All About Carcinoid (Neuroendocrine) Tumors

Autor: OncoLink Team
Contribuidor de contenido: Eric Shinohara, MD, MSCI
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What is a carcinoid tumor? 

A carcinoid tumor is a type of tumor that forms in the neuroendocrine system. The neuroendocrine system is made up of cells that are spread out over many organs in our body. They are called neuroendocrine cells because they act like both nerve and endocrine cells: 

  • Nerve cells use electrical impulses to send signals. 
  • Endocrine cells make up glands, such as the thyroid and pancreas. These glands secrete (release) hormones like thyroid hormone and insulin. These hormones can be used to communicate with other organs. Neuroendocrine tumors start in the cells of these organs that make hormones.

Carcinoid tumors commonly start in the gastrointestinal system (esophagus, stomach, intestines). Because carcinoid tumors come from hormone-producing tissues, many of the side effects of carcinoid tumors can be linked to incorrect levels of different hormones in the body.

Carcinoid tumors can start in a number of places in the body, which are broken down into three areas: 

  • Foregut: The lungs and the stomach.
  • Midgut: The small intestine, appendix, and the beginning of the large bowel (colon).

Hindgut: The end of the large bowel, the rectum, and the organs of the genitourinary tract (ovaries, testes).

What causes a carcinoid tumor and am I at risk?

Carcinoid tumors:

  • Seem to be slightly more common in women than men. 
  • Are most commonly diagnosed in people over the age of 60. 
  • Are more commonly found in the African American population. 

There are about 4,000 new cases of lung carcinoid tumors diagnosed each year and about 8,000 new cases of carcinoid tumors of the gastrointestinal system. Recent studies suggest that the number of cases of carcinoid tumors diagnosed each year has been increasing as a result of better testing to diagnose these tumors.

There are genetic syndromes that can be associated with carcinoid syndrome:

  • Multiple Endocrine Neoplasia (MEN) Type I: A rare genetic syndrome in which 10% of people affected by it develop carcinoid tumors. 
  • Zollinger-Ellison Syndrome: Can occur on its own or as part of MEN type I. This is a condition in which the lining of the stomach overproduces acid and increases the risk of carcinoid and other tumors. 
  • Von Hippel-Lindau Syndrome: Increases the risk of developing carcinoid in the pancreas.
  • Neurofibromatosis: Increases the risk for a carcinoid tumor of the stomach. 
  • Tuberous sclerosis: Can increase the risk for carcinoid tumor development. 
  • Atrophic gastritis: Causes long term inflammation of the stomach lining, putting you at increased risk for carcinoid of the stomach.

How can I prevent carcinoid tumors?

There is no known way to prevent carcinoid tumors. There may be a link between carcinoid tumors and smoking, so not starting or quitting smoking may reduce the risk of developing carcinoid tumors. Maintaining a healthy diet is a way to prevent many cancers, and possibly carcinoid tumors.

What screening tests are used for carcinoid tumors?

There are no specific screening tests for carcinoid tumors at this time.

What are the signs of carcinoid tumor?

In the early stages of growth, carcinoid tumors rarely have any signs or symptoms of their existence. The signs often don’t arise until the tumor has started to grow or there is metastasis (spread) to another organ. The signs of carcinoid tumors vary based on the organ from which they arise. Generally, early tumors are found incidentally (by chance) during an operation or procedure for another disease, such as during an appendectomy or a colonoscopy. If symptoms are present, such as abdominal discomfort, they tend to be vague. Symptoms that cause people to seek medical attention are uncommon. 

  • Carcinoid tumors in the small intestine: Can cause obstruction or intestinal bleeding.
  • Carcinoids of the colon: Can also cause intestinal bleeding. 
  • Carcinoids of the rectum (very end of the colon prior to the anus where stool is stored): Can be found during a rectal exam and can cause rectal pain or bleeding. 

Carcinoid tumors tend to be slow-growing, which causes the symptoms to be slow to show themselves. This leads to long time periods between the start of symptoms and diagnosis.

The most distinctive symptom of carcinoid tumors is known as carcinoid syndrome. Carcinoid syndrome is made up of a pattern of symptoms that are caused by the release of different substances (serotonin, histamine, and substance P, among others) by the carcinoid tumor. These symptoms can be brought on by certain foods, such as those high in tyramine, which is found in blue cheese and chocolates, and those that contain ethanol, such as wine and beer. 

