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Islet Cell Tumor
Pancreatic neuroendocrine tumours (PanNETs, PETs, or PNETs), often referred to as "islet cell tumours", or "pancreatic endocrine tumours" are neuroendocrine neoplasms that arise from cells of the endocrine (hormonal) and nervous system within the pancreas. PanNETs are a type of neuroendocrine tumor, representing about one-third of gastroenteropancreatic neuroendocrine tumors (GEP-NETs). Many PanNETs are benign, while some are malignant. Aggressive PanNET tumors have traditionally been termed "islet cell carcinoma". PanNETs are quite distinct from the usual form of pancreatic cancer, the majority of which are adenocarcinomas, which arise in the exocrine pancreas. Only 1 or 2% of clinically significant pancreas neoplasms are PanNETs. Types The majority of PanNETs are benign, while some are malignant. The World Health Organization (WHO) classification scheme places neuroendocrine tumors into three main categories, which emphasize the tumor grade rather than the anatomical origi ...
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Primitive Neuroectodermal Tumor
Primitive neuroectodermal tumor is a malignant (cancerous) neural crest tumor. It is a rare tumor, usually occurring in children and young adults under 25 years of age. The overall 5 year survival rate is about 53%. It gets its name because the majority of the cells in the tumor are derived from neuroectoderm, but have not developed and differentiated in the way a normal neuron would, and so the cells appear "primitive". PNET belongs to the Ewing family of tumors. Genetics Using gene transfer of SV40 large T-antigen in neuronal precursor cells of rats, a brain tumor model was established. The PNETs were histologically indistinguishable from the human counterparts and have been used to identify new genes involved in human brain tumor carcinogenesis. The model was used to confirm p53 as one of the genes involved in human medulloblastomas, but since only about 10% of the human tumors showed mutations in that gene, the model can be used to identify the other binding partners of SV40 ...
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Insulinoma
An insulinoma is a tumour of the pancreas that is derived from beta cells and secretes insulin. It is a rare form of a neuroendocrine tumour. Most insulinomas are benign in that they grow exclusively at their origin within the pancreas, but a minority metastasize. Insulinomas are one of the functional pancreatic neuroendocrine tumour (PNET) group ("functional" because it increases production of insulin). In the Medical Subject Headings classification, insulinoma is the only subtype of "islet cell adenoma". Beta cells secrete insulin in response to increases in blood glucose. The resulting increase in insulin acts to lower blood glucose back to normal levels, the point at which further secretion of insulin is stopped. In contrast, the secretion of insulin by insulinomas is rather independent of blood glucose; these tumours continue to secrete insulin, causing blood glucose levels to fall further below normal. As a result, patients present symptoms of low blood glucose (hypogly ...
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Hypokalemia
Hypokalemia is a low level of potassium (K+) in the blood serum. Mild low potassium does not typically cause symptoms. Symptoms may include feeling tired, leg cramps, weakness, and constipation. Low potassium also increases the risk of an abnormal heart rhythm, which is often too slow and can cause cardiac arrest. Causes of hypokalemia include vomiting, diarrhea, medications like furosemide and steroids, dialysis, diabetes insipidus, hyperaldosteronism, hypomagnesemia, and not enough intake in the diet. Normal potassium levels in humans are between 3.5 and 5.0 mmol/L (3.5 and 5.0 mEq/L) with levels below 3.5 mmol/L defined as hypokalemia. It is classified as severe when levels are less than 2.5 mmol/L. Low levels may also be suspected based on an electrocardiogram (ECG). The opposite state is called hyperkalemia that means high level of potassium in the blood serum. The speed at which potassium should be replaced depends on whether or not there are symptom ...
