Materiały źródłowe

• #1 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. […] Most PNETs are indolent but have malignant potential. […] The biological behavior of an individual PNET is unpredictable; higher tumor grade, lymph node and liver metastasis, and larger tumor size generally indicate a less favorable prognosis. […] If untreated, most PNETs grow and eventually metastasize to the liver; extensive liver metastasis is the most common cause of death for patients with PNETs. […] The biological behavior of an individual PNET is unpredictable; a higher tumor grade, lymph node and liver metastasis, and a larger primary tumor generally portend a less favorable prognosis. […] Non-functioning PNETs cause nonspecific symptoms, such as vague abdominal pain, and can be an incidental finding.

• #2 Pancreatic neuroendocrine tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5359348/

  Pancreatic neuroendocrine neoplasms (pNENs) are a heterogeneous group of tumors including well differentiated pancreatic neuroendocrine tumors (pNETs) and neuroendocrine carcinomas (pNECs). The incidence of pNENs has increased over the past few decades. […] Although the primary treatment for pNENs is surgical resection, there is still a lack of effective therapeutic options for patients with advanced unresectable pNENs. […] Patients with functional pNENs were diagnosed earlier than patients with nonfunctional pNENs (mean age of presentation 55 vs. 59 years) due to the different specific hormonal syndromes including gastrin, insulin, glucagon, somatostatin, vasoactive intestinal polypeptide (VIP), growth hormone-releasing factor and adrenocorticotrophic hormone. […] For the functional pNENs, the clinical presentations are mainly determined by the hypersecreted hormones produced by the tumor.

• #3 What Is a Pancreatic Neuroendocrine Tumor? | American Cancer Society

  https://www.cancer.org/cancer/types/pancreatic-neuroendocrine-tumor/about/what-is-pnet.html

  Pancreatic neuroendocrine tumors (pNETs) are a type of cancer that starts in the pancreas. […] Pancreatic neuroendocrine tumors start in neuroendocrine cells, a special cell found in the pancreas. […] Neuroendocrine tumors start in the endocrine cells of the pancreas. These cells make hormones, such as insulin. […] About half of pNETs make hormones that are released into the blood and cause symptoms. […] Non-functioning pNETs: These tumors dont make enough excess hormones to cause symptoms. Because they dont make excess hormones that cause symptoms, they can often grow quite large before they’re found. Symptoms that may occur when they grow to a large size include abdominal pain, poor appetite, and weight loss. […] The treatment and outlook for pNETs depend on the specific tumor type and the stage (extent) of the tumor, but the outlook is generally better than for pancreatic exocrine cancers.

• #4 Pancreatic Neuroendocrine Tumors: Slow-Growing and Often Curable – Southern Iowa Mental Health Center

  https://simhcottumwa.org/pancreatic-neuroendocrine-tumors-slow-growing-and-often-curable/

  Pancreatic cancer is often considered a death sentence, typically aggressive and usually caught late, but there is one type of pancreatic cancer that is far more treatable. […] Pancreatic NETs are often slow-growing compared to adenocarcinomas and have a better prognosis overall. […] Patients have a high rate of cure and patients with metastatic pancreatic NETs can often live for many years with a chronic cancer. […] Symptoms are often non-specific and can include fatigue, weight loss, nausea, vomiting, pain, jaundice (yellowing of skin), and new-onset diabetes. Some pancreatic NETs also produce hormones that can cause unique symptoms of hormone excess.

• #5 Pancreatic neuroendocrine tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5359348/

  Pancreatic neuroendocrine neoplasms (pNENs) are a heterogeneous group of tumors including well differentiated pancreatic neuroendocrine tumors (pNETs) and neuroendocrine carcinomas (pNECs). The incidence of pNENs has increased over the past few decades. […] Although the primary treatment for pNENs is surgical resection, there is still a lack of effective therapeutic options for patients with advanced unresectable pNENs. […] Patients with functional pNENs were diagnosed earlier than patients with nonfunctional pNENs (mean age of presentation 55 vs. 59 years) due to the different specific hormonal syndromes including gastrin, insulin, glucagon, somatostatin, vasoactive intestinal polypeptide (VIP), growth hormone-releasing factor and adrenocorticotrophic hormone. […] For the functional pNENs, the clinical presentations are mainly determined by the hypersecreted hormones produced by the tumor.

• #6 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 are either functional (produce hormones) or nonfunctional (produce no hormones). […] Functional neuroendocrine tumors cause the pancreas to overproduce hormones consequently causing hormone-related symptoms. The majority of PNETs are nonfunctional tumors. Nonfunctional tumors do not produce any hormones so they do not cause any hormone-related symptoms. As a result, these tumors are typically diagnosed once the tumor is advanced and is causing symptoms such as pain or jaundice. […] The tumors in the pancreas may be malignant and generally appear in individuals in their 30s or 40s.

• #7 Pancreatic neuroendocrine cancers (PancNETs) – Pancreatic Cancer UK

  https://www.pancreaticcancer.org.uk/information/just-diagnosed-with-pancreatic-cancer/pancreatic-neuroendocrine-tumours-pancnets/

  Some pancreatic neuroendocrine cancers produce higher levels of certain hormones, which can cause specific symptoms. These are called functioning neuroendocrine cancers. Up to 30 in 100 (30%) of pancreatic neuroendocrine cancers are functioning. […] Symptoms may include chest and/or tummy pain, acid reflux, heartburn, diarrhoea and tiredness. These combined symptoms are called Zollinger-Ellison syndrome. Excess gastrin may also cause irritation of the stomach or duodenum, leading to ulcers and bleeding. […] Symptoms include a distinct type of skin rash (redness and blisters), particularly on the face, tummy, bottom and feet. Glucagonomas can also cause diabetes (high blood sugar levels), diarrhoea (runny poo), weight loss, changes in mood, anaemia (low levels of red blood cells), blood clots, and sore mouth and tongue.

