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About carcinoid disease

What is carcinoid disease?

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 disease?

Fainting spells symptom was found in the carcinoid disease condition

Neuroendocrine cells are also located in other tissues, such as the lungs and stomach. The hormone they produce helps regulate digestion. But when a carcinoid tumor grows, it produces too much of this hormone and causes symptoms like diarrhea, flushing, wheezing, sweating, and heartburn.

  • Lightheadedness or fainting spells
  • Shortness of breath
  • Abdominal Pain or cramping
  • Rapid heartbeat (tachycardia)
  • Swelling in the legs and feet (edema)
  • Weight loss without trying to lose weight
  • Blood in your stool or urine
  • Diarrhea or constipation that won't go away
  • Pressure in the chest that is relieved by sitting up or leaning forward
  • Vomiting
  • Fatigue, weakness, and exhaustion
  • Sweating at night
  • Bloating after eating
  • Gastroparesis (sluggish emptying of the stomach)
  • Loss of muscle tone in your face, arms, and legs

What are the causes for carcinoid disease?

Carcinoid tumors are typically caused by neuroendocrine cells in the gastrointestinal tract.

  • These cells release hormones that help regulate blood pressure and digestive function.
  • In some cases, they may also be caused by environmental factors such as smoking or alcohol consumption.
  • Most people who develop carcinoid disease/tumors are older than 60 years old at the time of diagnosis; however, younger patients can also develop carcinoid tumors due to an autoimmune disorder called chronic inflammatory bowel disease (IBD).
  • In carcinoid tumors, serotonin and peptide histidine-derived or PHD2 hormones are overproduced and released into the bloodstream, causing symptoms associated with carcinoid disease.
  • It is also thought that changes in gene expression may play a role in carcinoid syndrome. Changes in gene expression are caused by an abnormal gain (overproduction) or loss (underproduction) of DNA material during cell division.
  • Age: Carcinoid tumors tend to occur in people over 50 years old.
  • Gender: Men and women are equally at risk for developing these tumors.
  • Ethnicity: Carcinoid tumors are more common among Caucasians and Japanese than in other ethnic groups.

What are the treatments for carcinoid disease?

There are two main treatments for carcinoid disease: surgery and medication.

  • Surgery is usually done to remove the tumor(s), but sometimes medications like octreotide (Sandostatin) are used as a treatment for both symptoms and cancer itself.
  • Surgery for carcinoid tumors can be either minimally invasive or open, depending on the location of the tumor and how severe it is.
  • The goal of surgery is to remove as much of the tumor as possible without damaging any vital organs.
  • Medical therapy involves using drugs to manage symptoms of carcinoid tumors and reduce their growth.
  • These drugs include octreotide (Sandostatin) and lanreotide (Somatuline), which are used to treat symptoms like diarrhea, flushing, sweating and wheezing, abdominal pain, and nausea, as well as other symptoms caused by carcinoid tumors like hiccups or coughing spills.
  • The most common treatment is an anti-diarrheal medication, which can help reduce diarrhea. This can help make your life more comfortable and keep you from having to go to the bathroom as often.
  • For more severe cases of carcinoid disease, doctors may prescribe medications that block the effects of serotonin. These medications can help reduce some symptoms like flushing and diarrhea.
  • If these treatments don't work or aren't enough to keep you comfortable, your doctor may recommend surgery.

What are the risk factors for carcinoid disease?

Carcinoid disease/tumors are typically found in the small intestine, but there are a few risk factors that can affect your chances of developing one.

The most common risk factor is being over 50 years old. In fact, more than half of all carcinoid tumors occur in people over 50. But there are other age-related risk factors as well: if you're over 60 and have a family history of colon cancer or breast cancer, you may be at an increased risk for developing carcinoid tumors.

In general, the risk factors for carcinoid disease include:

Age: Age is also a factor in the development of intestinal lymphoma, which is another type of tumor that can develop from a carcinoid tumor. Lymphoma is more likely to develop if you're between 60 and 70 years old.

Gender: Men are more likely than women to develop carcinoid tumors.

Ethnicity: African Americans and people of Asian descent have a higher risk of developing carcinoid tumors.

Family history: If someone in your family has been diagnosed with carcinoid tumors, then you may be more susceptible to developing them yourself.

If you have symptoms like diarrhea or constipation, bloody stool or vomit, unexplained weight loss or gain, unexplained fever or chills, and abdominal pain, talk with your doctor about whether you should get tested for carcinoid disease!

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