12 years. […] The treatment in MEN1-related PAs is not different from sporadic PAs and in contrast to previous assumptions, recent data show similar treatment responses to sporadic PAs. […] Therefore, currently Dutch females with MEN1 are recommended to undergo breast cancer screening from the age of 40, which is a decade earlier than the regular population screening program in the Netherlands.

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

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

  Treatment of pituitary tumours consists of drug therapy and/or surgery often in association with radiotherapy of the residual unresectable tumours. […] Medical treatment consists of dopamine agonists such as cabergoline, bromocriptine, pergolide and quinagolide. […] Individuals who have a 50% risk of having MEN1 syndrome, but whose genetic status is unknown, should undergo the following tests: biochemical evaluation of serum concentration of prolactin from age 5; biochemical screening of fasting total serum calcium concentration (corrected for albumin) from age 10; biochemical screening of fasting serum gastrin concentration, if the individual has symptoms of ZES (reflux, diarrhoea), from age 20; biochemical screening of fasting serum concentration of full-length PTH from age 10. […] In addition, it is possible to perform prophylactic thymectomy to prevent thymic carcinoids; it should be considered at the time of neck surgery for PHPT, particularly in men with MEN1 syndrome who are smokers and/or have relatives with thymic carcinoids. […] The final goal is to offer patients with MEN1 germline mutations an optimal cancer prevention and treatment program.

• #44 Multiple endocrine neoplasia type 1 | Endocrine Conditions

  https://www.yourhormones.info/endocrine-conditions/multiple-endocrine-neoplasia-type-1/

  How is multiple endocrine neoplasia type 1 treated? […] Exact treatment depends on the type of tumours present. If no abnormalities are detected then there is no need for any specific treatment. The three different kinds of tumours are treated in different ways: 1. Primary hyperparathyroidism is usually managed surgically although the timing of intervention remains controversial. Occasionally a medical treatment may be offered for the short or longer term. Usually an operation will involve removal of 3 or all 4 parathyroid glands and patients will then require life-long supplements of activated vitamin D and possibly calcium to maintain a normal healthy blood calcium level. 2. Pituitary tumours prolactinomas are usually managed with drugs called dopamine agonists such as cabergoline or bromocriptine. Surgery may occasionally be needed for large tumours that are unresponsive to medical treatment. Patients with acromegaly or Cushings disease nearly always require pituitary surgery to remove the tumour. Drugs such as somatostatin analogues may be used prior to surgery in acromegaly, or after if a patient is not cured, and radiotherapy may be necessary also if there is residual tumour after surgery (see the articles on acromegaly, Cushings disease and prolactinoma, for more information). Non-functioning pituitary tumours may undergo a period of observation depending upon their size and whether a patient’s vision is affected. Surgery and or radiotherapy are treatment options. 3. Pancreatic NETs gastrinomas are managed initially with medication (proton pump inhibitors), which reduces gastric acid secretion and therefore reduces ulceration. However, some patients have very severe disease and may still require surgery for symptom control. Pancreatic surgery to remove the tumour is the mainstay of treatment for insulinoma. Occasionally, a drug, diazoxide can be used while a patient is awaiting a definitive surgical procedure; long-term drug treatment is not usually used in the management of an insulinoma. In non-functioning pancreatic NETs, currently, the recommendation is to pursue surgery if tumours are larger than 2 cm in size, to consider surgery if tumours are 1-2 cm, and probably to opt for surveillance if tumours are 1 cm. Pancreatic surgery may include enucleation of the lesion only, removing a part of the pancreas, or part of the pancreas, stomach and duodenum (Whipples procedure), or in very extreme circumstances the whole pancreas, although this is never undertaken lightly due to the long-term complications for the patient. Adrenal nodules can often simply be monitored by imaging unless they behave suspiciously; as there is a small increased risk of adrenal cancer in MEN1. Non-pancreatic NETs in MEN1 are often treated surgically although other treatment modalities may be considered. Skin lesions are often treated as per patient preference.

• #45 Multiple Endocrine Neoplasia type 1 (MEN1) – Familial Isolated Pituitary Adenoma (FIPA)

  https://www.qmul.ac.uk/fipa-patients/pituitary-disorders/multiple-endocrine-neoplasia-type-1/

  Treatment with somatostatin analogues is also used. […] In general, surgery is the mainstay of treatment for these uncommon types of tumours. […] If treatment is needed, medicines known as a dopamine agonists (bromocriptine, cabergoline) can effectively shrink the tumour and lower the production of prolactin. Occasionally, prolactinomas do not respond well to this medication. In such cases, surgery, radiation, or both may be needed.

• #46 Multiple Endocrine Neoplasia Type 1 Symptoms, Doctors, Treatments, Advances & More | MediFind

  https://www.medifind.com/conditions/Multiple%20Endocrine%20Neoplasia%20Type%201/3557

  Multiple endocrine neoplasia (MEN) type I is a disease in which one or more of the endocrine glands are overactive or forms a tumor. It is passed down through families. […] What are the current treatments for Multiple Endocrine Neoplasia Type 1? […] Surgery to remove the diseased gland is often the treatment of choice. Medicines such as cabergoline and bromocriptine may be used instead of surgery for pituitary tumors that release the hormone prolactin. The parathyroid glands, which control calcium production, can be removed. However, it is difficult for the body to regulate calcium levels without these glands, so a total parathyroid removal is not done first in most cases. Medicine is available to decrease the excess stomach acid production caused by some tumors (gastrinomas), and to reduce the risk of ulcers. Hormone replacement therapy is given when entire glands are removed or do not produce enough hormones.

