Materiały źródłowe

• #1 Multiple Endocrine Neoplasia Type 1 – NIDDK

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

  MEN1 symptoms can differ from person to person, even among family members who have the disorder. The age at which signs or symptoms appear may also vary. […] As MEN1 almost always affects the parathyroid glands, the most common early symptoms are related to excess parathyroid hormone. These symptoms are often mild and may not be noticed for years. They include kidney stones, muscle weakness, tiredness, increased thirst and urination, depression, aches and pains in bones and joints, digestive problems and constipation. […] Tumors located in other endocrine glands can cause other symptoms, such as stomach ulcers, acid reflux, abdominal pain, frequent diarrhea, low blood glucose, enlarged and swollen hands and feet.

• #2 15 Top Multiple Endocrine Neoplasia Symptoms & Signs | MD Anderson Cancer Center

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

  Symptoms of multiple endocrine neoplasia (MEN) vary from person to person and depend on which glands are affected. […] Hyperparathyroidism, which means the parathyroid gland produces too much hormone. This may cause tiredness, weakness, muscle or bone pain, constipation, kidney stones or thinning of bones. Hyperparathyroidism is usually the first sign of MEN1 and typically occurs between the ages 20 and 25. Nearly 100% of people with MEN1 will develop hyperparathyroidism by the age of 50. […] High levels of gastrin, ulcers, inflammation of the esophagus, diarrhea and abdominal pain […] Headaches and changes in vision […] Problems with sexual function and fertility […] Acromegaly (enlargement of the bones) […] Cushings syndrome […] Excess production of breast milk.

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

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

  Insulinomas are the second most common functioning pancreatic neuroendocrine tumor in MEN1, developing at young age ( 35 years) in approximately 10-30% of patients. […] MEN1-associated anterior pituitary tumors most commonly secrete prolactin (60%), followed by tumors that secrete growth hormone (25%). […] Patients with multiple endocrine neoplasia type 1 (MEN1) have a decreased life expectancy, with a 50% probability of death by age 50 years. […] Malignant pancreatic neuroendocrine tumors and thymic carcinoid tumors have been associated with a marked increase in the risk of death in MEN1 patients. […] The peak incidence of symptoms in MEN1 is during the third decade of life in women and during the fourth decade of life in men. The reported age range is 5-81 years.

• #4 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

  Symptoms vary depending on which endocrine glands are affected. The symptoms are usually due to an overproduction of hormones. […] The most common symptoms are due to an overactive parathyroid gland. This often occurs in people in their 20s. Most people are diagnosed in their 40s as other endocrine glands are affected. […] High levels of calcium in the blood can make you: feel sick (nauseous), thirsty, drowsy, confused, unwell, constipated, need to pee (pass urine) often. […] If you have MEN1, you usually have a yearly MRI scan, CT scan or EUS of your pancreas and duodenum to check for tumours. […] Gastrinomas make large amounts of gastrin. This can cause you to have too much acid in the stomach. This can cause indigestion, diarrhoea and eventually stomach ulcers. […] Insulinomas make too much insulin. This can cause low blood sugar (hypoglycaemia). Low blood sugar may cause mild symptoms, such as feeling hungry, shaky or anxious. It can also cause more severe symptoms, such as fainting or seizures.

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

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

  Multiple endocrine neoplasia, type 1 (MEN 1) is a rare condition. It mainly causes tumors in the glands that make and release hormones. These are called the endocrine glands. The condition also can cause tumors in the small intestine and stomach. Another name for MEN 1 is Wermer’s syndrome. […] The extra hormones of MEN 1 can cause many symptoms. These symptoms may include tiredness, bone pain, broken bones, kidney stones, and ulcers in the stomach or intestines. […] Symptoms of multiple endocrine neoplasia, type 1 (MEN 1) can include the following: Tiredness. Bone pain or broken bones. Kidney stones. Ulcers in the stomach or intestines. Stomach pain. Muscle weakness. Depression. Acid reflux. Frequent diarrhea. […] Symptoms are caused by the release of too many hormones in the body.

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

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

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

• #7 Multiple endocrine neoplasia: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/multiple-endocrine-neoplasia/

  Multiple endocrine neoplasia type 1 frequently involves tumors of the parathyroid glands, the pituitary gland, and the pancreas. […] The most common sign of multiple endocrine neoplasia type 1 is overactivity of the parathyroid glands (hyperparathyroidism). Hyperparathyroidism disrupts the normal balance of calcium in the blood, which can lead to kidney stones, thinning of bones, nausea and vomiting, high blood pressure (hypertension), weakness, and fatigue. […] Multiple endocrine neoplasia type 4 appears to have signs and symptoms similar to those of type 1, although it is caused by mutations in a different gene. Hyperparathyroidism is the most common feature, followed by tumors of the pituitary gland, additional endocrine glands, and other organs.

• #8 Multiple Endocrine Neoplasia (MEN) | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/multiple-endocrine-neoplasia

  The symptoms of multiple endocrine neoplasia (MEN) may vary from child to child and depend on the type of the disease. Symptoms might mimic other, more common ailments. Sometimes your child may not experience symptoms but MEN type 1 or 2 may be suggested because of a family history. […] Some symptoms of MEN1 may include: Elevated calcium levels, Abdominal pain, vomiting, and weight changes due to pancreatic islet cell tumors, Headaches, visual disturbances, and problems with fertility from pituitary gland tumors. […] Children with MEN who had been treated for a tumor should visit a survivorship clinic yearly.

• #9 Multiple endocrine neoplasia (MEN) I: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/000398.htm

  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. […] Symptoms vary from person to person, and depend on which gland is involved. They may include: Abdominal pain, Anxiety, Black, tarry stools, Bloated feeling after meals, Burning, aching, or hunger discomfort in the upper abdomen or lower chest that is relieved by antacids, milk, or food, Decreased sexual interest, Fatigue, Headache, Lack of menstrual periods (in women), Loss of appetite, Loss of body or facial hair (in men), Mental changes or confusion, Muscle pain, Nausea and vomiting, Sensitivity to the cold, Unintentional weight loss, Vision problems, Weakness. […] The symptoms of peptic ulcer disease, low blood sugar, excess calcium in the blood, and pituitary dysfunction usually respond well to appropriate treatment. […] The tumors can keep coming back. Symptoms and complications depend on which glands are involved. Regular check-ups by your provider are essential. […] Contact your provider if you notice symptoms of MEN I or have a family history of this condition.