Carcinoid syndrome most commonly affects people with carcinoid tumors of the small bowel. However, these tumors do not cause the symptoms associated with carcinoid syndrome until cancer has spread to the liver (metastasized). This occurs because excess hormones produced by carcinoid tumors in the intestine are usually broken down by the liver. Carcinoids of the lung and other non-digestive tract organs can also sometimes cause carcinoid syndrome. Not all patients with carcinoid tumors will have symptoms of carcinoid syndrome. 

Carcinoid syndrome includes:

  • Flushing and telangiectasias: Most commonly flushing occurs in the face, but can affect the trunk as well, causing the skin to look reddish or purplish. With carcinoid syndrome, you will not sweat with flushing but will remain dry. Flushing can last minutes to hours and may become permanent. It is thought to be caused by the release of tachykinin or histamine by the carcinoid tumor. The most common tachykinin is substance P, which is a strong vasodilator (substances which open up blood vessels). Histamines also have vasodilatory effects. Telangiectasias are collections of tiny blood vessels which can develop close to the surface of the skin on the faces of people who have had carcinoid tumors for several years. They are most commonly found on the nose or upper lip and are purplish in color. They are thought to be due to chronic vasodilatation.
  • Diarrhea: Diarrhea, which can be extremely watery and explosive in nature, occurs in about 78% of people with carcinoid syndrome and may come with abdominal cramps and pain. The severity of diarrhea varies, occurring only a few times a day or up to 30 times or more a day. It appears to be related to serotonin release by the carcinoid tumor. Serotonin is a neurotransmitter normally made by the body, however in carcinoid syndrome, too much is produced. Serotonin causes increased secretion as well as increased motility of the gut.   
  • Rapid (Fast) heartbeat (tachycardia) and decreased blood pressure (hypotension): Dilation (opening) of blood vessels can lead to a rapid heartbeat and a drop in blood pressure. Dilation of blood vessels may also cause the flushing that comes with carcinoid syndrome. These symptoms may be caused by bradykinin, which acts as a blood vessel dilator.
  • Bronchospasm (Closing off of the airways): Symptoms from bronchospasm are similar to those a person with asthma may have. It can cause wheezing and a hard time breathing and may be linked with facial flushing. It may be related to the secretion of bradykinin or serotonin, but this is still being studied.
  • Pellagra: Pellagra usually shows as dry, scaly skin (dermatitis), dry cracked skin at the edges of the mouth (stomatitis), swollen tongue (glossitis), diarrhea and mental confusion (dementia). Pellagra is most commonly linked with low niacin (vitamin B3) levels, which is caused by low levels of tryptophan, an essential amino acid that can be converted into niacin. Tryptophan is used to make serotonin (a neurotransmitter) in the body, however, in carcinoid syndrome, so much tryptophan is being converted into serotonin that tryptophan levels decrease to the point where pellagra can occur.
  • Right-sided heart disease or failure: It is thought that high levels of serotonin in the bloodstream damages the heart. This can lead to lesions that cause fibrosis, particularly of the heart valves. This affects the right side of the heart when liver metastases are present. The left side of the heart is usually not affected because the lungs can break down serotonin. Right-sided heart failure can cause swelling (edema) in the arms and legs and enlargement of the heart.

How is a carcinoid tumor diagnosed?

Carcinoid tumors are often found incidentally (by chance) during an operation or a procedure. For example, during surgery to remove the appendix, it may be found that you have a carcinoid tumor of the appendix. However, if you have symptoms that are suspicious for carcinoid syndrome, tests can be ordered to confirm the cause. Some tests include:

24-hour urine collection test: One of the first tests ordered is usually a urine test to find serotonin breakdown products. 

  • The compound 5-hydroxyindoleacetic acid (5-HIAA) is measured in a 24-hour collection of your urine. 
  • You must avoid a number of foods and medications during the testing period. Some medications and foods can falsely raise or lower the 5-HIAA level. 
  • Normally 2 to 8 mg/day of 5-HIAA is found in urine. If you have carcinoid syndrome, you might have 5-HIAA values of greater than 100 mg/day. People with metastatic carcinoid tumors, but without carcinoid syndrome, tend to have elevated 5-HIAA levels, yet generally have lower levels than those with carcinoid syndrome. 
  • This test may not be useful for finding carcinoid tumors of the foregut (stomach and lungs) as they often do not produce 5-HIAA. In these cases, imaging studies may be useful, which will be discussed later.

Blood tests: If the urine test fails to produce a diagnosis, blood tests can be performed. 