• #47 Multiple endocrine neoplasia type 1 | Endocrine Conditions

  https://www.yourhormones.info/endocrine-conditions/multiple-endocrine-neoplasia-type-1/

  How is multiple endocrine neoplasia type 1 treated? […] Exact treatment depends on the type of tumours present. If no abnormalities are detected then there is no need for any specific treatment. The three different kinds of tumours are treated in different ways: 1. Primary hyperparathyroidism is usually managed surgically although the timing of intervention remains controversial. Occasionally a medical treatment may be offered for the short or longer term. Usually an operation will involve removal of 3 or all 4 parathyroid glands and patients will then require life-long supplements of activated vitamin D and possibly calcium to maintain a normal healthy blood calcium level. 2. Pituitary tumours prolactinomas are usually managed with drugs called dopamine agonists such as cabergoline or bromocriptine. Surgery may occasionally be needed for large tumours that are unresponsive to medical treatment. Patients with acromegaly or Cushings disease nearly always require pituitary surgery to remove the tumour. Drugs such as somatostatin analogues may be used prior to surgery in acromegaly, or after if a patient is not cured, and radiotherapy may be necessary also if there is residual tumour after surgery (see the articles on acromegaly, Cushings disease and prolactinoma, for more information). Non-functioning pituitary tumours may undergo a period of observation depending upon their size and whether a patient’s vision is affected. Surgery and or radiotherapy are treatment options. 3. Pancreatic NETs gastrinomas are managed initially with medication (proton pump inhibitors), which reduces gastric acid secretion and therefore reduces ulceration. However, some patients have very severe disease and may still require surgery for symptom control. Pancreatic surgery to remove the tumour is the mainstay of treatment for insulinoma. Occasionally, a drug, diazoxide can be used while a patient is awaiting a definitive surgical procedure; long-term drug treatment is not usually used in the management of an insulinoma. In non-functioning pancreatic NETs, currently, the recommendation is to pursue surgery if tumours are larger than 2 cm in size, to consider surgery if tumours are 1-2 cm, and probably to opt for surveillance if tumours are 1 cm. Pancreatic surgery may include enucleation of the lesion only, removing a part of the pancreas, or part of the pancreas, stomach and duodenum (Whipples procedure), or in very extreme circumstances the whole pancreas, although this is never undertaken lightly due to the long-term complications for the patient. Adrenal nodules can often simply be monitored by imaging unless they behave suspiciously; as there is a small increased risk of adrenal cancer in MEN1. Non-pancreatic NETs in MEN1 are often treated surgically although other treatment modalities may be considered. Skin lesions are often treated as per patient preference.

• #48 Multiple endocrine neoplasia 1 (MEN1) | Macmillan Cancer Support

  https://www.macmillan.org.uk/cancer-information-and-support/worried-about-cancer/pre-cancerous-and-genetic-conditions/multiple-endocrine-neoplasia-1-men1

  You may be given tablets or injections that reduce the amount of the hormone the tumour is producing. […] If your symptoms are not controlled by drugs, your doctor may talk to you about having an operation to remove the pituitary tumour. A surgeon usually does this through a small cut inside the nose. Most people fully recover from the operation within 2 weeks. […] Some people may need radiotherapy after surgery to destroy any remaining tumour cells.

• #49 Multiple endocrine neoplasia, type 1 (MEN 1) – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/men-1/diagnosis-treatment/drc-20446823

  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. These conditions and treatments may include: […] Pituitary tumors. These types of tumors may be treated with surgery or medicines. Rarely, radiation therapy is used. […] Hyperparathyroidism. Surgery to remove most of the parathyroid glands is the typical treatment for too much parathyroid hormone. […] Neuroendocrine tumors. These are tumors that form in specialized cells called neuroendocrine cells. With MEN 1, they’re in the pancreas or small intestine. Treatment depends on the type and size of the tumor. […] Hypoglycemic syndrome. This condition happens when tumors in the pancreas called insulinomas make too much insulin hormone. Too much insulin causes low levels of blood sugar that can be life-threatening. Treatment often involves surgery. Part of the pancreas may need to be removed too.

• #50 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

  Treatment of MEN 1 is primarily surgical, with subtotal parathyroidectomy; however, hyperparathyroidism frequently recurs. Octreotide and cinacalcet may help control recurrent or persistent postoperative hypercalcemia. […] Prolactinoma is usually managed with dopamine agonists; other pituitary tumors are treated surgically. […] Islet cell tumors are more difficult to manage because the lesions are often small and difficult to find, multiple lesions are common, and surgery often is not curative. […] The treatment of gastrin-secreting gastroenteropancreatic neuroendocrine tumors (GEP-NETs) is complex. When possible, the tumor is located and removed. […] In patients with insulinomas, if a single tumor cannot be found, distal subtotal pancreatectomy with enucleation of any palpable tumors in the head of the pancreas is recommended. Diazoxide or a somatostatin analogue (octreotide, lanreotide) may help treat hypoglycemia.

