Neuroendocrine tumors (NOT)

What are neuroendocrine tumors??

Neuroendocrine tumors are a large group of neoplasms, starting from hormone-producing cells of the neuroendocrine system. It consists of cells, which are a "cross" between endocrine and nerve cells. They are found all over the body, performing specific functions. In the lungs, for example, they control blood flow through the lungs, in the stomach and intestines regulate the motility of the organs and the passage of food through them.

The World Health Organization groups neuroendocrine tumors into three major categories:

  • well-differentiated tumors, benign or obscure behavior
  • well-differentiated tumors, malignant, but weakly invasive
  • poorly differentiated tumors, which include large cell neuroendocrine carcinomas and small cell carcinomas

There are also "mixed tumors", which have neuroendocrine traits, and epithelial carcinomas.

An important feature of the tumor is the place of origin. Neuroendocrine tumors are the most common, originating from the lungs and intestines. The other more common tumor is pheochromocytoma.

Because the term neuroendocrine tumors unites a large number of neoplasms, some of which are very rare, we will briefly look at some of the most common groups and species.

Carcinoid tumors

Carcinoids are slow-growing tumors, most commonly occurring in the small intestine, especially the ileum. Many carcinoids are asymptomatic and are found in abdominal surgery for other reasons.. Some tumors may even remain asymptomatic even after metastasis, while others may have serious complications while still young. This is most often due to the secretion of certain hormones.

Some carcinoid tumors secrete serotonin and substance P, which manifests itself with a characteristic symptom complex, called carcinoid syndrome: redness, diarrhea, wheezing, heart failure, colic of the abdominal organs, swelling and palpitations.

The secretion of other hormones is also possible. Overproduction of growth hormone leads to the development of acromegaly or gigantism, and cortisol overproduction - Cushing's syndrome.

Sometimes the tumor is identified after the onset of hemorrhage or by the symptoms of compression of neighboring organs. Tumors of the small intestine can make it difficult to pass food and cause intestinal obstruction.

Pancreatic endocrine tumors

They originate from the pancreas or neuroendocrine cells outside it. These tumors are different from pancreatic adenocarcinomas, which originate from the exocrine part of the gland.

Most endocrine tumors in the pancreas actively secrete hormones. They are referred to as functional, while tumors, which do not secrete hormones, are called non-functional.

Depending on the highly secreted hormone, functional pancreatic tumors can be divided into:

  • gastrinomas - secrete gastrin and lead to the development of Zollinger-Ellison syndrome
  • insulinomas - secrete insulin - hypoglycaemia and fatigue are observed
  • glucagonomes - glucagon has the opposite physiological effects to insulin, due to which there is an increase in blood glucose levels

Other types of tumors in this group are less common.


It is a tumor of the chromaffin cells in the adrenal cortex, which secrete adrenaline and noradrenaline. This leads to a sharp rise in blood pressure and heart rate. The condition may be associated with frequent hypertensive crises, potentially life-threatening. At 80% of the patients, the tumor was localized in only one of the adrenal glands, 10% of those affected have a tumor in both glands, and in the others 10% the tumor is outside the glands.

How is the diagnosis made??

Diagnosis of different tumors may include different tests and tests.

Generally speaking, in hormone-secreting tumors, the physiological effects of the relevant hormones give a good diagnostic direction to the treating clinician. When repeated measurements show a permanent increase in hormone levels, this raises the suspicion of the presence of the corresponding tumor. When looking for a tumor, blood tests often include measurement of relevant tumor markers.

In the diagnosis of non-secreting tumors relies more on imaging - radiography, CT, nuclear magnetic resonance, ultrasound or endoscopy. In some cases it is necessary to use different X-ray contrast materials. Imaging techniques are also used to visualize secretory tumors.

What is the treatment?

Treatment can vary widely, from monitoring and tracking non-invasive tumors to aggressive therapy or surgery for certain malignancies., highly invasive tumors. In Rule, treatment and patient care are carried out by a multidisciplinary team of specialists.

In general, surgery is recommended for non-metastatic tumors. It is often used to treat pheochromocytoma, which is combined with drug control of hypertension until tumor removal.

Along with the tumor, a certain amount is removed from the adjacent healthy tissue.

Radiation therapy is most often performed using a device outside the body, generating and directing high-energy rays to the tumor mass. In some cases, it is possible to implant such a device in the body. In Rule, treatment is applied, when the tumor has metastasized or is in such a place, which makes it inoperable.

Chemotherapy is a drug treatment, most often preventing tumor cell division. Chemotherapy regimens are usually standardized regimens of drug combinations, given in cycles. Side effects depend on the type of medication you are taking. The most common side effects are fatigue, increased risk of infections, loss of appetite and diarrhea. They usually pass after the end of the cycle.

In recent years, pharmaceutical companies are intensively developing t. the sun. targeted drugs, which affect only a certain abnormality of tumor cells, therefore have a weak effect on healthy cells and tissues in the body. This is related to improving the safety profile of medicines, less frequent occurrence of side effects, which are lighter and less serious, than with non-specific drugs. Targeted drugs most commonly affect certain proteins inside or outside cells.

The development of new drugs is being worked on intensively in the field of oncology, neurology, endocrinology and many others.

In parallel with the specific antitumor therapy, symptomatic treatment is often given, depending on the type and secretion of the tumor. For example, for pheochromocytomas, antihypertensive treatment is prescribed, with gastrinomitis - antisecretory treatment, which normalizes the acidity of gastric juice, somatostatin or octreotide are used in case of overgrowth of growth hormone, which block the release of growth hormone into the blood, and so called.

When treatment eliminates the tumor and traces of it cannot be detected using any diagnostic techniques, the patient has entered remission. This condition can be permanent or temporary. In temporary remissions, after a differently long period of clinical health, the tumor reappears. This may be in the same place as the original tumor - a local recurrence, near the original place - regional recurrence, or in a remote place - a distant recurrence.

Relapses most often involve repeated therapy, possibly with some changes and improvements.

Metastatic tumors are the biggest challenge from a therapeutic point of view. Their treatment most often combines surgery, radiotherapy, chemotherapy. Where possible, targeted treatment is also used.

The prognosis correlates well with the degree of tumor cell differentiation. In highly differentiated tumors, survival is usually long, in moderately differentiated there are intermediate levels and duration of survival, and the poorly differentiated have an unfavorable prognosis.

An important element of any anti-tumor treatment is the care of the patient's emotional and physical condition. The complex of events - social, curative, medicated and non-medicated, is called palliative care.


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