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Dehydration
In physiology, dehydration is a lack of total body water that disrupts metabolic processes. It occurs when free water loss exceeds intake, often resulting from excessive sweating, health conditions, or inadequate consumption of water. Mild dehydration can also be caused by immersion diuresis, which may increase risk of decompression sickness in divers. Most people can tolerate a 3-4% decrease in total body water without difficulty or adverse health effects. A 5-8% decrease can cause fatigue and dizziness. Loss of over 10% of total body water can cause physical and mental deterioration, accompanied by severe thirst. Death occurs with a 15 and 25% loss of body water.Ashcroft F, Life Without Water in Life at the Extremes. Berkeley and Los Angeles, 2000, 134-138. Mild dehydration usually resolves with oral rehydration, but severe cases may need intravenous fluids. Dehydration can cause hypernatremia (high levels of sodium ions in the blood). This is distinct from hypovolemia ...
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Vasoactive Intestinal Peptide
Vasoactive intestinal peptide, also known as vasoactive intestinal polypeptide or VIP, is a peptide hormone that is vasoactive in the intestine. VIP is a peptide of 28 amino acid residue (chemistry), residues that belongs to a Secretin family, glucagon/secretin superfamily, the ligand (biochemistry), ligand of class II G protein–coupled receptors. VIP is produced in many tissues of vertebrates including the Gut (zoology), gut, pancreas, neocortex, and suprachiasmatic nuclei of the hypothalamus in the brain. VIP stimulates contractility in the heart, causes vasodilation, increases glycogenolysis, lowers arterial blood pressure and relaxes the smooth muscle of Vertebrate trachea, trachea, stomach and gallbladder. In humans, the vasoactive intestinal peptide is encoded by the ''VIP'' gene. VIP has a half-life (t½) in the blood of about two minutes. Function In the digestive system In the digestive system, VIP seems to induce smooth muscle relaxation (lower esophageal sphincte ...
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VIPoma
A VIPoma or vipoma () is a rare endocrine tumor that overproduces vasoactive intestinal peptide (thus ''VIP'' + '' -oma''). The incidence is about 1 per 10,000,000 per year. VIPomas usually (about 90%) originate from the non-β islet cells of the pancreas. They are sometimes associated with multiple endocrine neoplasia type 1. Roughly 50–75% of VIPomas are malignant, but even when they are benign, they are problematic because they tend to cause a specific syndrome: the massive amounts of VIP cause a syndrome of profound and chronic watery diarrhea and resultant dehydration, hypokalemia, achlorhydria, acidosis, flushing and hypotension (from vasodilation), hypercalcemia, and hyperglycemia. This syndrome is called Verner–Morrison syndrome (VMS), WDHA syndrome (from watery diarrhea–hypokalemia–achlorhydria), or pancreatic cholera syndrome (PCS). The eponym reflects the physicians who first described the syndrome. Symptoms and signs The major clinical features are prolon ...
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Diarrhea
Diarrhea (American English), also spelled diarrhoea or diarrhœa (British English), is the condition of having at least three loose, liquid, or watery bowel movements in a day. It often lasts for a few days and can result in dehydration due to fluid loss. Signs of dehydration often begin with loss of the normal stretchiness of the skin and irritable behaviour. This can progress to decreased urination, loss of skin color, a fast heart rate, and a decrease in responsiveness as it becomes more severe. Loose but non-watery stools in babies who are exclusively breastfed, however, are normal. What is diarrhea? How is it caused, treated and prevented? (see also script)The most common cause is an infection of the intestines due to a virus, bacterium, or parasite—a condition also known as gastroenteritis. These infections are often acquired from food or water that has been contaminated by feces, or directly from another person who is infected. The three types of diarrhea ...
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Peptic Ulcer Disease
Peptic ulcer disease is when the inner part of the stomach's gastric mucosa (lining of the stomach), the first part of the small intestine, or sometimes the lower esophagus, gets damaged. An ulcer in the stomach is called a gastric ulcer, while one in the first part of the intestines is a duodenal ulcer. The most common symptoms of a duodenal ulcer are waking at night with upper abdominal pain, and upper abdominal pain that improves with eating. With a gastric ulcer, the pain may worsen with eating. The pain is often described as a burning or dull ache. Other symptoms include belching, vomiting, weight loss, or poor appetite. About a third of older people with peptic ulcers have no symptoms. Complications may include bleeding, perforation, and blockage of the stomach. Bleeding occurs in as many as 15% of cases. Common causes include infection with ''Helicobacter pylori'' and non-steroidal anti-inflammatory drugs (NSAIDs). Other, less common causes include tobacco smoking, ...