• #51 Multiple endocrine neoplasia, type 1 (MEN 1) – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/men-1/diagnosis-treatment/drc-20446823

  Zollinger-Ellison syndrome (ZES). ZES can result in tumors called gastrinomas that make too much stomach acid. This leads to ulcers and diarrhea. Healthcare professionals may prescribe medicine or try to remove the tumors with surgery. […] Other pancreatic neuroendocrine tumors. These tumors sometimes make other hormones that can cause health issues. Treatment of these types of tumors may involve medicine or surgery. Another treatment called ablation can be done to destroy irregular tissue that may be present. […] Metastatic neuroendocrine tumors. Tumors that spread are called metastatic tumors. Sometimes with MEN 1, tumors spread to the lymph nodes or liver. They may be treated with surgery. Surgery options include liver surgery or different types of ablation. […] Adrenal tumors. Most of these tumors can be watched with tests over time and not treated. But if the tumors make hormones or they’re large and thought to be cancerous, healthcare professionals recommend removing them. Often, the tumors can be removed with surgery that involves small incisions. This is known as minimally invasive surgery.

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

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

  Metastatic neuroendocrine tumors. Tumors that spread are called metastatic tumors. Sometimes with MEN 1, tumors spread to the lymph nodes or liver. They may be treated with surgery. Surgery options include liver surgery or different types of ablation. […] Adrenal tumors. Most of these tumors can be watched with tests over time and not treated. But if the tumors make hormones or they’re large and thought to be cancerous, healthcare professionals recommend removing them. Often, the tumors can be removed with surgery that involves small incisions. This is known as minimally invasive surgery. […] Carcinoid tumors. These slow-growing tumors in people with MEN 1 can form in the lungs, thymus gland and gastrointestinal tract. 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.

• #53 Multiple Endocrine Neoplasia Type 1 (MEN1) Treatment & Management: Approach Considerations, Consultations

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

  Surgical removal of the tumor is the treatment of choice for glucagonomas. […] Somatostatin analogs control symptoms in 80% of cases of VIPoma. Nevertheless, surgical cure should be attempted. […] If resectable, surgery is the treatment of choice for carcinoid tumors. […] Management is conservative for lipomas, facial angiofibromas, and collagenomas. Local excision can be performed if desired. […] Surgery is indicated for functioning tumors (eg. primary hyperaldosteronism or hypercortisolism), and nonfunctioning tumors with atypical features, size greater than 4 cm, or significant growth over a 6-month interval. […] Centers with expertise in MEN1 diagnosis and treatment are recommended for patients. Multiple consultations are generally necessary, including evaluation by specialists in endocrinology, gastroenterology, neurosurgery, general surgery, and dermatology.

• #54 Frontiers | Multiple Endocrine Neoplasia Type 1 (MEN1): An Update and the Significance of Early Genetic and Clinical Diagnosis

  https://www.frontiersin.org/journals/endocrinology/articles/10.3389/fendo.2019.00339/full

  Multiple endocrine neoplasia type 1 (MEN1) is a rare hereditary tumor syndrome inherited in an autosomal dominant manner and characterized by a predisposition to a multitude of endocrine neoplasms primarily of parathyroid, enteropancreatic, and anterior pituitary origin, as well as nonendocrine neoplasms. […] Despite advances in the diagnosis and treatment of MEN1-associated tumors, patients with MEN1 continue to have decreased life expectancy primarily due to malignant neuroendocrine tumors. The most recent clinical practice guidelines for MEN1, published in 2012, highlight the need for early genetic and clinical diagnosis of MEN1 and recommend an intensive surveillance approach for both patients with this syndrome and asymptomatic carriers starting at the age of 5 years with the goal of timely detection and management of MEN1-associated neoplasms and ultimately decreased disease-specific morbidity and mortality.

• #55 Multiple endocrine neoplasia, type 1 (MEN 1) – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/men-1/diagnosis-treatment/drc-20446823

  Carcinoid tumors. These slow-growing tumors in people with MEN 1 can form in the lungs, thymus gland and gastrointestinal tract. 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.

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

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

  Metastatic neuroendocrine tumors. Tumors that spread are called metastatic tumors. Sometimes with MEN 1, tumors spread to the lymph nodes or liver. They may be treated with surgery. Surgery options include liver surgery or different types of ablation. […] Adrenal tumors. Most of these tumors can be watched with tests over time and not treated. But if the tumors make hormones or they’re large and thought to be cancerous, healthcare professionals recommend removing them. Often, the tumors can be removed with surgery that involves small incisions. This is known as minimally invasive surgery. […] Carcinoid tumors. These slow-growing tumors in people with MEN 1 can form in the lungs, thymus gland and gastrointestinal tract. 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.

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

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

  Treatment of pituitary tumours consists of drug therapy and/or surgery often in association with radiotherapy of the residual unresectable tumours. […] Medical treatment consists of dopamine agonists such as cabergoline, bromocriptine, pergolide and quinagolide. […] Individuals who have a 50% risk of having MEN1 syndrome, but whose genetic status is unknown, should undergo the following tests: biochemical evaluation of serum concentration of prolactin from age 5; biochemical screening of fasting total serum calcium concentration (corrected for albumin) from age 10; biochemical screening of fasting serum gastrin concentration, if the individual has symptoms of ZES (reflux, diarrhoea), from age 20; biochemical screening of fasting serum concentration of full-length PTH from age 10. […] In addition, it is possible to perform prophylactic thymectomy to prevent thymic carcinoids; it should be considered at the time of neck surgery for PHPT, particularly in men with MEN1 syndrome who are smokers and/or have relatives with thymic carcinoids. […] The final goal is to offer patients with MEN1 germline mutations an optimal cancer prevention and treatment program.

