Neuroendocrine tumour

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What is Neuroendocrine tumour?

Neuroendocrine tumours (NETs) are tumours composed of neuroendocrine cells, which are cells that produce and secrete regulatory hormones and are present throughout the nervous and endocrine/hormonal systems. As neuroendocrine cells are distributed widely throughout the body, tumours of these cells can occur at many sites.

Examples of neuroendocrine tumours are:

Carcinoid tumour

Pancreatic endocrine tumour

Gastrinoma

Insulinoma

Glucagonoma

VIPoma (vasoactive intestinal polypeptide tumour)

Paraganglioma

Pheochromocytoma

Medullary thyroid carcinoma

Poorly differentiated small cell neuroendocrine carcinoma

Who gets Neuroendocrine tumour?

Neuroendocrine tumours are generally rare. The occurance rate is about 0.1-1 per million per year. Glucagonomas are among the rarest of the NETs.

Predisposing Factors

The exact cause is unknown.

Neuroendocrine tumours can arise spontaneously in a random fashion or as a result of genetic changes.

Neuroendocrine tumors may associated with the following conditions inherited genetically:

multiple endocrine neoplasia (MEN) types 1 and 2

von Hippel-Lindau (VHL) disease

von Recklinghausens neurofibromatosis (NF)

tuberculous sclerosis (TSC)

Progression

Neuroendocrine tumours are rare cancers that are often misdiagnosed. Because symptoms vary widely depending on where the cancer happens, a diagnosis is frequently made only after the cancer has spread/metastasised to other parts of the body.

Most neuroendocrine tumours are malignant, except insulinoma. They commonly spread to the lymph nodes and the liver. Some other uncommon sites of distant spread include the bone, lung, brain and other organs. Despite the ability to metastasise/spread, most malignant neuroendocrine tumours are slow-growing. An exception is the poorly differentiated small cell neuroendocrine carcinoma, which is highly malignant, fast-growing, but fortunately rare.

Depending on whether the neuroendocrine tumours lead to a specific clinical syndrome, they are termed "functionally active" and "functionally inactive". Functionally active NETs produce clinical symptoms because of excessive hormone release from the tumour cell. Examples of NETs that may be functionally active are insulinoma, gastrinoma, VIPoma, glucogonoma (producing the glucagonoma syndrome), carcinoid tumour (producing the carcinoid syndrome).

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