Caring for Carcinoid Foundation - Treating Carcinoid

"As the arbiter of high-quality cancer care, NCCN promotes the importance of continuous quality improvement and recognizes the significance of creating clinical practice guidelines appropriate for use by patients, clinicians, and other health care decision-makers. The primary goal of all NCCN initiatives is to improve the quality, effectiveness, and efficiency of oncology practice so patients can live better lives."

NCCN treatment guidelines cover these cancers:

Carcinoid Tumors

Multiple Endocrine Neoplasia, Type 1

Multiple Endocrine Neoplasia, Type 2

Islet Cell Tumors

Pheochromocytoma

Poorly Differentiated (High Grade or Anaplastic)/Small Cell/Atypical Lung Carcinoids

Treatment Guidelines - NCCN

Gut

Gut is a peer review journal for health professionals and researchers in gastroenterology and hepatology:

"Gut is a leading international journal in gastroenterology and has an established reputation for publishing first class clinical research of the alimentary tract, the liver, biliary tree and pancreas. Gut delivers up-to-date, authoritative, clinically oriented coverage of all areas in gastroenterology. Regular features include articles by leading authorities, reports on the latest treatments for diseases, reviews and commentaries."

Treatment guidelines - Gut

Additional Sources

Guidelines for the Management of Gastroenteropancreatic Neuroendocrine Tumours - Nordic Neuroendocrine Tumor Group (Part I)

Guidelines for the Management of Gastroenteropancreatic Neuroendocrine Tumours - Nordic Neuroendocrine Tumor Group (Part II)

Guidelines for the Diagnosis and Treatment of Neuroendocrine Gastrointestinal Tumors: A Consensus Statement on Behalf of the European Neuroendocrine Tumor Society (ENETS) - Neuroendocrinology (abstract)

Approach to Selecting Treatment

The American Cancer Society recommends the following approach to selecting treatment for carcinoid:

"After the carcinoid tumor is found and staged, the cancer care team will suggest one or more treatment plans. This is an important decision. It is also important for you to take time and think about all of the choices.

The main factors in selecting treatment options for gastrointestinal carcinoid tumors are the size and location of the tumor, whether it has spread to lymph nodes, liver, bones or other organs, whether there are any other serious medical conditions, and whether the tumor is causing bothersome symptoms.

It is often a good idea to seek a second opinion. A second opinion may provide more information and help the patient feel more confident about the treatment plan that is chosen."

Gastrointestinal Carcinoid Tumors: Treatment - National Cancer Institute

Considerations Concerning a Tailored, Individualized Therapeutic Management of Patients with (Neuro)endocrine Tumors of the Gastrointestinal Tract and Pancreas - Endocrine-Related Cancer

Diagnosis and Treatment of Carcinoid Tumors - Expert Review of Anticancer Therapy (abstract)

Therapeutic Options for Gastrointestinal Carcinoids - Clinical Gastroenterology and Hepatology (abstract)

Treatment Options

Chemotherapy

Chemotherapy options, according to the American Cancer Society, are the following:

"Chemotherapy uses anti-cancer drugs that are injected into a vein or a muscle or taken by mouth to kill cancer cells. These drugs enter the bloodstream and reach all areas of the body (called systemic treatment), making this treatment useful for some types of cancers that have spread or metastasized to organs other than the one where they started growing.

Unfortunately, carcinoid tumors are often not very sensitive to chemotherapy. Because of this, chemotherapy is generally used only for carcinoid tumors that have spread to other organs, are causing severe symptoms, and have not responded to other medications. Some of the chemotherapy drugs used in this situation include 5-fluorouracil (5-FU), doxorubicin, etoposide, dacarbazine, streptozotocin, cisplatin, and cyclophosphamide. Many cancers are treated with combinations of chemotherapy drugs. But in carcinoid tumors, using more than one drug has not been shown to be any more effective than using a single drug.

Chemotherapy drugs kill cancer cells but also damage some normal cells. Therefore, your doctors will pay careful attention to avoiding or minimizing side effects. These depend on the type of drugs, amount taken, and length of treatment. Temporary side effects might include nausea and vomiting, loss of appetite, loss of hair, and mouth sores. Because chemotherapy can damage the blood-producing cells of the bone marrow, you may have low blood cell counts. This can result in an increased risk of infection (due to a shortage of white blood cells), bleeding or bruising after minor cuts or injuries (due to a shortage of blood platelets), and fatigue or shortness of breath (due to low red blood cell counts).

Some side effects disappear within a few days after treatment. In addition, there are medicines that can help prevent or minimize treatment side effects. For example, your doctor can prescribe drugs to help prevent or reduce nausea and vomiting.

