Net-men-kanker.be
Neuroendocrine tumours (NETs) are neoplasms that arise from cells of the endocrine (hormonal) and nervous systems. Many are benign, while some are malignant. They most commonly occur in the intestine, where they are often called carcinoid tumours, but they are also found in the pancreas, lung and the rest of the body.
NETs are able to produce hormones and hormone-like substances (functioning tumours). NETs that do not produce hormones are known as non-functioning tumours. NETs are graded along a staging scale that describes how the tumour behaves. NETs that are graded I, II or III are relatively slow-growing. Grade IV, however, is a fast-growing aggressive tumour with a bad differentiation. These NETs are known as neuroendocrine carcinoma (NECs; see specific chapter)
For NETs in the pancreas, four subtypes are distinguished:
These four types only occur in NET patients with a functioning tumour in the pancreas. This is roughly half of all patients with a NET in the pancreas.
NETs can also occur in the small intestine, and these are also known as carcinoid tumours. They are mutations of serotonin-producing cells. Sometimes these tumours are discovered by chance during an appendectomy.
NETs that develop in the lungs also produce extra serotonin. In this case, the enterochromaffin cells are compromised.
NETs in the stomach occur less frequently than the types mentioned above, and are often found by chance. Three subtypes are distinguished:
NETs are rare, and some of the types only occur in a few patients every year. The past few years have seen a steady increase in the number of patients diagnosed with NET. The disease affects people of all ages, but is more prevalent in the over 45s, and there are more women than men who suffer from NET.
Since symptoms of NET often mirror symptoms of more common illnesses, NET is not always recognised as such. Sometimes patients have been suffering from symptoms for years without being diagnosed correctly.
Symptoms for NET in the pancreas:
Symptoms for NET in the small intestine:
Symptoms for NET in the lungs:
As in many types of cancer, it is impossible to pinpoint the cause of NET. In some cases, NET in the pancreas can have a genetic component, such as MEN1 syndrome or Von Hippel-Lindau syndrome.
If a GP suspects hormone-related cancer in a patient, they will refer him to a specialist, who will conduct several tests, such as tissue samples, blood and urine tests, as well as use imaging technologies such as ultrasounds, MRI scans, CAT scans, endoscopy, bronchoscopy and an octreotide scan. The latter is a method using a radioactive substance to find tumorous growths.
Once the research has been done, the grade and stage of the cancer can be determined. Grades I and II are relatively slow-growing and require a different approach than grade III. The grade also determines the outlook for the patient.
When the research has taken place, and the extent of the cancer has been established, the specialists will come up with a treatment plan. Given the rare nature of NET, it is highly advisable to undergo treatment in a specialised cancer clinic.
When the location of the first tumour is known, or in case all tumours can be removed by surgical means, treatment will primarily be focused on healing the patient. If this is no longer an option, palliative treatment will be initiated in order to ensure the patient is as comfortable as possible. The chosen treatment depends on type, location and stage of the NET and on the general health condition of the patient.
Here follows a non-exhaustive list of possible therapies:
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