Materiały źródłowe

• #1 Pancreatic Neuroendocrine Tumors (PNETs) – Pancreatic Cancer Action Network

  https://pancan.org/facing-pancreatic-cancer/about-pancreatic-cancer/types-of-pancreatic-cancer/endocrine-pancreatic-neuroendocrine-tumors/

  Pancreatic neuroendocrine tumors (pancreatic NETs or PNETs) account for less than 10% of all pancreatic tumors. They develop from the abnormal growth of endocrine (hormone-producing) cells in the pancreas called islet cells. […] About 30-75% of people with MEN1 will develop pancreatic neuroendocrine tumors. Gastrinomas are the most common pancreatic neuroendocrine tumors in individuals with MEN1. The second most common are insulinomas. The tumors in the pancreas may be malignant and generally appear in individuals in their 30s or 40s.

• #2 Pancreatic neuroendocrine tumors: biology, diagnosis, and treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3845620/

  Pancreatic neuroendocrine tumors (PNETs), a group of endocrine tumors arising in the pancreas, are among the most common neuroendocrine tumors. […] The genetic causes of familial and sporadic PNETs are somewhat understood, but their molecular pathogenesis remains unknown. […] The pathogenesis of PNETs is largely unknown but is growing as a research topic. […] Approximately 10% of all PNETs are components of familial endocrine tumor syndromes such as multiple endocrine neoplasia syndrome type 1 (MEN1), von Hippel-Lindau disease (VHL), neurofibromatosis type 1 (NF-1), and tuberous sclerosis (TSC). […] The etiology of PNETs within the context of these familial syndromes is the inherited germline loss of the respective tumor suppressor gene. […] Several studies have been performed on the pathogenesis of sporadic PNETs, which comprise 90% of all PNETs.

• #3 Pancreatic Neuroendocrine Tumor | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions/p/pancreatic-neuroendocrine-tumor.html

  A pancreatic neuroendocrine tumor is a rare type of cancer that starts in the pancreas. […] These tumors are fairly rare. Healthcare providers don’t know why people develop them. […] Experts don’t know what causes them.

• #4 Causes of Neuroendocrine Tumors

  https://www.webmd.com/cancer/neuroendocrine-tumors-causes

  The plain truth is, that experts don’t know exactly what causes neuroendocrine tumors (NETs). But a bunch of things can make you more likely to get them. […] If you have the condition, you may be more likely to get cancers of the parathyroid gland, pituitary gland, and pancreas, including pancreatic NETs. […] If you have MEN2, you’re more likely to get neuroendocrine tumors such as pheochromocytoma, medullary thyroid cancer, and parathyroid tumors. […] If you have NF1 you’re more likely to get NETs such as carcinoid tumors and pheochromocytoma. […] If you have this condition, you’re more likely to get pancreatic NETs or carcinoid tumors. […] Your immune system is your body’s defense against germs. Anything that weakens it, such as HIV or an organ transplant, can raise your risk for NETs. […] Some studies suggest that smoking increases the risk for carcinoid tumors of the small intestine.

• #5

  https://content-dev.irisoncology.com/library/pancreatic-neuroendocrine-tumors

  Pancreatic neuroendocrine tumors happen when cells in the pancreas develop changes in their DNA. A cell’s DNA holds the instructions that tell the cell what to do. The changes, which doctors call mutations, tell the cells to multiply quickly. The changes let the cells continue living when healthy cells would die as part of their natural life cycle. This causes many extra cells. The cells might form a mass called a tumor. Sometimes the cells can break away and spread to other organs, such as the liver. When cancer spreads, it’s called metastatic cancer. […] In pancreatic neuroendocrine tumors, the DNA changes happen in hormone-producing cells called islet cells. It’s not clear what causes the changes that lead to cancer. […] Factors that are associated with an increased risk of pancreatic neuroendocrine tumors include: A family history of pancreatic neuroendocrine tumors. If a family member was diagnosed with pancreatic neuroendocrine tumor, your risk is increased.

• #6 Neuroendocrine tumors – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/neuroendocrine-tumors/symptoms-causes/syc-20354132

  Neuroendocrine tumors are cancers that begin in specialized cells called neuroendocrine cells. […] The exact cause of neuroendocrine tumors isn’t known. These cancers begin in neuroendocrine cells that have traits similar to those of nerve cells and hormone-producing cells. Neuroendocrine tumors begin when neuroendocrine cells develop changes (mutations) in their DNA. The DNA inside a cell contains the instructions that tell the cell what to do. The changes tell the neuroendocrine cells to multiply rapidly and form a tumor. […] Some neuroendocrine tumors grow very slowly. Others are aggressive cancers that invade and destroy normal body tissue or spread (metastasize) to other parts of the body.

• #7 Pancreatic neuroendocrine tumor – Wikipedia

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

  Pancreatic neuroendocrine tumors (PanNETs), often referred to as „islet cell tumours,” are neuroendocrine neoplasms that arise from cells of the endocrine (hormonal) and nervous system within the pancreas. […] Pancreatic neuroendocrine tumors may arise in the context of multiple endocrine neoplasia type 1, Von Hippel-Lindau disease, neurofibromatosis type 1 (NF-1) or tuberose sclerosis (TSC). […] Analysis of somatic DNA mutations in well-differentiated pancreatic neuroendocrine tumors identified four important findings: as expected, the genes mutated in NETs, MEN1, ATRX, DAXX, TSC2, PTEN and PIK3CA, are different from the mutated genes previously found in pancreatic adenocarcinoma. […] One in six well-differentiated pancreatic NETs have mutations in mTOR pathway genes, such as TSC2, PTEN and PIK3CA. […] Mutations affecting a new cancer pathway involving ATRX and DAXX genes were found in about 40% of pancreatic NETs. […] ATRX/DAXX and MEN1 mutations were associated with a better prognosis.

• #8 KEGG DISEASE: Pancreatic neuroendocrine tumor

  https://www.genome.jp/dbget-bin/www_bget?ds:H00045

  Pancreatic neuroendocrine tumors (PNETs), also known as islet cell tumors, are rare neoplasms that arise in the endocrine tissues of the pancreas. They may occur sporadically or in association with a genetic syndrome, such as multiple endocrine neoplasia type 1 (MEN1), Von Hippel-Landau (VHL) syndrome, neurofibromatosis type 1, or tuberous sclerosis. […] The most frequent genetic alterations in PNET occur in MEN1 (Multiple Endocrine Neoplasia-1 Gene), DAXX/ATRX (Death-Domain Associated Protein/Mental Retardation Syndrome X-Linked Genes) and the mTOR pathway (Mammalian Target of Rapamycin). A germline mutation in the MEN1 tumor suppressor gene causes MEN1, the above-mentioned autosomal dominant hereditary syndrome. Mutations of DAXX and ATRX are common and related to altered telomeres but not to prognosis.

