1. They are totally different cancers despite an anatomical relationship. Although they can share a similar presentation, they can have different signs, different treatments, and vastly different prognostic outcomes. Anyone looking for useful information on either needs to be very careful on interpretation, they could end up with very bad advice and in some situations, become more concerned than they should be. (particularly with the prognostics).
2.Both Pancreatic Cancer and Neuroendocrine Cancer are diseases that need maximum publicity, both types of cancer have their own unique situations, thus why the awareness messages can be so vastly different. It’s really important, therefore, that publicity surrounding famous patients be attributed to the correct cancer type in order that the advocate organisations and supporters can gain maximum benefit to forward their causes. Unfortunately, thanks to doctors and media, this very often doesn’t work out in favour of Neuroendocrine Cancer due to the Human Anatomy of Neuroendocrine Cancer (this problem goes beyond the pancreas).
The pancreas is an organ that sits behind the stomach. It's shaped a bit like a fish with a wide head, a tapering body, and a narrow, pointed tail. In adults it's about 6 inches (15 centimeters) long but less than 2 inches (5 centimeters) wide.
The head of the pancreas is on the right side of the abdomen (belly), behind where the stomach meets the duodenum (the first part of the small intestine).
The body of the pancreas is behind the stomach.
The tail of the pancreas is on the left side of the abdomen next to the spleen.
Pancreatic neuroendocrine tumors, known as PNET, are also called islet cell tumors. They are a type of neuroendocrine cancer that starts in the pancreas. There are different kinds of PNET. Some PNET make and release different hormones. They also vary in size and how fast they grow.
PNET are different from other types of pancreatic cancer, because they form from neuroendocrine cells in your pancreas. Neuroendocrine cells are part of your body’s normal “control system.” When your brain and nervous system send messages, neuroendocrine cells release hormones and other chemical messengers to control bodily functions, like digestion, reproduction, airflow to the lungs, and responses to stress or injury.
There are many subtypes of PNET; most are slow growing, but some may be aggressive.
The most common type of pancreatic cancer, adenocarcinoma of the pancreas, starts when exocrine cells in the pancreas start to grow out of control. Most of the pancreas is made up of exocrine cells which form the exocrine glands and ducts. The exocrine glands make pancreatic enzymes that are released into the intestines to help you digest foods (especially fats). The enzymes are released into tiny tubes called ducts which eventually empty into the pancreatic duct. The pancreatic duct merges with the common bile duct (the duct that carries bile from the liver), and empties into the duodenum (the first part of the small intestine) at the ampulla of Vater.
Endocrine cells make up a smaller percentage of the cells in the pancreas. These cells make important hormones like insulin and glucagon (which help control blood sugar levels), and release them directly into the blood. Pancreatic neuroendocrine tumors start in the endocrine cells. See Pancreatic Neuroendocrine Tumor for more about this type.
A neuroendocrine tumor (NET) begins in the specialized cells of the body’s neuroendocrine system. These cells have traits of both hormone-producing endocrine cells and nerve cells. They are found throughout the body’s organs and help control many of the body’s functions. Hormones are chemical substances that are carried through the bloodstream to have a specific effect on the activity of other organs or cells in the body. Most NETs take years to develop and grow slowly. However, some NETs can be fast-growing. See the Grades section to learn more.
NETs can begin in any part of the body, including:
Gastrointestinal (GI) tract. NETs develop most commonly in the GI tract, specifically in the large intestine (20%), small intestine (19%), and appendix (4%). The GI tract plays a central role in digesting foods and liquid and in processing waste. GI tract NETs used to be called carcinoid tumors. Learn more about NET of the GI tract.
Lung. The lung is the second most common location of NETs. About 30% of NETs occur in the bronchial system, which carries air to the lungs. Lung NETs also used to be called carcinoid tumors. Learn more about NET of the lung.
Pancreas. Approximately 7% of NETs can develop in the pancreas, a pear-shaped gland located in the abdomen between the stomach and the spine. Pancreas NETs used to be called islet cell tumors. Learn more about NET of the pancreas.
