Is Carcinoid Cancer Fatal?

Small carcinoid tumors (neuroendocrine tumors) doctors catch early and remove may be completely cured. The survival rate drops to 80 percent five years after diagnosis for later-stage carcinoid tumors.

Carcinoid cancer is a type of neuroendocrine cancer that occurs when neuroendocrine cells grow out of control. Carcinoid tumors are rare and usually slow-growing tumors. Neuroendocrine cells produce hormones that control metabolism, digestion, and other bodily functions. 

Carcinoid tumors are usually found in the following parts of the body:

• Small intestine
• Stomach
• Pancreas
• Liver
• Appendix
• Rectum
• Lungs
• Colon

Types of carcinoid tumors include:

• Slow-growing tumors
• Most common type
• Usually remain small, under about an inch wide
• Don’t spread to other parts of the body
• Faster-growing tumors
• May grow more rapidly and grow larger
• Can spread (metastasize) to other parts of the body
• Hormone-secreting tumors
• Called “functioning” carcinoid tumors, they produce hormones including serotonin
• Excess serotonin and other hormones cause symptoms known as carcinoid syndrome

Many people with carcinoid tumors do not have symptoms. Symptoms of carcinoid tumor depend on where the tumor is located and whether it is producing hormones (carcinoid syndrome). 

Symptoms of carcinoid tumor in the digestive tract may include: 

• Abdominal pain or pressure 
• Nausea 
• Vomiting
• Diarrhea
• Change in bowel habits
• Blood in stool
• Weight loss
• Symptoms of appendicitis, such as pain in the abdomen (if the cancer is in the appendix)

Symptoms of carcinoid tumor in the lungs may include: 

• Trouble breathing
• Chest pain
• Wheezing 
• Cough/coughing up blood

Symptoms of carcinoid tumor in the lungs may be mistakenly diagnosed as pneumonia.

About 40% of people with carcinoid cancer will experience carcinoid syndrome (also called carcinoid crisis). Symptoms of carcinoid syndrome can include:

• Flushing 
  • Most common symptom
  • Skin redness or warmth
  • Usually occurs on the face, neck, or upper chest
  • Lasts 30 seconds to 30 minutes
  • Triggers for flushing episodes can include eating a big meal or drinking alcohol
• Rapid heartbeat
• Abdominal cramps
• Watery diarrhea
• Skin rash 
• Spider veins (telangiectasias), usually on the nose, cheek, or upper lip
• Trouble breathing/shortness of breath
• Wheezing
• Carcinoid heart disease, which affects the heart valves
  • May not always cause symptoms
  • When symptoms of carcinoid heart disease occur, they may include:
    • Fluid in the abdomen (ascites) and fluid in the feet (swelling or edema)
    • Trouble breathing/shortness of breath
    • Tiredness/fatigue
    • Low blood pressure
    • Heart murmur

Symptoms of carcinoid syndrome may be triggered by exercise, stress, and certain foods or drinks, such as alcohol (especially red wine), chocolate, and some cheeses.

The cause of carcinoid cancer is unknown. 

Risk factors for developing carcinoid cancer include: 

• A certain genetic condition called multiple endocrine neoplasia type 1 (MEN1)
• Smoking
• Race/gender
  • Gastrointestinal carcinoid tumors are more common among black people than white people
  • Black men have a higher risk than black women

Carcinoid cancer is diagnosed with a patient history and physical examination, along with tests such as: 

• Blood tests
  • Serotonin 
  • Chromogranin A (CgA)
• Urine tests
  • 5-HIAA (5-hydroxy indole acetic acid)
• Imaging tests
  • Computerized tomography (CT) scan
  • Magnetic resonance imaging (MRI) scan
  • Positron emission tomography (PET) scan
  • Chest X-ray
  • OctreoScan
• Biopsy

Treatment for carcinoid cancer depends on the symptoms, where the tumor is located, and how much the cancer has spread. 

Treatment for carcinoid cancer may include one or more of the following: 

• Surgery
  • Removal or destruction of large portions of the tumor (debulking) 
  • Cryoablation (freezing) or radiofrequency ablation (RFA) to destroy carcinoid tumor metastases in the liver when it is possible to remove them surgically
  • Chemoembolization to shut off the blood flow with its oxygen supply to the tumors and load them with tumor destroying and growth inhibiting chemotherapy
• Somatostatin analogs
  • Helps to stop the body from making too many hormones
  • May slow down the growth of the tumor when cancer cells have spread to other part of the body
  • Octreotide/lanreotide and vapreotide (Sandostatin, Sandostatin LAR)
• Targeted therapy
  • Peptide receptor radionuclide therapy (PRRT) 
• Chemotherapy, usually involves more than one drug in combination 
  • Leucovorin-fluorouracil and streptozotocin
  • Cytoxan-doxorubicin and cisplatin 
  • Dacarbazine-fluorouracil
  • Etoposide-cisplatin
  • Treatments under investigation: everolimus (Afinitor), sorafenib (Nexavar), sunitinib (Sutent), atiprimod, pasireotide (SOM230), bevacizumab (Avastin), temozolomide (Temodar), and capecitabine (Xeloda)
• Radiotherapy
  • Used for pain relief from tumors that have spread to the bones, but not for treating metastases or non-skeletal issues
• Supportive treatment
  • High protein diet
  • Vitamin supplements, particularly niacin and mineral supplements (such as potassium, magnesium, calcium, iron, and even salt) 
  • Anti-diarrheal medications 
  • Cyproheptadine (Periactin) to relieve diarrhea and flushing
  • Freshly grated nutmeg (1 teaspoon, 3 times a day) may help control diarrhea
  • Antihistamines and alpha-adrenergic blocking drugs such as phenoxybenzamine (Dibenzyline) to prevent carcinoid syndrome attacks
  • Avoid alcoholic beverages and physical and emotional stress that can trigger carcinoid crisis attacks
  • Avoid adrenaline-like drugs including certain asthma inhalers, nasal decongestants, and adrenaline itself
  • Corticosteroids such as prednisone, and chlorpromazine (Thorazine), or prochlorperazine (Compazine) may be used for certain very severe and prolonged carcinoid crises associated with bronchial (lung) carcinoids or some carcinoids of the stomach 
  • Black raspberry extract may have carcinoid inhibiting properties

Complications of carcinoid cancer include carcinoid syndrome, which can cause: 

• Low blood pressure (hypotension)
• Increased risk for strokes, deep venous thromboses (DVT), and other clotting disorders
• Gastrointestinal bleeding 
• Right-sided heart failure 
• Liver problems
• Acute renal failure (infrequent)
• Bowel obstruction (rare)
• Metastasis

Most carcinoid cancers grow slowly. In many cases, patients who have small tumors that have not spread and do not cause carcinoid syndrome can be cured with surgical removal of the tumor.  

For tumors that are somewhat larger and have spread to local tissues and local lymph nodes but are still totally removable surgically, the average survival ranges from 8 to 23 years. About 70 to 80% of patients with carcinoid tumors usually survive at least five years from diagnosis. 

Even when a tumor from the small intestine has spread (metastasized) and complete surgical removal is impossible, older data indicated about one half of patients survived an average of 5 years. Newer treatments may offer longer survival and improved quality of life.

For atypical carcinoids that are more aggressive than typical carcinoid tumor and spread more quickly, the prognosis is more uncertain. 

Neuroendocrine carcinomas, a rare, more malignant type of tumor, have a poor prognosis and can be fatal.