Carcinoid tumor

A carcinoid tumor is a type of neuroendocrine tumor that usually develops in the digestive gastrointestinal tract (such as the stomach or intestines) or in the lungs.

Carcinoid tumor with postobstructive pneumonia

In some cases, a carcinoid tumor develops in another part of the body, such as the pancreas, testicle (in men), or ovary (in women).

It is a slow-growing tumor that typically does not cause symptoms in the early stages, so a person may have the tumor for years before being diagnosed. In later stages, symptoms may vary depending on where the tumor is located.Symptoms of a GI carcinoid tumor may only develop if the tumor has spread to the liver.

Symptoms

The tumor may produce hormone-like substances serotonin that spread to the body and cause symptoms of carcinoid syndrome, such as flushing of the face and chest, diarrhea, and trouble breathing. People with a lung carcinoid tumor are less likely to have carcinoid syndrome, but may experience coughing, wheezing, or pneumonia

The tumor may also cause various symptoms if it has spread to other parts of the body.

Primary site of a carcinoid cancer of gut

cause

The cause of carcinoid tumors is currently unknown.

Atypical carcinoid tumor

Risk factors

There are some unavoidable risk factors that may increase a person's risk of developing the tumor. It is important to note that people with several risk factors may never develop cancer, while others with no risk factors may develop cancer. Possible risk factors for carcinoid tumors include:

• Family history of multiple endocrine neoplasia type 1 (MEN1). About 10% of gastrointestinal (GI) carcinoid tumors are thought to be associated with MEN1.
• Family history of neurofibromatosis type 1 (NF1).
• Family history of other genetic syndromes such as tuberous sclerosis complex and von Hippel Lindau disease.
• Other stomach conditions. People with diseases that damage the stomach and reduce stomach acid levels (in particular, pernicious anemia) have a greater risk of developing stomach carcinoid tumors.
• Race and gender. GI carcinoid tumors are more common among African Americans than among white people, and slightly more common among women than men. Lung carcinoid tumors are more common among white people than among people of other ethnicities.

Treatment

Surgery, if feasible, is the only curative therapy. If the tumor has metastasized (most commonly, to the liver) and is considered incurable, there are some promising treatment modalities, such as radiolabeled octreotide. The radiopharmaceutical 131I-mIBG (meta iodo benzyl guanidine) for arresting the growth of the tumors and prolonging survival in patients with liver metastases, though these are currently experimental.

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition.

• Everolimus (Brand name: Afinitor)Treatment of adult patients with progressive, well differentiated, nonfunctional,neuroendocrine tumors(NET) of gastrointestinal (GI) or lung origin, (excluding pancreatic) with unresectable, locally advanced or metastatic disease.
• Lutetium Lu 177 dotatate (Brand name: Lutathera)

Treatment of somatostatin receptor-positive gastroenteropancreatic neuroendocrine tumors (GEP-NETs), including foregut, midgut, and hindgut tumors in adults.

• Gallium (Brand name: Netspot)For use after radiolabeling with Ga 68, with positron emission tomography (PET) for localization of somatostatin receptor positive neuroendocrine tumors (NETs) in adult and pediatric patients.
• Octreotide (Brand name: Sandostatin LAR)Octreotide (Brand name: Sandostatin LAR)
• Lanreotide (Brand name: Somatuline Depot (injection))Treatment of patients with unresectable,well- or moderately-differentiated locally advanced or metastatic gastroenteropancreatic neuroendocrine tumors to improve progression-free survival.

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Peripheral typical carcinoid tumor - CT scan

Frequently asked questions

• What is the survival rate for carcinoid tumor?

• Is carcinoid cancer curable?

• What causes carcinoid tumors?

• Are carcinoid tumors benign or malignant?

• Can you die from carcinoid cancer?

• Does carcinoid mean cancer?

• Can carcinoid tumors be removed?

• Are carcinoid tumors terminal?

• How fast does a carcinoid tumor grow?

• What foods to avoid if you have carcinoid tumors?

• Can you see carcinoid tumors on a CT scan?

• Do carcinoid tumors cause pain?

• What is Stage 4 carcinoid cancer?

• What is the best treatment for carcinoid tumors?

• How do you feel when you have stomach cancer?

NIH genetic and rare disease info

• NIH info on Carcinoid tumor

Carcinoid tumor is a rare disease.

Carcinoid tumor Resources
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