• #8 Update on pancreatic neuroendocrine tumors – McKenna – Gland Surgery

  https://gs.amegroups.org/article/view/4839/html

  pNETs are divided into functional versus non-functional tumors with about 90% being classified as non-functional. Commonly, tumors are defined as non-functional if the patient does not suffer from symptoms due to hormone hyper-secretion, even if hormone levels are elevated on laboratory evaluation. Most non-functional pNETs present with symptoms due to mass effect, such as jaundice, weight loss, abdominal pain, palpable mass, nausea/emesis, pancreatitis, or back pain, and mimic the presentation of pancreatic adenocarcinoma. As routine diagnostic imaging becomes more prevalent, some patients present asymptomatically with an incidental finding on cross sectional imaging. Most patients present with metastatic (60%) or locally advanced disease (21%) […] As mentioned before, pNETs are functional 10% of the time. The presenting symptoms of functional tumors depend on the particular hormone that is being overproduced. The most common functional pNETs are insulinomas, gastrinomas, VIPomas, glucagonomas, and somatostatinomas. Insulinomas comprise 35-40% of all functional pNETs, and present with symptoms of episodic hyperinsulinemia classically referred to as Whipples triad: symptoms of hypoglycemia (weakness, sweating, tremors, palpitations, confusion, visual changes etc.) during fasting or exercise, documented hypoglycemia at time of symptoms, and symptom resolution with glucose administration. Gastrinomas make up 16-30% of functional pNETs and hyper-secrete gastrin resulting in Zollinger Ellison syndrome, the classic symptoms of which are refractory peptic ulcer disease and secretory diarrhea.

• #9 Pancreatic neuroendocrine cancers (PancNETs) – Pancreatic Cancer UK

  https://www.pancreaticcancer.org.uk/information/just-diagnosed-with-pancreatic-cancer/pancreatic-neuroendocrine-tumours-pancnets/

  Pancreatic neuroendocrine cancers may be called functioning or non-functioning. This depends on whether they produce higher levels of hormones, which cause symptoms. Most are non-functioning. This means that they dont produce more hormones than normal. […] Most pancreatic neuroendocrine cancers (60-90%) are called non-functioning pancreatic neuroendocrine cancers. They dont make higher levels of hormones. Rarely, they can cause symptoms that are similar to the symptoms of pancreatic ductal adenocarcinoma. These include pain, weight loss, jaundice and diarrhoea. […] Non-functioning neuroendocrine cancers may be harder to detect and diagnose than functioning neuroendocrine cancers. This is because they dont cause specific symptoms. They are often diagnosed during tests for another problem.

• #10 Pancreatic neuroendocrine tumor – Wikipedia

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

  Some PanNETs do not cause any symptoms, in which case they may be discovered incidentally on a CT scan performed for a different purpose. […] Symptoms such as abdominal or back pain or pressure, diarrhea, indigestion, or yellowing of the skin and whites of the eyes can arise from the effects of a larger PanNET tumor, either locally or at a metastasis. […] About 40% of PanNETS have symptoms related to excessive secretion of hormones or active polypeptides and are accordingly labeled as „functional”; the symptoms reflect the type of hormone secreted, as discussed below. […] Up to 90% of PanNETs are nonsecretory or nonfunctional, in which there is no secretion, or the quantity or type of products, such as pancreatic polypeptide (PPoma), chromogranin A, and neurotensin, do not cause a clinical syndrome although blood levels may be elevated.

• #11 Pancreatic neuroendocrine cancers (PancNETs) – Pancreatic Cancer UK

  https://www.pancreaticcancer.org.uk/information/just-diagnosed-with-pancreatic-cancer/pancreatic-neuroendocrine-tumours-pancnets/

  Pancreatic neuroendocrine cancers may be called functioning or non-functioning. This depends on whether they produce higher levels of hormones, which cause symptoms. Most are non-functioning. This means that they dont produce more hormones than normal. […] Most pancreatic neuroendocrine cancers (60-90%) are called non-functioning pancreatic neuroendocrine cancers. They dont make higher levels of hormones. Rarely, they can cause symptoms that are similar to the symptoms of pancreatic ductal adenocarcinoma. These include pain, weight loss, jaundice and diarrhoea. […] Non-functioning neuroendocrine cancers may be harder to detect and diagnose than functioning neuroendocrine cancers. This is because they dont cause specific symptoms. They are often diagnosed during tests for another problem.

• #12 Non functioning pancreatic neuroendocrine tumours (NETs) | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/types/pancreatic-nets/non-functioning

  Some people dont have any symptoms. Doctors can diagnose a non functioning pancreatic NET when looking for something else. […] You might have symptoms caused by the growth and spread of the tumour. These can include: tummy pain, indigestion (dyspepsia), yellowing of the skin and whites of the eyes (jaundice), unexplained weight loss, loss of appetite. […] Symptoms are often vague and can also be caused by other more common medical conditions. But its important to get them checked out by your doctor. […] Non functioning pancreatic NETs are very rare. Nearly 5 people in every million develop a non functioning pancreatic NET every year. […] Non functioning pancreatic NETs are the most common type of NET that develops in the pancreas. […] Non functioning pancreatic NETs are well differentiated cancers. This means the NET cells look abnormal. But they still have some similarities to normal neuroendocrine cells.

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

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

  Most pancreatic neuroendocrine tumors are nonfunctional and do not give rise to any hormone-related symptoms. […] The symptoms of pancreatic neuroendocrine tumors may vary depending on various factors, such as whether they are functional or non-functional, the size of the primary tumor, and how far they have spread (metastasized) in the body. […] Nonfunctional pancreatic neuroendocrine tumors: Because these tumors do not cause hormone-related symptoms, they are generally diagnosed incidentally or when the tumor grows large enough to press on adjacent structures or when it has metastasized to other sites in the body. They may cause symptoms, such as: Abdominal pain, Mass or lump in the abdomen, Unexplained weight loss, Diarrhea, Nausea, Indigestion, Jaundice (yellowing of the skin and eyes), Blood in stools or black-tarry stools.

• #14 Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment – NCI

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

  Pancreatic NETs may or may not cause signs or symptoms. […] Different types of pancreatic NETs have different signs and symptoms. […] Signs or symptoms can be caused by the growth of the tumor and/or by hormones the tumor makes or by other conditions. Some tumors may not cause signs or symptoms. […] A non-functional pancreatic NET may grow for a long time without causing signs or symptoms. It may grow large or spread to other parts of the body before it causes signs or symptoms, such as: Diarrhea. Indigestion. A lump in the abdomen. Pain in the abdomen or back. Yellowing of the skin and whites of the eyes. […] The signs and symptoms of a functional pancreatic NET depend on the type of hormone being made. […] Too much gastrin may cause: Stomach ulcers that keep coming back. Pain in the abdomen, which may spread to the back. The pain may come and go and it may go away after taking an antacid. The flow of stomach contents back into the esophagus (gastroesophageal reflux). Diarrhea.