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Abdominal Pain
Abdominal pain, also known as a stomach ache, is a symptom associated with both non-serious and serious medical issues. Since the abdomen contains most of the body's vital organs, it can be an indicator of a wide variety of diseases. Given that, approaching the examination of a person and planning of a differential diagnosis is extremely important. Common causes of pain in the abdomen include gastroenteritis and irritable bowel syndrome. About 15% of people have a more serious underlying condition such as appendicitis, leaking or ruptured abdominal aortic aneurysm, diverticulitis, or ectopic pregnancy. In a third of cases, the exact cause is unclear. Signs and symptoms The onset of abdominal pain can be abrupt, quick, or gradual. Sudden onset pain happens in a split second. Rapidly onset pain starts mild and gets worse over the next few minutes. Pain that gradually intensifies only after several hours or even days has passed is referred to as gradual onset pain. One can ...
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Gastrin
Gastrin is a peptide hormone that stimulates secretion of gastric acid (HCl) by the parietal cells of the stomach and aids in gastric motility. It is released by G cells in the pyloric antrum of the stomach, duodenum, and the pancreas. Gastrin binds to cholecystokinin B receptors to stimulate the release of histamines in enterochromaffin-like cells, and it induces the insertion of K+/H+ ATPase pumps into the apical membrane of parietal cells (which in turn increases H+ release into the stomach cavity). Its release is stimulated by peptides in the lumen of the stomach. Physiology Genetics In humans, the ''GAS'' gene is located on the long arm of the seventeenth chromosome (17q21). Synthesis Gastrin is a linear peptide hormone produced by G cells of the duodenum and in the pyloric antrum of the stomach. It is secreted into the bloodstream. The encoded polypeptide is preprogastrin, which is cleaved by enzymes in posttranslational modification to produce progastri ...
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Gastrinoma Triangle
Passaro's triangle or gastrinoma triangle is a presumptive region in the abdomen between three points: # Superior— porta hepatis (earlier—confluence of the cystic and common bile duct), # Inferior—junction of the second and third portion of duodenum, and # Medial—junction of the neck and body of the pancreas The pancreas (plural pancreases, or pancreata) is an Organ (anatomy), organ of the Digestion, digestive system and endocrine system of vertebrates. In humans, it is located in the abdominal cavity, abdomen behind the stomach and functions as a ... The importance of the triangle is because it is known as the source of origin of most gastrinomas. However, primary gastrinomas can also occur in the liver or extrahepatic bile ducts, commonly with metastasis to the local lymph nodes. The appellation is due to Edward Peter Passaro, an American surgeon, who explained it for the first time in the year 1984. eerakkody Y, Bell D, Gilcrease-Garcia B, et al. Gastrinoma tri ...
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Gastrinoma
Gastrinomas are neuroendocrine tumors (NETs), usually located in the duodenum or pancreas, that secrete gastrin and cause a clinical syndrome known as Zollinger–Ellison syndrome (ZES). A large number of gastrinomas develop in the pancreas or duodenum, with near-equal frequency, and approximately 10% arise as primary neoplasms in lymph nodes of the pancreaticoduodenal region ( gastrinoma triangle). Most gastrinomas are sporadic (75–80%), whereas approximately 20–25% are associated with multiple endocrine neoplasia type 1 (MEN-1). Over 50% of gastrinomas are malignant and can metastasize to regional lymph nodes and liver. One fourth of gastrinomas are related to multiple endocrine neoplasia type 1, Zollinger–Ellison syndrome, peptic ulcer disease. Signs and symptoms Gastrinoma in the early stages will have signs and symptoms of indigestion or similar to irritable bowel disease (IBD) such as: * Hypergastrinemia * Refractory or recurrent peptic ulcers involving duodenum * Ch ...
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