  • The blood test is used to measure 5-HIAA blood levels while you are fasting (having not eaten for a number of hours). 
  • Normal values vary from laboratory to laboratory but are generally between 101-283 ng/ml. 
  • Other blood tests can be used including tests for chromogranin A, bradykinin, kallikrein, and substance P. These tests are not available at all cancer centers.

Epinephrine (Adrenaline) Provocation Test: If the 5-HIAA blood test does not show a diagnosis, an epinephrine (adrenaline) provocation test can be performed. 

  • In this test, the healthcare provider tries to bring on symptoms linked with carcinoid syndrome by giving intravenous (IV) epinephrine. 
  • This test can confirm that a carcinoid tumor is causing your carcinoid syndrome symptoms almost 100% of the time. 
  • Your blood pressure, heart rate, and facial color are closely watched as epinephrine is given in your IV. The provider starts with 2 micrograms and goes up by 2 micrograms every five minutes until symptoms develop or a dose of 10 micrograms is reached.
  • If you have a carcinoid tumor, you will have flushing, increased heart rate, and decreased blood pressure at one of these doses. These symptoms often last for one to two minutes.
  • The test is stopped after you first experience these symptoms.

Pentagastrin Test: Pentagastrin is a polypeptide that can make your body secrete gastric acid and pepsin (a digestive enzyme). 

  • Intravenous (IV) pentagastrin is given and flushing symptoms are identified. 
  • This test can be used to detect not only midgut and hindgut tumors but also foregut tumors, unlike the other tests.

Imaging tests can be used to locate the tumor, once the diagnosis of carcinoid tumor has been made using one of the tests above. In cases where carcinoid syndrome is not present, imaging studies may be the first studies ordered to find the tumor. 

  • Barium studies can be used to find tumors; they coat the digestive tract and can outline tumors which can then be seen on X-ray. 
    • Barium swallows are used to locate tumors in the upper digestive tract such as the in the esophagus, stomach, and the first part of the small intestine. 
    • Barium enemas work in a similar fashion, with contrast injected into the rectum, and can be used to examine the rectum and colon for tumors. CT scans can be used to detect tumors throughout the body and CT guided needle biopsies can sometimes be used to obtain a biopsy for examination in the lab. When a carcinoid is biopsied or resected and examined under the microscope, it may be classified as either "typical" or "atypical" depending on certain features. Typical carcinoids tend to be less aggressive than atypical carcinoids.

Scans can also be used to identify where the tumor is located, and if it has spread. 

  • These scans use a compound, which is known to attach to carcinoid tumors. This compound is then attached to a radioactive substance. This combination is then injected intravenously (IV, into a vein) and is “taken up” by the carcinoid tumor. 
  • Special scanners then detect the radioactive substance to identify where the compound and tumor is. 
    • There are 2 types of scans that use a radioactive material that is attached to octreotide. This then attached to somatostatin receptors on NET cells. A PET scan is then able to "see" these areas.
    • An older scan, the I-131 MIBG scan, works in a similar fashion and can be used to locate the tumor. MIBG gathers in the carcinoid tumor and the attached I-131 can be detected using a scanner to localize the tumor. However, like the indium-111 octreotide scan, other neuroendocrine tumors can also be detected. This test is also not specific for carcinoid tumors. 
    • A PET scan using 5-hydroxytryptophan, a compound which is taken up by carcinoid tumors can also be used to identify where a tumor is located and if it has spread anywhere. Results from these imaging studies can be combined with CT scan results to better locate tumors.

Procedures to View Tumors: Once a tumor has been found, either incidentally during another surgery or with an imaging, urine, or blood test, your provider may suggest a different kind of procedure to better view the tumor. These may include:

  • Endoscopy can also be used to directly see tumors using a special camera attached to a flexible tube, allowing the provider to reach the esophagus and stomach.
  • Colonoscopy is done much like endoscopy and can be used to examine the rectum and colon directly. 
  • Bronchoscopy can be used to examine the airways of the lungs for tumors. 
  • EUS or endoscopic ultrasound uses a special type of scope (camera) that has an ultrasound probe at the end of it. The ultrasound uses sound waves to find how deep a tumor has invaded normal tissue.

Biopsies can be taken during any of these procedures and tested in the laboratory to identify carcinoid tumors. Unfortunately, none of these studies are very good at examining the majority of the small intestine, which is where many carcinoid tumors start. 

How is carcinoid tumor staged?

