Neuroendocrine tumors develop long and latently. The most important problem in their treatment is too late recognition. Until recently, only a few doctors knew about their existence. A neuroendocrine tumor is a very unusual tumor. Find out what you need to know about neuroendocrine tumors.

Neuroendocrine tumors(NET) is an atypical form of neoplastic disease. They are rare - only a few to a dozen or so people per million affect them during the year. However, there is also another truth: these types oftumorsusually go undiagnosed for a long time. They are diagnosed only whencanceris advanced, with metastases to the lymph nodes and the liver or other organs.

What do we know about neuroendocrine tumors?

Neuroendocrine tumors arise from endocrine cells that are dispersed throughout the human body and form a diffuse endocrine (endocrine) system. They can affect one organ or involve different organs. This name covers at least a dozen types of tumors, from benign to highly malignant, with rapid aggressive growth. The first, treated surgically, end with the patient's recovery. The latter must be treated in a combined manner with different therapies. Surgical treatment is usually not possible due to the initial stage of the tumor. There are intermediate forms with an advanced initial stage, but of slow growth, and these dominate. More than 70 percent of all neuroendocrine tumors are found in the digestive system, the remainder in the airways and in selected endocrine glands.

Important

Glossary

The endocrine system(endocrine) - a system that integrates the functions of individual organs with the nervous and immune systems through the production of hormones released into the blood.Somatostatin- growth hormone antagonist; blocks the secretion of growth hormone by the pituitary gland and inhibits the secretion of insulin.Serotonin- a hormone that acts, inter alia, in the function of an important neurotransmitter in the central nervous system and in the digestive system; The satisfaction associated with engaging in pleasant activities, such as eating chocolate, is possibly caused by the secretion of serotonin in the testesseam.

Neuroendocrine tumors of the digestive system

Tumors of the digestive system (GEP NETs) are characterized by a long, sometimes several years of development, asymptomatic. Most often they are small in size, so it is difficult to locate them in routine examinations. Metastasis, mainly to the liver and lymph nodes, usually appears before the onset of clinical symptoms. These features make a patient with a neuroendocrine tumor significantly different from a typical cancer patient in terms of psychology, explains Prof. Beata Kos-Kudła, head of the Department of Endocrinology in Zabrze and the Department of Pathophysiology and Endocrinology of the Medical University of Silesia in Katowice and chairman of the Council of Experts of the Polish Network of Neuroendocrine Tumors. - A man functions perfectly, considers himself fully he althy, and then suddenly he finds out that his entire liver is covered with metastases. This causes him a psychological shock. At this point and at a later stage of treatment, the attitude of the doctor to the patient plays a huge role, as well as the possibility of taking the patient under the care of a psychologist.

Neuroendocrine tumors: carcinoid

The best known and most common GEP cancer is carcinoid, a serotonin-secreting tumor. It was first described 100 years ago, and its name was meant to reflect its nature - a tumor "similar to cancer" microscopically but clinically benign. This disease manifests itself clinically with the symptoms of the so-called carcinoid syndrome: paroxysmal redness of the face, bothersome diarrhea, abdominal pain, shortness of breath, swelling, cyanosis and pain in the joints and muscles. Giving the right drugs to suppress hormone secretion usually completely clears the symptoms. The patient has a sense of recovery, can function normally, and his quality of life improves dramatically.

Worth knowing

Neuroendocrine tumors( NET ) are rare and atypical neoplasms whose cells have the ability to produce, store and secrete biogenic hormones and amines ( biologically active "relatives" of amino acids). These tumors develop from endocrine (endocrine) cells that are present in various parts of the body. NETs can occur in various organs and tissues, but 70% of them are located in the gastrointestinal tract.

Neuroendocrine tumors - diagnosis

The basic tool in the diagnosis of neuroendocrine tumors is the determination of the appropriate hormones in the blood:

  • Chromogranin A (CgA) concentration
  • concentration of 5-hydroxyindole acetic acid (5-HIAA)
  • concentration of neuronspecific enolase (NSE)
  • synaptophysin concentration

When a neuroendocrine tumor is suspected, the fact that about 80 percent of neuroendocrine tumors produce a characteristic protein on their surface - somatostatin type 2 receptor is used. By administering a somatostatin analogue labeled with a radioactive isotope - technetium, a tumor image can be obtained by scintigraphy (SRS) or, using gallium, PET positron emission tomography. In the diagnosis of NET tumors, the following are also used:

  • ultrasound examination with the use of an endoscope
  • computed tomography (CT)
  • magnetic resonance imaging (MR)

Pharmacological treatment of neuroendocrine tumors

The treatment of choice is surgery, which allows the complete removal of the neoplasm. For advanced lesions at diagnosis, surgery may not be possible. Then pharmacotherapy and chemotherapy are used.

Somatostatin analogues (octreotide and lantreotide) play a major role in the pharmacological treatment of hormonally active GEP tumors, currently considered the "gold standard" in GEP NET management - since their introduction to the treatment of carcinoid patients, the 5-year survival has increased from 18 to 67%. High hopes are also associated with the clinically tested radioisotope therapy with labeled somatostatin analogues (radioactive isotopes that attach to cancer cells together with the drug irradiate and destroy them). Newer and newer anti-cancer drugs are also being investigated in terms of their use in GEP tumors.

A method that is not commonly used in the treatment of this type of cancer is chemotherapy. Hence, it is extremely important to increase the awareness of physicians not to mechanically refer patients with GEP tumors to chemotherapy, which should be reserved mainly for rapidly developing GEP tumors.

References:

1. Violetta Rosiek, Beata Kos-Kudła, "Neuroendocrine tumors - early diagnosis in POZ", Medycyna po Diplie, February 20132. Beata Kos-Kudła, Anna Zemczak, "Contemporary methods of diagnosis and treatment of gastrointestinal neuroendocrine tumors" Endocrinology Poland, Volume 57, number 2/2006, pp. 174-1863. Marta Kunkel, "Gastrointestinal neuroedocrine tumors - characteristics, diagnosis, treatment", Doctor's Guide a 3/2005, pp. 107-1134. Beata Kos-Kudła (red) Neuroendocrine tumors of the digestive system. Gdańsk: Via Medica, 2010, pp 1-364

Category:

Oncology