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

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

  Symptoms of mild hyperparathyroidism include: Joint pain. Muscle weakness. Fatigue. Depression. Trouble concentrating. Loss of appetite. […] Symptoms of severe hyperparathyroidism include: Nausea and vomiting. Confusion and forgetfulness. Increased thirst and frequent urination. Constipation. Bone pain. […] Gastrinomas cause higher-than-normal levels of gastrin, which causes your stomach to release too much acid. Symptoms of gastrinomas include: Abdominal pain. Diarrhea. Acid reflux (esophageal reflux). Peptic ulcers. […] Insulinoma can cause low blood sugar (hypoglycemia), which can cause the following symptoms: Confusion. Shakiness. Sweating. Hunger. Anxiety. Heart palpitations. Temporary vision changes. […] Approximately 25% of people with MEN type 1 develop benign tumors in their pituitary gland.

• #11 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

  Pituitary tumours can cause symptoms by pressing on the optic nerve. This is the nerve between the eye and the brain. Pituitary tumours can cause headaches and changes in eyesight. Other symptoms can be caused by a change in normal hormone levels. […] Prolactinomas can cause: a milky discharge from the breasts, monthly periods to stop, problems with erections (erectile dysfunction), infertility. […] Tumours that make too much growth hormone can cause abnormal growth. Hands, feet, the lower jaw and brows can get larger. This is called acromegaly. These tumours may also cause high blood pressure, diabetes or excess sweating. […] High levels of ACTH can lead to a condition called Cushings syndrome. Symptoms include weight gain, a rounder face shape, and low mood (depression). It can also cause increased facial hair growth.

• #12 Multiple endocrine neoplasia type 1 | Endocrine Conditions

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

  Multiple endocrine neoplasia type 1 (MEN1) is a rare inherited disease, which is characterised by tumour development in the pituitary gland, parathyroid glands, and the pancreas. The three endocrine tumours which constitute MEN1 are: 1. Primary hyperparathyroidism the vast majority of patients with MEN1 will have problems with over activity of the parathyroid glands (hyperparathyroidism) by the age of 50 years. This overproduction of parathyroid hormone results in high calcium levels in the blood and urine. Symptoms include tiredness, depression, stomach ulcers, abdominal pain and non-specific aches and pains, and if left untreated, it can result in thinning of bones (osteoporosis) and kidney stones. Very rarely, patients with MEN1 may have a parathyroid cancer. It is important to note that most patients with hyperparathyroidism do not develop all of the above symptoms especially if diagnosed early.

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

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

  Primary hyperparathyroidism (PHPT) is the most common clinical manifestation of MEN1, affecting more than 95% of all MEN1 patients. The age of the onset (typically between 20 and 25 years of age) of MEN1-associated parathyroid tumours is about three decades earlier than that of sporadic parathyroid adenoma; these tumours are generally characterised by multiglandular hyperplasia. […] Symptoms of PHPT in MEN1 are the same as those of sporadic PHPT. PHPT in MEN1 manifests with hypercalcaemia as a result of overproduction of parathyroid hormone (PTH) by tumoural and supernumerary parathyroid glands. […] The common clinical manifestations of hypercalcaemia include: central nervous system altered mental status, including lethargy, depression, decreased alertness and confusion; gastrointestinal tract anorexia, constipation, nausea and vomiting; kidneys polyuria, nycturia, polydipsia, impaired concentrating ability, dehydration, hypercalciuria and increased risk for kidney stones; skeleton increased bone resorption and increased fracture risk, mainly in women who manifest PHPT before 35 years of age; cardiovascular system hypertension, shortened QT interval.

• #13 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. It occurs in approximately one in 30,000 individuals. […] MEN1 syndrome is characterised by the occurrence of primary tumours involving two or more endocrine tissues within a single patient. It encompasses tumours of the parathyroids (95% of cases), pancreatic islets (from 30 to 80% of cases) and anterior pituitary (from 15 to 90% of cases). […] Hyperparathyroidism is the most common and usually the first clinical manifestation of MEN1. Gastrinoma and carcinoids represent the most frequent causes of mortality. The onset of the MEN1-associated primary hyperparathyroidism and the onset of MEN1-associated gastrinoma and insulinoma anticipate the onset of the corresponding sporadic counterparts of three and one decades, respectively.

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

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

  Symptoms of prolactinomas include: Changes in menstruation unrelated to menopause, such as irregular periods (menstruation) or no periods (amenorrhea). Infertility. Milky discharge from the nipples when not pregnant or breastfeeding called galactorrhea. Loss of interest in sex. […] Symptoms of hyperparathyroidism include: Joint pain. Muscle weakness. Fatigue. Depression. Trouble concentrating. Loss of appetite. Nausea and vomiting. Increased thirst and frequent urination. Constipation. Bone pain.

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

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

  Not all people with MEN1 will have all these symptoms. Some people may have no symptoms. Others may have many symptoms. […] Change in mental status (tired, depressed, less alert, confused) […] Weight loss […] Constipation […] Nausea and vomiting […] Urinating more than normal […] Dehydration (not drinking enough fluids) […] Weak bones […] High blood pressure […] Heart rhythm problems […] Kidney stones […] For women: a lack of periods (amenorrhea) or abnormal production of milk in the breasts (galactorrhea) […] For men: a loss of sex drive […] Cushing syndrome: obesity, diabetes, hypertension, bone loss, and depression […] For pituitary gland tumors: taller than normal (gigantism); overgrowth of the hands, feet, and face (acromegaly) […] Pain in the abdomen (belly) […] Nausea and vomiting […] Loss of appetite […] Diarrhea […] Heartburn (esophageal reflux) […] Peptic ulcers.