In the past, carcinoid tumors did not have a formal staging system. They were staged as either:

  • Localized- The tumor has not spread beyond the wall of the organ or place of origin.
  • Regional- The tumor has spread beyond the confines of the organ to adjacent tissues or lymph nodes. 
  • Distant metastatic spread- The tumor has spread to distant organs and tissues.

Now, the American Joint Committee on Cancer Staging (AJCC) has included carcinoid in a formal staging system which includes the stomach, duodenum, ampulla, jejunum, ileum, colon/rectum, pancreas, and appendix. Although there is now a staging system, the classification of tumors into localized, regional, or metastatic is still relevant in terms of thinking about prognosis and treatment options. Please see the end of this article for complete staging.

How are carcinoid tumors treated?

The treatment for carcinoid tumors depends on where the tumor is, your general health, and medical history. The treatment of choice for people with localized carcinoid is often surgery to remove the tumor. The role of chemotherapy and radiotherapy has been debated, particularly when a complete surgical resection is done. Treatment information and symptoms of each type of carcinoid tumor will be discussed by location.

Foregut

Symptoms of foregut carcinoids include stomach ulcers, abdominal (belly) pain, and bleeding in your intestines. They are rarely associated with carcinoid syndrome.

  • Bronchial: People can present with cough, coughing up of blood (hemoptysis), wheezing, chest pain, or pneumonia. These tumors appear to be more common in women and are rarely associated with carcinoid syndrome. Tumors tend to be small, however, there are often multiple tumors. Surgery to remove the lesion is the treatment of choice for this tumor. Usually, only a small portion of the lung needs to be removed, but sometimes with larger lesions, an entire lobe of the lung may need to be removed. If there has been regional spread to the lymph nodes, a lymph node dissection will also be needed. Rarely, the tumor will be localized to only the interior of the airway passage. If this happens, the tumor may be resected with bronchoscopy. Bronchoscopy can also be used to guide a laser to destroy the tumor if you are having trouble breathing because the tumor has blocked your airway. Generally, this is only used in people who cannot have surgery.
  • Stomach: Carcinoids of the stomach can be broken into three types, type 1 through 3. 
    • Type 1 gastric carcinoids are associated with atrophic gastritis (chronic inflammation of the stomach) and pernicious anemia (anemia due to low levels of B12). Tumors that are less than 1 cm in size can often be treated with endoscopic resection with frequent endoscopies every 6-12 months. For tumors larger than 1 cm or if there are more than five lesions, a part of the stomach may need to be resected.
    • Type 2 gastric carcinoid tumors are associated with Zollinger-Ellison syndrome or multiple endocrine neoplasias (MEN) type 1. Zollinger-Ellison syndrome is a disease where there is too much secretion of gastrin, leading to increased secretion of hydrochloric acid in the stomach. MEN type I is one of three types of hereditary syndromes that affect endocrine tissues (hormone-producing tissues). In MEN type I, the parathyroid, the pancreas, and the pituitary gland are predisposed to tumor formation. Surgery to remove the antrum of the stomach, which releases gastrin, may be recommended to reduce gastrin secretion, leading to slowing or stopping tumor growth.
    • Type 3 gastric carcinoid tumors occur sporadically, meaning that they are not associated with other conditions, unlike type 1 and 2 gastric carcinoids. They also tend to produce serotonin, unlike type 1 and 2 gastric carcinomas, which can lead to carcinoid syndrome. The treatment is also surgical resection of the tumor.

Midgut

Symptoms associated with midgut carcinoids include vague abdominal (belly) pain, diarrhea, nausea, vomiting, fatigue, and obstruction (blockage) of the intestinal tract. They are associated with carcinoid syndrome about 10% of the time. The gut can fold in on itself, where a part of the gut slides into another part of the intestine (intussusception) resulting in obstruction of the intestine. The tumor itself may grow large enough to cause obstruction of the intestine as well.

