Neuroendocrine Tumors

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Neuroendocrine Tumors

The multidisciplinary team at Johns Hopkins is dedicated to providing the best cutting-edge care to our patients with neuroendocrine tumors of the pancreas.

Neuroendocrine tumors run in some families (called "familial" or "hereditary"), and some produce hormones that can cause symptoms (called "functional" or "syndromic"). In addition, neuroendocrine tumors can arise in many different organs, and some neuroendocrine tumors are aggressive, while others are relatively slow growing. Simply put, the complexity of managing patients with a neuroendocrine tumor requires the multidisciplinary expertise at centers such as Johns Hopkins.

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Our Team

Our team includes highly-experienced surgeons, radiologists, interventional radiologists, pathologists, medical oncologists, radiation oncologists, gastroenterologists and geneticists is supported by a dedicated team of nurses, nurse practitioners and physician assistants. Members of our team are recognized as international experts in their fields, and the group at Hopkins emphasizes teamwork in their approach to patient care.

One thing that is special about the multidisciplinary team approach at Johns Hopkins is that it is not a "one-size-fits-all" approach. Instead, we recognize that no two patients with a neuroendocrine tumor are alike, and we treat the patient who has a tumor, not just the tumor in isolation. Most importantly, we care about, and are dedicated to, our patients.

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About Neuroendocrine Tumors

Neuroendocrine tumors are treated very differently from other cancers in the body. The cells of neuroendocrine tumors are recognized as being "neuroendocrine" (having neuroendocrine differentiation) because they either produce a hormone or because the cells of the tumor contain small granules (called neuroendocrine granules) that are characteristic of normal cells that produce hormones.

Neuroendocrine tumors are important to recognize for four reasons:

Islets of Langerhans - illustration by Kyoungran Eun

1. Some neuroendocrine tumors are functional (they produce hormones)

Many, but not all, neuroendocrine tumors produce hormones and release these hormones into the blood stream. Neuroendocrine tumors that produce hormones are called "functional" or "syndromic."

Syndromic neuroendocrine tumors are those neuroendocrine tumors that cause clinical signs and symptoms because they produce and release a hormone into the blood stream. Hormones are chemicals made by cells that can stimulate other cells in the body into action. These hormones produced by neuroendocrine tumors can cause significant symptoms and complications that have to be managed. For example, “insulinoma” is a neuroendocrine tumor of the pancreas that, as the name suggests, releases the hormone insulin into the blood stream. The insulin released by insulinomas can act on other cells in the body to greatly lower blood sugar (glucose) levels.

2. Some Neuroendocrine Tumors Run in Families

Many, but not all, neuroendocrine tumors arise as a part of an inherited genetic predisposition. Neuroendocrine tumors that are caused by an inherited genetic predisposition are called familial. In these syndromes a person inherits a defective copy a a neuroendocrine-tumor causing gene from one parent. For example, individuals with the “multiple endocrine neoplasia, type 1 (MEN 1)” syndrome inherit a genetic change in the gene MEN1 that predisposes them to develop neuroendocrine tumors of the pancreas, parathyroid glands, pituitary and upper gastrointestinal tract. These inherited genetic predispositions are important to recognize because they have implications for other family members (they too may be at risk), and because neuroendocrine tumors can arise in more than one organ, and all of the at-risk organs should be monitored.

The management of patients with a neuroendocrine tumor therefore includes a good understanding of the role that family cancer history can play in these diseases.

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3. Neuroendocrine Tumors are Diagnosed Differently from Other Tumors of the Pancreas

The tools and tests used to diagnose neuroendocrine tumors are slightly different from those to diagnose the more common forms of pancreatic cancer. The specific tests can be used to diagnose neuroendocrine tumors range from specific blood tests that detect the hormones released by neuroendocrine tumors, to specific imaging tests that take advantage of the differences between neuroendocrine tumors and other tumor types.

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4. Neuroendocrine Tumors are Treated Differently from Other Tumors of the Pancreas

Neuroendocrine tumors are treated in very specific ways. Because some neuroendocrine tumors grow slowly, many neuroendocrine tumors are treated surgically. Other therapies specifically target unique receptors made by neuroendocrine tumors.