• #8 Molecular Basis of Pancreatic Neuroendocrine Tumors

  https://www.mdpi.com/1422-0067/25/20/11017

  The studies addressing the NET genome clearly indicate the existence of a group of non-functioning NETs enriched in MEN1/DAXX/ATRX mutations, structural aberrations, mTOR pathway alterations, positive ALT status, and unfavorable prognosis. […] The clinically-advantageous group is also non-functional, has MEN1 mutations, chromosome 11 LoH, and limited copy-number events. […] The poor prognosis associated with tumors harboring mutations in MEN1/ATRX/DAXX genes is at odds with the findings of certain studies.

• #9 Pancreatic neuroendocrine tumors: biology, diagnosis, and treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3845620/

  Pancreatic neuroendocrine tumors (PNETs), a group of endocrine tumors arising in the pancreas, are among the most common neuroendocrine tumors. […] The genetic causes of familial and sporadic PNETs are somewhat understood, but their molecular pathogenesis remains unknown. […] The pathogenesis of PNETs is largely unknown but is growing as a research topic. […] Approximately 10% of all PNETs are components of familial endocrine tumor syndromes such as multiple endocrine neoplasia syndrome type 1 (MEN1), von Hippel-Lindau disease (VHL), neurofibromatosis type 1 (NF-1), and tuberous sclerosis (TSC). […] The etiology of PNETs within the context of these familial syndromes is the inherited germline loss of the respective tumor suppressor gene. […] Several studies have been performed on the pathogenesis of sporadic PNETs, which comprise 90% of all PNETs.

• #10 Neuroendocrine pancreatic cancer: An overview

  https://www.medicalnewstoday.com/articles/neuroendocrine-pancreatic-cancer

  Neuroendocrine tumors (NETs) are a type of cancerous growth affecting cells that send hormones around the body. Pancreatic neuroendocrine tumors (PNETs) grow in the cells of the pancreas that produce hormones to control blood sugar. […] The causes of PNETs are unclear. Around 90% of PNETs are sporadic, meaning they occur randomly. […] Cancer occurs due to genetic changes. These changes may switch on genes, called oncogenes, that help cells grow, divide, and die. These changes may also turn off tumor suppressor genes that control cell division or repair damaged DNA. […] Certain syndromes related to changes in three tumor suppressor genes may be responsible for inherited cases of PNETs. These syndromes include: multiple endocrine neoplasia type 1 (MEN1) syndrome, with changes in the MEN1 gene causing most cases of inherited PNETs; Von Hippel-Lindau (VHL) syndrome, which typically causes slow-growing, nonfunctioning PNETs at earlier ages; neurofibromatosis type 1 (NF1) syndrome, which mostly causes somatostatinomas, a very rare type of PNET. […] It is also possible to acquire gene changes after birth, sometimes through exposure to chemicals that cause cancer. This may occur through smoking. However, the causes of acquired gene changes are often not clear.

• #11 What Causes Pancreatic Neuroendocrine Tumor? | American Cancer Society

  https://www.cancer.org/cancer/types/pancreatic-neuroendocrine-tumor/causes-risks-prevention/what-causes.html

  Scientists dont know exactly what causes most pancreatic neuroendocrine tumors (pNETs), but they have found several risk factors that can make a person more likely to get this disease. […] Some of these risk factors affect the DNA of cells in the neuroendocrine system in the pancreas, which can result in abnormal cell growth and may cause cancers to form. […] Although 90% of pNETs are sporadic (random), some people inherit gene changes from their parents that raise their risk of pancreatic NET. […] Sometimes these gene changes are part of syndromes that increase risks for other health problems, as well. […] Syndromes related to changes in three tumor suppressor genes are responsible for many inherited cases of pNETs: […] Most gene mutations related to neuroendocrine tumors of the pancreas are random. […] If these random mutations occur after a person is born, they are called acquired. […] These acquired gene mutations sometimes result from exposure to cancer-causing chemicals (like those found in tobacco smoke).

• #12 Genetic Causes of Neuroendocrine Tumors by Dr. Run Yu – Carcinoid Cancer Foundation

  https://www.carcinoid.org/genetic-causes-neuroendocrine-tumors-dr-run-yu/

  A common question that patients with neuroendocrine tumors (NETs) ask is whether their NETs are caused by inheriting a defective gene. […] Most patients with pancreatic NETs do not have a clear genetic cause. In a recent study of ours, only about 5% of the patients with pancreatic NETs have a clear genetic cause. The most common genetic syndrome that causes pancreatic NETs is the multiple endocrine neoplasia type 1 (MEN1). […] The second most common genetic syndrome is the von Hippel-Lindau disease (VHL). […] Two other genetic syndromes, neurofibromatosis type 1 (NF1) and tuberous sclerosis complex (TSC) occasionally cause pancreatic NETs. […] The fifth genetic syndrome, Mahvash disease, which we discovered several years ago based on our clinical practice, causes pancreatic NETs and very high glucagon levels.

• #13 Inherited syndromes involving pancreatic neuroendocrine tumors – Geurts – Journal of Gastrointestinal Oncology

  https://jgo.amegroups.org/article/view/39073/html

  Inherited syndromes are important to recognize in the setting of a pancreatic neuroendocrine tumor (PNET) as there are significant implications for the patients medical management and opportunity for early detection of subsequent manifestations. […] Although most PNETs are sporadic, approximately 10% are due to an inherited syndrome, which include multiple endocrine neoplasia type 1 (MEN1), multiple endocrine neoplasia type 4 (MEN4), von Hippel-Lindau disease (VHL), neurofibromatosis type 1 (NF1), and tuberous sclerosis complex (TSC). […] The unique aspects to management, challenges in hereditary disease recognition and accurate diagnosis, and rarity of these syndromes are all reasons to support referral to high-volume centers with the experience and knowledge to treat patients with hereditary endocrine neoplasia syndromes.