When the carcinoid tumor has spread to the liver, it is sometimes treated by directly injecting the chemotherapy drug into the artery that supplies blood to the liver. This approach exposes the liver metastases to high doses of the drug and limits exposure of the rest of the body. This can avoid many of the side effects described above. Sometimes the chemotherapy drug is injected together with a material that plugs up the artery. When the arteries leading to them are blocked, the tumors become starved for nutrients and oxygen and many die off. This can be more effective when combined with chemotherapy. This combined approach is called chemoembolization."

Chemoembolization - RadiologyInfo

Selective Hepatic Artery Embolization for Treatment of Patients With Metastatic Carcinoid and Pancreatic Endocrine Tumors - Cancer Control

Interferon

Interferon options, according to the American Cancer Society, are the following:

"These are naturally occurring substances that normally activate the body's immune system. They also slow the growth of tumor cells. Alpha-interferon is helpful in occasionally shrinking some metastatic carcinoid tumors, slowing the growth of many others, and improving symptoms of carcinoid syndrome. Its usefulness is sometimes limited by its flu-like side effects, which may be severe. The drug is given by injection."

Octreotide (Sandostatin) and Lanreotide

Octreotide (Sandostatin) and Lanreotide options, according to the American Cancer Society, are the following:

"Several medications are available for controlling the symptoms of carcinoid syndrome (problems arising from the release of substances produced by some of these tumors and found through blood and urine tests) in patients with metastatic carcinoid tumors. Octreotide and lanreotide are agents chemically related to a natural hormone, somatostatin. They are very helpful in treating the flushing, diarrhea, and wheezing from carcinoid syndrome. Although these drugs rarely shrink carcinoid tumors, they often slow or stop their growth. Although this is not curative, it can prolong life.

The main side effects of these medications are pain at the site of the injection, and rarely, stomach cramps, nausea, vomiting, headaches, dizziness, and fatigue. These drugs have become available in long-acting injections that need to be given only once a month. These longer acting preparations may help patients more than the short acting ones. A recent study found that patients taking them lived longer than patients on the short acting preparations."

How Sandostatin Works - Novartis (video)

Sandostatin Support Guide - Novartis

Sandostatin Dosing Flow Chart - Novartis

Sandostatin LAR: Q & A - Novartis

Sandostatin LAR: Injecting with Success - Novartis

Order free video of Sandostatin LAR: Injecting with Success - Novartis

Consensus Report on the Use of Somatostatin Analogs for the Management of Neuroendocrine Tumors of the Gastroenteropancreatic System - Annals of Oncology

Radiation

Radiation options, according to the American Cancer Society, are the following:

"External-beam radiation therapy: This is the type of radiation used most often for most types of gastrointestinal cancer. It is like having a regular x-ray except it takes longer and involves much higher amounts of radiation. Patients typically have treatments for 5 days a week for several weeks. Unfortunately, radiation therapy often is not very effective against most gastrointestinal carcinoid tumors. It is used primarily to treat pain from carcinoid tumors that have spread to the bones or other parts of the body.

The main side effects of gastrointestinal radiation therapy are fatigue (tiredness), nausea, vomiting, diarrhea, and mild temporary, sunburn-like skin changes."

Radiofrequency Ablation

Radiofrequency ablation, according to the American Cancer Society, is a procedure to destroy liver metastases:

"These methods are often useful in destroying carcinoid metastases that have spread to the liver, especially if the number or location of the liver metastases makes surgical removal difficult or impossible. CT scan images are used to guide a needle precisely into the tumor deposits. The cells are then destroyed by injecting concentrated alcohol through the needle, or liquid nitrogen can be used to cool the needle and kill the carcinoid cells by freezing. One new approach, called radiofrequency ablation, uses high-energy radio waves for treatment. A thin, needle-like probe temporarily placed into the tumor releases these radio waves. Placement of the probe is accurately guided by CT scans. The probe releases high frequency alternating current that destroys the cancer cells."

Radiofrequency Ablation: How It Works - Cleveland Clinic

Radiofrequency Ablation: A Treatment Option for Inoperable Liver Cancer - Cleveland Clinic

Treating Cancer with Radiofrequency Ablation - Society of Interventional Radiology

Radiofrequency Ablation of Liver Tumors - RadiologyInfo

Selective Internal Radiation Therapy (SIRT)

Selective Internal Radiation Therapy (SIRT), according to University of Maryland Greenebaum Cancer Center, is a promising new treatment option:

"Selective Internal Radiation Therapy (SIRT) is a revolutionary treatment for advanced liver cancer that utilizes new technologies to deliver radiation directly to the site of tumors.

Conventional radiotherapy can only be applied to limited areas of the body, and it adversely affects nearby tissues. SIRT, on the other hand, involves the delivery of millions of microscopic radioactive spheres, called SIR-Spheres�, directly to the site of the liver tumors, where they selectively irradiate the tumors. The targeted nature of SIRT enables doctors to deliver up to 40 times more radiation to the liver tumors than would be possible using conventional radiotherapy.