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News & Updates

FDA Approves Lutetium for the Treatment of Neuroendocrine Tumors

January 2018

In an exciting advance, the FDA today approved Lutetium for the treatment of neuroendocrine tumors, including neuroendocrine tumors of the pancreas. Lutetium is a peptide receptor radionucleotide therapy. It targets somatostatin receptor positive neuroendocrine tumors. The approval is based on a randomized phase 3 clinical trial.

News Archive ▼

April 2014

In the April 2014 issue of the Journal of the American College of Surgeons, Dr. Choti and colleagues reported that the genetic change of "alternative lengthening of telomeres (ALT+)," can be used to predict the organ of origin in patients who have a neuroendocrine tumor in their liver. In some patients it can be hard to determine if a neuroendocrine tumor in the liver originated in the pancreas, or, for example, in the small bowel. Choti and colleagues used a molecular test for ALT and found that those tumors that originated in the pancreas and spread to the liver were more likely to have ALT than were tumors that arose in the small bowel and spread to the liver. Although a research test, it is hoped that one day this approach will help patients with a metastatic neuroendocrine tumor of unknown origin. PMID: 24655849

April 2014

Collaborators from Johns Hopkins and Memorial Sloan-Kettering Cancer Center reported in the April 2014 issue of the American Journal of Surgical Pathology their experience treating patients with aggressive ("poorly differentiated") neuroendocrine tumors of the pancreas. The authors studied 44 patients with poorly differentiated neuroendocrine tumors and found that they did significantly worse than did patients with well-differentiated (low grade) neuroendocrine tumors. Eighty-eight percent of the patients with poorly differentiated neuroendocrine tumors metastatic disease at presentation, and an additional 7% later developed metastases. Follow-up information was available for 43 patients; 33 died of disease, with an average survival of only 11. The 5-year survival rate was 16.1%. This study highlights the need to accurately grade neuroendocrine tumors, and suggests that patients with poorly differentiated neuroendocrine tumors need to be treated aggressively. PMID: 24503751

November 2013

In the November 2013 issue of the American Journal of Surgical Pathology, Dr. Ralph Hruban and colleagues from the team at Johns Hopkins reviewed the microscopic grading of 297 pancreatic neuroendocrine tumors. Grading of these tumors is performed microscopically and is determined by how fast the tumor cells are dividing. The team found that the combination of two methods for determining how fast the tumor cells are dividing (the "mitotic rate" and the "Ki67 labeling index") was better than either method alone. This study highlights the importance of good pathological review in the evaluation of neuroendocrine tumors. PMID: 24121170

October 2013

Dr. Joanna Law and colleagues at Johns Hopkins report a new way to localize small neuroendocrine tumors of the pancreas in the October issue of Surgical Endoscopy. In this paper Dr. Law and colleagues show that small “fiducials” placed at the time of endoscopic ultrasound can help guide surgeons to the precise location of small neuroendocrine tumors. This more precise localization has the potential for increasing the precision of surgery and reducing the amount of pancreatic tissue that needs to be resected. PMID: 23636530

May 2013

Dr. Trevor Ellison and colleagues reported in the Annals of Surgery the 26-year experience at Johns Hopkins on the surgical treatment of pancreatic neuroendocrine tumors. In this tour de force, Dr. Ellison and colleagues found that tumor grade (how fast the tumor cells are dividing) is the most important predictor of long-term outcome for patients with a neuroendocrine tumor of the pancreas. Importantly, they propose a simple prognostic tool that integrates grade and patient sex. This study reinforces the importance of tumor grade, and highlights the extraordinary experience the Hopkins team has in treating neuroendocrine tumors. PMID: 23673766

February 2013

The team at Johns Hopkins has successfully sequenced all known human genes in a series of medullary carcinomas of the thyroid. As reported in the Journal of clinical endocrinology, Nishant Agrawal and colleagues at Johns Hopkins, in a real tour de force, sequenced the exomes (all 21,000 known human genes) of a series of 17 clinically well-characterized medullary cancers of the thyroid. They then validated their findings in a series of 40 additional cancers. The authors found that the genes RET, HRAS and KRAS are frequently mutated (the DNA is altered abnormally) in these cancers. This study is important because it defines the genetic abnormalities driving the formation of medullary cancers of the thyroid. J Clin Endocrinol Metab. 2013 Feb;98(2):E364-9. PMID: 23264394