• #14

  https://content-dev.irisoncology.com/library/pancreatic-neuroendocrine-tumors

  Syndromes present at birth that increase the risk of tumors. Some syndromes that are passed from parents to children can increase the risk of pancreatic neuroendocrine tumors. Examples of these include multiple endocrine neoplasia, type 1 (MEN 1), von Hippel-Lindau (VHL) disease, neurofibromatosis 1 (NF1) and tuberous sclerosis. These inherited syndromes are caused by changes in the DNA. These changes allow cells to grow and divide more than needed. […] There’s no way to prevent pancreatic neuroendocrine tumors. If you develop this type of cancer, you didn’t do anything to cause it.

• #15 Pancreatic Neuroendocrine Tumors (PNETs) – Pancreatic Cancer Action Network

  https://pancan.org/facing-pancreatic-cancer/about-pancreatic-cancer/types-of-pancreatic-cancer/endocrine-pancreatic-neuroendocrine-tumors/

  Pancreatic neuroendocrine tumors (pancreatic NETs or PNETs) account for less than 10% of all pancreatic tumors. They develop from the abnormal growth of endocrine (hormone-producing) cells in the pancreas called islet cells. […] About 30-75% of people with MEN1 will develop pancreatic neuroendocrine tumors. Gastrinomas are the most common pancreatic neuroendocrine tumors in individuals with MEN1. The second most common are insulinomas. The tumors in the pancreas may be malignant and generally appear in individuals in their 30s or 40s.

• #16 Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/pancreatic/hp/pnet-treatment-pdq

  Most pancreatic NETs are sporadic, but some occur as part of the autosomal dominant multiple endocrine neoplasia type 1 (MEN1) inherited syndrome. This syndrome consists of tumors of the anterior pituitary, parathyroid, and endocrine pancreas glands and is a result of inactivation of the MEN1 tumor suppressor gene located on chromosome 11q13. […] In patients with MEN1 in which 85% have pancreatic islet cell tumors, 90% have hyperparathyroidism, and 65% have pituitary tumors, and they are less likely to be cured by pancreatic resection than are patients with sporadic islet cell tumors. […] Most islet cell cancers are functional, but about 15% are nonfunctional, with presentations similar to the far more common exocrine adenocarcinomas of the pancreas. […] Nonfunctional tumors tend to present at later clinical stages with symptoms attributable to mass effect or metastases.

• #17 Inherited syndromes involving pancreatic neuroendocrine tumors – Geurts – Journal of Gastrointestinal Oncology

  https://jgo.amegroups.org/article/view/39073/html

  The current genetic testing technologies, including MEN1 gene sequencing and deletion/duplication analysis, identify a pathogenic variant in approximately 90% of familial cases and 65% in apparently sporadic instances of MEN1 syndrome. […] Genetic testing is highly reliable and should be offered to individuals meeting diagnostic criteria. […] The prevalence of PNET in individuals with VHL syndrome ranges from 9% to 17% and the tumors are most often nonfunctional and may be numerous. […] Risk factors for PNET metastasis in VHL syndrome include: greatest tumor diameter 3 cm, blood type O, tumor doubling time 500 days, and pathogenic missense variants or any pathogenic variant in exon 3 of the VHL gene. […] PNETs in the setting of VHL syndrome typically have a better prognosis compared to sporadic PNETs.

• #18 Neuroendocrine pancreatic cancer: An overview

  https://www.medicalnewstoday.com/articles/neuroendocrine-pancreatic-cancer

  Neuroendocrine tumors (NETs) are a type of cancerous growth affecting cells that send hormones around the body. Pancreatic neuroendocrine tumors (PNETs) grow in the cells of the pancreas that produce hormones to control blood sugar. […] The causes of PNETs are unclear. Around 90% of PNETs are sporadic, meaning they occur randomly. […] Cancer occurs due to genetic changes. These changes may switch on genes, called oncogenes, that help cells grow, divide, and die. These changes may also turn off tumor suppressor genes that control cell division or repair damaged DNA. […] Certain syndromes related to changes in three tumor suppressor genes may be responsible for inherited cases of PNETs. These syndromes include: multiple endocrine neoplasia type 1 (MEN1) syndrome, with changes in the MEN1 gene causing most cases of inherited PNETs; Von Hippel-Lindau (VHL) syndrome, which typically causes slow-growing, nonfunctioning PNETs at earlier ages; neurofibromatosis type 1 (NF1) syndrome, which mostly causes somatostatinomas, a very rare type of PNET. […] It is also possible to acquire gene changes after birth, sometimes through exposure to chemicals that cause cancer. This may occur through smoking. However, the causes of acquired gene changes are often not clear.

• #19 Inherited syndromes involving pancreatic neuroendocrine tumors – Geurts – Journal of Gastrointestinal Oncology

  https://jgo.amegroups.org/article/view/39073/html

  Less commonly, pancreatic involvement in neurofibromatosis (NF1) and tuberous sclerosis complex (TSC) may be observed. […] GEP-NET tumors may be reported in up to 10% of patients with NF1 syndrome, most frequently nonfunctioning somatostatinomas arising from the duodenum where there is risk for obstruction. […] Both functional and nonfunctional PNETs are a rare manifestation, involved in only 1% of cases. […] The discoveries and increased knowledge from our understanding of hereditary endocrine neoplasia syndromes provide a model to help scientists and clinicians have insight into the pathogenesis of sporadic PNETs as well.

• #20 Genetic Causes of Neuroendocrine Tumors by Dr. Run Yu – Carcinoid Cancer Foundation

  https://www.carcinoid.org/genetic-causes-neuroendocrine-tumors-dr-run-yu/

  A common question that patients with neuroendocrine tumors (NETs) ask is whether their NETs are caused by inheriting a defective gene. […] Most patients with pancreatic NETs do not have a clear genetic cause. In a recent study of ours, only about 5% of the patients with pancreatic NETs have a clear genetic cause. The most common genetic syndrome that causes pancreatic NETs is the multiple endocrine neoplasia type 1 (MEN1). […] The second most common genetic syndrome is the von Hippel-Lindau disease (VHL). […] Two other genetic syndromes, neurofibromatosis type 1 (NF1) and tuberous sclerosis complex (TSC) occasionally cause pancreatic NETs. […] The fifth genetic syndrome, Mahvash disease, which we discovered several years ago based on our clinical practice, causes pancreatic NETs and very high glucagon levels.