  • Small intestine: Carcinoid tumors of the small intestine most commonly start near the end of the small intestine (terminal ileum), where it attaches to the colon. People can present with bowel obstruction or ischemic bowel (cutoff of the blood supply to the bowel). Carcinoid tumors of the small intestine tend to metastasize (spread) to the lymph nodes and liver. The surgeon often resects the area with the lesion as well as the regional lymph nodes (lymph nodes which drain the area the tumor was in). During the operation, the surgeon inspects the entire length of the bowel to check for multiple tumors. Even in people with metastatic disease, surgery is generally recommended to prevent symptoms, such as obstruction, from developing.
  • Colon: Carcinoid of the colon can cause decreased appetite, weight loss, and abdominal pain. Treatment is the surgical removal of the entire colon or in some cases local excision of the tumor. 
  • Appendix: Carcinoid tumors are the most common tumors of the appendix. As with most carcinoid tumors, nearly all are discovered incidentally during surgery. Carcinoid tumors of the appendix generally do not cause symptoms. When the tumor causes obstruction leading to appendicitis or if there are metastases, symptoms may be present. Appendicitis is rare, as most carcinoid tumors are in the end of the appendix, not close to the opening, so obstruction tends to be rare. In smaller tumors, appendectomy alone is sufficient to treat the tumor. If the tumor is larger, portions of the colon may need to be removed along with the appendix.

Hindgut

Symptoms associated with hindgut carcinoids are similar to other rectal cancers. Change in bowel habits, rectal bleeding and intestinal obstruction (blockage) can occur. Carcinoids of the hindgut are rarely associated with carcinoid syndrome. Hindgut tumors can also arise in the genitourinary system which includes the kidneys and gonads (ovaries or testes).

  • Rectal: Most of the tumors of the rectum are discovered during colonoscopy or during a digital rectal exam. There can sometimes be rectal bleeding or pain. Carcinoid syndrome is rare. The extent of surgery needed to treat rectal cancer is unclear. For smaller tumors, fulguration (burning of the tumor) can be used to treat the tumor. Larger lesions may need complete resection of the rectum. However, it is unclear if this improves survival compared with excision of the tumor alone, as many of these tumors have spread to other organs already.
  • Ovary: A majority of carcinoids of the ovary start as part of another ovarian tumor known as a teratoma. Teratomas are made up of tissues that are normally present in other organs such as bone, brain, lung, and liver, among others. Carcinoid tumors that arise in teratomas tend to have a better prognosis. Ovarian carcinoids can also cause carcinoid syndrome without liver metastasis.

Regional Spread 

When the tumor has spread to the lymph nodes around the primary region, surgery is usually attempted to remove all of the tumor as well as the nodes around the tumor that have the disease. If this is not possible, surgery is still often done to prevent symptoms from developing, such as obstruction of the intestine.

Distant Metastasis

Surgery cannot be used to cure the disease at this stage. Surgery is still used to prevent symptoms, such as obstruction, from developing but the focus is on treatments that can slow the progression of the disease and alleviate symptoms.

Radiation Therapy

Radiation therapy is the use of high energy x-rays. These x-rays are similar to those used for diagnostic x-rays, but they are of much higher energy. The high energy of x-rays in radiation therapy results in damage to the DNA of cells. Cancer cells divide faster than healthy cells, and so their DNA is more likely to be damaged than that of normal cells. Cancer cells are generally less able to repair damaged DNA than normal cells are, so cancer cells are killed more easily by radiation than normal cells are. Radiation therapy treats cancers by killing cancer cells while killing fewer cells in normal, healthy tissue. Radiation therapy is rarely used in the curative treatment of carcinoid. It may be used to treat metastasis that develops in the bones or other parts of the body, and to relieve pain caused by these metastases.

Chemotherapy

Chemotherapy refers to medications that are usually given intravenously (IV, into a vein) or in pill form. Chemotherapy travels throughout the bloodstream of the body to kill cancer cells. This is one of the big advantages of chemotherapy. If cancer cells have broken off from the tumor and are somewhere else inside the body, chemotherapy has a chance at killing them, while radiation does not. No single chemotherapy has been found to be effective. Clinical trials that use combinations of chemotherapies are being studied. The use of chemotherapy is limited to carcinoids, which have spread to other organs or are causing severe symptoms that are not responding to other medications. Some of the chemotherapies that are used include streptozocin5-fluorouracilcisplatinetoposide, and doxorubicin, among others. In some cases, immunotherapy or targeted therapy medications can be used. 

A directed form of chemotherapy, called chemoembolization, can be used to treat liver metastasis. A catheter (a hollow, flexible tube) is threaded through an artery that leads to the liver (intra-arterial therapy) and chemotherapy is delivered through the catheter to the arteries that feed the tumor. This allows a high dose of chemotherapy to be delivered directly to the tumor while sparing the rest of the body from high doses of chemotherapy. Another form of intra-arterial therapy delivers a compound which blocks off the artery along with the chemotherapy. Chemoembolization allows the direct delivery of chemotherapy but also cuts off the blood supply to the tumor. Radiofrequency ablation (RFA) can also be used to destroy liver tumors. A probe, which produces high-frequency radio waves, is placed in the tumor which can heat it, destroying the tumor.