February 2013

In the February 2013 issue of the journal, The American Journal of Surgical Pathology, Dr. Chad McCall and colleagues from the Multidisciplinary Neuroendocrine Tumor Team at Johns Hopkins reported that the correct microscopic grading of pancreatic neuroendocrine tumors plays an important role in establishing the prognosis for patients. Dr. McCall and his colleagues found that it is important that pathologists use "Ki-67 stain" to assess tumor grade, in addition to the “old-fashioned” method of simply counting dividing cells (mitoses). PMID: 24121170

August 2012

Dr. Siva Raman and colleagues in Radiology at Johns Hopkins report in the August 2012 issue of the American Journal of Radiology on the imaging appearance of pancreatic neuroendocrine tumors, as well as a number of mimics on computerized tomography (CT). They demonstrate that pancreatic neuroendocrine tumors have a distinct appearance on imaging, and emphasize that experience is needed because a number of other entities can mimic the appearance of a neuroendocrine tumor radiologically. PMID: 22826391

March 2011

In an exciting breakthrough, Dr. Y. Jiao and colleagues at Johns Hopkins reported in the March 2011 issue of Science that they successfully sequenced the exomes (all 21,000 known human genes) in a series of pancreatic neuroendocrine tumors. This represents a major advance in our understanding of the gene changes that drive these tumors. Dr. Jiao and colleagues discovered a new cancer pathway (ATRX and DAXX mutations leading to the alternative lengthening of telomeres) and several gene changes (mutations) that may be targetable (treatable) with currently available drugs. PMID: 21252315

An inherited genetic variation in DNA that you are born with

Third Degree Relatives - First cousins, great-aunts and uncles

Second Degree Relatives - Aunts, uncles, grandparents, nieces and nephews

First Degree relatives - Blood relatives in your immediate family: parents, children, and siblings

Also known as a pancreatoduodenectomy, the Whipple procedure is the surgery typically performed to remove cancers of the head of the pancreas (the part of the pancreas on the right side of your body). It typically involves the surgical removal of the head of the pancreas, a portion of the duodenum and a portion of the bile ducts.

This is an experimental type of treatment. It is a medication made of killed or weakened cells, organisms or manufactured materials, which is used to boost the body's immune system. Ideally, this will allow the body to fight and kill the cancer cells more effectively. Vaccines include whole killed cancer cells or specific proteins from the cancer.

Unable to be surgically removed. This usually means that the cancer has spread beyond the areas that can be removed surgically.

The part of the pancreas that bends backwards, hooking around two very important blood vessels, the superior mesenteric artery and vein. The word "uncinate" comes from the word uncus which means "hook."

A painless procedure in which high frequency sound waves are used to generate pictures of the inside of the body. An ultrasound devise can be placed at the end of a scope, and the scope inserted into the duodenum, providing very detailed pictures of the pancreas. This is called "endoscopic ultrasound."

This term simply refers to a "mass" or neoplasm. For example, a collection of pus is a tumor. This is a general term that can refer to either benign or malignant growths.

A clot within the blood vessels. It may occlude (block) the vessel or may be attached to the wall of the vessel without blocking the blood flow.

An inflammation of the veins accompanied by thrombus formation. It is sometimes referred to as Trousseau's sign.

The long thin part of gland in the left part of abdomen, near the spleen.

A slender hollow tube inserted into the body to relieve a blockage. For example, pancreas cancers often grow into the bile duct as the bile duct passes through the pancreas. This can block the flow of bile and cause the patient to become jaundiced. In these cases the flow of bile can be reestablished by placing a stent into the bile duct, through the area of blockage.

Excessive amounts of fat in the stool. Sometimes this can appear as an oil slick on top of the toilet water after the patient has had a bowel movement. It can be a sign that the pancreas isn't functioning well.

A classification system that is used to describe the extent of disease. Clinicians use it to predict the likely survival of a patient.