• #58 Update on the clinical management of multiple endocrine neoplasia type 1

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9540817/

  The option of chemoprevention for PanNETs in MEN1 is interesting and a recent small observational cohort study compared lanreotide with a standard of care active surveillance. […] Prophylactic thymectomy during initial parathyroidectomy is advocated to reduce the risk of subsequent thNETs, although the risk is not abolished. […] Current guidelines recommend that patients with MEN1 are screened for thoracic NETs by thoracic imaging every 12 years. […] The treatment in MEN1-related PAs is not different from sporadic PAs and in contrast to previous assumptions, recent data show similar treatment responses to sporadic PAs. […] Therefore, currently Dutch females with MEN1 are recommended to undergo breast cancer screening from the age of 40, which is a decade earlier than the regular population screening program in the Netherlands.

• #59 Multiple endocrine neoplasia, type 1 (MEN 1) – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/men-1/diagnosis-treatment/drc-20446823

  Zollinger-Ellison syndrome (ZES). ZES can result in tumors called gastrinomas that make too much stomach acid. This leads to ulcers and diarrhea. Healthcare professionals may prescribe medicine or try to remove the tumors with surgery. […] Other pancreatic neuroendocrine tumors. These tumors sometimes make other hormones that can cause health issues. Treatment of these types of tumors may involve medicine or surgery. Another treatment called ablation can be done to destroy irregular tissue that may be present. […] Metastatic neuroendocrine tumors. Tumors that spread are called metastatic tumors. Sometimes with MEN 1, tumors spread to the lymph nodes or liver. They may be treated with surgery. Surgery options include liver surgery or different types of ablation. […] Adrenal tumors. Most of these tumors can be watched with tests over time and not treated. But if the tumors make hormones or they’re large and thought to be cancerous, healthcare professionals recommend removing them. Often, the tumors can be removed with surgery that involves small incisions. This is known as minimally invasive surgery.

• #60 Preclinical drug studies in MEN1-related neuroendocrine neoplasms (MEN1-NENs) in: Endocrine-Related Cancer Volume 27 Issue 9 (2020)

  https://erc.bioscientifica.com/view/journals/erc/27/9/ERC-20-0127.xml

  Neuroendocrine neoplasms (NENs) occur usually as sporadic tumours; however, rarely, they may arise in the context of a hereditary syndrome, such as multiple endocrine neoplasia type 1 (MEN1), an autosomal dominant disorder characterised by the combined development of pancreatic NENs (pNENs) together with parathyroid and anterior pituitary tumours. […] The therapeutic decision for sporadic pNENs patients is multi-disciplinary and complex: based on the grade and stage of the tumor, various options (and their combinations) are considered, such as surgical excision (either curative or for debulking aims), biological drugs (somatostatin analogues), targeted therapies (mTOR inhibitors or tyrosine kinases (TK)/receptors inhibitors), peptide receptor radioligand therapy (PRRT), chemotherapy, and liver-directed therapies.

• #61 Frontiers | Multiple Endocrine Neoplasia Type 1 (MEN1): An Update and the Significance of Early Genetic and Clinical Diagnosis

  https://www.frontiersin.org/journals/endocrinology/articles/10.3389/fendo.2019.00339/full

  The recommended surgical approach for the treatment of MEN1-associated PHPT is subtotal parathyroidectomy with removal of 3.5 parathyroid glands. […] The use of surgical resection is also limited in advanced metastatic disease, with treatment involving several approaches that may include medical therapy (with everolimus, tyrosine kinase inhibitors such as sunitinib, or chemotherapy), peptide radioreceptor therapy with lutetium-177-labeled somatostatin analog, chemotherapy, or liver directed therapies (such as embolization, chemoembolization, or radioembolization). […] The treatment approach for both glucagonomas and VIPomas is similar to that for insulinomas as described above.

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

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

  Proton pump inhibitors, which inhibit stomach acid production, are used for medical treatment of gastrinomas. […] Treatment for insulinomas may involve surgical removal of a tumour (if over 2cm), or partial/complete removal of the pancreas. […] Tumours may metastasise to the liver, and may be treated by chemotherapy, newer targeted therapies (for example, tyrosine kinase inhibitors) or radio ablation.

• #63 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

  Somatostatin analogs also can block hormone secretion from other nongastrin-secreting pancreatic tumors, including carcinoid tumors, and are well tolerated. […] Palliative treatments for metastatic pancreatic tumors include hepatic debulking surgery and hepatic artery chemoembolization. […] Because MEN 1 tumors do not occur simultaneously, patients tend to have a high fear of disease occurrence for additional tumors in themselves or for disease beginning in their relatives. Studies indicate that this leads to lower quality of life (as measured by emotional and physical well-being), suggesting that medical care should also address and treat the psychological burden of this condition.

