All About Carcinoid and Neuroendocrine Tumors

Eric Shinohara, MD, MSCI
Updated by: Lara Bonner Millar, MD
Abramson Cancer Center of the University of Pennsylvania
Last Modified: June 28, 2016

What is a Carcinoid Tumor?

The neuroendocrine system is comprised of cells that are spread throughout several organ systems, including the lungs and digestive system. They are called neuroendocrine cells because they have characteristics of both nerve and endocrine cells. Nerve cells use electrical impulses to transmit signals. Endocrine cells make up glands, such as the thyroid and pancreas, that secrete hormones (thyroid hormone and insulin), which can be used to communicate with other organs. Neuroendocrine tumors start in the cells of these hormone producing organs.

A carcinoid tumor is a type of neuroendocrine tumor, which most commonly arises in the gastrointestinal system (esophagus, stomach, intestines). About 10% of carcinoid tumors arise in the lungs. Because carcinoid tumors originate from hormone producing tissues, many of the side effects of carcinoid tumors can be linked to the inappropriate release of various hormones into the blood stream.

Carcinoid tumors can arise in a number of locations, which are categorized into three areas: the foregut, midgut and hindgut. The foregut includes the lungs and the stomach, the midgut is comprised of the small intestine, appendix, and the beginning of the large bowel (colon), and the hindgut is composed of the end of the large bowel, the rectum and the organs of the genitourinary tract (ovaries, testes).

What causes carcinoid tumor and am I at risk?

Carcinoid tumors appear to be slightly more common in women than men. It is most commonly diagnosed in people over the age of 60. It is more commonly found in the African American population. About 4,000 new cases of lung carcinoid tumors are 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 a year have been increasing as a result of better testing to diagnose these tumors.

There are genetic syndromes that can be associated with carcinoid syndrome. The first is 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. The genetic abnormality that causes Von Hippel-Lindau syndrome increases the risk of developing carcinoid in the pancreas. A condition called neurofibromatosis increases the risk for carcinoid tumor of the stomach. Tuberous sclerosis is another rare condition that can increase risk for carcinoid tumor development. A history of atrophic gastritis, which causes long term inflammation of the stomach lining, puts you at increased risk for carcinoid of the stomach.

How can I prevent carcinoid tumors?

Presently, there is no way to prevent carcinoid tumors. There may be an association between carcinoid tumors and smoking, hence 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 available?

There are no specific screening tests or schedules for carcinoid tumor at this time.

What are the signs of Carcinoid Tumor?

In the early stages of growth, carcinoid tumors rarely present any signs or symptoms of their existence. The signs typically don’t arise until the tumor has started to grow or there is metastasis to another organ. The signs of carcinoid tumor vary based on the organ from which they arise. Generally, early tumors are found incidentally during an operation or procedure for another disease, such as during an appendectomy or a colonoscopy. If symptoms are present, they tend to be relatively vague, such as abdominal discomfort. Symptoms that cause people to seek medical attention are uncommon. In the small intestine, carcinoid tumors 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 rectal exam and can cause rectal pain or bleeding. Carcinoid tumors tend to be slow growing, which also contributes to the slow evolution of symptoms. This leads to long intervals between the start of symptoms and diagnosis.

The most distinctive symptom of carcinoid tumors, known as carcinoid syndrome, occurs in about 10% of people with carcinoid tumor. Carcinoid syndrome is made up of a pattern of symptoms that are caused by the release of a variety of substances (serotonin, histamine, and substance P, among others) by the carcinoid tumor. These symptoms can be precipitated 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 liver metastasis develops. 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, on occasion, cause carcinoid syndrome.

Carcinoid syndrome includes:
  • Flushing and telangiectasias: Most commonly flushing occurs in the face, but can affect the trunk as well, causing a reddish or purplish appearance to the skin. 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 potent vasodilator (substances which open up blood vessels). Histamines also have vasodilatory effects. Telangiectasias are collections of tiny blood vessels which can develop superficially 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 be accompanied by abdominal cramps and pain. The severity of the diarrhea is variable, occurring only a few times a day or up to 30 times or more a day. It appears to be related to serotonin secretion by the carcinoid tumor. Serotonin is a neurotransmitter normally made by the body, however in carcinoid syndrome it is produced in great excess. Serotonin causes increased secretion as well as increased motility of the gut.   
  • Rapid heartbeat (tachycardia) and decreased blood pressure (hypotension): Dilation of blood vessels can lead to a rapid heartbeat and a drop in blood pressure. Dilation of blood vessels may also be responsible for the flushing associated 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 an asthmatic may experience. It can cause wheezing and difficulty breathing and may be associated with facial flushing. It may be related to the secretion of bradykinin or serotonin, but this is still being studied.
  • Pellagra: Pellagra usually manifests 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 associated with a niacin (vitamin B3) deficiency, which is caused by a deficiency of tryptophan, an essential amino acid that can be converted into niacin. Tryptophan is normally used to make serotonin (a neurotransmitter) in the body, however in people with 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 blood stream damages the heart, leading to lesions which cause fibrosis, particularly of the heart valves. This generally 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 symptoms include swelling (edema) in the extremities and enlargement of the heart.