A flat, scale-like cell. Although most pancreatic cancers look like ducts under the microscope, a small fraction look like squamous cells.

A maroon colored, rounded organ in the upper left part of the abdomen, near the tail of the pancreas. This organ is part of your immune system and filters the lymph and blood in your body. It is often removed during the distal pancreatectomy surgical procedure.

A long (20 foot) tube that stretches from the stomach to the large intestine. It helps absorb nutrients from food as the food is transported to the large intestine. There are three sections: the duodenum, the jejunum and the ileum. Due to its proximity to the pancreas, the duodenum is the section of the small intestine most often affected by pancreatic cancer.

An infection of the blood. This can be life-threatening and is often treated with antibiotics.

A malignant tumor that looks like connective tissues (bone, cartilage, muscle)under the microscope. Sarcomas are extremely rare in the pancreas.

Able to be removed surgically. Usually this means that the cancer is confined to areas typically removed surgically.

The use of high-energy waves similar to x-rays to treat a cancer. Radiation therapy is usually used to treat a local area of disease and often is given in combination with chemotherapy.

A thick ring of muscle (a sphincter) between the stomach and duodenum. This sphincter helps control the release of the stomach contents into the small intestine.

A forecast for the probable outcome of a disease based on the experience of large numbers of other patients with similar stage disease. Importantly, making a prognosis is not an exact science. Some patients with poor prognosis beat the odds and live longer than anyone would have predicted. Steve Dunn's Cancer Guide has an excellent article on statistics and prognoses and stories of other cancer patients.

A cancer in the organ where it started in. A primary cancer of the pancreas is one that started in the pancreas as opposed to a cancer that started somewhere else and only later spread to the pancreas.

The biochemical study of plants; concerned with the identification, biosynthesis, metabolism of chemical constituents of plants; especially in regards to natural products.

Around the ampulla of Vater in the duodenum. The peri-ampullary region is comprised of 4 structures; the ampulla, the duodenum, the bile duct and the head of the pancreas. It is sometimes difficult to tell which structure a tumor originated in. In such cases the diagnosis will be a peri-ampullary tumor.

A medical doctor specially trained to study disease processes. Pathologists make the microscopic diagnosis that is used to establish the diagnosis of cancer.

A term used to describe certain tumors which grow in finger-like projections. Pathologists use this term to describe some precancerous lesions in the pancreas (intraductal papillary mucinous neoplasm).

Any treatment that reduces the severity of a disease or its symptoms. Palliative care is often a part of the treatment plan for patients with advanced pancreatic cancer.

An oblong organ located between the stomach and the spine. The pancreas secretes enzymes needed for the digestion of food and it produces hormones such as insulin and glucagon which help control blood sugar.

A surgically created opening in an organ that can also be referred to as an anastamosis. Sometimes when surgeons remove a segment of bowel they create an ostomy to allow for the bowel contents to exit the body.

A medical doctor who specializes in the treatment of tumors. Oncologists often treat patients with pancreatic cancer with chemotherapy.

An abnormal new growth of tissue that grows more rapidly than normal cells and will continue to grow if not treated. These growths will compete with normal cells for nutrients. This is a general term that can refer to benign or malignant growths. It is a synonym for the word tumor.

Chemotherapy and radiation therapy that is given to patients before surgery. Some centers feel that the use of neoadjuvant therapy improves local and regional control of disease and that it may make more patients surgical candidates.

The thin section of the pancreas between the head and the body of the gland.

An alteration in the DNA of a cell. Think of it as a typographically error in the DNA code.

A cancer that has spread from one organ to another. Pancreas cancer most frequently metastasizes to the liver. In general, cancers that have metastasized are generally not treated surgically, but instead are treated with chemotherapy and/or radiation therapy.

A cancer that has the potential of invading nearby tissues, spreading to other organs (metastasizing) and possibly leading to the patient's death.

A painless method for visualizing internal organs. A tube-like machine with a powerful magnet generates images of the inside of the body. It does not involve the use of Xrays.