• #64 A New Medical Therapy for Multiple Endocrine Neoplasia Type 1? – touchENDOCRINOLOGY

  https://touchendocrinology.com/endocrine-oncology/journal-articles/a-new-medical-therapy-for-multiple-endocrine-neoplasia-type-1/

  Nevertheless, there has remained a distinct lack of other specific medical therapies for pNETs in patients with MEN1, either as prophylaxis against metastasis or to retard initial progression. […] A very recent publication has raised the prospect of a defined medical treatment specifically targeted at patients with MEN1, which may significantly influence the progression of pNETs. […] The article also reported that the first three patients with MEN1 enrolled in the study showed signs of improvement: one 43-year-old man had a progressive pNET that had been stable for 11 months; a second 39-year-old man had a recurrent pNET in the head of the pancreas, which almost disappeared and showed diminished uptake on 68Ga-dotatate scanning; a third 40-year-old man demonstrating progressive disease on a somatostatin analogue and everolimus, showed stable disease for 8 months on leflunomide.

• #65 A New Medical Therapy for Multiple Endocrine Neoplasia Type 1? – touchENDOCRINOLOGY

  https://touchendocrinology.com/endocrine-oncology/journal-articles/a-new-medical-therapy-for-multiple-endocrine-neoplasia-type-1/

  Leflunomide has been used for the treatment of rheumatoid arthritis, with reputedly few adverse effects, and has suggested anti-tumour activity. […] The current preliminary results on patients with advanced pNETs demonstrated some clinical activity, although the animal studies indicate that the earlier in disease course that leflunomide is used, the more likely it is to be effective. […] From that point of view, it may be that this re-purposed agent, which is reputedly inexpensive, could play a more important role in the prophylaxis of pNETs in MEN1. […] Summarizing the above results, this study may provide new ideas and strategies for the treatment of patients with MEN1-mutated tumours and the prevention of MEN1-induced lethality, which are important aims in current research.

• #66 Preclinical drug studies in MEN1-related neuroendocrine neoplasms (MEN1-NENs) in: Endocrine-Related Cancer Volume 27 Issue 9 (2020)

  https://erc.bioscientifica.com/view/journals/erc/27/9/ERC-20-0127.xml

  Despite the advances in their treatment, the life expectancy of MEN1-patients remains shorter than normal population (mean age at death ~55 years) […] The existing therapeutic options for the various NENs have not been formally evaluated in MEN1 patients, being extrapolated from non-MEN1 NENs patients. […] The optimal treatment for these patients is challenging, as MEN1-NENs are multiple, multicentric, pose a higher metastatic potential and are relatively insensitive to treatment. […] The choice of optimal anti-tumour therapies for MEN1-NENs patients is challenging and needs the involvement of experienced multi-disciplinary teams inside referral centers of excellence. […] The use of epigenetic-targeting compounds may therefore have utility in MEN1-associated tumours. […] Preclinical studies have indicated that JQ1, an inhibitor of the bromo and extra terminal domain (BET) family of proteins that bind to acetylated histone residues to promote gene transcription may have efficacy in pancreatic, bronchial and pituitary NENs as in vitro studies revealed that JQ1 decreased proliferation and increased apoptosis of pancreatic, pituitary and bronchial NEN cell lines, as well as reducing ACTH secretion from the ACTH-secreting pituitary cell lines.

• #67 Preclinical drug studies in MEN1-related neuroendocrine neoplasms (MEN1-NENs) in: Endocrine-Related Cancer Volume 27 Issue 9 (2020)

  https://erc.bioscientifica.com/view/journals/erc/27/9/ERC-20-0127.xml

  Furthermore, in vivo, assessment using a pancreatic -cell-specific conditional Men1-knockout mouse model that develops pNENs, revealed that JQ1 decreased proliferation and increased apoptosis of pNENs. […] Pasireotide (SOM230) is effective for the treatment of pancreatic neuroendocrine tumors (PNETs) in a multiple endocrine neoplasia type 1 (MEN1) conditional knockout mouse model. […] Pasireotide decreased proliferation and increased apoptosis of pNENs, suppressed tumour growth and tumor number, increased mice survival and resulted in prevention of tumor development. […] Gene therapy for MEN1-NENs may represent a promising cutting-edge therapy. However, there are major issues to be solved including optimal delivery, risks of several off-target/adverse effects including immune response and mutagenesis and efficacy.

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

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

  How is multiple endocrine neoplasia (MEN) treated? 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). […] There is currently no cure for multiple endocrine neoplasia, but it is manageable. Healthcare providers treat the changes in each gland as they happen with surgery or with medications.

• #69 Multiple endocrine neoplasia 1 (MEN1) | Macmillan Cancer Support

  https://www.macmillan.org.uk/cancer-information-and-support/worried-about-cancer/pre-cancerous-and-genetic-conditions/multiple-endocrine-neoplasia-1-men1

  A team of specialists meet to talk about the best treatment for you. They are called a multidisciplinary team (MDT). […] Your treatment will depend on the type of tumour and where it is. You may need surgery to remove tumours, or drugs to control symptoms. […] The main treatment for parathyroid tumours is surgery. Some people may have monitoring with blood tests to check calcium levels, or medicines to control the blood calcium levels. […] If you have a gastrinoma, your doctors will usually give you tablets that reduce the amount of acid made by the stomach. This helps prevent more serious problems, such as stomach ulcers. Some people may have other treatments such as surgery, hormone treatment or chemotherapy. […] You may be offered treatment with drugs to reduce insulin levels. Or you may have surgery to remove the affected area of the pancreas.