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

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

  Multiple endocrine neoplasia type 1 (MEN1) is a rare genetic disorder that can be passed down from parents to their children (inherited). People with MEN1 have a higher risk of developing certain kinds of tumors. These tumors are usually non-cancerous (benign) but may sometimes be cancerous (malignant). […] People with MEN1 may also have tumors in other parts of their bodies, which are not in the endocrine system. These are known as non-endocrine tumors. […] People with MEN1 have an increased risk of developing endocrine and non-endocrine tumors. Nearly all people with MEN1 develop some type of endocrine tumor during their lifetime. […] MEN1 can affect many areas of the body, so people with this condition may have a variety of symptoms. Their symptoms will vary, depending on where tumors are located and the amount of hormones their tumor makes.

• #16

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

  MEN1 is typically characterised by tumours in the following locations of the body: […] About 95% of people with MEN1 develop tumours in the parathyroid glands by age 50. These four pea-sized glands produce the parathyroid hormone, which helps to maintain the right balance of calcium and phosphate in your body. Over time, MEN1 can affect all four glands. MEN1-related tumours cause the parathyroid glands to become overactive, producing too much parathyroid hormone. This condition, called hyperparathyroidism, is the most common complication associated with MEN1. […] Excess parathyroid hormone can cause calcium levels in your blood to rise too high. Complications may include: fractures, kidney stones, muscle weakness, tiredness or confusion, increased thirst and urination, depression, aches and pains in bones and joints, constipation or abdominal pain.

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

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

  Multiple endocrine neoplasia type 1 (MEN1) is characterized by the combination of parathyroid tumors, pancreatic islet cell tumors, and anterior pituitary tumors. Most MEN1 tumors are not aggressive, and many of them (particularly nonfunctioning tumors) follow a long-term indolent course, remaining asymptomatic for years. Nevertheless, patients with untreated MEN1 have a decreased life expectancy, with a 50% probability of death by age 50 years. […] Primary hyperparathyroidism, due to hyperplasia and/or adenoma of parathyroid glands, is the most common manifestation of multiple endocrine neoplasia type 1 (MEN1) and occurs in approximately 90% of all patients. […] Common symptoms associated with hypercalcemia include polydipsia, polyuria, constipation, and generalized malaise. […] Pancreatic islet cell tumors represent the second most common manifestation of MEN1, occurring in 30-80% of patients.

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

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

  Multiple endocrine neoplasia, type 1 (MEN 1) is an autosomal dominant syndrome characterized by hyperplasia or adenomas of the parathyroid glands, pancreatic islet cell tumors (also known as pancreatic neuroendocrine tumors), and/or pituitary gland tumors. Hyperparathyroidism and asymptomatic hypercalcemia commonly occur. […] A patient with a MEN 1 gene mutation and one of the MEN 1 tumors is at risk of developing any of the other tumors later on. Age at onset ranges from 4 to 81 years, but peak incidence occurs in the 20s to 40s. Men and women are equally affected. […] Hyperparathyroidism is present in 95% of patients. Asymptomatic hypercalcemia is the most common manifestation, but about 25% of patients have evidence of nephrolithiasis or nephrocalcinosis. […] Pancreatic islet cell tumors (also known as pancreatic neuroendocrine tumors) occur in 30 to 90% of patients. The most common functional enteropancreatic tumor in MEN 1 is the gastrinoma, which can arise from the pancreas or the duodenum. Up to 80% of patients with MEN 1 have either multiple peptic ulcers due to gastrin-stimulated increased gastric acid secretion or asymptomatic elevated gastrin levels.

• #19 Multiple endocrine neoplasia type 1 | Endocrine Conditions

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

  2. Pituitary tumours about 30-40% of patients with MEN1 develop pituitary tumours. These tumours may produce excess hormones (functional pituitary tumours). The most common functional pituitary tumours diagnosed in patients with MEN1 are prolactinomas (milk hormone producing tumors), occurring in about 20% of patients with MEN1. These tumours produce excess prolactin (milk hormone). In women, prolactinomas may cause breast milk production without pregnancy, lack of periods and may lead to infertility. In men, prolactinomas can cause impotence and infertility. […] 3. Pancreatic neuroendocrine tumours (NETs) these tumours occur in up to 60% of patients with MEN1 and may be i) functioning making different hormones characterised by different symptoms described below, or ii) non-functioning. […] Gastrinomas these are the most common functioning pancreatic NET in MEN1 and occur in up to 40% of patients. The excess secretion of gastrin by a gastrinoma causes the over-production of acid in the stomach leading to stomach/duodenal ulcers (sometimes known as Zollinger-Ellison syndrome). Patients can present with severe bleeding from these ulcers, and may develop strictures of the upper gastrointestinal tract as well as diarrhoea.

• #20 Multiple Endocrine Neoplasia Type 1 (MEN1) Clinical Presentation: History and Physical Examination

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

  The presentation in multiple endocrine neoplasia type 1 (MEN1) varies from patient to patient. Patients may be asymptomatic, or may present with signs and symptoms related to the endocrine organs involved and the hormones secreted, as follows: Hyperparathyroidism is usually the initial clinical manifestation of MEN1 […] Some patients initially present with Zollinger-Ellison syndrome (ZES). Gastrinoma symptoms caused by ZES include diarrhea and upper abdominal pain due to peptic ulcers and esophagitis; complications include ulcer perforation or bleeding. […] Insulinomas also may be identified prior to primary hyperparathyroidism-associated hypercalcemia. Hypoglycemia after a fast or exertion, with improvement in symptoms after sugar intake, is the classic presentation. […] MEN1 patients with hyperparathyroidism usually present with mild hypercalcemia, and rarely develop nephrolithiasis. Other manifestations include bone abnormalities and musculoskeletal complaints. Patients may report increased thirst and urination and/or constipation. In more severe hypercalcemia, generalized weakness and alterations of mental status may develop.