Not all patients with carcinoid tumors will present with symptoms of carcinoid syndrome. The specific symptoms of carcinoid tumors of each location in the body will be highlighted in the treatment section.

How is carcinoid tumor diagnosed?

Generally, carcinoid tumors are found incidentally during an operation or a procedure. For instance, during surgery to remove the appendix it may be found that the patient has a carcinoid tumor of the appendix. However, if a person has symptoms, which are suspicious for carcinoid syndrome, tests can be ordered to confirm the cause.

One of the first tests ordered is usually a urine test to detect serotonin breakdown products. In particular, the compound 5-hydroxyindoleacetic acid (5-HIAA) is measured in a 24 hour collection of the person's urine. The person being tested must avoid a number of foods and medications during the testing period which can falsely elevate or decrease the 5-HIAA level. Normally 2 to 8 mg/day of 5-HIAA is found in urine, however, up to 30 mg/day can be found in people with malabsorptive disease or who have recently eaten tryptophan rich foods (chocolate, certain types of cheeses). Generally people with carcinoid syndrome have 5-HIAA values of greater than 100 mg/day, though not always. 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 detecting 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.

If the urine test fails to produce a diagnosis, blood tests can be performed. The blood test is used to determine 5-HIAA blood levels while the person is fasting. 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, among others, however these tests are not available at all hospitals.

Generally, if the 5-HIAA blood test fails to produce a diagnosis, an epinephrine (adrenaline) provocation test can be performed. In this test, the physician tries to induce symptoms associated with carcinoid syndrome by giving intravenous (IV) epinephrine. This test can confirm that a carcinoid tumor is causing the person's carcinoid syndrome symptoms nearly 100% of the time. The person's blood pressure, heart rate and facial color are closely monitored as epinephrine is given IV. The physician starts with 2 micrograms and goes up by 2 micrograms every five minutes until symptoms develop or a dose of 10 micrograms is reached. People with a carcinoid tumor will experience flushing, increased heart rate and decreased blood pressure at one of these doses and these symptoms generally last for one to two minutes. The test is stopped after the person first experiences these symptoms.

Another test uses pentagastrin, a polypeptide that can stimulate the secretion of gastric acid and pepsin (a digestive enzyme). Intravenous pentagastin is given and flushing symptoms are identified. The advantage of this test is that it can be used to detect not only midgut and hindgut tumors, but also foregut tumors, unlike the tests mentioned previously.

Imaging tests can be used to locate the tumor, once the diagnosis of carcinoid tumor has been established using one of the above mentioned. Alternatively, imaging studies may be the initial studies ordered to detect the tumor, particularly in cases where carcinoid syndrome is not present. Based on the suspected location of the tumor, several tests can be used. 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.

Endoscopy can also be used to directly visualize tumors using a special camera attached to a flexible tube, allowing the physician to reach the esophagus and stomach. A colonoscopy is done in a similar fashion and can be used to examine the rectum and colon directly. Bronchoscopy can be used to examine the airways of the lungs for tumors. Another special type of scope (camera) has an ultrasound probe at the end of it (called EUS or endoscopic ultrasound). The ultrasound uses sound waves to detect how deep a tumor has invaded normal tissue. Biopsies can be taken during any of these exams and examined in the labratory 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 arise. 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.

Several different types of 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 and is “taken up” by the carcinoid tumor. Special scanners then detect the radioactive substance to identify where the compound- and tumor - is. An example of this is the indium-111 octreotide scan, which can be used to find the tumor. Octreotide is similar to somatostatin, a compound which can bind to somatostatin receptors, which are found on carcinoid tumors. However, there are other types of tumors, which can express somatostatin receptors; hence this study is not 100% specific for carcinoid tumors. The indium-111 is a radioactive compound, which is attached to the somatostatin, allowing the localization of the octreotide to be determined using a special scanner. An older scan, the I-131 MIBG scan, works in a similar fashion and can be used to locate the tumor. MIBG accumulates 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. Hence 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.

How is carcinoid tumor staged?

In the past, carcinoid tumors did not have a formal staging system. They were staged as either localized, which means that the tumor has not spread beyond the wall of the organ or place of origin; regional, which is when the tumor has spread beyond the confines of the organ to adjacent tissues or lymph nodes, or distant metastatic spread, which indicates that 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 appropriate treatment options. Please see the end of this article for complete staging.