• #15 Neuroendocrine tumours | Cancer Council

  https://www.cancer.org.au/cancer-information/types-of-cancer/rare-cancers/neuroendocrine-tumours

  Most neuroendocrine tumours develop slowly over several years but they can also be aggressive and grow quickly, spreading to other parts of the body and causing problems. […] In the early stages neuroendocrine tumours may not cause symptoms. If you do have symptoms, it will depend on where in the body the tumour is and if the tumour cells are producing hormones. You may experience general symptoms such as fatigue and loss of appetite. […] Symptoms may include: low blood sugar levels (hypoglycaemia) causing shaking, sweating, confusion, dizziness and rapid heartbeat; high blood sugar levels (hyperglycaemia) which may cause increased urination, excessive thirst and blurred vision; indigestion (heartburn) and stomach ulcers; rash in the groin area; unexplained weight loss; nausea or vomiting; changes in bowel habits, including diarrhoea or pale, foul-smelling stools that are hard to flush away.

• #16 Non functioning pancreatic neuroendocrine tumours (NETs) | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/types/pancreatic-nets/non-functioning

  Some people dont have any symptoms. Doctors can diagnose a non functioning pancreatic NET when looking for something else. […] You might have symptoms caused by the growth and spread of the tumour. These can include: tummy pain, indigestion (dyspepsia), yellowing of the skin and whites of the eyes (jaundice), unexplained weight loss, loss of appetite. […] Symptoms are often vague and can also be caused by other more common medical conditions. But its important to get them checked out by your doctor. […] Non functioning pancreatic NETs are very rare. Nearly 5 people in every million develop a non functioning pancreatic NET every year. […] Non functioning pancreatic NETs are the most common type of NET that develops in the pancreas. […] Non functioning pancreatic NETs are well differentiated cancers. This means the NET cells look abnormal. But they still have some similarities to normal neuroendocrine cells.

• #17 Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment – NCI

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

  Too much insulin may cause: Low blood sugar. This can cause blurred vision, headache, and feeling lightheaded, tired, weak, shaky, nervous, irritable, sweaty, confused, or hungry. Fast heartbeat. […] Too much glucagon may cause: Skin rash on the face, stomach, or legs. High blood sugar. This can cause headaches, frequent urination, dry skin and mouth, or feeling hungry, thirsty, tired, or weak. Blood clots. Blood clots in the lung can cause shortness of breath, cough, or pain in the chest. Blood clots in the arm or leg can cause pain, swelling, warmth, or redness of the arm or leg. Diarrhea. Weight loss for no known reason. Sore tongue or sores at the corners of the mouth. […] Pancreatic NETs can recur (come back) after they have been treated. The tumors may come back in the pancreas or in other parts of the body.

• #18 Signs and Symptoms of PNETs | American Cancer Society | American Cancer Society

  https://www.cancer.org/cancer/types/pancreatic-neuroendocrine-tumor/detection-diagnosis-staging/signs-and-symptoms.html

  Having one or more of the symptoms below does not mean you have a pancreatic neuroendocrine tumor (pNET). In fact, many of these symptoms are more likely to be caused by other conditions. Still, if you have any of them, its important to have them checked by a doctor so the cause can be found and treated, if needed. […] PNETs often release excess hormones into the bloodstream. Different types of tumors make different hormones, and cause different symptoms. […] These tumors make gastrin, a hormone that tells the stomach to make more acid. Too much gastrin causes a condition known as Zollinger-Ellison syndrome, in which the stomach makes too much acid. This leads to stomach ulcers, which can cause pain, nausea, and loss of appetite. Severe ulcers can bleed. Even if the bleeding is mild, it can lead to anemia (too few red blood cells), which can make a person feel tired and be short of breath. If the bleeding is more severe, it can make stools black and tarry. Severe bleeding can itself be life-threatening.

• #19 Pancreatic Neuroendocrine Tumor | Cedars-Sinai

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

  Nonfunctioning pancreatic NETs dont cause symptoms until theyre big enough to put pressure on other organs, such as the bile ducts. […] The symptoms of functional pancreatic NETs depend on what type you have because the symptoms are caused by the overproduction of hormones. […] Symptoms are caused by too much stomach acid and include: Acid reflux, Stomach ulcers that keep coming back, Pain in the belly or back, Diarrhea. […] Symptoms are caused by low blood sugar levels and include: Blurred vision, Weakness, Extreme tiredness (fatigue), Headache, Feeling lightheaded or dizzy, Shakiness, Irritability, Hunger, Fast heartbeat. […] Symptoms are caused by high blood sugars and include: Diarrhea, Weight loss, Mouth dryness, Sores at the corner of the mouth or on the tongue, Skin rash or dry skin, Headaches, Feeling very thirsty and hungry, Frequent urination, Feeling tired, weak, or dizzy, Blood clots.

• #20 Pancreatic Neuroendocrine Tumours | Pancreatic Cancer Awareness | PCA

  https://pancreaticcanceraction.org/about-pancreatic-cancer/what-is-pancreatic-cancer/pancreatic-neuroendocrine-tumours/

  Pancreatic neuroendocrine tumours (pNETs) form in the endocrine cells of the pancreas where insulin and other hormones are produced. These tumours are called pancreatic neuroendocrine tumours (or pNETs) or islet cell tumours. They are much less common than exocrine tumours and divided into functional or non-functional tumours. Functioning tumours produce hormones and therefore are normally detected earlier, they make up around a third of the endocrine tumours diagnosed. Non-functional tumours do not produce hormones but are more likely to be cancerous. […] Insulinoma Pancreatic islet cells Tumours in insulin producing cells. This leads to over production of insulin and the symptoms of diabetes and low blood sugar such as headaches, frequency urinating, thirst, sweating, vision problems, confusion and fits.

• #21 Update on pancreatic neuroendocrine tumors – McKenna – Gland Surgery

  https://gs.amegroups.org/article/view/4839/html

  pNETs are divided into functional versus non-functional tumors with about 90% being classified as non-functional. Commonly, tumors are defined as non-functional if the patient does not suffer from symptoms due to hormone hyper-secretion, even if hormone levels are elevated on laboratory evaluation. Most non-functional pNETs present with symptoms due to mass effect, such as jaundice, weight loss, abdominal pain, palpable mass, nausea/emesis, pancreatitis, or back pain, and mimic the presentation of pancreatic adenocarcinoma. As routine diagnostic imaging becomes more prevalent, some patients present asymptomatically with an incidental finding on cross sectional imaging. Most patients present with metastatic (60%) or locally advanced disease (21%) […] As mentioned before, pNETs are functional 10% of the time. The presenting symptoms of functional tumors depend on the particular hormone that is being overproduced. The most common functional pNETs are insulinomas, gastrinomas, VIPomas, glucagonomas, and somatostatinomas. Insulinomas comprise 35-40% of all functional pNETs, and present with symptoms of episodic hyperinsulinemia classically referred to as Whipples triad: symptoms of hypoglycemia (weakness, sweating, tremors, palpitations, confusion, visual changes etc.) during fasting or exercise, documented hypoglycemia at time of symptoms, and symptom resolution with glucose administration. Gastrinomas make up 16-30% of functional pNETs and hyper-secrete gastrin resulting in Zollinger Ellison syndrome, the classic symptoms of which are refractory peptic ulcer disease and secretory diarrhea.

