Neuroendocrine Cancer - Treatments, Symptoms, Causes

Neuroendocrine tumors (NETs) arise from cells in the neuroendocrine system, which consists of endocrine cells (or hormone-producing cells) and nerve cells. Neuroendocrine cells are mostly found in organs such as the digestive system (stomach, intestines, etc.) and lungs, and they perform functions such as regulating air circulation and blood flow in the lungs and controlling the speed of food passing through the digestive system.

Unlike other types of tumors, two important elements stand out in NETs. Firstly, they are composed of dispersed cells throughout the endocrine system rather than of cells found in a particular organ or tissue. Secondly, the neuroendocrine cells produce bioactive substances and hormones that control important body functions. NETs may cause a variety of symptoms by overproducing these substances, so multiple specialists including an endocrinologist, a gastroenterologist, an oncologist, and a radiologist may need to follow the treatment for the correct diagnosis.

Statistics show that approximately 2.5 to 5 out of every 100,000 people are diagnosed with NETs each year (incidence). 

Causes of Neuroendocrine Cancers – What Are The Risk Factors?

Risk factors for NETs include lifestyle factors, acquired habits, and environmental factors known to increase the likelihood of developing the disease. We can reduce risk factors associated with cancer by paying careful attention to our lifestyle, habits, and exposure to environmental factors, and we can reduce risk factors associated with cancer.

Smoking, drinking alcohol, and other risk factors are habits we acquire in life and can be changed. Certain risk factors, such as age and family history, cannot be changed. On the other hand, having a risk factor does not mean you will get cancer. It can also be said that you can get cancer even without any risk factors.

Therefore, it is important to know the risk factors involved with neuroendocrine cancers. This way, you can reduce the risk by making small changes in your life. If you have any risk factors regarding neuroendocrine cancer, do not neglect to have routine checkups regularly. This way, you have the opportunity for early diagnosis and effective treatment of cancer.

What Are The Symptoms Of Neuroendocrine Tumors?

1. High blood pressure, fever, headache, sweating, palpitations, nausea, vomiting, and rapid heartbeat are symptoms seen in patients with both neuroendocrine tumors and pheochromocytomas.
2. In addition, patients with Merkel cell tumors may have painless, firm bumps on the skin that can turn red, pink, or blue.
3. Also, neuroendocrine tumors that have gastrointestinal, pancreatic, or familial characteristics may exhibit certain symptoms such as:

a. Hyperglycemia (high blood sugar that increases hunger and thirst, and causes frequent urination)

b. Hypoglycemia (low blood sugar that causes weakness, irritability, dizziness or loss of balance, seizures, and fainting)

c. Diarrhea, pain, appetite and weight loss, cough and hoarseness, jaundice, rash on the skin.

Neuroendocrine Tumor Treatment With Surgery 

Surgical intervention is the main treatment for both pheochromocytoma and Merkel cell neuroendocrine cancers. A margin method is used to draw a small border around the healthy tissue surrounding the tumor and the tumor is removed. Laparoscopic surgery (closed surgery) is one of the surgical methods used for pheochromocytoma treatment.

In addition, since surgery is the only treatment option for neuroendocrine tumors of the pancreas, all regional pancreatic neuroendocrine tumors should be removed if possible.

Laparoscopic Surgery For Neuroendocrine Tumors 

In laparoscopic surgery, a laparoscope with a camera at the end is inserted through one of the 3-4 small incisions and guides the surgery. Surgical tools are inserted through the other incisions and the surgery is performed while being visualized.

Radiation Therapy For Neuroendocrine Tumors 

Radiation therapy is a method of killing cancer cells using high-energy X-rays. The most commonly used radiation therapy is external radiation therapy, which involves directing radiation directly to the tumor from outside the body. When radiation therapy is carried out using an implant placed inside the body, it is called internal radiation therapy or brachytherapy. A high dose of radiation is delivered to the cancerous area by placing a highly powered radioactive source on the cancer cells, minimizing side effects by causing less harm to healthy tissue. Radiation therapy is used when neuroendocrine tumors spread or when surgery is difficult or impossible due to their location.

Chemotherapy and Targeted Therapy For Neuroendocrine Tumors 

Chemotherapy kills cancer cells using drugs. Generally, in this method, cancer cells' growth and division are halted. Systemic chemotherapy reaches cancer cells throughout the body through the bloodstream and destroys them. This is accomplished by administering one or more combinations of drugs intravenously or orally at certain intervals. In the chemotherapy treatment of poorly differentiated neuroendocrine cancers, small-cell drugs are used to treat the cancer cells.

In recent years, studies have shown that not all diagnosed tumors have the same target. A series of tests can be conducted to identify the genes, proteins, and other factors that the tumor has in order to find the most effective treatment. This allows for the planning of the most effective treatment specific to that tumor. Research to increase the effectiveness of targeted therapy is ongoing.
Two new targeted therapies, Sunitinib (Sutent) and Everolimus (Afinitor), have been beneficial in patients with pancreatic neuroendocrine tumors. Before implementing a treatment plan with these drugs, you can talk to your doctor to learn more about possible side effects and how to manage them.

Other Drug Treatments For Neuroendocrine Tumors

Phaeochromocytoma is usually treated with alpha-adrenergic blockers (drugs often used to lower blood pressure) in addition to surgery. Beta-blockers can be used to control rapid or irregular heartbeat.

Drugs used for cancer treatment are constantly being evaluated. Talk to your doctor to learn more about the drug treatment and potential side effects. Familiarize yourself with the results of previous research on the drugs to be used.

Treatment With İnterferon-Alpha

Interferons are natural substances that activate the body's immune system. These substances also slow down the development of tumor cells. Interferon-alpha, which is synthetically produced in the laboratory and used in cancer treatment, can be applied from time to time to reduce or slow the development of metastatic neuroendocrine tumors and reduce symptoms of carcinoid tumors.

Immune Checkpoint İnhibitors (Immunotherapies) 

Studies combining anti-CTLA4 drugs such as ipilimumab and other immunotherapy agents for neuroendocrine tumors are ongoing, but there is currently no approved immune checkpoint suppressor in this field.