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A neuroendocrine tumor

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(NET) is a rare type of tumor that arises from specialized body cells called neuroendocrine cells. These cells have traits of both nerve cells and hormone-producing cells, and release hormones into the blood in response to signals from the nervous system.[1][2] Because a neuroendocrine tumor arises from cells that produce hormones, the tumor can also produce hormones.[2]
Neuroendocrine tumors can develop anywhere in the body, but most occur in the digestive tract, pancreas, rectum, lungs, or appendix.[1][2][3] They can be non-cancerous (benign) or cancerous (malignant).[1] They usually grow slowly over many years, but there are fast-growing forms.Rar E[2][4]
There are many types of neuroendocrine tumors, but most are classified as one of two main types:[2][3]Cards
  • Carcinoid tumors - NETs that most commonly arise in the digestive tract, lungs, appendix or thymus. They can also grow in the lymph nodes, brain, bone, gonads (ovaries and testes) or skin.[2][5]
  • Pancreatic neuroendocrine tumors (also called islet cell tumors) - NETs that typically arise in the pancreas, although they can occur outside the pancreas.[6]

Roboform import from lastpass. A pheochromocytoma is another, rarer type of NET that usually develops in the adrenal gland, but can also arise in other parts of the body.[2]

Signs and symptoms depend on the tumor's type, size and location; whether it produces hormones; and whether it has spread to other parts of the body (metastasized).[2] NETs are typically described as functional or nonfunctional. Functional NETs produce a specific set of symptoms due to the production of excess hormones, while non-functional NETs generally do not cause specific symptoms. In many cases, a person has no symptoms until the tumor spreads to the liver and/or impairs the function of an organ or system. This can make NETs very hard to diagnose.[7]
The majority of NETs are not inherited and occur sporadically in people with no family history of NETs.[3] However, some NETs are associated with a hereditary cancer or tumor syndrome such as multiple endocrine neoplasia type 1 (most commonly), Von Hippel-Lindau disease, tuberous sclerosis, or neurofibromatosis type 1 (NF1). Inheritance of each of these is autosomal dominant.[3]
Treatment of NETs depends on many factors such as the tumor's type, location, aggressiveness, and hormone-producing capabilities; as well as whether it has metastasized.[1] Management options may include surveillance, surgery to remove the tumor and/or surrounding tissue, and various non-surgical therapies to shrink the tumor, stop it from growing, or manage symptoms.[8][9][10]

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Last updated: 4/25/2019