• #70 Multiple Endocrine Neoplasia Type 1 (MEN1) Treatment & Management: Approach Considerations, Consultations

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

  Surgical removal of the tumor is the treatment of choice for glucagonomas. […] Somatostatin analogs control symptoms in 80% of cases of VIPoma. Nevertheless, surgical cure should be attempted. […] If resectable, surgery is the treatment of choice for carcinoid tumors. […] Management is conservative for lipomas, facial angiofibromas, and collagenomas. Local excision can be performed if desired. […] Surgery is indicated for functioning tumors (eg. primary hyperaldosteronism or hypercortisolism), and nonfunctioning tumors with atypical features, size greater than 4 cm, or significant growth over a 6-month interval. […] Centers with expertise in MEN1 diagnosis and treatment are recommended for patients. Multiple consultations are generally necessary, including evaluation by specialists in endocrinology, gastroenterology, neurosurgery, general surgery, and dermatology.

• #71 Role of Nutrition in the Management of Patients with Multiple Endocrine Neoplasia Type 1

  https://www.mdpi.com/2072-6643/16/11/1576

  This review aims to outline nutritional indications for MEN1 patients, to direct them towards the “right diet” to follow, both as prevention and as treatment of the main nutritional deficiencies they may encounter, emphasizing the importance that MEN1 patients should be followed a multidisciplinary team that includes not only the endocrinologist, the surgeon, the oncologist, the radiotherapist, but also the nutritionist/dietitian. […] The nutritionist/dietitian must take into account these clinical aspects, in drawing up the MEN1 patient’s food plan, paying attention primarily to the amount of dairy dietary calcium intake, but also granting a correct intake of phosphorus, magnesium, and vitamin D, all factors that regulate bone metabolism. […] Dietary sodium levels should be limited to less than 1.5 g per day in adults, as recommended by the LARN 2014.

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

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

  Monitoring and follow-up care for MEN1 depends on how severe the disease is, the location of any tumors that may arise, and a persons overall health. Care may involve managing symptoms with medications or hormones. Patients may also have surgery to remove their tumors if the tumor presses on nearby organs or produces very high levels of hormones in their bodies. […] Some tumors, like carcinoid tumors, may require treatments such as chemotherapy. […] Because MEN1 is a complex condition, parents should discuss screening options for their child with an experienced health care provider who knows this condition well. The goal of screening is to find and treat tumors early. Doing so allows the best outcome for patients. There are clinics throughout the country that specialize in taking care of people with MEN.

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

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

  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.

• #74 Update on the clinical management of multiple endocrine neoplasia type 1

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9540817/

  The option of chemoprevention for PanNETs in MEN1 is interesting and a recent small observational cohort study compared lanreotide with a standard of care active surveillance. […] Prophylactic thymectomy during initial parathyroidectomy is advocated to reduce the risk of subsequent thNETs, although the risk is not abolished. […] Current guidelines recommend that patients with MEN1 are screened for thoracic NETs by thoracic imaging every 12 years. […] The treatment in MEN1-related PAs is not different from sporadic PAs and in contrast to previous assumptions, recent data show similar treatment responses to sporadic PAs. […] Therefore, currently Dutch females with MEN1 are recommended to undergo breast cancer screening from the age of 40, which is a decade earlier than the regular population screening program in the Netherlands.

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

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

  Treatment of pituitary tumours consists of drug therapy and/or surgery often in association with radiotherapy of the residual unresectable tumours. […] Medical treatment consists of dopamine agonists such as cabergoline, bromocriptine, pergolide and quinagolide. […] Individuals who have a 50% risk of having MEN1 syndrome, but whose genetic status is unknown, should undergo the following tests: biochemical evaluation of serum concentration of prolactin from age 5; biochemical screening of fasting total serum calcium concentration (corrected for albumin) from age 10; biochemical screening of fasting serum gastrin concentration, if the individual has symptoms of ZES (reflux, diarrhoea), from age 20; biochemical screening of fasting serum concentration of full-length PTH from age 10. […] In addition, it is possible to perform prophylactic thymectomy to prevent thymic carcinoids; it should be considered at the time of neck surgery for PHPT, particularly in men with MEN1 syndrome who are smokers and/or have relatives with thymic carcinoids. […] The final goal is to offer patients with MEN1 germline mutations an optimal cancer prevention and treatment program.

• #76 Role of Nutrition in the Management of Patients with Multiple Endocrine Neoplasia Type 1

  https://www.mdpi.com/2072-6643/16/11/1576

  Multiple endocrine neoplasia type 1 (MEN1) is a rare syndrome caused by inactivating mutations in the MEN1 tumor suppressor gene. […] It is important to remember the fundamental role that diet plays as a primary prevention tool, together with a healthy and active lifestyle in preventing osteoporosis/osteopenia and reducing the risk of developing kidney stones due to hypercalciuria, two frequent clinical complications in MEN1 patients. […] Therefore, the nutritionist’s aim should be to devise a dietary plan that takes into consideration each single patient, educating them about a healthy and active lifestyle, and accompanying them through various life stages by implementing strategies that can enhance their quality of life. […] Nutrition can be a valuable aid, together with a healthy and active lifestyle, in the prevention of osteoporosis, and in reducing the risk of developing kidney stones, two frequent clinical complications in patients with MEN1 syndrome and PHPT.

