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Neuroendocrine tumors (NETs) are neoplasms that arise from cells of the endocrine and nervous systems.They most commonly occur in the intestine, where they are often called carcinoid tumors, but they are also found in the pancreas, lung, and the rest of the body.
Gastrinoma is the second most common functional pancreatic neuroendocrine tumor (pNET), with a yearly incidence of approximately 0.5 to 21.5 cases per a million of people worldwide. [5] Gastrinomas are located predominantly in the duodenum (70%) and pancreas (25%). [ 20 ]
Carcinoid syndrome is a paraneoplastic syndrome comprising the signs and symptoms that occur secondary to neuroendocrine tumors (formerly known as carcinoid tumors). [1] The syndrome is caused by neuroendocrine tumors most often found in the gut releasing biologically active substances into the blood causing symptoms such as flushing and diarrhea, and less frequently, heart failure, vomiting ...
Due to presence of these tumors, DIPNECH is classified as a pre-malignant condition. [1] Although there have been reports of atypical carcinoids with local lymph node involvement, there are no reports of more aggressive neuroendocrine tumors, such as large cell neuroendocrine or small cell lung cancer, associated with DIPNECH. [3]
The tumor typically produces serotonin, Tachykinin peptides and other substances, which cause flushing, tachycardia, diarrhea and in some cases fibrosis of the heart valves. [citation needed] There are often several small and highly fibrotic tumors present in the intestine. The tumors often spread to the mesenteries and the liver. [citation needed]
Endocrine-Related Cancer was founded as Reviews on Endocrine-Related Cancer, the in-house journal of Zeneca (now AstraZeneca) published by its parent company ICI Pharmaceuticals. It was taken over by the Society for Endocrinology in 1994 when it was renamed, and the remit was extended to include a limited number of original research articles to ...
Neuroendocrine tumors can arise 7–16 years after the initial onset of symptoms, therefore the name ROHHADNET (which includes these tumors) has not been widely accepted as it is believed this name would lead to further misdiagnosis. Treatment for these neuroendocrine tumors requires surgical removal, typically performed by a pediatric oncologist.
According to the North American Neuroendocrine Tumor Society, the prevalence of pheochromocytoma is between 1:2,500 and 1:6,500, meaning that for every 2,500–6,500 people, there is (on average) one person with pheochromocytoma. [170] In the United States, this equates to an annual incidence (new cases per year) of 500 to 1,600 cases. [170]