• #21 Multiple endocrine neoplasia type 1 – Wikipedia

  https://en.wikipedia.org/wiki/Multiple_endocrine_neoplasia_type_1

  Most islet cell tumors secrete pancreatic polypeptide, the clinical significance of which is unknown. Gastrin is secreted by many non-cell tumors (increased gastrin secretion in MEN 1 also often originates from the duodenum). Increased gastrin secretion increases gastric acid, which may inactivate pancreatic lipase, leading to diarrhea and steatorrhea. Increased gastrin secretion also leads to peptic ulcers in 50% of MEN 1 patients. Usually the ulcers are multiple or atypical in location, and often bleed, perforate, or become obstructed. Peptic ulcer disease may be intractable and complicated. Among patients presenting with Zollinger-Ellison syndrome, 20 to 60% have MEN 1. […] A severe secretory diarrhea can develop and cause fluid and electrolyte depletion with non-cell tumors. This complex, referred to as the watery diarrhea, hypokalemia and achlorhydria syndrome (VIPoma) has been ascribed to vasoactive intestinal polypeptide, although other intestinal hormones or secretagogues (including prostaglandins) may contribute. Hypersecretion of glucagon, somatostatin, chromogranin, or calcitonin, ectopic secretion of ACTH resulting in Cushing’s syndrome, and hypersecretion of somatotropinreleasing hormone (causing acromegaly) sometimes occur in non-cell tumors. All of these are rare in MEN 1. Nonfunctioning pancreatic tumors also occur in patients with MEN 1 and may be the most common type of pancreatoduodenal tumor in MEN 1. The size of the nonfunctioning tumor correlates with risk of metastasis and death.

• #22 Multiple endocrine neoplasia type 1 – Wikipedia

  https://en.wikipedia.org/wiki/Multiple_endocrine_neoplasia_type_1

  Neoplasia in the pituitary gland can manifest as prolactinomas whereby too much prolactin is secreted, suppressing the release of gonadotropins, causing a decrease in sex hormones such as testosterone. Pituitary tumor in MEN1 can be large and cause signs by compressing adjacent tissues. […] Pancreatic tumors associated with MEN-1 usually form in the beta cells of the islets of Langerhans, causing over-secretion of insulin, resulting in low blood glucose levels (hypoglycemia). However, many other tumors of the pancreatic Islets of Langerhans can occur in MEN-1. One of these, involving the alpha cells, causes over-secretion of glucagon, resulting in a classic triad of high blood glucose levels (hyperglycemia), a rash called necrolytic migratory erythema, and weight loss. Gastrinoma causes the over-secretion of the hormone gastrin, resulting in the over-production of acid by the acid-producing cells of the stomach (parietal cells) and a constellation of sequelae known as Zollinger-Ellison syndrome. Zollinger-Ellison syndrome may include severe gastric ulcers, abdominal pain, loss of appetite, chronic diarrhea, malnutrition, and subsequent weight loss.

• #23 Multiple endocrine neoplasia syndromes – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/866

  Multiple endocrine neoplasia syndromes are hereditary tumour syndromes with distinct patterns of organ involvement. […] Medical management of hormonal hypersecretion is important for symptom control. […] Morbidity and mortality result from both hormonal hypersecretion and metastases. […] Multiple endocrine neoplasia (MEN) syndromes are hereditary tumour syndromes of variable neoplastic patterns and characterised by the development of multiple endocrine tumours. […] Clinical features of kidney stones (MEN1/2) […] Facial angiofibromas or collagenomas (MEN1) […] Clinical features of acromegaly (MEN1) […] Clinical features of thyrotoxicosis (MEN1) […] Unexplained flushing (MEN2) […] Infertility (MEN1) […] Visual changes (MEN1) […] Irregular menses (MEN1) […] Anxiety (MEN1/2) […] Heat intolerance (MEN1) […] Confusion (MEN1/2) […] Dehydration (MEN1/2) […] Gastrointestinal bleeding (MEN2) […] Hepatomegaly (MEN2).

• #24 Multiple endocrine neoplasia type 1 | Endocrine Conditions

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

  Insulinomas occur in about 10% of patients with MEN1. They are tumours of the beta cells in the pancreas and by overproducing insulin they cause a low blood sugar level (hypoglycaemia). The symptoms are typically sweating, shakiness, hunger, dizziness and anxiety but can progress to confusion and even loss of consciousness. […] Some of the pancreatic tumours in MEN1 are non-functioning (non-functioning pancreatic NETs) and are only found by surveillance imaging. Pancreatic NETs in MEN1 are often multiple and can be malignant; they represent the commonest cause of death for patients with MEN1. Non-functioning pancreatic NETs are the most common pancreatic NET in MEN1 and probably carry the greatest risk of malignancy. […] Manifestations of MEN1 can vary greatly, even within families, and tumours can manifest from the first decade of life. Life-long biochemical and radiological screening should be offered to all MEN1 patients on the grounds that detection and treatment of early disease may improve prognosis and life expectancy. Patients should be cared for in a specialist centre under the guidance of an experienced multidisciplinary team. Outlook is dependent upon which tumours a patient may develop, and what treatments are recommended. Some pancreatic NETs are malignant, which can be life-limiting. Overall, MEN1 has a reduced life expectancy.

• #25 MEN Syndromes | Endocrinology and Diabetes

  https://health.ucdavis.edu/conditions/multiple-endocrine-neoplasia-syndromes

  MEN syndrome symptoms depend on the type and affected glands. Some mild symptoms can be easy to dismiss. […] Signs you have a MEN syndrome include: […] Digestive problems, including constipation, diarrhea, stomach pain, reflux and nausea […] Fatigue and muscle weakness […] Increased thirst and urination […] Joint and bone pain […] Kidney (renal) stones […] Muscle weakness […] Swollen hands and feet. […] Certain MEN syndromes can cause dangerously high blood pressure or extremely low blood sugar. Seek immediate care if you experience: […] Confusion […] Heart palpitations […] Profuse sweating […] Shakiness […] Vision changes.

• #26 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

  Pituitary tumors occur in 15 to 42% of patients with MEN 1. From 25 to 90% are prolactinomas. About 25% of pituitary tumors secrete growth hormone or growth hormone and prolactin. Excess prolactin may cause galactorrhea in affected women, and excess growth hormone causes acromegaly clinically indistinguishable from sporadically occurring acromegaly. […] Local tumor expansion may cause visual disturbance, headache, and hypopituitarism. […] Carcinoid tumors, particularly those derived from the embryologic foregut, occur in 5 to 15% of patients with MEN 1. Adrenal adenomas occur in up to 33% of patients and may be bilateral. […] Multiple subcutaneous and visceral lipomas, angiofibromas, meningiomas, ependymomas, and collagenomas may also occur. […] 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, suggesting that medical care should also address and treat the psychological burden of this condition.