• #22 Signs and Symptoms of PNETs | American Cancer Society | American Cancer Society

  https://www.cancer.org/cancer/types/pancreatic-neuroendocrine-tumor/detection-diagnosis-staging/signs-and-symptoms.html

  Having one or more of the symptoms below does not mean you have a pancreatic neuroendocrine tumor (pNET). In fact, many of these symptoms are more likely to be caused by other conditions. Still, if you have any of them, its important to have them checked by a doctor so the cause can be found and treated, if needed. […] PNETs often release excess hormones into the bloodstream. Different types of tumors make different hormones, and cause different symptoms. […] These tumors make gastrin, a hormone that tells the stomach to make more acid. Too much gastrin causes a condition known as Zollinger-Ellison syndrome, in which the stomach makes too much acid. This leads to stomach ulcers, which can cause pain, nausea, and loss of appetite. Severe ulcers can bleed. Even if the bleeding is mild, it can lead to anemia (too few red blood cells), which can make a person feel tired and be short of breath. If the bleeding is more severe, it can make stools black and tarry. Severe bleeding can itself be life-threatening.

• #23 Symptoms of neuroendocrine tumours (NETs) | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/neuroendocrine-tumours/signs-and-symptoms

  Zollinger-Ellison syndrome is a condition caused by too much gastrin in the body. This increases the amount of stomach acid made, which can eventually lead to ulcers of the stomach or small intestine. Zollinger-Ellison syndrome may happen in people with gastrin-producing NETs of the pancreas or small intestine (gastrinoma). The signs or symptoms of Zollinger-Ellison syndrome include: pain in the abdomen; severe diarrhea; heartburn; nausea and vomiting, sometimes vomiting blood; weight loss.

• #24 Signs and Symptoms of PNETs | American Cancer Society | American Cancer Society

  https://www.cancer.org/cancer/types/pancreatic-neuroendocrine-tumor/detection-diagnosis-staging/signs-and-symptoms.html

  If the stomach acid reaches the small intestine, it can damage the cells of the intestinal lining and break down digestive enzymes before they have a chance to digest food. This can cause diarrhea and weight loss. […] Excess glucagon can raise blood sugar, sometimes leading to diabetes. This can cause symptoms such as feeling thirsty and hungry, and having to urinate often. […] The symptom that brings most people with glucagonomas to their doctor is a rash called necrolytic migratory erythema. This red rash with swelling and blisters often moves from place to place on the skin. […] Too much insulin leads to low blood sugar, which can cause symptoms like weakness, confusion, sweating, and rapid heartbeat. When blood sugar gets very low, it can lead to a person passing out or even going into a coma and having seizures.

• #25 Pancreatic Neuroendocrine Tumours | Pancreatic Cancer Awareness | PCA

  https://pancreaticcanceraction.org/about-pancreatic-cancer/what-is-pancreatic-cancer/pancreatic-neuroendocrine-tumours/

  Glucagonoma Pancreatic islet cells Tumours in the cells that produce hormones to prompt the body to produce glucagon. This raises blood sugar levels in the body and therefore when produced to excess result in diabetes symptoms, damage to nerves, metabolic problems, mouth sores and anaemia. Patients often experience rashes, blisters and skin conditions.

• #26 Update on pancreatic neuroendocrine tumors – McKenna – Gland Surgery

  https://gs.amegroups.org/article/view/4839/html

  The most common presenting symptom of glucagonomas is a dermatitis called migratory necrolytic erythema, consisting of erythematous lesions that become necrotic and develop pigmented scarring. Other common symptoms from glucagonomas include glucose intolerance, weight loss, diarrhea, and deep vein thrombosis (DVT). Together these symptoms are sometimes referred to as the 4D syndrome (dermatitis, diabetes, diarrhea, DVT). VIPomas secrete vasoactive intestinal polypeptide and result in symptoms of large volume watery diarrhea and hypokalemia. Somatostatinomas comprise less than 5% of pNETs. They secrete somatostatin and can cause diabetes mellitus, gallbladder disease, diarrhea/steatorrhea, anemia, and weight loss. Somatostatinomas have the subtlest syndrome of any of the functional pNETs and rarely is the syndrome present in its entirety in a single patient.

• #27 Pancreatic neuroendocrine cancers (PancNETs) – Pancreatic Cancer UK

  https://www.pancreaticcancer.org.uk/information/just-diagnosed-with-pancreatic-cancer/pancreatic-neuroendocrine-tumours-pancnets/

  Some pancreatic neuroendocrine cancers produce higher levels of certain hormones, which can cause specific symptoms. These are called functioning neuroendocrine cancers. Up to 30 in 100 (30%) of pancreatic neuroendocrine cancers are functioning. […] Symptoms may include chest and/or tummy pain, acid reflux, heartburn, diarrhoea and tiredness. These combined symptoms are called Zollinger-Ellison syndrome. Excess gastrin may also cause irritation of the stomach or duodenum, leading to ulcers and bleeding. […] Symptoms include a distinct type of skin rash (redness and blisters), particularly on the face, tummy, bottom and feet. Glucagonomas can also cause diabetes (high blood sugar levels), diarrhoea (runny poo), weight loss, changes in mood, anaemia (low levels of red blood cells), blood clots, and sore mouth and tongue.

• #28 Pancreatic Neuroendocrine Tumor | Cedars-Sinai

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

  Symptoms include: Weight loss, Stomach cramps or pain, Large amounts of watery diarrhea, Flushing (redness) of the face, chest, and neck, Dehydration, which can cause dizziness, low urine output, dry skin and mouth, and tiredness, Symptoms linked to low potassium levels, such as muscle weakness, aches, cramps, numbness, tingling, frequent urination, thirst, and irregular heartbeat (palpitations). […] Symptoms include: Diarrhea, Gallstones, Fatty, bad-smelling stools that float, Yellowing of the skin or eyes (jaundice), Unexplained weight loss, High blood sugar symptoms, such as weakness, hunger, confusion, headaches, frequent urination, and dry skin and mouth.