• #21

• #22 Pancreatic Neuroendocrine Tumor Risk Factors | American Cancer Society

  https://www.cancer.org/cancer/types/pancreatic-neuroendocrine-tumor/causes-risks-prevention/risk-factors.html

  Several factors can affect a persons chance of getting a pancreatic neuroendocrine tumor (pNET). […] Smoking comes with health risks, including an increased risk for pNETs. Most research shows that heavy smoking increases risk, but some studies show that any history of smoking could put you at risk. […] Some studies have shown a link between heavy alcohol use and pNETs. This link appears to be mostly related to functioning pNETs rather than non-functioning pNETs. Heavy alcohol use can also lead to conditions such as chronic pancreatitis, which may increase pNET risk. […] PNETs seem to run in some families. In some, the high risk is caused by an inherited syndrome. In other families, the gene causing the increased risk is not known. If family history is a risk factor, it usually involves a first-degree relative (parent, sibling, child), a family history of pNET, or a family history of any cancer.

• #23 Pancreatic Neuroendocrine Tumor Risk Factors | American Cancer Society

  https://www.cancer.org/cancer/types/pancreatic-neuroendocrine-tumor/causes-risks-prevention/risk-factors.html

  Several factors can affect a persons chance of getting a pancreatic neuroendocrine tumor (pNET). […] Smoking comes with health risks, including an increased risk for pNETs. Most research shows that heavy smoking increases risk, but some studies show that any history of smoking could put you at risk. […] Some studies have shown a link between heavy alcohol use and pNETs. This link appears to be mostly related to functioning pNETs rather than non-functioning pNETs. Heavy alcohol use can also lead to conditions such as chronic pancreatitis, which may increase pNET risk. […] PNETs seem to run in some families. In some, the high risk is caused by an inherited syndrome. In other families, the gene causing the increased risk is not known. If family history is a risk factor, it usually involves a first-degree relative (parent, sibling, child), a family history of pNET, or a family history of any cancer.

• #24 Pancreatic Neuroendocrine Tumor: Causes, Symptoms, and More

  https://resources.healthgrades.com/right-care/cancer/neuroendocrine-pancreatic-cancer

  Researchers have not yet discovered what causes neuroendocrine pancreatic cancer. However, they have identified several factors that can increase your risk. […] Certain lifestyle changes may decrease your risk of developing pancreatic NETs. However, you may inherit certain risk factors that can’t be changed. The risk factors for pancreatic NETs include: Alcohol: Heavy alcohol consumption can damage your pancreas and increase your risk. Smoking: Research shows that a history of smoking can increase your risk. […] Family history: Having a first-degree relative — such as a parent, sibling, or child — with neuroendocrine pancreatic cancer can increase your risk. Inherited genetic syndromes: Inherited genetic syndromes can increase your risk, such as: neurofibromatosis type 1, multiple endocrine neoplasia type 1 (MEN1), Von Hippel-Lindau (VHL) syndrome. […] Chronic pancreatitis: Long-term inflammation of the pancreas can increase your risk. Diabetes: Pancreatic NETs are more common in people with diabetes.

• #25 Pancreatic Neuroendocrine Tumor Risk Factors | American Cancer Society

  https://www.cancer.org/cancer/types/pancreatic-neuroendocrine-tumor/causes-risks-prevention/risk-factors.html

  Several factors can affect a persons chance of getting a pancreatic neuroendocrine tumor (pNET). […] Smoking comes with health risks, including an increased risk for pNETs. Most research shows that heavy smoking increases risk, but some studies show that any history of smoking could put you at risk. […] Some studies have shown a link between heavy alcohol use and pNETs. This link appears to be mostly related to functioning pNETs rather than non-functioning pNETs. Heavy alcohol use can also lead to conditions such as chronic pancreatitis, which may increase pNET risk. […] PNETs seem to run in some families. In some, the high risk is caused by an inherited syndrome. In other families, the gene causing the increased risk is not known. If family history is a risk factor, it usually involves a first-degree relative (parent, sibling, child), a family history of pNET, or a family history of any cancer.

• #26

• #27 Pancreatic Neuroendocrine Tumor Risk Factors | American Cancer Society

  https://www.cancer.org/cancer/types/pancreatic-neuroendocrine-tumor/causes-risks-prevention/risk-factors.html

  Inherited gene changes (mutations) can be passed from parent to child. Sometimes these changes result in syndromes that increase risks for other cancers (or other health problems). […] PNETs are more common in people with diabetes. The reason for this is not known. Most of the risk is found in people with type 2 diabetes. […] Chronic pancreatitis, a long-term inflammation of the pancreas, is linked with an increased risk of pancreatic NETs. […] Excess weight or obesity could be a risk factor for pNET. Studies so far are inconclusive. […] Some older studies have suggested that drinking coffee might increase the risk of pNET, but more recent studies have not confirmed this.

• #28 Risks and causes of neuroendocrine cancer| Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/risks-causes

  We dont know what causes most neuroendocrine cancers. […] The possible risks and causes of NETs and NECs are different. […] Most neuroendocrine cancers are not inherited. […] But there are some rare inherited conditions that can run in families. These can increase your risk of developing some types of NET. […] People with MEN1 have a higher risk of developing NETs in the pancreas. […] VHL is a rare inherited condition caused by a change in the von Hippel-Lindau gene. It can affect different parts of the body. People with VHL have an increased risk of developing different types of tumours. These include pancreatic NETs. […] Researchers think that people with tuberous sclerosis may have a higher risk of developing a pancreatic NET. […] There is some evidence that people with diabetes have an increased risk of developing a pancreatic NET. […] Some stomach NETs are linked to a condition called atrophic gastritis. This is a long term condition that causes inflammation of the stomach. Research has shown that there is a link between atrophic gastritis and type 1 stomach NET.

• #29 Pancreatic Neuroendocrine Tumor Risk Factors | American Cancer Society

  https://www.cancer.org/cancer/types/pancreatic-neuroendocrine-tumor/causes-risks-prevention/risk-factors.html

  Inherited gene changes (mutations) can be passed from parent to child. Sometimes these changes result in syndromes that increase risks for other cancers (or other health problems). […] PNETs are more common in people with diabetes. The reason for this is not known. Most of the risk is found in people with type 2 diabetes. […] Chronic pancreatitis, a long-term inflammation of the pancreas, is linked with an increased risk of pancreatic NETs. […] Excess weight or obesity could be a risk factor for pNET. Studies so far are inconclusive. […] Some older studies have suggested that drinking coffee might increase the risk of pNET, but more recent studies have not confirmed this.

