Neuroendocrine Cancer
What is neuroendocrine cancer?
Neuroendocrine tumors are cancers that begin in specialized cells called neuroendocrine cells. Neuroendocrine cells have traits similar to those of nerve cells and hormone-producing cells.
Neuroendocrine tumors are rare and can occur anywhere in the body. Most neuroendocrine tumors occur in the lungs, appendix, small intestine, rectum and pancreas.
There are many types of neuroendocrine tumors. Some grow slowly and some grow very quickly. Some neuroendocrine tumors produce excess hormones (functional neuroendocrine tumors). Others don’t release hormones or don’t release enough to cause symptoms (nonfunctional neuroendocrine tumors).
Diagnosis and treatment of neuroendocrine tumors depend on the type of tumor, its location, whether it produces excess hormones, how aggressive it is and whether it has spread to other parts of the body.
Types
- Adrenal cancer is a rare disease that starts in the adrenal glands—small, triangular glands atop the kidneys that produce hormones regulating metabolism, blood pressure, and other vital functions. Early detection may lead to a cure, but once it spreads, treatment focuses on slowing its progression. Most adrenal gland growths, such as adrenal adenomas, are benign (non-cancerous).
- Carcinoid tumors are a type of slow-growing cancer that can arise in several places throughout your body. Carcinoid tumors, which are one subset of tumors called neuroendocrine tumors, usually begin in the digestive tract (stomach, appendix, small intestine, colon, rectum, called Gastrointestinal neuroendocrine tumors or GI-NETs) or in the lungs (lung neuroendocrine tumors). These tumors often don’t cause signs and symptoms until late in the disease. Carcinoid tumors can produce and release hormones into your body that cause signs and symptoms such as diarrhea or skin flushing.
- Merkel cell carcinoma is a rare type of skin cancer. It most often appears as a bump on the face, head or neck. Merkel cell carcinoma also is called neuroendocrine carcinoma of the skin. This type of cancer often happens in people older than 50. Long-term sun exposure or a weakened immune system may raise the risk of getting this cancer. It tends to grow fast and to spread quickly to other parts of the body. Treatment may depend on whether the cancer has spread beyond the skin.
- Pancreatic neuroendocrine tumors (PNETs) are rare cancers that begin in the hormone-producing islet cells of the pancreas, a gland behind the stomach that aids in digestion. Also called islet cell cancers, PNETs can be functional (produce excess hormones, e.g., insulinoma, gastrinoma, glucagonoma) or nonfunctional (do not produce extra hormones). Most PNETs are nonfunctional.
- Paraganglioma is a rare tumor that forms from nerve cells found throughout the body, most often in the head, neck, abdomen, or pelvis. While usually benign (non-cancerous), some paragangliomas can be cancerous and spread to other parts of the body.
Symptoms
The condition can affect your breasts in different ways. Some breast cancer symptoms are very distinctive. Others may simply seem like areas of your breast that look very different from any other area. Breast cancer may not cause noticeable symptoms either. But when it does, symptoms may include:
- Pain from a growing tumor
- A growing lump you can feel under the skin
- Feeling unusually tired
- Losing weight without trying
- Fatigue.
- Stomach pain.
- Diarrhea.
- Nausea and vomiting.
- Shortness of breath.
- Coughing (sometimes with blood).
Causes
NETs form when neuroendocrine cells begin to divide and multiply uncontrollably. Eventually, they become tumors that can affect the organs where the cells are located. Researchers don’t know what triggers the abnormal cell growth that causes neuroendocrine tumors.
Risk factors
- Multiple endocrine neoplasia (MEN): This condition causes overactive tumors to form in various organs and endocrine system glands. Type 1 (MEN1) is the most common cancer syndrome associated with NETs. It’s especially common in people with pancreatic NETs (P-NETs). Von Hippel-Lindau syndrome: This condition increases your risk of benign and cancerous tumor growth in various parts of your body.
- Von Hippel-Lindau syndrome This condition increases your risk of benign and cancerous tumor growth in various parts of your body.
- Neurofibromatosis type 1: This condition causes tumors to grow on your skin and nerves, affecting your eyes and other parts of your body.
- Tuberous sclerosis complex: This condition causes tumors to grow on your skin and nerves, affecting your eyes and other parts of your body.
- Cowden syndrome: This condition causes benign tumor-like growths and increases your risk of some cancers.
Staging
NETs are staged using the TNM system, where:
- T (tumor):Size of the original tumor
- T (tumor):Size of the original tumor
- N (node): Whether cancer is present in lymph nodes
- M (metastasis):Whether cancer has spread to other body parts
Generally, NETs are classified as localized, regional, or metastatic, with lower stages associated with better survival rates.
- The extent (size) of the tumor (T): How large is the cancer? Has it grown into nearby areas?
- The spread to nearby lymph nodes (N): Has the cancer spread to nearby lymph nodes? If so, how many?
- The spread (metastasis) to distant sites (M): Has the cancer spread to distant organs such as the lungs or liver?
- Estrogen Receptor (ER) status: Does the cancer have the protein called an estrogen receptor?
- Progesterone Receptor (PR) status: Does the cancer have the protein called a progesterone receptor?
- HER2 status: Does the cancer make too much of a protein called HER2?
- Grade of the cancer (G): How much do the cancer cells look like normal cells?
5-Year Survival Rate
At a glance:
| Estimated new cases in 2024 | 83,190 |
| % of all new cancer cases | 4.2% |
| Estimated deaths in 2024 | 16,840 |
| % of all cancer deaths | 6.9% |
| 5-year relative survival (2014–2020) | 91.2% |
The 5-year relative survival rates for bladder cancer are as follows:
- >99% for localized breast cancer (there is no sign that the cancer has spread outside of the breast).
- 87% for regional breast cancer (the cancer has spread outside the breast to nearby structures or lymph nodes).
- 32% for distant breast cancer (he cancer has spread to distant parts of the body, such as the lungs, liver or bones).