How are carcinoid tumors treated?

The treatment for carcinoid tumors is dependent upon where the tumor is, the general health of the person and the person’s medical history. Generally, the treatment of choice for people with localized carcinoid is surgical excision of the tumor. The role of chemotherapy and radiotherapy has been debated, particularly when a complete surgical resection is performed. Treatment information and symptoms of each type of carcinoid tumor will be discussed by location.

Foregut

Symptoms associated with foregut carcinoids include peptic ulcers, abdominal pain and intestinal bleeding. 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. Surgical resection of the lesion is the treatment of choice for this tumor. Usually only a small portion of the lung needs to be removed, however in larger lesions an entire lobe of the lung may need to be removed. In the event that there has been regional spread to the lymph nodes, an extensive lymph node dissection will also be required to optimally treat the tumor. Rarely, the tumor will be localized to only the interior of the airway passage. In this very select group of people the tumor may be resected via bronchoscopy. Bronchoscopy can also be used to guide a laser to destroy tumor in individuals, who are having trouble breathing because the tumor has blocked an airway. Generally, this is not a curative procedure and is only used in people who can not 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 leading to loss of the glandular cells of the stomach) and pernicious anemia (anemia due to a B12 deficiency). Tumors which are less than 1 cm in size can usually be treated with endoscopic resection with close endoscopic surveillance every 6-12 months. For tumors larger than 1 cm or if there are more than five lesions, a portion 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 increased secretion of gastrin, resulting in increased secretion of hydrochloric acid in the stomach. MEN type I is one of three types of hereditary syndromes which affect endocrine tissues (hormone producing tissues). In MEN type I, the parathyroid, the pancreas, and the pituitary 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 suppression of 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 choice of treatment is also surgical resection of the tumor.
Midgut

Symptoms associated with midgut carcinoids include vague abdominal pain, diarrhea, nausea, vomiting, fatigue, and obstruction of the intestinal tract. They are associated with carcinoid syndrome approximately 10% of the time. The gut can fold in on itself, where a portion of the gut slides into another portion 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 arise towards 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 to the lymph nodes and liver, even when small, though the risk of metastasis does increase for larger tumors. The surgeon generally 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 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 are generally asymptomatic; only when the tumor causes obstruction leading to appendicitis or if there are metastases, are symptoms generally found. Appendicitis is generally rare as most carcinoid tumors are in the end of the appendix, not close to the opening, and hence obstruction tends to be rare. Nonetheless, about 1 in 300 people who undergo appendectomies have carcinoid tumors. 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 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. Occasionally, there can be rectal bleeding or pain. Carcinoid syndrome is rare. The extent of surgery necessary to treat rectal cancer is unclear. For smaller tumors, fulguration (burning of the tumor) can be used to treat the tumor. Larger lesions or ones that have crossed through the wall of the rectum have been treated with complete resection of the rectum, using the same surgical techniques used for other rectal cancers. 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 arise 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 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, which have 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 comes in the form of high energy x-rays. These x-rays are similar to those used for diagnostic x-rays, but they are of a much high 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. Additionally, 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 exploits this difference to treat cancers by killing cancer cells, while killing fewer cells in normal, healthy tissue.Radiation therapy is rarely used in the curative treatment of carcinoid and is generally used to treat metastasis that may develop in the bones or other parts of the body to relieve pain.

Chemotherapy

Chemotherapy refers to medications that are usually given intravenously or in pill form. Chemotherapy travels throughout the bloodstream and throughout 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 the chance killing them, while radiation does not. Unfortunately, no single chemotherapy has been found to be effective. Clinical trials that use combinations of chemotherapies may be of greater efficacy. Generally 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 streptozocin, 5-fluorouracil (5-FU), cisplatin, etoposide, and doxorubicin, among others.

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. Radio frequency 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 inhibit 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, octereotide and lanreotide are used. They function similarly to somatostatin but are more potent inhibitors. There are also formulations which are long acting which allow longer intervals between injections of the drug. Most of the side effects occur 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 that control the symptoms of carcinoid syndrome are commonly used. Medications which boost the immune system may also be used to slow tumor growth.Interferons are compounds which regulate the activity of the immune system. Interferons are usually produced by the body to fight viral or bacterial infections; hence 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, and H1 antagonist which is a drug which blocks histamine and serotonin is effective at relieving symptoms of carcinoid syndrome. The most common side effect is drowsiness. At times a person will 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, causing 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 performed using an ultrasound (sound waves) machine to determine how well the heart is functioning and can be used to monitor the heart's condition. If heart damage has occurred, medications which 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, particularly the legs (edema) and as it progresses 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 heart beat to make it more efficient

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 given. Generally, your physician may have you follow up every 3-6 months to determine 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 if symptoms develop warranting an immediate imaging study. Your physician may also order an echocardiogram periodically to evaluate heart function. Other tests, such as blood tests for serotonin and other products produced by carcinoid tumors, may also be checked regularly.