Hormone Therapy

Several analogs of the hormone somatostatin are used to control the symptoms of carcinoid syndrome. Somatostatin regulates the endocrine system and can block the release of other hormones. It can be used to block the flushing, diarrhea, and wheezing associated with carcinoid syndrome. Most commonly, the somatostatin analogs octreotide and lanreotide are used.

They work much like somatostatin but are more powerful blockers. There are also long-acting forms that allow longer time periods between injections of the drug. Most of the side effects happen at the injection site; however, people can have nausea, vomiting, fatigue, cramps, and headaches. Generally, these drugs do not shrink tumors, but they can slow their growth and prolong survival.

Other Medications

Other treatments can control the symptoms of carcinoid syndrome. Medications that boost the immune system may also be used to slow tumor growth. Interferons are compounds that regulate the activity of the immune system. Interferons are usually produced by the body to fight viral or bacterial infections. Treatment with interferons can cause flu-like symptoms. They are delivered by injection and can sometimes shrink tumors and reduce carcinoid syndrome symptoms.

Recent studies have suggested that cyproheptadine helps relieve symptoms of carcinoid syndrome. Cyproheptadine is an H1 antagonist, which is a drug that blocks histamine and serotonin. The most common side effect is drowsiness. You may require more than an H1 antagonist and may also need an H2 antagonist to manage symptoms. An example is ranitidine, which can also be useful for treating itching and flushing related to carcinoid syndrome.

Treatment of the Heart

Damage to the heart can occur due to prolonged exposure to serotonin. This can cause problems with the heart valves and ultimately heart failure. Octreotide and other somatostatin analogs can be used to control serotonin levels to limit heart damage. Echocardiograms are done using an ultrasound (sound waves) machine to determine how well the heart is working and can be used to monitor the heart's condition. If heart damage has occurred, medications that are commonly used in heart failure can be used. Heart failure occurs when the heart is unable to circulate blood as effectively as a normal heart. This can cause fluid to build up in the body, especially the legs (edema). As it progresses, it can cause fluid to build up in the lungs as well (pulmonary edema). Treatments for this include diuretics (water pill), which draw excess water out of the body and medications to help regulate the heartbeat.

Clinical Trials

There are clinical research trials for most types of cancer, and every stage of the disease. Clinical trials are designed to determine the value of specific treatments. Trials are often designed to treat a certain stage of cancer, either as the first form of treatment offered or as an option for treatment after other treatments have failed to work. They can be used to evaluate medications or treatments to prevent cancer, detect it earlier, or help manage side effects. Clinical trials are extremely important in furthering our knowledge of this disease. It is through clinical trials that we know what we do today, and many exciting new therapies are currently being tested. Talk to your provider about participating in clinical trials in your area. You can also explore currently open clinical trials using the OncoLink Clinical Trials Matching Service.

Follow Up Care and Survivorship

Follow up testing varies widely depending on the location of the carcinoid tumor, the stage of the disease at diagnosis, and the initial treatment is given. Generally, your provider may have you follow up every 3-6 months to see if symptoms of carcinoid syndrome or recurrence have developed. Imaging studies such as CT or MRI scans are usually done every 6-12 months or sooner if symptoms develop. Your provider may also order an echocardiogram every so often to test heart function. Other tests, such as blood tests for serotonin and other products produced by carcinoid tumors, may also be checked regularly.

Fear of recurrence, financial impact of cancer treatment, employment issues, and coping strategies are common emotional and practical issues experienced by bladder cancer survivors. Your healthcare team can identify resources for support and management of these practical and emotional challenges faced during and after cancer.

Cancer survivorship is a relatively new focus of oncology care. With some 17 million cancer survivors in the US alone, there is a need to help patients transition from active treatment to survivorship. What happens next, how do you get back to normal, what should you know and do to live healthy going forward? A survivorship care plan can be a first step in educating yourself about navigating life after cancer and helping you communicate knowledgeably with your healthcare providers. Create a survivorship care plan today on OncoLink.

Resources for More Information

The Carcinoid Cancer Foundation.

Offers online education and resources for patients, caregivers, and health care professionals.

www.carcinoid.org

The North American Neuroendocrine Tumor Society (NANETS).