• #27 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

  Pancreatic neuroendocrine tumors (pNETs) are estimated to occur in 30–80% of patients with MEN1, and in up to 80–100% of patients in postmortem studies. […] Gastrinoma is the most common functional enteropancreatic tumor in MEN1 and may lead to ZES (hypergastrinemia with recurrent peptic ulcerations), with ZES being the initial clinical manifestation in up to 40% of patients with MEN1. […] Insulinomas are the second most common type of functional enteropancreatic neoplasm in patients with MEN1, representing 10–30% of pNETs in this group. […] The prevalence of anterior pituitary tumors in patients with MEN1 varies widely among series between 10 and 60%, and was noted to be 42% and 38.1% in two large cohorts. […] Several other neoplasms occur with increased frequency in MEN1 including foregut carcinoid tumors, adrenocortical tumors, cutaneous tumors, meningiomas and ependymomas, tumors of smooth muscle, and very rarely pheochromocytomas. […] The most recent Endocrine Society clinical practice guidelines for MEN1 recommend a comprehensive surveillance scheme commencing at the age of 5, with the goal of early detection and management of MEN1-associated manifestations and tumors.

• #28 Multiple Endocrine Neoplasia Type 1 (MEN1) Clinical Presentation: History and Physical Examination

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

  Clinical manifestations of anterior pituitary tumors are similar to those of sporadic pituitary adenomas and depend on hormone secretion and tumor size. Mass effects from tumor growth include headache and visual-field defects. Hormonal effects may include the following: Prolactinomas may cause erectile dysfunction or decreased libido in men, while women may develop amenorrhea and galactorrhea. […] Growth hormone-secreting tumors can cause acromegaly and result in enlargement of the hands, feet, and jaw; this last can manifest with increased drooling and larger spaces between teeth. […] Corticotropin-producing tumors cause excess cortisol secretion; patients can present with large, purplish abdominal stretch marks; increased bruising; and proximal muscle weakness.

• #29 Multiple Endocrine Neoplasia | MD Anderson Cancer Center

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

  MEN1 tends to cause tumors in the following parts of the body: […] Parathyroid gland: Almost all people with MEN1 develop parathyroid gland tumors. These are usually the first glands affected by MEN1. The four parathyroid glands are near the thyroid gland in the front of the neck. MEN1 tumors may cause them to make too much parathyroid hormone (PTH). This is called hyperparathyroidism, and it leads to high levels of calcium in the blood. This is called hypercalcemia. If hypercalcemia is not treated, you may develop kidney stones or kidney damage, and your bones may become thin. […] Pituitary gland: MEN1 can cause benign (non-cancerous) tumors in the front part of the pituitary gland. The most common is prolactinoma. People with MEN1 can develop other pituitary tumors that do not make hormones or that secrete other hormones such as growth hormone, adrenocorticotropin hormone and thyroid stimulating hormone. Symptoms of a pituitary tumor are usually due to the tumor pressing on other nearby structures and can include headaches and changes in vision.

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

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

  The incidence of pituitary adenomas MEN1 patients varies from 15 to 90%. Generally, symptoms depend on the level of pituitary hormone produced and/or compression effects due to size of the tumour. […] Prolactinomas induce galactorrhoea, amenorrhoea and infertility in women, and hypogonadism, sexual dysfunction and, more rarely, gynecomastia in men.

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

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

  Pancreatic tumours occur in about 30-80% of MEN1 patients and are the second most frequently expressed clinical manifestation of MEN1. They are characterised by multiple nodular lesions developed at an early age. […] The majority of these tumours produce excessive amounts of hormone (gastrin, insulin, glucagons, somatostatin, neurotensin or vasoactive intestinal polypeptide (VIP)) and are associated with distinct clinical syndromes. […] Approximately 40% of MEN1 patients have gastrinoma that manifests as Zollinger-Ellison syndrome (ZES) which usually occurs before age 40 years (about one decade earlier than sporadic gastrinomas). ZES may lead to upper abdominal pain, diarrhoea, oesophageal reflux, vomiting and acid-peptic or duodenal ulcers and, more rarely, heartburn and weight loss. […] These insulin-secreting tumours arise in about 10% of MEN1 patients, often in association with gastrinomas, and have approximately one decade earlier onset than that of sporadic insulinomas.

• #31 Multiple Endocrine Neoplasia | Tampa General Hospital

  https://www.tgh.org/institutes-and-services/conditions/multiple-endocrine-neoplasia

  Prolactinomas (tumors that produce prolactin) can cause headaches, vision changes, nausea, vomiting, infertility and reduced interest in sex. For men, they can also cause erectile dysfunction, and for women, they can also cause galactorrhea (milky nipple discharge) and menstrual changes (e.g., amenorrhea or irregular periods).

• #32 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Multiple-Endocrine-Neoplasia-Type-1-(MEN1).aspx

  There is a wide range of symptoms that may affect a patient with MEN1, depending on the endocrine that is affected and the changes in the production of hormones. Increases in the following hormones may cause these symptoms: […] Prolactin: increased production of milk by the breast, amenorrhea in women, reduced testosterone levels in men […] Growth hormone: increased growth of the jaw and other tissues […] Adrenocorticotropic hormone: increased production of cortisol […] Gastrin: increased risk of stomach ulcers […] Glucagon: increased risk of diabetes mellitus […] Parathyroid hormone: increased risk of hypercalcemia and kidney stones […] Vasoactive intestinal peptide: severe diarrhea.

• #33 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/

  The collection of symptoms associated with gastrinomas is called Zollinger-Ellison syndrome. […] The pituitary gland develops a tumour in about one in four people with MEN1. […] High prolactin levels can cause excessive production of breast milk or interfere with fertility in women or with sex drive and fertility in men.