• #30

  https://www.iowaclinic.com/specialties/surgery/general-surgery/pancreatic-surgery/net/

  The exact cause of neuroendocrine tumors (NETs) is not always clear, but several factors may contribute to their development, including: […] Genetic mutations or inherited syndromes such as multiple endocrine neoplasia type 1 (MEN1) or neurofibromatosis type 1 (NF1). […] Chronic inflammation of the pancreas, such as in cases of chronic pancreatitis, may increase the risk of developing pancreatic NETs.

• #31

• #32 How Rare Are Pancreatic Neuroendocrine Tumors? Symptoms, Causes

  https://www.medicinenet.com/how_rare_are_pancreatic_neuroendocrine_tumors/article.htm

  Pancreatic neuroendocrine tumors arise when cells in the endocrine part of the pancreas grow out of control. […] The exact cause of pancreatic neuroendocrine tumors is unknown. […] There are no known environmental or lifestyle factors that may cause them. […] Certain inherited conditions, however, may be associated with these tumors. […] They include multiple endocrine neoplasia type I (MEN1), neurofibromatosis type I (NF-1), tuberous sclerosis, and von Hippel-Lindau syndrome (VHL). […] Pancreatic neuroendocrine tumors are more common in males than in females. […] Having a family history of pancreatic neuroendocrine tumors may increase your risk of having these tumors.

• #33 Pancreatic neuroendocrine tumors: biology, diagnosis, and treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3845620/

  Pancreatic neuroendocrine tumors (PNETs), a group of endocrine tumors arising in the pancreas, are among the most common neuroendocrine tumors. […] The genetic causes of familial and sporadic PNETs are somewhat understood, but their molecular pathogenesis remains unknown. […] The pathogenesis of PNETs is largely unknown but is growing as a research topic. […] Approximately 10% of all PNETs are components of familial endocrine tumor syndromes such as multiple endocrine neoplasia syndrome type 1 (MEN1), von Hippel-Lindau disease (VHL), neurofibromatosis type 1 (NF-1), and tuberous sclerosis (TSC). […] The etiology of PNETs within the context of these familial syndromes is the inherited germline loss of the respective tumor suppressor gene. […] Several studies have been performed on the pathogenesis of sporadic PNETs, which comprise 90% of all PNETs.

• #34 Pancreatic Neuroendocrine Cancer vs. Pancreatic Cancer – NETRF

  https://netrf.org/2018/11/07/pancreatic-neuroendocrine-cancer-vs-pancreatic-cancer/

  Neuroendocrine tumors arise from endocrine cells in the pancreas, which cluster together like an island and are called islet cells. These cells play an important role in regulating the body’s blood sugar. […] One type of pancreatic neuroendocrine tumor, insulinoma, may lead to too much insulin and cause blurred vision, headache, fast heartbeat, and feeling lightheaded, tired, weak, shaky, nervous, irritable, sweaty, confused, or hungry. […] Another type, called glucagonoma, can cause high blood sugar and cause headaches, frequent urination, dry skin, and mouth, or feeling hungry, thirsty, tired, or weak. […] Neuroendocrine tumors (NETs) are an uncommon cancer of the neuroendocrine cells, which receive messages from the nervous system and then release hormones into the bloodstream. When a neuroendocrine cell becomes cancerous, it divides uncontrollably, without stopping, forming tumors. […] Since they arise in hormone-producing cells, a neuroendocrine tumor can overproduce hormones and release them into the bloodstream, causing a range of symptoms.

• #35 Pancreatic Neuroendocrine Cancer vs. Pancreatic Cancer – NETRF

  https://netrf.org/2018/11/07/pancreatic-neuroendocrine-cancer-vs-pancreatic-cancer/

  Neuroendocrine tumors arise from endocrine cells in the pancreas, which cluster together like an island and are called islet cells. These cells play an important role in regulating the body’s blood sugar. […] One type of pancreatic neuroendocrine tumor, insulinoma, may lead to too much insulin and cause blurred vision, headache, fast heartbeat, and feeling lightheaded, tired, weak, shaky, nervous, irritable, sweaty, confused, or hungry. […] Another type, called glucagonoma, can cause high blood sugar and cause headaches, frequent urination, dry skin, and mouth, or feeling hungry, thirsty, tired, or weak. […] Neuroendocrine tumors (NETs) are an uncommon cancer of the neuroendocrine cells, which receive messages from the nervous system and then release hormones into the bloodstream. When a neuroendocrine cell becomes cancerous, it divides uncontrollably, without stopping, forming tumors. […] Since they arise in hormone-producing cells, a neuroendocrine tumor can overproduce hormones and release them into the bloodstream, causing a range of symptoms.

• #36 Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/pancreatic/hp/pnet-treatment-pdq

  Although nonfunctional tumors do not produce specific clinical syndromes, they may secrete inactive amine and peptide products such as: Neurotensin, Alpha-subunit of human chorionic gonadotropin (alpha-hCG), Neuron-specific enolase, Pancreatic polypeptide, Chromogranin A. […] The potential long delays between initial symptoms and diagnosis and the varied effects of the polypeptides secreted often necessitate involvement of multiple surgical and medical subspecialties. […] In patients with indolent, slow-growing, metastatic islet cell tumors, the best therapy may be careful observation, and no treatment until palliation is required. […] The approach to treatment often depends on the results of preoperative localization studies and findings at exploratory laparotomy. […] Patients with hepatic-dominant disease and substantial symptoms caused by tumor bulk or hormone-release syndromes may benefit from procedures that reduce hepatic arterial blood flow to metastases (hepatic arterial occlusion with embolization or with chemoembolization).

• #37 Pancreatic neuroendocrine tumors | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/pancreatic-neuroendocrine-tumours-2?lang=us

  Pancreatic neuroendocrine tumors (pNET), also known as endocrine tumors of the pancreas, arise from pancreatic ductal stem cells and include some distinct tumors that match the cell type of origin. […] Most tumors are sporadic. Approximately 10% are associated with multiple endocrine neoplasia type 1 (MEN1), von Hippel Lindau disease, tuberous sclerosis, and neurofibromatosis type 1. […] The term „syndromic” may be preferred over „functioning” since it is becoming increasingly clear that most tumors are functional (i.e. produce hormones), but either do not produce enough hormone or produce an ineffective form of the hormone, so that they may not produce a clinical syndrome. […] Biological behavior also depends on the cell of origin.