NANETS goal is to improve neuroendocrine tumor disease management through research. The site offers education and resources for patients.

https://www.nanets.net/

Appendix: Complete Staging Charts

AJCC 7th Edition (8th ed., 2017)

Stomach, TNM

Primary Tumor (T)

TX

Primary tumor cannot be assessed

T0

No evidence of primary tumor

T1

Invades the lamina propria or submucosa and less than or equal to 1 cm in size 

T2

Invades the muscularis propria or greater than 1 cm in size

T3

Invades through the muscularis propria into subserosal tissue without penetration of overlying serosa

T4

Invades visceral peritoneum (serosa) or other organs or adjacent structures 

(For any T, add (m) for multiple tumors).

Regional Lymph Nodes (N)

NX

Regional lymph nodes cannot be assessed

N0

No regional lymph node metastasis

N1

Regional lymph node metastasis

Distant Metastasis (M)

M0

No distant metastases

M1

Distant Metastasis

M1a

Metastasis confined to liver

M1b

Metastases in at least one extrahepatic site (e.g., lung, ovary, nonregional lymph node, peritoneum, bone)

M1c

Both hepatic and extrahepatic metastases 

Duodenum/Ampulla, TNM

Primary Tumor (T)

TX

Primary tumor not assessed

T1

Tumor invades the mucosa or submucosa only and is ≤1 cm (duodenal tumors); Tumor ≤1 cm and confined within the sphincter of Oddi (ampullary tumors) 

T2

Tumor invades the muscularis propria or is >1 cm (duodenal);
Tumor invades through sphincter into duodenal submucosa or muscularis propria, or is >1 cm (ampullary) 

T3

Tumor invades the pancreas or peripancreatic adipose tissue 

T4

Tumor invades the visceral peritoneum (serosa) or other organs 

(For any T, add (m) for multiple tumors).

Regional Lymph Nodes (N)

NX

Regional lymph nodes cannot be assessed

N0

No regional lymph node metastasis

N1

Regional lymph node involvement

Distant Metastases (M)

M0

No distant metastases

M1

Distant metastasis

M1a

Metastasis confined to liver 

M1b

Metastases in at least one extrahepatic site (e.g., lung, ovary, nonregional lymph node, peritoneum, bone) 

M1c

Both hepatic and extrahepatic metastases 

Jejunum/Ileum, TNM

Primary Tumor

TX

Primary tumor cannot be assessed

T0

No evidence of primary tumor

T1

Invades lamina propria or submucosa and less than or equal to 1 cm in size 

T2

Invades muscularis propria or greater than 1 cm in size 

T3

Invades through the muscularis propria into subserosal tissue without penetration of overlying serosa 

T4

Invades visceral peritoneum (serosal) or other organs or adjacent structures 

(For any T, add (m) for multiple tumors).

Regional Lymph Nodes

NX

Regional lymph nodes cannot be assessed

N0

No regional lymph node metastasis

N1

Regional lymph node metastasis less than 12 nodes 

N2

Large mesenteric masses (>2 cm) and/or extensive nodal deposits (12 or greater), especially those that encase the superior mesenteric vessels 

Distant Metastasis

M0

No distant metastases

M1

Distant metastasis

M1a

Metastasis confined to liver 

M1b

Metastases in at least one extrahepatic site (e.g., lung, ovary, nonregional lymph node, peritoneum, bone) 

M1c

Both hepatic and extrahepatic metastases 

Colon or Rectum, TNM

Primary Tumor

TX

Primary tumor cannot be assessed

T0

No evidence of primary tumor

T1

Tumor invades the lamina propria or submucosa and is ≤2 cm 

T1a

Tumor <1 cm in greatest dimension 

T1b

Tumor size 1-2cm in greatest dimension

T2

Tumor invades the muscularis propria or is >2 cm with invasion of the lamina propria or submucosa 

T3

Tumor invades through the muscularis propria into subserosal tissue without penetration of overlying serosa 

T4

Tumor invades the visceral peritoneum (serosa) or other organs or adjacent structures 

(For any T, add (m) for multiple tumors).