• #34 Multiple endocrine neoplasia type 1 – Wikipedia

  https://en.wikipedia.org/wiki/Multiple_endocrine_neoplasia_type_1

  Pituitary tumors occur in 15 to 42% of MEN 1 patients. From 25 to 90% are prolactinomas. About 25% of pituitary tumors secrete growth hormone or growth hormone and prolactin. Excess prolactin may cause galactorrhea, and excess growth hormone causes acromegaly clinically indistinguishable from sporadically occurring acromegaly. About 3% of tumors secrete ACTH, producing Cushing’s disease. Most of the remainder are nonfunctional. Local tumor expansion may cause visual disturbance, headache, and hypopituitarism. Pituitary tumors in MEN 1 patients appear to be larger and behave more aggressively than sporadic pituitary tumors. […] Overactivity of the parathyroid gland (hyperparathyroidism) is the most common sign of this disorder. Hyperparathyroidism disrupts the normal balance of calcium in the blood, which can lead to kidney stones, thinning of the bones (osteoporosis), high blood pressure (hypertension), loss of appetite, nausea, weakness, fatigue, and depression.

• #35

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

  About 40% of people with MEN1 develop tumours in the pancreas, duodenum (small intestine), or other parts of the digestive tract. Many different types of tumours may develop at the same time. Many of these tumours produce hormones while others do not. Some tumours may be cancerous. […] In people with MEN1, the two most common tumours of the digestive tract are: gastrinomas and insulinomas. […] Nearly one in three people with MEN1 develop tumours in the front part of the pituitary gland called the anterior lobe. […] In people with MEN1, the two most common pituitary tumours are: prolactinomas and growth hormone (GH) tumours. […] Health problems caused by excessive GH include hypertension, diabetes, heart disease, stroke, arthritis, carpal tunnel syndrome and tumours of the colon or rectum. […] While individuals with MEN1 have an increased risk of the tumours listed above, it does not mean that they will definitely develop tumours associated with MEN1.

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

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

  MEN1-gastrinomas usually include a malignant component and in approximately 50% of subjects with MEN1 has metastasized before diagnosis. […] MEN1-insulinomas are almost always benign. […] They are frequent in MEN1 syndrome. […] The presence of a meningioma has been reported in 8% of 74 individuals with MEN1; the meningiomas were mainly asymptomatic and 60% showed no growth. […] 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. […] Individuals with MEN1 syndrome have a significantly increased risk of premature death, justifying surveillance of those with MEN1 mutations and/or a family history of MEN1 syndrome. […] Longer life expectancy in MEN1 syndrome is likely to result in a rising cumulative morbidity and mortality from MEN1 syndrome-associated malignancies, which currently account for approximately 30% of deaths in MEN1 syndrome.

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

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

  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.

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

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

  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.

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

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

  People with MEN type 1 develop tumors in multiple glands of their endocrine system. The most common affected areas include: […] Most tumors associated with MEN type 1 are benign (noncancerous), but some tumors can be cancerous (malignant) and can spread to other areas of your body (metastasize). […] Endocrine glands that are affected by tumors usually release excessive amounts of hormones into your bloodstream, which can result in a variety of symptoms and health issues. […] Since people with MEN type 1 can develop several different tumors, symptoms can vary widely from person to person even within members of the same family and identical twins. In addition, people with MEN type 1 can develop tumors and symptoms at different ages, and symptoms can range from none (asymptomatic) or mild to severe and life-threatening.

• #40

  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. Patients with MEN1 have a high risk for primary hyperparathyroidism (PHPT) with a penetrance of nearly 100%, pituitary adenomas (PitAd) in 40% of patients, and neuroendocrine neoplasms (NEN) of the pancreas (40% of patients), duodenum, lung, and thymus. […] Overall penetrance is 98% by age 80, with 95% of the patients developing PHPT, 41% PNEN, and 30% PitAd. The median age at diagnosis is 31 years (range 10-65) for PHPT, 41 (13-73) for DP-NEN, and 28 (14-60) for PitAd. […] Mortality is MEN1-related in 70% of cases, primarily due to thymic and duodenopancreatic NEN (DP-NEN). […] PHPT is the most common manifestation in MEN1 patients, reaching a penetrance of almost 100%, with age at diagnosis as early as 10 years of age but typically at an older age, as the median age at presentation is 31 years.

• #41 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

  Primary hyperparathyroidism (PHPT), due to parathyroid hyperplasia and/or adenoma, is the most common and often earliest endocrine manifestation in MEN1 and occurs primarily in the third decade of life with 100% penetrance by the age of 50. […] MEN1-associated neoplasms were diagnosed as early as within the first 5 years of life, though the majority were diagnosed after the age of 10 with increasing disease penetrance with age. […] Despite advances in the diagnosis and treatment of MEN1-associated tumors, patients with this syndrome continue to have a decreased life expectancy compared to the rest of the population, with a mean age of death of 55–60 years. […] The most common cause of death has shifted from the complications of hormone-excess states, primarily due to gastrinomas, to malignant NETs, most notably pNETs and th-NETs.

• #42 Multiple Endocrine Neoplasia Type 1 | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/multiple-endocrine-neoplasia-type-1

  Multiple endocrine neoplasia type 1 (MEN1) is a genetic condition that causes benign and malignant tumors in endocrine (hormone producing) and non-endocrine tissues. It occurs in approximately 1 in 30,000 individuals. […] Individuals with MEN1 are at increased risk to develop endocrine and non-endocrine tumors. These tumors are often benign (non-cancerous); however, they may require removal or treatment as they can cause symptoms due to pressure on nearby organs or excess hormone secretion. […] Almost all individuals with a germline MEN1 mutation will develop features of the condition. Approximately 50 percent of individuals with MEN1 will develop features associated with MEN1 by age 20, and more than 95 percent of individuals with MEN1 will develop features by age 50.

• #43 Multiple endocrine neoplasia – Wikipedia

  https://en.wikipedia.org/wiki/Multiple_endocrine_neoplasia

  Multiple Endocrine Neoplasia type 1 (MEN1) is a rare hereditary endocrine cancer syndrome characterized primarily by tumors of the parathyroid glands (95% of cases), endocrine gastroenteropancreatic (GEP) tract (30-80% of cases), and anterior pituitary (15-90% of cases). […] 50% of patients develop signs and symptoms by 20 years of age and more than 95% have symptoms by 40 years of age. There is significant intra- and inter-familial variability in the age of onset, the severity of disease, and tumor types. […] Many endocrine tumors in MEN1 are benign and cause symptoms by overproduction of hormones or local mass effects, while other MEN1 tumors are associated with an elevated risk for malignancy. About one-third of patients affected with MEN1 will die early from MEN1-related cancer or associated malignancy. Entero-pancreatic gastrinomas and thymic and bronchial carcinoids are the leading cause of morbidity and mortality. Consequently, the average age of death in untreated individuals with MEN1 is significantly lower (55.4 years for men and 46.8 years for women) than that of the general population.