Normal, round, raisin to grape-sized collections of lymphocytes (white blood cells) found throughout the body. Lymph nodes are connected to each other by lymphatic vessels. They normally help fight infection, but also are one of the first sites to which cancers spread. In general, the spread of cancer to lymph nodes portends a worse prognosis for the patient. There are exceptions to this.

A primary pancreatic cancer that has spread to regional lymph nodes and/or resectable (removable) tissues. Removable tissues include some lymph nodes and parts of the duodenum and stomach that are routinely removed in some surgical treatments for pancreatic cancer.

The largest organ in the body, located in the right upper part of the abdomen. It performs many life-maintaining functions including the production of bile. The liver detoxifies the blood of drugs, alcohol and other harmful chemicals. It processes nutrients absorbed by the intestine and stores essential nutrients, vitamins and minerals. Bilirubin is a chemical produced when old or damaged blood cells breakdown. The liver chemically process the bilirubin so that it can dissolve in water and be excreted through the urine. When this process is disrupted, jaundice can develop.

A technique that surgeons can use to visualize and even biopsy (take tissue samples of) organs inside of the abdomen without making large incisions. Very small incisions are made in the belly and small tubes (called trocars) are then inserted. Gas is pumped in through one of the tubes to create enough space to work in. The surgeon inserts a small camera through one of the tubes and examines the lining and contents of the abdominal cavity by looking at the projected image on the television screen. With specially designed laparascopic instruments, biopsies and fluid samples can be taken for examination. Some surgeons feel that this technique can help "stage" a patient less invasively than with open surgery.

Yellowing of the skin or yellowing of the whites of the eyes caused by the accumulation of bile pigments (usually due to an obstruction of the bile ducts).

A hormone produced by the endocrine cells of the islets of Langerhans cells of the pancreas. Insulin acts to lower blood sugar levels.

A term used to indicate that cancerous cells are present in the duct but have not yet invaded deeper tissues.

The widest part of the pancreas. It is found in the right part of abdomen, nestled in the curve of the duodenum, which forms an impression in the side of the pancreas.

A hormone produced by the endocrine (islets of Langerhans) cells of the pancreas. When blood sugar levels are low, glucagon acts to raise blood sugar levels.

Gemzar is the trade name for the chemotherapy drug gemcitabine. It is frequently used to treat pancreatic cancer. It has been shown, in controlled clinical trials, to improve quality of life.

A green pear-shaped organ located on the right side of the abdomen just under the liver. The gallbladder is essentially a reservoir for holding bile.

A chemotherapeutic drug commonly used to treat pancreatic cancer.

The exocrine cells (acinar cells) of the pancreas produce and transport chemicals that will exit the body through the digestive system.

The chemicals that the exocrine cells produce are called enzymes. They are secreted in the duodenum where they assist in the digestion of food.

A test used to visualize and examine the pancreas and bile ducts. A tube is inserted through a patient's nose (or throat), down through the esophagus and stomach then into the small intestine (duodenum). There, a small probe is inserted into the ampulla of Vater. A dye is injected through the probe and into the pancreatic and bile ducts. X-rays are then taken to visualize the pancreatic and bile ducts. these ducts can be seen as white structures (this is because the injected dye is opaque). Because pancreas cancers often block the pancreatic and/or bile ducts, this technique can be useful in establishing a diagnosis of pancreas cancer.

A chemical that causes a reaction in other substances, in this case as a part of the digestive process.

A medical doctor who specializes in the treatment of hormonal abnormalities.

These are specialized cells that produce hormones released into the bloodstream. For example, the islets of Langerhans are endocrine cells in the pancreas that produce the hormone insulin. This hormone helps control blood sugar(glucose) levels.

Some rare tumors of the pancreas, the endocrine (Islet Cell) tumors, can produce these same hormones. It is very important that these rare tumors be properly diagnosed because it will determine the treatment and prognosis.

Surgical removal of a structure or part of a structure. For example, pancreatectomy is the surgical removal of the pancreas (or a portion of it).

The first portion of the small intestine. It is about 1 foot long. It is the part of the intestinal track that comes after the stomach.

A small anatomic structure. This is essentially a tube that carries various bodily fluids. The pancreatic duct runs the full length of the pancreas and drains into the duodenum.