• #29 Symptoms of Pancreatic Neuroendocrine Tumors – Pancreatic Cancer Action Network

  https://pancan.org/facing-pancreatic-cancer/symptoms/symptoms-of-neuroendocrine-tumors/

  Pancreatic neuroendocrine tumors (pancreatic NETs or PNETs), also called pancreatic endocrine tumors or islet cell tumors, are tumors that form from the abnormal growth of hormone-producing cells in the pancreas called islet cells. The pancreas produces hormones that regulate blood sugar level, stomach acid production, the rate of food absorption and amount of water in the intestines. Pancreatic neuroendocrine tumors are either functional (produce hormones) or nonfunctional (do not produce hormones). […] Functional pancreatic neuroendocrine tumors may cause the pancreas to overproduce certain hormones, resulting in a number of symptoms. The following table describes symptoms specific to functional pancreatic neuroendocrine tumors. […] Gastrinoma (Zollinger-Ellison Syndrome) gastrin Acid reflux, burning abdominal pain, diarrhea, excess fat in the stools and weight loss. […] Glucaganoma glucagon High blood sugar, severe swelling or irritation of the skin, mouth sores, anemia and weight loss. […] Insulinoma insulin Low blood sugar, which can cause heart palpitations, shakiness, perspiration, confusion and seizures. […] Somatostatinoma somatostatin Because this hormone suppresses production of a variety of other hormones, symptoms are non-specific. They include diabetes, gallstones, weight loss, diarrhea, excess fat in the stools, nausea and vomiting. […] Vasoactive Intestinal Peptide Releasing Tumor (VIPoma) (Verner-Morrison Syndrome) vasoactive intestinal peptide (VIP) Severe watery diarrhea, which can lead to low blood potassium levels causing muscle weakness, fatigue and nausea. […] Nonfunctional pancreatic neuroendocrine tumors do not cause the pancreas to overproduce hormones. They are generally detected because of pain or jaundice caused by the large tumor size.

• #30 Signs and Symptoms of PNETs | American Cancer Society | American Cancer Society

  https://www.cancer.org/cancer/types/pancreatic-neuroendocrine-tumor/detection-diagnosis-staging/signs-and-symptoms.html

  Symptoms of this type of tumor can include belly pain, nausea, poor appetite, weight loss, diarrhea, symptoms of diabetes (feeling thirsty and hungry, and having to urinate often), gallstones, and jaundice (yellowing of the skin and eyes). The early symptoms of a somatostatinoma are mild and are more often caused by other things, so these tumors tend to be diagnosed at an advanced stage. Often, they are not found until they spread to the liver, when they cause problems like jaundice and pain. […] Too much VIP can lead to problems with diarrhea. This may be mild at first, but get worse over time. By the time they are diagnosed, most people have severe, watery diarrhea. […] These tumors often make serotonin or its precursor, 5-HTP. Carcinoid tumors often don’t cause symptoms until they spread outside the pancreas. When these tumors do spread, it is most often to the liver. There, the cancer cells can release hormones directly into the blood. This can cause the carcinoid syndrome, with symptoms including flushing (redness and warmth in the face or neck), diarrhea, wheezing, and rapid heart rate.

• #31 Pancreatic neuroendocrine cancers (PancNETs) – Pancreatic Cancer UK

  https://www.pancreaticcancer.org.uk/information/just-diagnosed-with-pancreatic-cancer/pancreatic-neuroendocrine-tumours-pancnets/

  Symptoms include lots of watery diarrhoea, dehydration, low levels of potassium in the blood (hypokalaemia), tummy pain, bloating, and a flushed face. […] This can lead to symptoms including gallstones, steatorrhoea (pale, oily, poo), anaemia, tummy pain, high blood sugar levels and jaundice. […] This can cause symptoms of Cushings disease, including weight gain, easy bruising, anaemia, depression, increased risk of infection and darkened skin. […] This can cause symptoms including high calcium levels, tummy pain, feeling and being sick, constipation, bone pain, osteoporosis, fatigue, and confusion. […] This can cause symptoms including diarrhoea, abdominal pain and weight loss.

• #32 Signs and Symptoms of PNETs | American Cancer Society | American Cancer Society

  https://www.cancer.org/cancer/types/pancreatic-neuroendocrine-tumor/detection-diagnosis-staging/signs-and-symptoms.html

  Symptoms of this type of tumor can include belly pain, nausea, poor appetite, weight loss, diarrhea, symptoms of diabetes (feeling thirsty and hungry, and having to urinate often), gallstones, and jaundice (yellowing of the skin and eyes). The early symptoms of a somatostatinoma are mild and are more often caused by other things, so these tumors tend to be diagnosed at an advanced stage. Often, they are not found until they spread to the liver, when they cause problems like jaundice and pain. […] Too much VIP can lead to problems with diarrhea. This may be mild at first, but get worse over time. By the time they are diagnosed, most people have severe, watery diarrhea. […] These tumors often make serotonin or its precursor, 5-HTP. Carcinoid tumors often don’t cause symptoms until they spread outside the pancreas. When these tumors do spread, it is most often to the liver. There, the cancer cells can release hormones directly into the blood. This can cause the carcinoid syndrome, with symptoms including flushing (redness and warmth in the face or neck), diarrhea, wheezing, and rapid heart rate.

• #33 Signs and Symptoms of PNETs | American Cancer Society | American Cancer Society

  https://www.cancer.org/cancer/types/pancreatic-neuroendocrine-tumor/detection-diagnosis-staging/signs-and-symptoms.html

  Symptoms of this type of tumor can include belly pain, nausea, poor appetite, weight loss, diarrhea, symptoms of diabetes (feeling thirsty and hungry, and having to urinate often), gallstones, and jaundice (yellowing of the skin and eyes). The early symptoms of a somatostatinoma are mild and are more often caused by other things, so these tumors tend to be diagnosed at an advanced stage. Often, they are not found until they spread to the liver, when they cause problems like jaundice and pain. […] Too much VIP can lead to problems with diarrhea. This may be mild at first, but get worse over time. By the time they are diagnosed, most people have severe, watery diarrhea. […] These tumors often make serotonin or its precursor, 5-HTP. Carcinoid tumors often don’t cause symptoms until they spread outside the pancreas. When these tumors do spread, it is most often to the liver. There, the cancer cells can release hormones directly into the blood. This can cause the carcinoid syndrome, with symptoms including flushing (redness and warmth in the face or neck), diarrhea, wheezing, and rapid heart rate.

