What Is a Neuroendocrine Neoplasm?

A neuroendocrine neoplasm is a kind of abnormal growth developing from neuroendocrine cells, which are part of the body's hormone-producing endocrine system. The growth may be a benign tumor, which means it is not cancer, or a malignant tumor, which is cancerous. Most often, a neuroendocrine neoplasm develops in the digestive system, but these tumors can also occur in glands such as the adrenals, the respiratory system and reproductive organs. Some neuroendocrine tumors produce hormones, leading to a variety of symptoms, but some are non-functioning. Neuroendocrine neoplasia is rare, and the most frequently occurring tumor type is known as a carcinoid tumor.

Although they are found in different areas of the body, carcinoid tumors commonly develop from neoplastic cells in the gut wall. In children, the majority of these tumors are found in the appendix. When this type of neuroendocrine neoplasm occurs in adults, it arises most frequently inside the small intestine or appendix.

Symptoms associated with a carcinoid tumor can vary, depending on its location and whether it produces hormones. This can make diagnosis difficult. In the gut, symptoms such as nausea and abdominal pain can occur, while a tumor in the lungs could cause breathlessness and coughing.

Sometimes, tumors secrete the hormone serotonin, resulting in what is known as carcinoid syndrome. A syndrome is a collection of symptoms and, in carcinoid syndrome, wheezing, flushing, diarrhea and weight loss occur. The treatment of this type of neuroendocrine neoplasm varies according to its cell type, location, size and whether it has spread or not.

Another type of neuroendocrine neoplasm is known as a pancreatic endocrine tumor. These growths develop from cells which are found in the pancreas and sometimes in other parts of the gut. Some are non-functional but others produce hormones, giving rise to different syndromes. For example, a tumor may produce the hormone insulin, leading to what is called insulinoma syndrome. The patient may then experience symptoms of low blood sugar, such as weakness, shaking, headaches and confusion.

A gastrinoma is another kind of pancreatic endocrine tumor, which produces the hormone gastrin. As gastrin stimulates gastric acid production, a patient with a gastrinoma may experience ulcers developing in the stomach, esophagus and small intestine. This could lead to symptoms of pain, bleeding in the stomach, abdominal cramps and diarrhea. The treatment of this kind of neuroendocrine neoplasm depends on its size and position, and whether it has spread. Management of all types of neuroendocrine tumors may involve one or all of a range of treatments, including drugs, surgery, chemotherapy, radiotherapy and other procedures.