This onocological disease can fool even doctors for a long time: symptoms appear, disappear

I live!

Five years ago, Julius was diagnosed with a neuroendocrine tumor. One day intense internal bleeding began. Doctors searched for the cause for a long time until they found the culprit: neuroendocrine cancer of the small intestine. It was already the size of a golf ball.

“If I hadn’t started bleeding, a few months later the tumor would have blocked my intestine. Therefore, the surgeons removed part of the intestine along with the tumor, and then part of the liver, because there was metastasis, ”says the man.

Then the doctors prescribed medicine. Now, five years later, a man feels good, he just has to choose the food: he had to love soups and stews, he has to eat more raw grains. You can work too, you just get tired faster so you stick to your daily routine, take longer breaks.

“These problems are just a trifle, I live!” – the interlocutor rejoices.

League – Zebras

“Cells of neuroendocrine origin can be found in various places in the human body. Therefore, neuroendocrine tumors can develop in various organs: pancreas, small or large intestine, stomach, lungs, skin, etc. ”Says the doc. Head of the Department of Oncology at LSMU Kaunas Clinics and Head of the Center for Neuroendocrine Tumors. Rasa Jančiauskienė.

This is because neuroendocrine cells are present in almost the entire human body.

“Usually cancer symptoms are caused by the growing mass of the tumor. In the case of neuroendocrine tumors, symptoms depend not only on the size of the tumor, but also on the biologically active substances they produce, such as serotonin, kallikrein, adrenocorticotropic hormone, histamine, and the like. These substances cause a variety of symptoms: diarrhea, hot flashes, bronchial asthma-like syndrome, potassium deficiency and related symptoms, diabetes-like symptoms or, conversely, low blood sugar due to increased secretion insulin and consequent weakness or loss of consciousness. Not in vain is this disease equated with a zebra, there are many expressions of it ”, continues the doctor.

About half of all neuroendocrine tumors produce biologically active substances.

Neuroendocrine tumors generally develop in the gastrointestinal tract (small intestine, growth of worms, pancreas), as well as in the lungs.

What causes are unknown

Studies in the United States and the United Kingdom show that the incidence of neuroendocrine tumors has increased significantly in the last 4 decades: 11-18 times in the stomach, 10 times in the rectum and 5 times in the small intestine. However, neuroendocrine tumors remain a rare disease. In Lithuania, they are approved each year for about three hundred people.

“Researchers so far have not found an answer as to why neuroendocrine tumors develop. However, some facts are known. Omeprazole, a popular drug for heartburn, has been shown to affect the development of some gastrointestinal neuroendocrine tumors. .

Long-term use of this drug kills so-called enterochromaffin cells, which then form tumors, such as polyps. The good news is that when you stop taking this drug, the person recovers without treatment, – explains the doc. R. Jančiauskienė. – Some people have a hereditary predisposition to neuroendocrine tumors.

There are syndromes of MEN1 and MEN2 – multiple endocrine neoplasia, in which benign and malignant tumors of a neuroendocrine nature develop in various organs. In such families, a person can develop cancer of various organs during his life: thyroid gland, pancreas, gastrointestinal tract, etc. These people are often aware of their propensity to get sick and visit oncologists or endocrinologists more often. “

Hard to identify

Studies by British researchers show that an average of 53.8 months elapse between the onset of the first symptoms and the diagnosis.

The most common early symptoms are pain, changes in bowel movements, and weakness.

About a third of the patients experienced weight loss. Because these symptoms are common in a variety of diseases, about 80 percent. all patients visited the family doctor up to 11 times from the onset of symptoms to diagnosis of the disease.

58 percent of people were able to visit second-level specialists 3 times before diagnosis. 30 percent Cases of the disease became apparent when acute conditions developed that required emergency care.

Why are neuroendocrine tumors difficult to detect?

“There is an aphorism: when you hear the hooves snoring, you think the horse is running away, not the zebra. Since the symptoms and syndromes caused by neuroendocrine tumors are common to other diseases, of course, doctors hope to detect those diseases other than cancer. Even with state-of-the-art research, the primary tumor site may not be found in about 5 percent of cases. patients. This applies not only to neuroendocrine tumors, but also to other types of tumors, ”says the specialist.

