About carcinoid cancer

What is carcinoid cancer?

Carcinoid syndrome is a disease consisting of a combination of symptoms, physical manifestations, and abnormal laboratory findings. Carcinoid syndrome is seen in individuals who have an underlying carcinoid tumour with spread to the liver. Carcinoid tumors are well-differentiated neuroendocrine tumours with secretory properties, releasing serotonin, along with a number of other active peptides. These tumors can arise anywhere along the primitive gut and are therefore found in the bronchial tree (airways) and along the gastrointestinal tract. The tumor cells can also migrate (metastasize) to the liver.

Carcinoid tumors most commonly occur in the small intestine and appendix, but 10% originate in the lung. Other affected areas include the rectum, colon, pancreas, stomach, ovary, thymus, kidney, prostate, breast and elsewhere. These slow-growing malignancies tend to spread to lymph nodes and the liver but can also metastasize to lung, bone, brain, and skin.

Only about 10% of the people with a carcinoid tumor will develop carcinoid syndrome. Major symptoms of this syndrome include hot, red facial flushing, diarrhea and wheezing. Carcinoid syndrome occurs when the tumor produces excessive amounts of serotonin in an individual with liver metastases. In patients who have no spread to the liver, the serotonin released by an intestinal tumor will be broken down to an inactive substance; thus, carcinoid syndrome does not occur.



What are the symptoms for carcinoid cancer?

Diarrhea symptom was found in the carcinoid cancer condition

Signs and symptoms of carcinoid lung tumors include:

  • Chest pain
  • Wheezing
  • Shortness of breath
  • Diarrhea
  • Redness or a feeling of warmth in your face and neck (skin flushing)
  • Weight gain, particularly around the midsection and upper back
  • Pink or purple marks on the skin that look like stretch marks

Carcinoid tumors in the digestive tract

Signs and symptoms of carcinoid tumors in the digestive tract include:

  • Abdominal pain
  • Diarrhea
  • Nausea, Vomiting and inability to pass stool due to intestinal blockage (bowel obstruction)
  • Rectal bleeding
  • Rectal pain
  • Redness or a feeling of warmth in your face and neck (skin flushing)



What are the causes for carcinoid cancer?

It's not clear what causes carcinoid tumors. In general, cancer occurs when a cell develops mutations in its DNA. The mutations allow the cell to continue growing and dividing when healthy cells would normally die.

The accumulating cells form a tumor. Cancer cells can invade nearby healthy tissue and spread to other parts of the body.

Doctors don't know what causes the mutations that can lead to carcinoid tumors. But they know that carcinoid tumors develop in neuroendocrine cells.

Neuroendocrine cells are found in various organs throughout the body. They perform some nerve cell functions and some hormone-producing endocrine cell functions. Some hormones that are produced by neuroendocrine cells are histamine, insulin and serotonin.



What are the treatments for carcinoid cancer?

Treatment for a carcinoid tumor depends on the tumor's location, whether cancer has spread to other areas of the body, the types of hormones the tumor secretes, your overall health and your own preferences.

Carcinoid tumor treatment options may include:

  • Surgery. When detected early, a carcinoid tumor may be removed completely using surgery. If carcinoid tumors are advanced when discovered, complete removal may not be possible. In some situations, surgeons may try to remove as much of the tumor as possible, to help control signs and symptoms.
  • Medications to control excess hormones. Using medications to block hormones secreted by the tumor may reduce the signs and symptoms of carcinoid syndrome and slow tumor growth.

    Octreotide (Sandostatin, Bynfezia Pen) and lanreotide (Somatuline Depot) are given as injections under the skin. Side effects from either medication may include abdominal pain, bloating and diarrhea. Telotristat (Xermelo) is a pill that is sometimes used in combination with octreotide or lanreotide to further try to improve the symptoms of carcinoid syndrome.

  • Chemotherapy. Chemotherapy uses strong drugs to kill tumor cells. It can be given through a vein in your arm or taken as a pill. Chemotherapy is sometimes recommended for treating advanced carcinoid tumors that can't be removed with surgery.
  • Targeted drug therapy. Targeted drug treatments focus on specific abnormalities present within tumor cells. By blocking these abnormalities, targeted drug treatments can cause tumor cells to die. Targeted drug therapy is usually combined with chemotherapy for advanced carcinoid tumors.
  • Drugs that deliver radiation directly to the cancer cells. Peptide receptor radionuclide therapy (PRRT) combines a drug that seeks out cancer cells with a radioactive substance that kills them. In PRRT for carcinoid tumors, the drug is injected into your body, where it travels to the cancer cells, binds to the cells and delivers the radiation directly to them. This therapy may be an option for people with advanced carcinoid tumors.
  • Treatment for cancer that spreads to the liver. Carcinoid tumors commonly spread to the liver. Treatments may include surgery to remove part of the liver, blocking blood flow to the liver (hepatic artery embolization), and using heat and cold to kill cancer cells. Radiofrequency ablation delivers heat treatments that cause carcinoid tumor cells in the liver to die. Cryoablation uses cycles of freezing and thawing to kill cancer cells.

 



What are the risk factors for carcinoid cancer?

Factors that increase the risk of carcinoid tumors include:

  • Older age. Older adults are more likely to be diagnosed with a carcinoid tumor than are younger people or children.
  • Sex. Women are more likely than men to develop carcinoid tumors.
  • Family history. A family history of multiple endocrine neoplasia, type 1 (MEN 1), increases the risk of carcinoid tumors. In people with MEN 1 multiple tumors occur in glands of the endocrine system.



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