Insulinomas
These tumors release insulin, a hormone that lowers sugar levels in your blood. They may cause the following symptoms:
Glucagonomas
These tumors release glucagon, a hormone that raises sugar levels in your blood. They may cause the following symptoms:
Gastrinomas
These tumors release gastrin, a hormone that signals your stomach to produce digestive acids and enzymes. They may cause the following symptoms:
These tumors release vasoactive intestinal peptide, a substance that stimulates the release of water and electrolytes in your intestines. They may cause the following symptoms:
Abdominal pain and cramping
Dehydration
Somatostatinomas
These tumors release somatostatin, a hormone that stops the release of other hormones, including gastrin, insulin, and glucagon. They may cause the following symptoms:
Neuroendocrine tumors that do not release hormones are called "nonfunctional." These tumors in your pancreas may cause the following symptoms:
NETs can also begin in other organs. In about 15% of cases, a primary site cannot be found. Sometimes, NETs may develop in or on the adrenal glands. These rare types of NETs are called pheochromocytoma and paraganglioma. Other types of tumors that begin in hormone-producing cells are also described in their own sections on Cancer.Net, including thyroid cancer, adrenal gland tumors, and pituitary gland tumors.
A tumor begins when the DNA of healthy cells is damaged, causing the cells to change and grow out of control, forming a mass. A tumor can be cancerous or benign. A cancerous tumor is malignant, meaning it can grow and spread to other parts of the body if it is not found early and treated. A benign tumor means the tumor can grow but will not spread. A benign tumor usually can be removed without it causing much harm.
It is difficult to tell if pheochromocytoma and paraganglioma are benign or malignant, even after surgically removing the tumor and examining it under a microscope. The only sure way to know if a pheochromocytoma or paraganglioma is malignant is if it has spread, called metastasis, or if it comes back, called a recurrence.
Pheochromocytomas and paragangliomas are considered a subset of a group of tumors called neuroendocrine tumors (NETs). A NET begins in the specialized cells of the body’s neuroendocrine system. These cells have traits of both hormone-producing endocrine cells and nerve cells. They are found throughout the body’s organs and help control many of the body’s functions. Hormones are chemical substances that are carried through the bloodstream to have a specific effect on the activity of other organs or cells in the body. All NETs have the potential to become metastatic. Most NETs take years to develop and grow slowly. However, some NETs can be fast-growing.
A pheochromocytoma is a rare NET of the adrenal glands. The body has 2 of these small, yellowish glands. One is on top of each kidney. Adrenal glands have 2 main parts that function separately: the outer adrenal cortex and the inner adrenal medulla. Each part produces a different set of hormones.
A pheochromocytoma begins in the chromaffin cells of the adrenal medulla. The cells release hormones called catecholamines during times of stress. Adrenaline and noradrenaline, which increase blood pressure and heart rate, are 2 of those catecholamines. A pheochromocytoma can cause uncontrolled surges of extra adrenaline and noradrenaline into the blood. Even though an estimated 90% of pheochromocytomas remain localized to the area they began, these hormone surges can still lead to life-threatening health problems, such as a stroke, heart attack, hemorrhage, or sudden death.
Most people develop a pheochromocytoma in 1 adrenal gland. Some people develop a tumor in both glands. There can also be multiple tumors in a gland. Pheochromocytomas usually grow slowly.
A pheochromocytoma is called a primary adrenal gland tumor because it starts inside an adrenal gland.
A paraganglioma is a rare NET that develops from the same type of cells that pheochromocytomas do. However, paragangliomas form outside the adrenal glands. These tumors are also called extra-adrenal paragangliomas. The majority of paragangliomas form in the abdomen. They can also form in other parts of the body, including near the carotid artery, along nerve pathways in the head and neck, and in the chest, heart, abdomen, pelvis, and bladder. Paragangliomas are less common than pheochromocytomas. An estimated 60% remain localized and 40% may spread, though that cannot be determined at the time of diagnosis or from the pathology evaluation Paragangliomas are typically slow growing.
Maintain a healthy lifestyle
Continue your daily routine
Your family members and caregivers will be there for you throughout this journey. Don't be afraid to ask them for help. They can help you with daily activities and also help you cope with your feelings. They understand that the most important thing is for them to be there for you.
Your loved ones can help:
Let the people in your life know how you feel and what you are going through. Asking them to be more involved can help you continue your regular routines.