“We not only need experience in the detection and treatment of these tumors, but also special tests, because it depends on whether the patient can choose the most appropriate treatment,” says Alvydas Česas, head of the Oncology Chemotherapy Clinic at Klaipėda University Hospital.

“Unfortunately, we still cannot do modern research to detect neuroendocrine tumors in Lithuania very precisely, because we do not have an isotope producing device, a small ‘nuclear power plant’, which allows the production of the latest radioactive materials that can detect tumors. very rare and very specific neuroendocrine.

We are currently conducting studies in Lithuania with SPECTA devices, which allow us to detect some neuroendocrine tumors, but unfortunately not all, and the sensitivity of these studies is not high. The new radionuclides would not only allow a more accurate diagnosis of neuroendocrine tumors, but would also introduce a new generation of radionuclide therapies for therapeutic purposes. It is quite expensive, but if you were to transport these very rapidly decaying radioactive materials from abroad, the price would increase dramatically. This is not only very expensive but also inconvenient because isotopes can only be used for a very short time. These radiological examinations are very important because they determine the treatment tactics, ”says A. Česas.

The video studies available now show that the tumor is present, but do not show what it is, so doctors can mistake neuroendocrine tumors for other tumors and prescribe conventional antitumor therapies that unfortunately do not work for these tumors.

You can also recover

Doctor of the National Cancer Institute, oncologist-chemotherapist dr. Edita Baltruškevičienė says that it is important for people with neuroendocrine tumors to receive treatment in specialized centers, because special diagnostic tools are needed to diagnose the disease and patients with other tumors are treated differently.

“The treatment of neuroendocrine tumors includes methods and tools frequently used in oncology: surgical treatment, chemotherapy, innovative biological therapies and specific treatments, treatment with somatostatin analogues, radionuclide therapy, etc. The choice of method depends on the location of the primary tumor, the spread of the disease and the malignancy of the tumor ”, says the doctor.

Surgery is very important in these diseases, as it can give the patient a chance to heal. When the disease has spread, the objective is to eliminate not only the primary tumor but also the metastases, since reducing the volume of tumor derivatives reduces the incidence of carcinoid syndrome induced by the tumor, facilitating the control of the disease with drugs .

In the case of carcinoid syndrome, for no obvious reason, the patient has a fever, redness and many times the person does not know why he has diarrhea, etc., explains the doctor. – A specific class of biologics, somatostatin analogs, alleviate these effects and significantly improve the patient’s quality of life. The important thing is that the medication can be used for a long time, this treatment is well tolerated, the medication can be administered by the patient at home ”.

Somatostatin analogs were previously thought to only save by relieving symptoms of carcinoid syndrome, but they have also been shown to inhibit the division of tumor cells, thereby controlling the disease.

Chemotherapy, targeted therapy is generally given for pancreatic, lung neuroendocrine tumors, or for detection of high-grade tumors.

“We have a large number of patients who, even after metastasis, have been living for two or three, some and six years to control their symptoms and have not survived for a year before,” Ches says, adding that the results of the Treatment would be even better if the isotopes used in diagnosis could be adapted for treatment: they are injected into the body, travel to the tumor, and kill it by irradiation. Now, to be treated in this way, patients are sent abroad.

How to shorten the path of patients

Dr. E. Baltruškevičienė states that neuroendocrine tumors are detected late due to the atypical course of oncological disease and symptoms, which are often confused with symptoms of other diseases.

“We specialists can contribute to the solution of this problem by reminding family doctors and other specialists about this rare and exceptional disease, making information known to society and educating patients. The aim is to draw attention to and assess the presence of a possible neuroendocrine tumor in the face of the unique symptoms of carcinoid syndrome ”, says the doctor.

“Neuroendocrine tumors are silent killers: the first symptoms appear when the cancer has already spread. That is why it is important that doctors listen to the patient’s complaints and think about this cancer, because there are no complaints without a reason,” says A. Česas.

“In Lithuania, there are so-called” green corridors “in cancer care institutions. Patients suspected or diagnosed with cancer for the first time, for example, when a microscope confirms the presence of cancer cells under a microscope, are admitted to the consultation of an oncologist or other tertiary specialists as a priority, recalls the director of the doc of the Center for Neuroendocrine Tumors, R. Jančiauskienė. “Unfortunately, various restrictions on quarantine have made access to doctors very difficult.”