• #38 Pancreatic neuroendocrine tumors: biology, diagnosis, and treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3845620/

  It is generally assumed that the loss of a tumor suppressor gene or the gain of an Oncogene is the mechanism by which chromosomal alterations cause PNETs, but stochastic chromosomal number changes are also possible. […] The exact pathogenetic mechanism that leads to PNET tumorigenesis in these syndromes, however, is unclear. […] It is therefore plausible that PNETs develop from precursor (pre-malignant) lesions, such as hyperplasia and microadenoma, in familial PNET syndromes and in some sporadic cases.

• #39 Molecular Basis of Pancreatic Neuroendocrine Tumors

  https://www.mdpi.com/1422-0067/25/20/11017

  The origin of neuroendocrine tumors remains obscure. […] The use of genetic profiling technologies has revealed significant inter-patient heterogeneity between patients within the same WHO subtype during pancreatic neuroendocrine tumor research. […] High molecular heterogeneity is observed in grade 1 and 2 groups. […] The development of new therapeutics is ongoing, and new models to facilitate this process are being developed. […] Identifying patients with higher risk of metastasis and recurrence may influence the decision on radical resection, chemotherapy use and frequency of postoperative monitoring. […] A multitude of studies have illustrated the complexity and ubiquity of alterations in neoplasms, highlighting the need for multidimensional analysis of the pathology. […] The search for activated molecular pathways revealed that the complement and coagulation cascades, retinol metabolism, steroid hormone biosynthesis and drug metabolism were up-regulated in mutant NETs.

• #40 Pancreatic neuroendocrine tumors: biology, diagnosis, and treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3845620/

  Pancreatic neuroendocrine tumors (PNETs), a group of endocrine tumors arising in the pancreas, are among the most common neuroendocrine tumors. […] The genetic causes of familial and sporadic PNETs are somewhat understood, but their molecular pathogenesis remains unknown. […] The pathogenesis of PNETs is largely unknown but is growing as a research topic. […] Approximately 10% of all PNETs are components of familial endocrine tumor syndromes such as multiple endocrine neoplasia syndrome type 1 (MEN1), von Hippel-Lindau disease (VHL), neurofibromatosis type 1 (NF-1), and tuberous sclerosis (TSC). […] The etiology of PNETs within the context of these familial syndromes is the inherited germline loss of the respective tumor suppressor gene. […] Several studies have been performed on the pathogenesis of sporadic PNETs, which comprise 90% of all PNETs.

• #41 Neuroendocrine pancreatic cancer: An overview

  https://www.medicalnewstoday.com/articles/neuroendocrine-pancreatic-cancer

  Neuroendocrine tumors (NETs) are a type of cancerous growth affecting cells that send hormones around the body. Pancreatic neuroendocrine tumors (PNETs) grow in the cells of the pancreas that produce hormones to control blood sugar. […] The causes of PNETs are unclear. Around 90% of PNETs are sporadic, meaning they occur randomly. […] Cancer occurs due to genetic changes. These changes may switch on genes, called oncogenes, that help cells grow, divide, and die. These changes may also turn off tumor suppressor genes that control cell division or repair damaged DNA. […] Certain syndromes related to changes in three tumor suppressor genes may be responsible for inherited cases of PNETs. These syndromes include: multiple endocrine neoplasia type 1 (MEN1) syndrome, with changes in the MEN1 gene causing most cases of inherited PNETs; Von Hippel-Lindau (VHL) syndrome, which typically causes slow-growing, nonfunctioning PNETs at earlier ages; neurofibromatosis type 1 (NF1) syndrome, which mostly causes somatostatinomas, a very rare type of PNET. […] It is also possible to acquire gene changes after birth, sometimes through exposure to chemicals that cause cancer. This may occur through smoking. However, the causes of acquired gene changes are often not clear.

• #42 Pancreatic neuroendocrine tumors: biology, diagnosis, and treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3845620/

  It is generally assumed that the loss of a tumor suppressor gene or the gain of an Oncogene is the mechanism by which chromosomal alterations cause PNETs, but stochastic chromosomal number changes are also possible. […] The exact pathogenetic mechanism that leads to PNET tumorigenesis in these syndromes, however, is unclear. […] It is therefore plausible that PNETs develop from precursor (pre-malignant) lesions, such as hyperplasia and microadenoma, in familial PNET syndromes and in some sporadic cases.

• #43 Pancreatic neuroendocrine tumor – Wikipedia

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

  Pancreatic neuroendocrine tumors (PanNETs), often referred to as „islet cell tumours,” are neuroendocrine neoplasms that arise from cells of the endocrine (hormonal) and nervous system within the pancreas. […] Pancreatic neuroendocrine tumors may arise in the context of multiple endocrine neoplasia type 1, Von Hippel-Lindau disease, neurofibromatosis type 1 (NF-1) or tuberose sclerosis (TSC). […] Analysis of somatic DNA mutations in well-differentiated pancreatic neuroendocrine tumors identified four important findings: as expected, the genes mutated in NETs, MEN1, ATRX, DAXX, TSC2, PTEN and PIK3CA, are different from the mutated genes previously found in pancreatic adenocarcinoma. […] One in six well-differentiated pancreatic NETs have mutations in mTOR pathway genes, such as TSC2, PTEN and PIK3CA. […] Mutations affecting a new cancer pathway involving ATRX and DAXX genes were found in about 40% of pancreatic NETs. […] ATRX/DAXX and MEN1 mutations were associated with a better prognosis.

• #44 Pancreatic neuroendocrine tumor – Wikipedia

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

  Pancreatic neuroendocrine tumors (PanNETs), often referred to as „islet cell tumours,” are neuroendocrine neoplasms that arise from cells of the endocrine (hormonal) and nervous system within the pancreas. […] Pancreatic neuroendocrine tumors may arise in the context of multiple endocrine neoplasia type 1, Von Hippel-Lindau disease, neurofibromatosis type 1 (NF-1) or tuberose sclerosis (TSC). […] Analysis of somatic DNA mutations in well-differentiated pancreatic neuroendocrine tumors identified four important findings: as expected, the genes mutated in NETs, MEN1, ATRX, DAXX, TSC2, PTEN and PIK3CA, are different from the mutated genes previously found in pancreatic adenocarcinoma. […] One in six well-differentiated pancreatic NETs have mutations in mTOR pathway genes, such as TSC2, PTEN and PIK3CA. […] Mutations affecting a new cancer pathway involving ATRX and DAXX genes were found in about 40% of pancreatic NETs. […] ATRX/DAXX and MEN1 mutations were associated with a better prognosis.

