Can You Die from Carcinoid Syndrome?

Carcinoid syndrome is a group of symptoms that occur when some neuroendocrine tumors (also called carcinoid tumors) overproduce hormones such as serotonin. 

Neuroendocrine tumors are rare and usually slow-growing tumors arising from neuroendocrine cells. Neuroendocrine cells produce hormones that control metabolism, digestion, and other bodily functions. Neuroendocrine tumors can start growing in the digestive tract, lungs, appendix, thymus, and other organs, and may spread to other parts of the body.

About 40% of people who have carcinoid cancer will experience carcinoid syndrome. Symptoms of carcinoid syndrome can include:

• Flushing 
  • Skin redness or warmth
  • Most common symptom
  • Lasts 30 seconds to 30 minutes
  • Usually occurs on the face, neck, or upper chest
  • 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
• Wheezing
• Trouble breathing/shortness of breath
• 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

Carcinoid syndrome is caused by an overproduction of hormones such as serotonin by a neuroendocrine tumor, resulting in the characteristic symptoms. 

Carcinoid syndrome commonly occurs when a neuroendocrine tumor that starts in the small intestine spreads to the liver, where the hormones produced by the tumor can enter the bloodstream, which can result in symptoms.

In addition to a patient history and physical examination, tests used to diagnose carcinoid syndrome include: 

• Urine tests 
• Blood tests
  • 5-HIAA (5-hydroxyindoleacetic acid) 
  • Chromogranin A (CgA) 
• Imaging tests
  • Computerized tomography (CT) scan 
  • Magnetic resonance imaging (MRI) scan
  • Iodine-123-meta-iodobenzylguanidine scan (MIBG)
  • Endoscopy 
  • Ultrasound
• Echocardiogram (“echo”) 
• Pancreastatin Test, Substance P and Neurokinin A Tests
• Full body imaging tests: Somatostatin Receptor Scintigraphy (SRS) Test (Octreoscan)

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

Treatment aimed at controlling symptoms of carcinoid syndrome includes:

• Anti-diarrheal medications
• Medicines that block neuroendocrine tumors from producing the substances that cause symptoms
• Dietary modifications
  • Avoid foods and substances with high levels of amines, nitrogen-containing organic compounds, or adrenaline-like substances or alcohol that can trigger the release of serotonin and other hormones from carcinoid tumors
  • Foods to avoid include: 
    • Fatty meats, smoked or salted fish, aged cheeses, chocolate, and red wine
    • Foods with high serotonin levels such as eggs, cheese, pineapple, nuts, bananas, tomatoes, kiwi fruit, and plums
• Adjusting prescription medications
  • Some drugs can affect amine or adrenaline levels
  • Ask your doctor about this
  • Never stop taking a prescribed medication without first talking to your doctor
• Palliative therapy (supportive care) to help manage pain, maintain quality of life, and reduce stress that comes with a serious illness
  • Focuses on treating symptoms, side effects, and emotional aspects of illness
  • Examples of palliative therapy include:
    • Surgery to reduce tumor size and reduce pain 
    • Counseling to manage mental stress and emotional burdens
  • Treatment for carcinoid heart disease
    • May involve surgery to replace a diseased heart valve with a healthy heart valve

Treatments for neuroendocrine tumors may help relieve symptoms of carcinoid syndrome and include: 

• Surgery to remove all or part of a neuroendocrine tumor
• Embolization  
  • Blocks a neuroendocrine tumor's blood supply
  • Only used for tumors in the liver
• Ablation therapy 
  • Kills cancer cells
  • Only used for tumors in the liver
• Biologic therapy 
  • Interferon 
• Chemotherapy 
  • CAPTEM, a combination of two anti-cancer medications, capecitabine and temozolomide
  • 5-fluorouracil (5-FU), alone or in combination with streptozotocin 
  • Doxorubicin, streptozotocin, dactinomycin, and dacarbazine 
• Immunotherapy 
  • PD-1 inhibitors
• Radiation delivered directly to the tumor
  • Lutetium Lu 177-Dotatate (Lutathera)
  • Administered intravenously (IV)
  • Usually used on advanced neuroendocrine tumors, when other treatments do not work
• Somatostatin analogs (SSA) 
  • Injections can help lesson symptoms including diarrhea and flushing
  • Octreotide (Sandostatin Depot, Sandostatin LAR, Bynfezia Pen) 
  • Lanreotide (Somatuline Depot)
• Telotristat ethyl (Xermelo) targets overproduction of serotonin and is given in combination with a somatostatin analog when the SSA alone is not fully effective
• Targeted therapies 
  • Angiogenesis inhibitors (AI) including sunitinib (Sutent) and bevacizumab (Avastin)
  • Tyrosine kinase inhibitors (TKI) including sorafenib (Nexavar) and sunitinib (Sutent) 
  • mTOR inhibitors such as everolimus (Afinitor)

Complications of malignant carcinoid syndrome include: 

• 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