Regional Lymph Nodes (N)

NX

Regional lymph nodes cannot be assessed

N0

No regional lymph node metastasis

N1

Regional lymph node metastasis

Distant Metastases (M)

M0

No distant metastases

M1

Distant metastasis

M1a

Metastasis confined to liver 

M1b

Metastases in at least one extrahepatic site (e.g., lung, ovary, nonregional lymph node, peritoneum, bone)

M1c

Both hepatic and extrahepatic metastases 

Anatomic Stage/Prognostic Groups

Stage 1

T1

N0

M0

Stage IIA

T2

N0

M0

Stage IIB

T3

N0

M0

Stage IIIA

T4

N0

M0

Stage IIIB

T1

N1

M0

T2

N1

M0

T3

N1

M0

T4

N1

M0

Stage IV

TX, T0

Any N

M1

T1

Any N

M1

T2

Any N

M1

T3

Any N

M1

T4

Any N

M1

Pancreatic, TNM

Primary Tumor (T)

TX

Primary tumor cannot be assessed

T1

Tumor limited to the pancreas, <2 cm

T2

Tumor limited to the pancreas,* 2−4 cm

T3

Tumor limited to the pancreas,* >4 cm; or tumor invading the duodenum or common bile duct

T4

Tumor invading adjacent organs (stomach, spleen, colon, adrenal gland) or the wall of large vessels (celiac axis or the superior mesenteric artery)

Regional Lymph Nodes (N)

NX

Regional lymph nodes cannot be assessed

N0

No regional lymph node metastasis

N1

Regional lymph node involvement

Distant Metastases (M)

M0

No distant metastases

M1

Distant metastasis

M1a

Metastasis confined to liver 

M1b

Metastases in at least one extrahepatic site (e.g., lung, ovary, nonregional lymph node, peritoneum, bone)

M1c

Both hepatic and extrahepatic metastases 

Anatomic Stage/Prognosis

Stage I

T1

N0

M0

Stage II

T2

N0

M0

T3

N0

M0

Stage III

T4

N0

M0

Any T

N1

M0

Stage IV

Any T

Any N

M1

Appendix, TNM

Primary Tumor (T)

TX

Primary tumor cannot be assessed

T0

No evidence of primary tumor

T1

Tumor 2 cm or less in greatest dimension

T2

Tumor more than 2 cm but less than or equal to 4 cm

T3

Tumor more than 4 cm or with subserosal invasion or involvement of the mesoappendix

T4

Tumor perforates the peritoneum or directly invades other adjacent organs or structures (excluding direct mural extension to adjacent subserosa of adjacent bowel), e.g., abdominal wall and skeletal muscle

Regional Lymph Nodes (N)

NX

Regional lymph nodes cannot be assessed

N0

No regional lymph node metastasis

N1

Regional lymph node metastasis

Distant Metastases (M)

M0

No distant metastases

M1

Distant metastasis

M1a

Metastasis confined to liver 

M1b

Metastases in at least one extrahepatic site (e.g., lung, ovary, nonregional lymph node, peritoneum, bone)

M1c

Both hepatic and extrahepatic metastases 

Anatomic Stage/Prognostic Groups

Stage I

T1

N0

M0

Stage II

T2, T3

N0

M0

Stage III

T1

T2

T3

T4

T4

N1

N1

N1

N0

N1

M0

M0

M0

M0

M0

Stage IV

TX, T0

T1

T2

T3

T4

Any N

M1

(Found at https://www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf)

Referencias

American Cancer Society, Detailed Guide: Gastrointestinal Carcinoid Tumors.

Carcinoid Tumor Guide. Cancer.net. 2017. Found at: https://www.cancer.net/cancer-types/carcinoid-tumor

Kunz PL et al. Consensus Guidelines for the Management and Treatment of Neuroendocrine Tumors. Pancreas. 2013 May. 42(4):557-577.

National Comprehensive Cancer Network. Neuroendocrine Tumors. Version 2.2018. Found at: http://www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf

National Institute of Health. National Cancer Institute. Gastrointestinal Carcinoid Tumors Treatment-Patient Version (PDQ ®). 2016. Found at: http://www.cancer.gov/types/gi-carcinoid-tumors/patient/gi-carcinoid-treatment-pdq#section/_57

National Institute of Health, U.S. National Library of Medicine. Medline Plus. Carcinoid Syndrome. 2014. Found at: https://www.nlm.nih.gov/medlineplus/ency/article/000347.htm

Oberg K, et al. Nuclear medicine in the detection, staging and treatment of gastrointestinal carcinoid tumors. Best Practice & Research Clinical Endocrinology and Metabolism. 2005. 19(2):265-276.

Santacroce L et al. Malignant Carcinoid Syndrome Treatment and Management. Medscape. 2016.

The North American Neuroendocrine Tumor Society (NANETS). Found at: https://www.nanets.net/

Vinik A et al. NANETS Consensus Guidelines for the Diagnosis of Neuroendocrine Tumor. Pancreas. 2010. 39(6): 713-734. 

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