• #45 Molecular Basis of Pancreatic Neuroendocrine Tumors

  https://www.mdpi.com/1422-0067/25/20/11017

  The origin of neuroendocrine tumors remains obscure. […] The use of genetic profiling technologies has revealed significant inter-patient heterogeneity between patients within the same WHO subtype during pancreatic neuroendocrine tumor research. […] High molecular heterogeneity is observed in grade 1 and 2 groups. […] The development of new therapeutics is ongoing, and new models to facilitate this process are being developed. […] Identifying patients with higher risk of metastasis and recurrence may influence the decision on radical resection, chemotherapy use and frequency of postoperative monitoring. […] A multitude of studies have illustrated the complexity and ubiquity of alterations in neoplasms, highlighting the need for multidimensional analysis of the pathology. […] The search for activated molecular pathways revealed that the complement and coagulation cascades, retinol metabolism, steroid hormone biosynthesis and drug metabolism were up-regulated in mutant NETs.

• #46 Molecular Basis of Pancreatic Neuroendocrine Tumors

  https://www.mdpi.com/1422-0067/25/20/11017

  The studies addressing the NET genome clearly indicate the existence of a group of non-functioning NETs enriched in MEN1/DAXX/ATRX mutations, structural aberrations, mTOR pathway alterations, positive ALT status, and unfavorable prognosis. […] The clinically-advantageous group is also non-functional, has MEN1 mutations, chromosome 11 LoH, and limited copy-number events. […] The poor prognosis associated with tumors harboring mutations in MEN1/ATRX/DAXX genes is at odds with the findings of certain studies.

• #47 Pancreatic neuroendocrine tumors: biology, diagnosis, and treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3845620/

  It is generally assumed that the loss of a tumor suppressor gene or the gain of an Oncogene is the mechanism by which chromosomal alterations cause PNETs, but stochastic chromosomal number changes are also possible. […] The exact pathogenetic mechanism that leads to PNET tumorigenesis in these syndromes, however, is unclear. […] It is therefore plausible that PNETs develop from precursor (pre-malignant) lesions, such as hyperplasia and microadenoma, in familial PNET syndromes and in some sporadic cases.

• #48

  https://journals.lww.com/md-journal/fulltext/2021/10080/pancreatic_neuroendocrine_tumor_producing.41.aspx

  Functional pancreatic neuroendocrine tumors (pNETs) rarely produce vasopressin. […] This case illustrated the potential ectopic production of vasopressin resulting in SIAD in pNETs, highlighting the adoption of gallium-68-labeled tetraazacyclododecanetetraacetic acid-Dphel-Tyr3-octreotate positron emission tomography-computed tomography and vasopressin immunohistochemical staining in the evaluation of the etiology of SIAD. […] The pNET ectopically producing vasopressin was clinically suspected and confirmed in pathology, highlighting the adoption of 68Ga-DOTATATE PET-CT and immunohistochemical staining for vasopressin in evaluating the etiology of SIAD. […] In our patient, we found an inappropriate release of vasopressin by the pNET, which was an unusual cause of SIAD. […] Therefore, in this patient, low level of the renin-angiotensin-aldosterone system, hypopituitarism, and the inappropriate production of vasopressin by the pNET jointly resulted in hyponatremia. […] Our case highlights pNETs are possibly 1 of the causes of hyponatremia.

• #49 Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/pancreatic/hp/pnet-treatment-pdq

  Although nonfunctional tumors do not produce specific clinical syndromes, they may secrete inactive amine and peptide products such as: Neurotensin, Alpha-subunit of human chorionic gonadotropin (alpha-hCG), Neuron-specific enolase, Pancreatic polypeptide, Chromogranin A. […] The potential long delays between initial symptoms and diagnosis and the varied effects of the polypeptides secreted often necessitate involvement of multiple surgical and medical subspecialties. […] In patients with indolent, slow-growing, metastatic islet cell tumors, the best therapy may be careful observation, and no treatment until palliation is required. […] The approach to treatment often depends on the results of preoperative localization studies and findings at exploratory laparotomy. […] Patients with hepatic-dominant disease and substantial symptoms caused by tumor bulk or hormone-release syndromes may benefit from procedures that reduce hepatic arterial blood flow to metastases (hepatic arterial occlusion with embolization or with chemoembolization).

• #50 Pancreatic Neuroendocrine Tumor With Humoral Hypercalcemia and High Tumor PD-L1 Score

  https://www.cancernetwork.com/view/pancreatic-neuroendocrine-tumor-with-humoral-hypercalcemia-and-high-tumor-pd-l1-score

  Pancreatic neuroendocrine tumors (NETs) are a heterogeneous group of neoplasms. […] Hypercalcemia due to malignancy can occur in about 20% to 30% of patients with cancer. The secretion of parathyroid hormone-related protein (PTH-rP) is among the causes of malignant hypercalcemia and has seldom been associated with hypercalcemia of NETs. […] Although humoral hypercalcemia can be caused by any tumor, its presence in patients with pancreatic NETs is extremely rare. […] An elevated calcium level with a suppressed intact PTH level and an elevated PTH-rP level was another rather unusual feature of our case. Malignancy-related hypercalcemia is rather uncommon in pancreatic NETs. […] PTH-rP is an autocrine, paracrine, and endocrine peptide involving calcium transportation and/or signaling expressed in response to a variety of physiological stimuli. […] Our patients calcium level normalized with intravenous bisphosphonate and isotonic saline; however, her PTH-rP level remained elevated (34 pmol/L) while her intact PTH level remained suppressed (1.4 pg/mL) for 2 months into her hypercalcemia diagnosis and treatment.

• #51 Causes of Neuroendocrine Tumors

  https://www.webmd.com/cancer/neuroendocrine-tumors-causes

  The plain truth is, that experts don’t know exactly what causes neuroendocrine tumors (NETs). But a bunch of things can make you more likely to get them. […] If you have the condition, you may be more likely to get cancers of the parathyroid gland, pituitary gland, and pancreas, including pancreatic NETs. […] If you have MEN2, you’re more likely to get neuroendocrine tumors such as pheochromocytoma, medullary thyroid cancer, and parathyroid tumors. […] If you have NF1 you’re more likely to get NETs such as carcinoid tumors and pheochromocytoma. […] If you have this condition, you’re more likely to get pancreatic NETs or carcinoid tumors. […] Your immune system is your body’s defense against germs. Anything that weakens it, such as HIV or an organ transplant, can raise your risk for NETs. […] Some studies suggest that smoking increases the risk for carcinoid tumors of the small intestine.