• #77 Role of Nutrition in the Management of Patients with Multiple Endocrine Neoplasia Type 1

  https://www.mdpi.com/2072-6643/16/11/1576

  This review aims to outline nutritional indications for MEN1 patients, to direct them towards the “right diet” to follow, both as prevention and as treatment of the main nutritional deficiencies they may encounter, emphasizing the importance that MEN1 patients should be followed a multidisciplinary team that includes not only the endocrinologist, the surgeon, the oncologist, the radiotherapist, but also the nutritionist/dietitian. […] The nutritionist/dietitian must take into account these clinical aspects, in drawing up the MEN1 patient’s food plan, paying attention primarily to the amount of dairy dietary calcium intake, but also granting a correct intake of phosphorus, magnesium, and vitamin D, all factors that regulate bone metabolism. […] Dietary sodium levels should be limited to less than 1.5 g per day in adults, as recommended by the LARN 2014.

• #78 Role of Nutrition in the Management of Patients with Multiple Endocrine Neoplasia Type 1

  https://www.mdpi.com/2072-6643/16/11/1576

  Lifelong replacement therapy with digestive enzymes derived from the porcine exocrine pancreas is the intervention of choice for the management of malabsorption due to exocrine pancreas insufficiency. […] Somatostatin analogues (SSAs), octreotide and lanreotide, were shown to be effective in MEN1, to both reduce excessive hormone secretion, controlling NET-derived endocrine syndromes, and to control tumor growth, reducing tumor size, and are, thus, commonly used in MEN1 patients with GEP-NETs not suitable for surgery, those who refused the surgical intervention, or to shrink tumor before surgery. […] Currently, nutritional and vitamin status is a neglected area for MEN1 patients with DP-NETs. […] In conclusion, it is essential to underline the importance of managing MEN1 patients through a multidisciplinary approach, which should include also the figure of the nutritionist/dietitian, a specific aspect that is currently missing in the global management of MEN1 syndrome.

• #79 Multiple Endocrine Neoplasia Syndromes – Endocrinology | Conditions, Treatments & Specialty | Geisinger

  https://www.geisinger.org/patient-care/conditions-treatments-specialty/multiple-endocrine-neoplasia-syndromes

  Get treatment for multiple endocrine neoplasia syndromes […] Multiple endocrine neoplasia syndromes treatment […] Our team of endocrine specialists is experienced in diagnosing and treating all kinds of endocrine conditions, including multiple endocrine neoplasia syndromes. Depending on the type and severity of your condition, one or more of the following treatments may be recommended. […] You can make several lifestyle changes that your doctor may suggest to help manage your symptoms. These may include: Increasing sleep, Exercise, Change in diet, Reducing stress, Emotional support […] Your doctor may recommend prescription medications to help manage your symptoms and treat your endocrine conditions. This may include medications to regulate the amount of hormones your endocrine system produces.

• #80 Frontiers | Multiple Endocrine Neoplasia Type 1 (MEN1): An Update and the Significance of Early Genetic and Clinical Diagnosis

  https://www.frontiersin.org/journals/endocrinology/articles/10.3389/fendo.2019.00339/full

  Multiple endocrine neoplasia type 1 (MEN1) is a rare hereditary tumor syndrome inherited in an autosomal dominant manner and characterized by a predisposition to a multitude of endocrine neoplasms primarily of parathyroid, enteropancreatic, and anterior pituitary origin, as well as nonendocrine neoplasms. […] Despite advances in the diagnosis and treatment of MEN1-associated tumors, patients with MEN1 continue to have decreased life expectancy primarily due to malignant neuroendocrine tumors. The most recent clinical practice guidelines for MEN1, published in 2012, highlight the need for early genetic and clinical diagnosis of MEN1 and recommend an intensive surveillance approach for both patients with this syndrome and asymptomatic carriers starting at the age of 5 years with the goal of timely detection and management of MEN1-associated neoplasms and ultimately decreased disease-specific morbidity and mortality.

• #81 Multiple endocrine neoplasia type 1 (MEN1): Not only inherited endocrine tumors | Genetics in Medicine

  https://www.nature.com/articles/gim2009126

  MEN1-insulinomas are almost always benign. […] A prospective endoscopic ultrasonographic evaluation of the frequency of nonfunctioning pancreatic tumors in patients with MEN1 suggested that their frequency is higher (55%) than previously thought. […] Their cancer progression risk is very low. […] Nonendocrine tumors associated with MEN1 syndrome include skin tumors such as facial angiofibromas, collagenomas, lipomas, CNS tumors such as meningiomas, ependymomas, and leiomyomas. […] Counseling of patients and closely related family members has a central role in the management of hereditary diseases, such as MEN1 syndrome. […] The advantages of DNA analysis are that it requires a single blood sample and does not need to be repeated in theory, because the analysis is independent of the age of the individual and provides an objective result.