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

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

  Multiple endocrine neoplasia type 1 (MEN1) is an inherited condition that leads to growth of mainly benign (non-cancerous) tumours of the endocrine glands. The features of MEN1 vary depending on the types of tumours that occur, and the hormones produced. Features usually present in adulthood; manifestation before teenage years is very rare. The most common presenting feature of MEN1 is hyperparathyroidism, with raised calcium levels owing to growth of the parathyroid glands. Onset of hyperparathyroidism usually occurs after the age of 20. Over 90% of patients with MEN1 will develop hyperparathyroidism by the age of 50. Growths in the parathyroid gland result in hyperparathyroidism (raised parathyroid hormone (PTH) levels) and hypercalcaemia. Hypercalcaemia can lead a wide range of symptoms including thirst, lethargy, aches and pains, muscle weakness and constipation. If left untreated, the long-term effects of hypercalcaemia can include osteoporosis and renal stones. Around 30% of patients affected by MEN1 will develop a pituitary tumour. The effect of the tumour will depend on which hormones are produced. Tumours may secrete prolactin (causing infertility issues), growth hormone (causing the size of the jawbone, hands and feet to grow) and ACTH (causing the adrenal gland to overproduce cortisol). Some tumours may affect vision through compression of the optic nerve. Regular surveillance is needed to screen for MEN1-associated tumours and their hormonal effects. Screening may include a medical review for assessment of symptoms, biochemical screening to check the level of PTH /calcium and other hormones, and imaging of the pituitary/pancreatic gland.

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

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

  Hyperparathyroidism is the presenting feature of MEN1 in about 80% of patients. […] Pancreatic endocrine tumours usually present between the ages of 15 and 50 if not identified by screening. […] Pancreatic endocrine tumours, particularly gastrinomas, become malignant in about half of patients with MEN1. […] The average age of death in individuals with MEN1 is significantly lower (55.4 years for men and 46.8 years for women) than that of the general population. […] Untreated, patients may die from peptic ulcer disease, metastatic endocrine pancreatic carcinoma, or foregut carcinoid malignancy.

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

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

  Malignant progression of parathyroid tumors is not a frequent feature of MEN1 syndrome, even if some reports described the presence of parathyroid carcinoma in patients with MEN1. […] Pituitary tumors may occur as the first clinical manifestation of MEN1 syndrome in 25% of simplex and in 10% of familial cases. […] The occurrence of anterior pituitary tumors in MEN1 syndrome may range between 10 and 60% depending on the study. […] Symptoms such as nerve compression, headache, and hypopituitarism may also result from pituitary mass effects. […] Although the MEN1 French-Belgian Registry reported that 32% of pituitary macroadenomas were locally invasive, malignant degeneration of MEN1-associated pituitary tumors is an infrequent event. […] Approximately 40% of individuals with MEN1 syndrome have gastrinoma(s), which manifests as ZES. Clinical findings can include upper abdominal pain, diarrhea, esophageal reflux, and acid-peptic ulcers; if not properly diagnosed or treated, as occurred in the past, ulcer perforation can develop from hypergastrinemia, even without prior symptoms.

• #47 Multiple endocrine neoplasia type 1 – Wikipedia

  https://en.wikipedia.org/wiki/Multiple_endocrine_neoplasia_type_1

  Multiple endocrine neoplasia type 1 (MEN-1 aka Wermer Syndrome) is one of a group of disorders, the multiple endocrine neoplasias, that affect the endocrine system through development of neoplastic lesions in pituitary, parathyroid gland and pancreas. Individuals suffering from this disorder are prone to developing multiple endocrine and nonendocrine tumors. […] Hyperparathyroidism is present in 90% of patients. Asymptomatic hypercalcemia is the most common manifestation: about 25% of patients have evidence of nephrolithiasis or nephrocalcinosis. In contrast to sporadic cases of hyperparathyroidism, diffuse hyperplasia or multiple adenomas are more common than solitary adenomas. […] Pancreatic islet cell tumors are today the major cause of death in persons with MEN-1. Tumors occur in 60-80% of persons with MEN-1 and they are usually multicentric. Multiple adenomas or diffuse islet cell hyperplasia commonly occurs. About 30% of tumors are malignant and have local or distant metastases. About 10-15% of islet cell tumors originate from a -cell, secrete insulin (insulinoma), and can cause fasting hypoglycemia. -cell tumors are more common in patients 40 years of age.

• #48 Multiple endocrine neoplasia type 1 (MEN I; endocrine adenomatosis; Wermer’s syndrome) | Healthengine Blog

  https://healthinfo.healthengine.com.au/multiple-endocrine-neoplasia-type-1-endocrine-adenomatosis-or-wermers-syndrome

  Multiple Endocrine Adenomatosis or Wermers syndrome is a disorder of the endocrine glands; the parathyroids are the earliest and most often affected by MEN1. […] In patients with MEN1, sometimes more than one group of endocrine glands, such as the parathyroid, the pancreas, and the pituitary become overactive at the same time. Most people who develop overactivity of only one endocrine gland do not have MEN1. In MEN1, all four parathyroid glands tend to be overactive. They release too much parathyroid hormone, leading to excess calcium in the blood. […] Gastrin is another hormone that can be oversecreted in people with MEN1. The gastrin comes from one or more tumors in the pancreas and small intestine. Gastrin normally circulates in the blood, causing the stomach to secrete enough acid needed for digestion. If exposed to too much gastrin, the stomach releases excess acid, leading to the formation of severe ulcers in the stomach and small intestine. Too much gastrin can also cause serious diarrhea. About one in three patients with MEN1 has gastrin-releasing tumors, called gastrinomas. (Zollinger-Ellison syndrome). The pituitary gland becomes overactive in about one of four persons with MEN1. This overactivity can usually be traced to a very small, benign tumor in the gland that releases too much prolactin, called a prolactinoma. High prolactin can cause excessive production of breast milk or it can interfere with fertility in women or with sex drive and fertility in men. […] There is no cure for MEN1 itself, but most of the health problems caused by MEN1 can be recognized at an early stage and controlled or treated before they become serious problems.