The anti-cancer effect is concentrated in the liver and there is little effect on cancer at other sites such as the lungs or bones."

About Selective Internal Radiation Therapy - Greenebaum Cancer Center

About SIR-Spheres� Microspheres - Sirtex

Selective Internal Radiation Therapy with SIR-Spheres� in Patients with Nonresectable Liver Tumors - Cancer Biotherapy & Radiopharmaceuticals

Surgery

Surgery options, according to the American Cancer Society, are the following:

Local excision: This operation removes the primary tumor and some surrounding normal tissue. The edges of the defect are then sewn together. This usually doesn�t cause any prolonged problem with eating or bowel movements. This operation is usually done for small carcinoid tumors (no larger than 2 centimeters, or about 3/4 inch). The most common example of this is when an appendectomy is done and the carcinoid tumor is discovered after the surgery.
Electrofulguration: This treatment destroys a tumor by heating it with electric current. It is sometimes used for small rectal carcinoid tumors.
More extensive excision: When the carcinoid tumor is larger than 2 centimeters, then most surgeons prefer to do a larger operation to make sure they remove the entire tumor. This also gives them the opportunity to see whether the cancer has invaded other tissues so they can get to these areas and remove the invading tumor.
- Segmental colon resection or hemicolectomy: This operation removes between 1/3 and 1/2 of the colon, as well as nearby blood vessels and lymph nodes.
- Low anterior resection: This operation is used for some tumors of the upper part of the rectum. It removes some of the rectum and the remaining ends are sewn together, without much impact on digestive function.
- Abdominoperineal resection: This surgery is for large or very invasive cancers of the lower part of the rectum. After this operation, the end of the colon is connected to the surface of the front of the abdomen and waste is eliminated from the body through this opening called a colostomy.
- Liver resection: This is an operation to remove one or a few metastases from the liver. It is not usually expected to cure the cancer but is often helpful in reducing symptoms of carcinoid syndrome.
Liver transplantation: This is a rarely used treatment that may be effective for young patients with carcinoid tumors that have only spread to the liver.

Operative Resection of Primary Carcinoid Neoplasms in Patients with Liver Metastases Yields Significantly Better Survival - Surgery

Surgical Treatment of Advanced-Stage Carcinoid Tumors: Lessons Learned - Annals of Surgery

Surgery for Midgut Carcinoid - Endocrine-Related Cancer

Hepatic Surgery for Metastases from Neuroendocrine Tumors - Surgical Oncology Clinics of North America (abstract)

Surgical Treatment of Neuroendocrine Metastases to the Liver: A Plea for Resection to Increase Survival (abstract)

Carcinoid Treatment Centers

The following centers treat carcinoid and neuroendocrine tumors:

USA

Cedars-Sinai

Dana-Farber Cancer Institute

Fox Chase Cancer Center

Indiana University - Simon Cancer Center

Johns Hopkins University - Sidney Kimmel Comprehensive Cancer Center

Louisiana State University - Stanley S. Scott Cancer Center

Mayo Clinic

MD Anderson Cancer Center

Memorial Sloan-Kettering Cancer Center

Moffitt Cancer Center

Mount Sinai Medical Center

Ohio State University - James Cancer Hospital

Stanford University

University of Iowa - Holden Comprehensive Cancer Center

University of Maryland - Greenebaum Cancer Center

University of Pittsburgh Medical Center

Washington University - Siteman Cancer Center

Yale University

Europe

Center for Neuroendocrine Tumors - Bad Berka (Germany)

Uppsala University (Sweden)

Cancer Hospitals and Institutes

The leading cancer hospitals in the United States are the following:

Top 50 Cancer Hospitals - U.S. News & World Report

U.S. News & World report ranked the Top 50 cancer hospitals in the United States:

Full ranking of cancer hospitals - U.S. News & World Report

Designated Cancer Centers - National Cancer Institute

The National Cancer Institute (NCI) designates Cancer Centers according to the following performance criteria:

"The Cancer Centers Program of the NCI supports major academic and research institutions throughout the United States to sustain broad based, coordinated, interdisciplinary programs in cancer research.

These institutions are characterized by scientific excellence and capability to integrate a diversity of research approaches to focus on the problem of cancer. The NCI and its Cancer Centers Program are dedicated to the advancement of cancer research to ultimately impact on the reduction of cancer incidence, morbidity, and mortality."

Designated Cancer Centers - National Cancer Institute

Questions to Ask Your Doctor

Before receiving carcinoid treatment, the American Cancer Society recommends asking the following questions of your doctor:

What kind of carcinoid tumor do I have?
What is the stage of my carcinoid tumor and what does that mean to me?
What treatment choices do I have?
What do you recommend and why?
Based on what you've learned about my carcinoid tumor, what is my prognosis?
What risks or side effects are there to the treatments you suggest?
What are the chances of recurrence of my carcinoid tumor with these treatment plans?
What should I do to be ready for treatment?