• #34 Neuroendocrine Tumors (NETs): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/22006-neuroendocrine-tumors-net

  Neuroendocrine tumors (NETs) are a group of uncommon tumors that start in specialized cells in your neuroendocrine system. Theyre most common in your GI tract, lungs and pancreas but can form wherever neuroendocrine cells are in your body. […] Often, neuroendocrine tumors dont cause symptoms until a tumor impacts an organ. When this happens, symptoms vary based on location. NET symptoms may include: Fatigue. Stomach pain. Diarrhea. Nausea and vomiting. Shortness of breath. Coughing (sometimes with blood). […] Carcinoid syndrome happens when a neuroendocrine tumor in your GI tract or lungs releases too many hormones. It can cause a range of unpleasant symptoms, including flushing (sudden warmth and redness) of your head and neck, wheezing and diarrhea. […] NETs are often mistaken for other less serious conditions, so it may take some time before youre diagnosed. Once you learn you have a neuroendocrine tumor, your prognosis, or expected outcome, depends on several factors, including the type of NET you have and whether your tumor has spread.

• #35 9 Top Neuroendocrine Tumor Symptoms & Signs | MD Anderson Cancer Center

  https://www.mdanderson.org/cancer-types/neuroendocrine-tumors/neuroendocrine-tumor-symptoms.html

  Since neuroendcrine tumors (NETs) can form anywhere in the body, symptoms will vary depending on where the tumor is located. […] The symptoms of pancreatic NETs are similar to other pancreatic cancers. […] Symptoms of gastrointestinal nets are similar to those of other GI tract cancers. These include: Rectal bleeding and/or blood in the stool, A change in size, shape or color of the stool, Discomfort or urge to have a bowel movement when there is no need, Bloating or a feeling of fullness, Unexplained weight loss, Pain in the abdomen or lower back. […] Functional NETs are NETs that produce hormones. Like all NETs, these can appear anywhere in the body. The hormones they release can cause physical symptoms and lead to changes in behavior. In addition to symptoms related to their location, the symptoms of functional NETs may include: Persistent flushing of the face, Persistent, often severe diarrhea, Severe gastric ulcers, High blood sugar or low blood sugar, Changes in bathroom habits, including diarrhea and frequent urination, Dizziness or shakiness, Anxiety or confusion.

• #36 Pancreatic neuroendocrine cancers (PancNETs) – Pancreatic Cancer UK

  https://www.pancreaticcancer.org.uk/information/just-diagnosed-with-pancreatic-cancer/pancreatic-neuroendocrine-tumours-pancnets/

  Symptoms include lots of watery diarrhoea, dehydration, low levels of potassium in the blood (hypokalaemia), tummy pain, bloating, and a flushed face. […] This can lead to symptoms including gallstones, steatorrhoea (pale, oily, poo), anaemia, tummy pain, high blood sugar levels and jaundice. […] This can cause symptoms of Cushings disease, including weight gain, easy bruising, anaemia, depression, increased risk of infection and darkened skin. […] This can cause symptoms including high calcium levels, tummy pain, feeling and being sick, constipation, bone pain, osteoporosis, fatigue, and confusion. […] This can cause symptoms including diarrhoea, abdominal pain and weight loss.

• #37 Neuroendocrine pancreatic cancer: An overview

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

  PNETs can also cause an excess of adrenocorticotropic hormone (ACTH), which can lead to Cushing syndrome. This can cause a range of symptoms, including vision loss, headache, a fatty lump on the back of the neck, weight gain in the trunk, face, and neck but thin arms and legs, a tendency to bruise easily, thin skin, purple stretch marks on the abdomen or chest, fine hair growth on the face, arms, or upper back, slow-healing cuts and sores, increased risk of bone fractures, irritability, anxiety, depression. […] The outlook for a person with PNET depends on many factors, including the type of PNET, the location of the tumor on the pancreas, whether the tumor has spread to distant organs, whether the person has MEN1 syndrome, whether the cancer has returned or if a doctor has diagnosed it for the first time, the person’s age and health status.

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

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

  Neuroendocrine tumors don’t always cause signs and symptoms at first. The symptoms you might experience depend on the location of your tumor and whether it produces excess hormones. […] In general, neuroendocrine tumor signs and symptoms might include: Pain from a growing tumor, A growing lump you can feel under the skin, Feeling unusually tired, Losing weight without trying. […] Neuroendocrine tumors that produce excess hormones (functional tumors) might cause: Skin flushing, Diarrhea, Frequent urination, Increased thirst, Dizziness, Shakiness, Skin rash. […] 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.

• #39 Neuroendocrine tumours | Cancer Council

  https://www.cancer.org.au/cancer-information/types-of-cancer/rare-cancers/neuroendocrine-tumours

  Most neuroendocrine tumours develop slowly over several years but they can also be aggressive and grow quickly, spreading to other parts of the body and causing problems. […] In the early stages neuroendocrine tumours may not cause symptoms. If you do have symptoms, it will depend on where in the body the tumour is and if the tumour cells are producing hormones. You may experience general symptoms such as fatigue and loss of appetite. […] Symptoms may include: low blood sugar levels (hypoglycaemia) causing shaking, sweating, confusion, dizziness and rapid heartbeat; high blood sugar levels (hyperglycaemia) which may cause increased urination, excessive thirst and blurred vision; indigestion (heartburn) and stomach ulcers; rash in the groin area; unexplained weight loss; nausea or vomiting; changes in bowel habits, including diarrhoea or pale, foul-smelling stools that are hard to flush away.

• #40 Neuroendocrine Tumor Stages and Survival Rate

  https://www.cancercenter.com/cancer-types/neuroendocrine-tumors/stages

  Cancer is found in the pancreas and hasnt spread. The tumor size is smaller than 2 centimeters (cm) wide. […] The tumor is still only in the pancreas, but it is greater than 2 cm wide. It hasnt yet spread to nearby lymph nodes or distant body parts, but it may have grown into the bile duct that carries digestive fluid through the pancreas to the small intestine, or into the upper part of the small intestine. […] The tumor may or may not have spread to nearby lymph nodes, but it hasnt yet spread to distant parts of the body. Or, it may or may not have begun to grow outside the pancreas into blood vessels or nearby organs. […] The cancer has spread to distant parts of the body. […] More than 20 percent of NETs have already spread to other parts of the body by the time they are diagnosed. NETs metastasize most often to the liver, peritoneal cavity or bone.