• #82 Childhood Multiple Endocrine Neoplasia (MEN) | Dana-Farber Cancer Institute

  https://www.dana-farber.org/cancer-care/types/childhood-multiple-endocrine-neoplasia

  We provide two programs for children with MEN1 and MEN2, including our Endocrine-Oncology Program and Boston Children’s Hospital Thyroid Center. Once in our care, we build a team to treat your child that includes pediatric experts in the areas of oncology, endocrinology, genetics, and surgery. […] Treatment for MEN1 and MEN2 includes: […] Surgery: We use surgery to remove any existing tumors. We may also perform surgery to prevent medullary thyroid cancer from developing in patients with MEN2. […] Radiation therapy: Often, we’ll use radiation therapy before or after surgery to damage or eliminate cancer cells and shrink tumors. […] Chemotherapy: We may also use chemotherapy to further destroy or shrink cancer cells. […] Genetic counseling: We help your child and family cope with the stress of hereditary cancer diagnosis while discussing practical considerations such as future reproductive options. […] We also provide children with continued, comprehensive care in our pediatric cancer survivorship programs. We conduct regular medical evaluations and provide patient and family education, support groups, and counseling services.

• #83 Facts for people and families with Multiple Endocrine Neoplasia type 1

  https://www.eviq.org.au/cancer-genetics/consumer-information/3447-facts-for-people-and-families-with-multiple-e

  This fact sheet contains general information about Multiple Endocrine Neoplasia type 1 (MEN type 1). Each person should be referred to a genetic service for further information and advice. […] To find tumours and cancer early, people with MEN type 1 should have a blood test to check their hormone levels and a physical examination with a doctor every year from age 10 years. […] To find tumours and cancer early, people with MEN type 1 should have imaging to check their brain at age 10 years. This imaging should then be done every four years from age 20 years. Imaging to check the pancreas, duodenum, chest and adrenal glands should be done every 2 years from age 20 years. This involves having MRI scans. […] Family members (including children) of someone with a faulty MEN1 gene can have genetic testing to check who has the faulty gene and who does not. Their doctor can refer them to a genetic service to find out more about their chance of developing cancer and what genetic testing involves. […] People who decide not to have genetic testing should still get advice about managing their chance of developing cancer.

• #84 Multiple Endocrine Neoplasia Type 1 (09.08.2024)

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

  As an alternative to surgery for small NF-pNENs, radiofrequency ablation has recently been declared a successful treatment, especially for elderly patients. […] Functioning dpNENs include gastrinomas, insulinomas, vasoactive intestinal peptide (VIP)-secreting tumors (VIPomas), and glucagonomas. […] 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. […] If a gastrinoma is confirmed by clinical and biochemical findings, some experts recommend drug therapy with proton pump inhibitors (PPIs) alone. […] When deciding on a treatment, the preference of the fully informed patient should always be taken into account. […] 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. […] 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. Screening programs in specialized centers can help to detect tumor manifestations in time and provide organ-sparing treatment.

• #85 Frontiers | Multiple Endocrine Neoplasia Type 1 (MEN1): An Update and the Significance of Early Genetic and Clinical Diagnosis

  https://www.frontiersin.org/journals/endocrinology/articles/10.3389/fendo.2019.00339/full

  Multiple endocrine neoplasia type 1 (MEN1) is a rare hereditary tumor syndrome inherited in an autosomal dominant manner and characterized by a predisposition to a multitude of endocrine neoplasms primarily of parathyroid, enteropancreatic, and anterior pituitary origin, as well as nonendocrine neoplasms. […] Despite advances in the diagnosis and treatment of MEN1-associated tumors, patients with MEN1 continue to have decreased life expectancy primarily due to malignant neuroendocrine tumors. The most recent clinical practice guidelines for MEN1, published in 2012, highlight the need for early genetic and clinical diagnosis of MEN1 and recommend an intensive surveillance approach for both patients with this syndrome and asymptomatic carriers starting at the age of 5 years with the goal of timely detection and management of MEN1-associated neoplasms and ultimately decreased disease-specific morbidity and mortality.

• #86 Multiple Endocrine Neoplasia | MD Anderson Cancer Center

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

  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. This means we are able to offer clinical trials, which may be difficult to find in other programs.

• #87

• #88 Multiple Endocrine Neoplasia Type 1 (09.08.2024)

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

  As an alternative to surgery for small NF-pNENs, radiofrequency ablation has recently been declared a successful treatment, especially for elderly patients. […] Functioning dpNENs include gastrinomas, insulinomas, vasoactive intestinal peptide (VIP)-secreting tumors (VIPomas), and glucagonomas. […] 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. […] If a gastrinoma is confirmed by clinical and biochemical findings, some experts recommend drug therapy with proton pump inhibitors (PPIs) alone. […] When deciding on a treatment, the preference of the fully informed patient should always be taken into account. […] 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. […] 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. Screening programs in specialized centers can help to detect tumor manifestations in time and provide organ-sparing treatment.

• #89 Comprehensive treatment of rare multiple endocrine neoplasia type 1: A case report

  https://www.wjgnet.com/2307-8960/full/v8/i12/2647.htm

  Since there were no obvious surgical contraindications, the MDT recommended a radical excision of the lesions to alleviate hypoglycemia symptoms and improve the quality of life for the patient. […] After surgery, all diabetes factors with abnormal serum levels were returned to normal levels. […] The progression of MEN1 was successfully controlled by MDT in the current study. […] If a patient is diagnosed with primary hyperparathyroidism with pituitary tumors or gastrointestinal NET, and the family history shows similar symptoms, MEN1 should be considered, and genetic testing is recommended.

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