• #52 Risks and causes of neuroendocrine cancer| Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/risks-causes

  We dont know what causes most neuroendocrine cancers. […] The possible risks and causes of NETs and NECs are different. […] Most neuroendocrine cancers are not inherited. […] But there are some rare inherited conditions that can run in families. These can increase your risk of developing some types of NET. […] People with MEN1 have a higher risk of developing NETs in the pancreas. […] VHL is a rare inherited condition caused by a change in the von Hippel-Lindau gene. It can affect different parts of the body. People with VHL have an increased risk of developing different types of tumours. These include pancreatic NETs. […] Researchers think that people with tuberous sclerosis may have a higher risk of developing a pancreatic NET. […] There is some evidence that people with diabetes have an increased risk of developing a pancreatic NET. […] Some stomach NETs are linked to a condition called atrophic gastritis. This is a long term condition that causes inflammation of the stomach. Research has shown that there is a link between atrophic gastritis and type 1 stomach NET.

• #53 What Causes Pancreatic Neuroendocrine Tumor? | American Cancer Society

  https://www.cancer.org/cancer/types/pancreatic-neuroendocrine-tumor/causes-risks-prevention/what-causes.html

  Scientists dont know exactly what causes most pancreatic neuroendocrine tumors (pNETs), but they have found several risk factors that can make a person more likely to get this disease. […] Some of these risk factors affect the DNA of cells in the neuroendocrine system in the pancreas, which can result in abnormal cell growth and may cause cancers to form. […] Although 90% of pNETs are sporadic (random), some people inherit gene changes from their parents that raise their risk of pancreatic NET. […] Sometimes these gene changes are part of syndromes that increase risks for other health problems, as well. […] Syndromes related to changes in three tumor suppressor genes are responsible for many inherited cases of pNETs: […] Most gene mutations related to neuroendocrine tumors of the pancreas are random. […] If these random mutations occur after a person is born, they are called acquired. […] These acquired gene mutations sometimes result from exposure to cancer-causing chemicals (like those found in tobacco smoke).

• #54 Pancreatic neuroendocrine tumors | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/pancreatic-neuroendocrine-tumours-2?lang=us

  Pancreatic neuroendocrine tumors (pNET), also known as endocrine tumors of the pancreas, arise from pancreatic ductal stem cells and include some distinct tumors that match the cell type of origin. […] Most tumors are sporadic. Approximately 10% are associated with multiple endocrine neoplasia type 1 (MEN1), von Hippel Lindau disease, tuberous sclerosis, and neurofibromatosis type 1. […] The term „syndromic” may be preferred over „functioning” since it is becoming increasingly clear that most tumors are functional (i.e. produce hormones), but either do not produce enough hormone or produce an ineffective form of the hormone, so that they may not produce a clinical syndrome. […] Biological behavior also depends on the cell of origin.

• #55 Pancreatic Neuroendocrine Tumor

  https://www.ahn.org/services/cancer/types/pancreatic-neuroendocrine-tumor-pnet

  While the evidence is less conclusive than genetic factors, there is some indication that PanNETs can result from certain risk factors. […] Some studies suggest a possible link between exposure to certain chemicals and an increased risk of PanNETs. […] Some chronic medical conditions have been suggested as potential risk factors, though evidence remains limited and often correlational, not necessarily causal. […] The incidence of PanNETs increases with age, suggesting that the accumulation of genetic and/or environmental damage over time may play a role. […] Despite these lifestyle choices not being strongly connected to PanNETs, its still important to quit smoking, have a healthy diet, and limit or abstain from alcohol. […] Unlike many other cancers, there’s no strong evidence linking smoking to an increased risk of PanNETs. […] While a healthy diet is always beneficial for overall health, there’s no strong evidence to suggest a direct link between specific dietary factors and the risk of PanNETs. […] The evidence for a link between alcohol consumption and PanNET risk is weak and inconclusive.

• #56 Molecular Basis of Pancreatic Neuroendocrine Tumors

  https://www.mdpi.com/1422-0067/25/20/11017

  The origin of neuroendocrine tumors remains obscure. […] The use of genetic profiling technologies has revealed significant inter-patient heterogeneity between patients within the same WHO subtype during pancreatic neuroendocrine tumor research. […] High molecular heterogeneity is observed in grade 1 and 2 groups. […] The development of new therapeutics is ongoing, and new models to facilitate this process are being developed. […] Identifying patients with higher risk of metastasis and recurrence may influence the decision on radical resection, chemotherapy use and frequency of postoperative monitoring. […] A multitude of studies have illustrated the complexity and ubiquity of alterations in neoplasms, highlighting the need for multidimensional analysis of the pathology. […] The search for activated molecular pathways revealed that the complement and coagulation cascades, retinol metabolism, steroid hormone biosynthesis and drug metabolism were up-regulated in mutant NETs.

• #57

  https://content-dev.irisoncology.com/library/pancreatic-neuroendocrine-tumors

  Syndromes present at birth that increase the risk of tumors. Some syndromes that are passed from parents to children can increase the risk of pancreatic neuroendocrine tumors. Examples of these include multiple endocrine neoplasia, type 1 (MEN 1), von Hippel-Lindau (VHL) disease, neurofibromatosis 1 (NF1) and tuberous sclerosis. These inherited syndromes are caused by changes in the DNA. These changes allow cells to grow and divide more than needed. […] There’s no way to prevent pancreatic neuroendocrine tumors. If you develop this type of cancer, you didn’t do anything to cause it.

• #58 Pancreatic neuroendocrine tumors | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/pancreatic-neuroendocrine-tumors

  Pancreatic neuroendocrine tumors happen when cells in the pancreas develop changes in their DNA. A cell’s DNA holds the instructions that tell the cell what to do. The changes, which doctors call mutations, tell the cells to multiply quickly. The changes let the cells continue living when healthy cells would die as part of their natural life cycle. This causes many extra cells. The cells might form a mass called a tumor. Sometimes the cells can break away and spread to other organs, such as the liver. When cancer spreads, it’s called metastatic cancer. […] In pancreatic neuroendocrine tumors, the DNA changes happen in hormone-producing cells called islet cells. It’s not clear what causes the changes that lead to cancer. […] There’s no way to prevent pancreatic neuroendocrine tumors. If you develop this type of cancer, you didn’t do anything to cause it.

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