• #41 Where Do Pancreatic Neuroendocrine Tumors Metastasize? Lifespan Chart

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

  Pancreatic neuroendocrine tumors can metastasize to any organ in the body; however, the most common organ is the liver. […] Liver failure resulting from metastasis from these tumors is the most common cause of death in patients affected by the tumor. […] The lifespan of a patient with a pancreatic neuroendocrine tumor depends on the stage and grade of their cancer. […] If the tumor has spread to distant areas of the body, such as the liver, the survival rate is 25 percent.

• #42 Signs and Symptoms of PNETs | American Cancer Society | American Cancer Society

  https://www.cancer.org/cancer/types/pancreatic-neuroendocrine-tumor/detection-diagnosis-staging/signs-and-symptoms.html

  These tumors don’t make excess hormones, so they don’t cause symptoms in early stages and often grow quite large before they are found. Most of these cause problems as they get larger or spread outside the pancreas. Symptoms can be like those from exocrine pancreas cancers, including jaundice (yellowing of the eyes and skin), belly pain, and weight loss. […] When pNETs spread, it is most often to the liver. This can enlarge the liver, which can cause pain and loss of appetite. It can also affect liver function, sometimes leading to jaundice (yellowing of the skin and eyes) and abnormal blood test results. These cancers can also spread to other organs and tissues. The symptoms depend on where the cancer is growing. For example, cancer spread to the lungs can cause shortness of breath or a cough. Spread to bones can cause pain in those areas.

• #43 Signs and Symptoms of PNETs | American Cancer Society | American Cancer Society

  https://www.cancer.org/cancer/types/pancreatic-neuroendocrine-tumor/detection-diagnosis-staging/signs-and-symptoms.html

  These tumors don’t make excess hormones, so they don’t cause symptoms in early stages and often grow quite large before they are found. Most of these cause problems as they get larger or spread outside the pancreas. Symptoms can be like those from exocrine pancreas cancers, including jaundice (yellowing of the eyes and skin), belly pain, and weight loss. […] When pNETs spread, it is most often to the liver. This can enlarge the liver, which can cause pain and loss of appetite. It can also affect liver function, sometimes leading to jaundice (yellowing of the skin and eyes) and abnormal blood test results. These cancers can also spread to other organs and tissues. The symptoms depend on where the cancer is growing. For example, cancer spread to the lungs can cause shortness of breath or a cough. Spread to bones can cause pain in those areas.

• #44 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. […] Most PNETs are indolent but have malignant potential. […] The biological behavior of an individual PNET is unpredictable; higher tumor grade, lymph node and liver metastasis, and larger tumor size generally indicate a less favorable prognosis. […] If untreated, most PNETs grow and eventually metastasize to the liver; extensive liver metastasis is the most common cause of death for patients with PNETs. […] The biological behavior of an individual PNET is unpredictable; a higher tumor grade, lymph node and liver metastasis, and a larger primary tumor generally portend a less favorable prognosis. […] Non-functioning PNETs cause nonspecific symptoms, such as vague abdominal pain, and can be an incidental finding.

• #45 Non functioning pancreatic neuroendocrine tumours (NETs) | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/types/pancreatic-nets/non-functioning

  There are 3 grades of pancreatic neuroendocrine tumours (NETs) grade 1, 2 and 3. Grade 1 grow slowly, and grade 3 grow quickly. Grade 2 grow at a moderate speed. […] You have tests to find out the size of the NET and whether it has spread. This is your stage. Doctors use your stage to plan your treatment. […] Your prognosis will depend on the type of NET you have and what stage it is.

• #46 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. […] Most PNETs are indolent but have malignant potential. […] The biological behavior of an individual PNET is unpredictable; higher tumor grade, lymph node and liver metastasis, and larger tumor size generally indicate a less favorable prognosis. […] If untreated, most PNETs grow and eventually metastasize to the liver; extensive liver metastasis is the most common cause of death for patients with PNETs. […] The biological behavior of an individual PNET is unpredictable; a higher tumor grade, lymph node and liver metastasis, and a larger primary tumor generally portend a less favorable prognosis. […] Non-functioning PNETs cause nonspecific symptoms, such as vague abdominal pain, and can be an incidental finding.

• #47 Neuroendocrine Tumors (NETs): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/22006-neuroendocrine-tumors-net

  NETs are curable when your healthcare provider can remove all signs of the tumor with surgery. But one of the biggest challenges with this diagnosis is that although most NETs are slow-growing, theyve often spread by the time theyre caught. […] Neuroendocrine tumors and their treatment can take a toll on your body. You may have to manage common symptoms like fatigue feeling extremely tired day after day and diarrhea throughout your treatment.

• #48 Neuroendocrine Tumor Stages and Survival Rate

  https://www.cancercenter.com/cancer-types/neuroendocrine-tumors/stages

  According to the American Cancer Society, the overall five-year relative survival rate is 53 percent. For pancreatic NETs contained to the pancreas, the five-year relative survival rate is 95 percent. If the cancer has grown into nearby tissues or lymph nodes, the five-year relative survival rate is 72 percent. For pancreatic NETs that have spread to distant body parts, the five-year relative survival rate is 23 percent.

• #49 Neuroendocrine Tumor Stages and Survival Rate

  https://www.cancercenter.com/cancer-types/neuroendocrine-tumors/stages

  According to the American Cancer Society, the overall five-year relative survival rate is 53 percent. For pancreatic NETs contained to the pancreas, the five-year relative survival rate is 95 percent. If the cancer has grown into nearby tissues or lymph nodes, the five-year relative survival rate is 72 percent. For pancreatic NETs that have spread to distant body parts, the five-year relative survival rate is 23 percent.

• #50 Treating Pancreatic Neuroendocrine Tumors – Let’s Win Pancreatic Cancer

  https://letswinpc.org/treatments/living-longer-pancreatic-neuroendocrine-tumors/

  They are words you dont often hear together: Metastatic cancer and hope. […] Even in the metastatic setting, there are many treatment options. It is possible to keep setting the clock back on the progression of the disease, says Hodul, surgical oncologist at Moffitt Cancer Center in Tampa, Florida, and Associate Professor in the Division of Oncologic Sciences at the University of South Florida. […] Most PNETs are nonfunctional, however, and may not be detected until the tumors have progressed to a later stage, or spread to other organs. […] The disease progression is very different from other types of cancer, and should give hope to PNET patients. Treatment can be very beneficial in most cases, even in metastatic disease.