Cancer survivorship is a relatively new focus of oncology care. With some 15 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, care-givers 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/

 

Complete Staging Charts

AJCC 7th Edition (7th ed., 2010)

 

Stomach, TNM

Primary Tumor (T)

TX

Primary tumor cannot be assessed

T0

No evidence of primary tumor

Tis

Carcinoma in situ/dysplasia (tumor size less than 0.5mm), confined to mucosa

T1

Tumor invades lamina propria or submucosa and 1cm or less in size

T2

Tumor invades muscularis propria or more than 1cm in size

T3

Tumor penetrates subserosa

T4

Tumor invades visceral peritoneum (serosal) or other organs or adjacent structures.

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

 

Regional Lymphnodes (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

 

Duodenum/Ampulla/Jejunum/Ileum, TNM

Primary Tumor (T)

TX

Primary tumor cannot be assessed

T0

No evidence of primary tumor

T1

Tumor invades lamina propria or submucosa and size 1cm or less (sm. Intestine tumors); tumor 1cm or less (ampullary tumors)

T2

Tumor invades through the muscularis propria or size greater than 1cm (small intestinal tumors); tumor greater than 1cm (ampullary tumors)

T3

Tumor invades through the muscularis propria into subserosal tissue without penetration of overlying serosa (jejunal or ileal tumors) or invades pancreas or retroperitoneum (ampullary or duodenal tumors) or into non-peritonealized tissues

T4

Tumor invades visceral peritoneum (serosa) or invades 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 metastasis

 

Distant Metastases (M)

M0

No distant metastases

M1

Distant metastasis

 

Colon or Rectum, TNM

Primary Tumor

TX

Primary tumor cannot be assessed

T0

No evidence of primary tumor

T1

Tumor invades lamina propria or submucosa and size 2cm or less

T1a

Tumor size less than 1cm in greatest dimension

T1b

Tumor size 1-2cm in greatest dimension

T2

Tumor invades muscularis propria or size more than 2cm with invasion of lamina propria or submucosa

T3

Tumor invades through the muscularis propria into the subserosa, or into the non-peritonealized pericolic or perirectal tissues

T4

Tumor invades the peritoneum 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 metastasis

 

Distant Metastases (M)

M0

No distant metastases

M1

Distant metastasis

 

Anatomic Stage/Prognostic Groups

Stage 0

Tis

N0

M0

Stage I

T1

N0

M0

Stage IIA

T2

N0

M0

Stage IIB

T3

N0

M0

Stage IIIA

T4

N0

M0

Stage IIIB

Any T

N1

M0

Stage IV

Any T

Any N

M1

 

Pancreatic, TNM

Primary Tumor (T)

TX

Primary tumor cannot be assessed

T0

No evidence of primary tumor

Tis

Carcinoma in situ

T1

Tumor limited to the pancreas, 2cm or less in greatest dimension

T2

Tumor limited to the pancreas, more than 2cm in greatest dimension

T3

Tumor extends beyond the pancreas but without involvement of the celiac axis or the superior mesenteric artery

T4

Tumor involves the celiac axis or the superior mesenteric artery (unresectable primary tumor)

 

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

 

Anatomic Stage/Prognosis

Stage 0

Tis

N0

M0

Stage IA

T1

N0

M0

Stage IB

T2

N0

M0

Stage IIA

T3

N0

M0

Stage IIB

T1

T2

T3

N1

N1

N1

M0

M0

M0

Stage III

T4

Any N

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 2cm or less in greatest dimension

T1a

Tumor 1cm or less in greatest dimension

T1b

Tumor more than 1cm but not more than 2cm

T2

Tumor more than 2cm but not more than 4cm or with extension to the cecum

T3

Tumor more than 4cm or with extension to the ileum

T4

Tumor directly invades other adjacent organs or structures

 

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

 

Anatomic Stage/Prognostic Groups

Stage I

T1

N0

M0

Stage II

T2, T3

N0

M0

Stage III

T4

Any T

N0

N1

M0

M0

Stage IV

Any T

Any N

M1

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

References

American Cancer Society, Detailed Guide: Gastrointestinal Carcinoid Tumors.

Carcinoid Tumor: Diagnosis. Cancer.net. 2015. Found at: http://www.cancer.net/cancer-types/carcinoid-tumor/diagnosis

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 1.2015. 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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