The chemical in every cell that carries genetic information.

A dome shaped muscle that separates the lungs and heart from the abdomen. This muscle assists in breathing.

The disease in which the body is unable to appropriately control blood sugar (glucose) levels. This may be caused by failure of the pancreas to produce adequate amounts of insulin.

A fluid filled sac. Some tumors of the pancreas, including the serous cystadenomas and intraductal papillary mucinous neoplasms, form cysts. Cysts have a distinct appearance in CT scans. They are important to recognize because the treatment of cystic tumors can differ from that for solid tumors.

A dye, taken by mouth or injected, that is sometimes used during x-ray examinations to highlight areas that otherwise might not be seen.

A way to image internal organs. A series of x-ray pictures taken by a machine that encircles the body like a giant tube. Computers are then used to generate cross-sectional images of the inside of the body.

The treatment of a cancer by chemicals. For pancreatic cancer these include: Gemzar (Gemcitabine), 5-flurouracil, leukovorin, taxol, and others.

A small, flexible tube inserted into the body to inject or suck out fluids.

A cancer-causing chemical. Cigarette smoke contains a number of carcinogens.

A malignant tumor. It has the potential of invading into the adjacent tissues, spreading to other organs and may eventually lead to the patient's death.

A dramatic weight loss and general wasting that occurs during chronic disease.

A blood marker for pancreas cancer. It is not a good screening test for diagnosing possible pancreas cancers in individuals without symptoms. Instead, it can be useful in following the progress of patients known to have a cancer by measuring how their cancer is responding to treatment.

The middle part of the pancreas between the neck and the tail. The superior mesenteric blood vessels run behind this part of the gland.

The removal and microscopic examination of a small tissue sample.

A duct that carries bile from the liver to the intestine. This term may refer to the hepatic, cystic or common bile duct.

A green fluid produced by the liver that helps digest fats. It is transported from the liver to the duodenum by the bile duct. When the flow of bile is blocked, patients may become jaundiced (yellow skinned).

Tumors which are non-cancerous. These generally grow slowly and do not invade adjacent organs or spread (metastasize) beyond the pancreas.

The collection of excess amounts of fluid in the abdominal cavity (belly). It often is a sign that the cancer has spread to either the liver or to the portal vein that goes to the liver, or that the cancer involves the internal lining of the abdomen. If normal liver function is affected, a complex set of biochemical checks and balances is disrupted and abnormal amounts of fluid are retained.

The large artery that carries oxygen-rich blood from the heart. From the heart it arches backwards and descends into the abdomen where it gives off many branches to supply the organs. The superior mesenteric artery is a major branch of the aorta that can be involved by pancreatic cancer.

A radiographic technique used to visualize blood vessels. A contrast medium (a dye) is usually injected into the vessels to make them appear white on the x-rays.

A condition marked by a diminished apetite and aversion to food. Often results in physical signs of wasting.

A condition characterized by a deficiency in red blood cells. This can lead to fatigue among other symptoms.

A surgical joining of two hollow structures. It is similar to attaching two ends of a garden hose. For example, a gastrojejunostomy is a surgical procedure that connects the stomach and the jejunum (small intestine.)

This widening of the pancreatic duct as it reaches the duodenum is an landmark for physicians. It is where the bile duct and pancreatic duct join before draining into the duodenum (small intestine). Tumors in the head of the pancreas may squeeze this duct partially or completely closed. This can lead to problems with digestion and jaundice.

Chemotherapy given to patients after their cancers have been surgically removed. It is a secondary treatment given to supplement surgical treatment. (see Neoadjuvant chemotherapy which is chemotherapy given before surgery)

A benign (non-cancerous) tumor made up of cells that form glands (collections of cells surrounding an empty space).

The form of cancer that most people are talking about when they refer to "cancer of the pancreas." These tumors account for 75% of all pancreas cancers.

Microscopically, adenocarcinomas form glands. These tumors can grow large enough to invade nerves which can cause back pain. They also frequently spread (metastasize) to the liver or lymph nodes. If this happens the tumor may be considered unresectable.

A pus-filled cavity. Usually caused by an infection.

The portion of the body between the diaphragm and the pelvis.