• #51 Overview of Gastrointestinal and Pancreatic Neuroendocrine Tumors (NETs) – Endocrine and Metabolic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/endocrine-and-metabolic-disorders/gastrointestinal-and-pancreatic-neuroendocrine-tumors-nets/overview-of-gastrointestinal-and-pancreatic-neuroendocrine-tumors-nets

  Hypersecretion of these substances can cause autonomic, neuromuscular, and mental status changes that together make up the carcinoid syndrome. […] Endocrinologically inert NETs are suspected because of their symptoms and signs (eg, pain, luminal bleeding, gastrointestinal obstruction). […] Prognosis for neuroendocrine tumors depends on primary site, grade, and stage. Despite metastatic disease, NETs are slow growing, and survival of 10 to 15 years is not unusual.

• #52 Pancreatic Neuroendocrine Tumors – PNETs | Choose the Right Test

  https://arupconsult.com/content/pancreatic-neuroendocrine-tumors

  Pancreatic neuroendocrine tumors (PNETs) can present in a variety of ways. Patients with nonfunctional PNETs (the more common type) may be asymptomatic, even though the tumors may be large and at an advanced stage when detected. […] Functional PNETs secrete hormones, and this secretion leads to clinical syndromes such as insulinoma, gastrinoma or Zollinger-Ellison syndrome, glucagonoma, somatostatinoma, or vasoactive intestinal polypeptide (VIP)-secreting tumors (referred to as VIPoma). Patients with insulinomas may present with hypoglycemia, those with gastrinoma may have recurring peptic ulcers, patients with glucagonoma or somatostatinoma may present with hyperglycemia or diabetes mellitus, and those with VIPoma may have watery diarrhea or hypokalemia. […] Approximately 21-42% of patients with PNETs experience disease recurrence, and in some cases, the disease recurs a number of years after initial diagnosis and treatment. Follow-up testing is recommended 12 weeks to 12 months after resection (and sooner if symptoms appear) and every 6-12 months thereafter for up to 10 years. Monitoring should include biochemical marker testing, particularly if a functional syndrome was diagnosed. Chromogranin A may be useful to assess treatment response, detect disease progression, and identify disease recurrence.

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

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

  The distinction between functioning and non-functioning PNETs is based on clinical presentation, and there is no absolute difference in hormone expression between functioning and non-functioning PNETs. […] Most patients survive many years after diagnosis. […] The treatment strategy for PNETs has undergone a paradigm shift in the last 10-20 years. […] The aggressive approach has 4 components: surgery, locoregional therapy, systemic therapy, and complication control. […] Surgical removal of primary, non-metastatic PNET is the only clinical cure, and surgical debulking of liver PNET metastases reduces the hormone secretion from functioning PNETs and the tumor mass effects of all PNETs. […] Most PNETs are indolent but have malignant potential. […] The true natural history of PNETs has not been studied systemically. […] The clinical presentation, diagnosis, and treatment of specific types of PNETs and familial PNET syndromes, including the novel Mahvash disease, are summarized.

• #54 Pancreatic Neuroendocrine Tumor

  https://myhealth.umassmemorial.org/BreatheEasy/134,40

  For tumors that cant be removed by surgery, or that have spread to other parts of the body, other treatments may be used to shrink the tumor, destroy it, or control the symptoms its causing. These include: […] Active surveillance if the tumor is slow-growing and not cancer […] […] Chemotherapy […] […] Hormone therapy […] […] Targeted therapy […] […] Radiofrequency ablation, or using heat to destroy the tumor […] […] Cryosurgical ablation, or freezing the cancer cells […] […] Your healthcare provider uses medicines to help manage your symptoms from the pancreatic NET. These may include: […] Medicines for stomach ulcers […] […] Therapy or over-the-counter remedies to deal with diarrhea […] […] IV fluids to help prevent dehydration […] […] Medicines or other strategies to keep your blood sugar in a healthy range […]

• #55 Surgical Management of Pancreatic Neuroendocrine Tumors

  https://www.mdpi.com/2072-6694/15/7/2006

  A correlation between tumor size and risk of malignant characteristics has been demonstrated. Nonfunctional PNETs that are symptomatic, large (greater than 2 cm), and with atypical features such as pancreatic duct dilatation, should undergo surgical resection. However, the management of small nonfunctioning tumors has been an area of debate given their typically relatively indolent behavior. […] While additional tumor characteristics such as Ki-67 proliferative index could ideally guide decision making regarding resection, at present tumor size has been the most reliable determinant of tumor progression for well-differentiated PNET. […] Randomized data to guide management of small, incidentally discovered PNETs are difficult to obtain due to their low incidence and relatively indolent course. A recent international, prospective, nonrandomized study attempts to address this, enrolling patients with asymptomatic small nonfunctional PNETs (2 cm or less).

• #56 Surgical Management of Pancreatic Neuroendocrine Tumors

  https://www.mdpi.com/2072-6694/15/7/2006

  The decision to pursue surgical resection for these patients included patient preference, younger age, tumor size greater than 1 cm, and presence of main pancreatic duct dilation. At last follow-up, 2% of patients had tumor progression and no patients had metastatic disease. […] These preliminary results provide additional, prospective evidence that active surveillance is a safe strategy for patients with small nonfunctional PNETs.

• #57 Pancreatic neuroendocrine tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5359348/

  For the patients with poorly differentiated pNEC, the role of surgery is limited, because many cases are unresectable and most resectable cases have a high risk of recurrence or metastasis. […] While the primary treatment for pNENs is surgical, the treatment of patients with advanced or metastatic disease requires a multidisciplinary approach. […] The pNENs demonstrate a relative sensitivity to chemotherapy. […] The poorly differentiated (G3) pNECs have a better response than the well differentiated (G1/G2) pNETs. […] SSAs have shown a significant impact on functional pNENs patients with hormonal symptoms. […] PRRT is a newer treatment option that can be used for tumors that express a high density of somatostatin receptors on somatostatin receptor imaging. […] pNENs are highly vascularized neoplasms and express an abundance of VEG-F and platelet-derived growth factor (PDGF) receptors.

• #58 Shedding light on pancreatic neuroendocrine tumors – UChicago Medicine

  https://www.uchicagomedicine.org/forefront/cancer-articles/shedding-light-on-pancreatic-neuroendocrine-tumors

  The most common treatment is surgery. If the tumor is still localized and has not spread, we remove tumors greater than 2 centimeters. […] If the tumor has spread, surgery is still common. […] Recent, more advanced therapies include the new and promising PRRT therapy, or peptide receptor radionuclide therapy. PRRT is a radioactive infusion given every other month for eight months that targets the tumor cells without having to affect other organs. It has minimal side effects and is a promising therapy because it can shrink the tumor in up to 30-40% of patients with pancreatic neuroendocrine tumors.

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