• #49 Multiple Endocrine Neoplasia | MD Anderson Cancer Center

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

  Prolactinomas can interfere with sexual function and fertility, and tumors secreting growth hormone over time can cause acromegaly (enlargement of the bones). Adrenocorticotropin-producing tumors can cause Cushing’s syndrome. Pituitary tumors generally respond well to medication; however, in some instances surgical removal of the tumor or radiation is necessary. […] Pancreas: Tumors also may form in the islet cells of the pancreas and the lining of the duodenum (the first portion of the small intestine), which can secrete several hormones involved with endocrine function. Tumors that develop in the pancreas can be benign or malignant. However, malignancy is rare before the age of 30. […] Gastrinomas are the most common functional pancreatic tumor in people with MEN1 and can cause Zollinger-Ellison syndrome (ZES). Symptoms of ZES include elevated levels of gastrin, ulcers, inflammation of the esophagus, diarrhea and abdominal pain.

• #50 Multiple Endocrine Neoplasia | MD Anderson Cancer Center

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

  Multiple endocrine neoplasia type 1 (MEN1), also called multiple endocrine adenomatosis or Wermer’s syndrome, is found in one in 30,000 people. It can affect people of any age, ethnic group or gender. It is caused by mutations in the MEN1 gene, which is a tumor suppressor gene. Mutations of the MEN1 gene „disable” tumor suppression, causing unregulated cell division that leads to tumor formation. All children of a parent with MEN1 have a 50% chance of developing the disease. […] In MEN1, tumors grow in certain glands of the endocrine system. They tend to develop in more than one gland. If you have only one affected endocrine gland, you probably do not have MEN1. […] While these tumors usually are benign, they may cause problems by releasing too much hormone or growing against other parts of the body. However, about half of people with MEN1 will eventually develop cancer.

• #51 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.

• #52 Multiple endocrine neoplasia type 1: Early diagnosis is very important

  https://www.wjgnet.com/1007-9327/full/v31/i6/99613.htm

  In this manuscript, we comment on a recent publication by Yuan et al. This article provides a detailed scientific diagnostic process for a multiple endocrine neoplasia type 1 patient, thus offering strong guidance for clinical practice. However, we believe that the authors should also provide information on the patient’s long-term prognosis. […] Due to its rarity and diverse clinical presentations, the early diagnosis of multiple endocrine neoplasia type 1 (MEN1) is challenging. […] Primary hyperparathyroidism is the most common and first manifestation of MEN1; however, it is often asymptomatic, with elevated serum calcium levels and urinary stones representing important diagnostic clues. GEP-NETs are the second most common clinical manifestation of MEN1, with gastrinomas being the most frequently observed types of GEP-NETs. Gastrinomas in MEN1 are usually small; moreover, in most cases, they are located in the submucosa of the duodenum and often result in gastrointestinal symptoms such as abdominal pain and diarrhoea.

• #53 Multiple endocrine neoplasia type 1 (patient information) – wikidoc

  https://www.wikidoc.org/index.php/Multiple_endocrine_neoplasia_type_1_(patient_information)

  Multiple endocrine neoplasia (MEN) type I is a disease passed down through families, in which one or more of the endocrine glands are overactive or form a tumor. Endocrine glands most commonly involved include pancreas, parathyroid and pituitary. […] What are the symptoms of multiple endocrine neoplasia type 1? Visual disturbance, Headache, Constipation, Nausea, Vomiting, Dehydration, Lethargy, Depression, Confusion, Anorexia. […] Seek urgent medical care if there are symptoms of MEN 1 or its complications. […] MEN1 cannot be cured, but regular testing can detect the problems caused by MEN1 tumors many years before serious complications develop. […] Most people with MEN1 have a long and productive life. Most of the tumors are benign while some pancreatic tumors may become cancerous and lower life expectancy.

• #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

  The diagnosis of MEN1 can be made on the basis of clinical, familial, and/or genetic criteria. […] The degree of hypercalcemia in these individuals is usually mild and patients may be asymptomatic or present with polydipsia, polyuria, constipation, malaise, altered mentation, hypertension, shortened QT interval, peptic ulcer disease, urolithiasis, and/or decreased bone mineral density with increased fracture risk. […] There is a high prevalence of urolithiasis and early bone mineral loss in young individuals with MEN1-associated PHPT, and bone or renal complications are progressively more frequent, extensive, and severe in long-standing PHPT cases and in those associated with gastrinoma. […] The recommended surgical approach for the treatment of MEN1-associated PHPT is subtotal parathyroidectomy with removal of 3.5 parathyroid glands.

• #55 Multiple endocrine neoplasia type 1 in patients with gastroenteropancreatic neuroendocrine tumors: An opportunity for early diagnosis and appropriate management

  https://www.spandidos-publications.com/10.3892/mco.2020.2074

  The long delay before a diagnosis of MEN1 is established has been previously reported in studies from countries including Brazil, Japan, The Netherlands, United States, Greece, and France/Belgium. […] The delay in the diagnosis of an index case may have a serious impact on patient morbidity and prognosis. Undiagnosed PHPT may evolve into a brown tumor and low BMD, with subsequent bone pain and increased risk of fractures. Nephrolithiasis, which is another common manifestation, may cause pain, increased risk of urinary tract infection, and renal dysfunction. […] Prolactinomas and somatotropinomas (acromegaly) are the most common PitNET tumors in the MEN1 context, and they may be associated with visual field defects, cardiovascular morbidity and mortality, notably acromegaly. These manifestations are time-dependent and directly associated with late diagnosis.

• #56 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. […] 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. […] Currently, pNETs are the leading cause of MEN1-related death. […] The high prevalence and malignant potential of pNETs in MEN1 emphasize the need for an evidence-based screening programme at an early stage to enable meticulous follow-up and timely intervention to prevent metastatic disease. […] 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.

• #57 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 results of the study demonstrate that leflunomide may be an effective drug for the treatment of MEN1-mutated tumours. […] Leflunomide also attenuated the spontaneous incidence of pNETS in the MEN1-KO mice, especially when treated early as opposed to its late addition. […] 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. […] 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.

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