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VERIFIED CONTENTAuthor: lek. Agnieszka Michalak

Rare cancers are a group of diseases that occur less frequently than in 6 out of 100,000 people. patients. There is also the concept of neoplasms that are particularly rare, when the incidence is less than one in 100,000 patients. Other criteria such as age and gender are also used to define the concept of a rare neoplasm. Therefore, this group also includes neoplasms that rarely occur in a given age or gender. What is the incidence of rare cancers in Poland and what is their treatment?

Rare neoplasmsis a group of 190 types of cancer. One hundred of them are sarcomas of soft tissues, bones and viscera. Other diagnoses are neuroendocrine tumors, as well as specific types of CNS neoplasms, genital neoplasms, skin neoplasms, head and neck neoplasms, and neoplasms of the gastrointestinal tract and internal secretion.

Rare neoplasms - stats

Rare neoplasms, as the name suggests, occur infrequently and therefore access to detailed data on them was very difficult until recently.

The RARECARE project, funded by the European Union and associating doctors from all over Europe, was established to develop statistics on the incidence, prognosis and cure rates and to assess the scale of the problem of rare cancers in Europe. Based on the data collected as part of this project, it was estimated that rare cancers account for 22% of the total number of cancer patients. all cases of malignant neoplasms.

This means approximately 541,000 new diagnoses per year and 4,300,000 patients living with a diagnosis of a rare cancer. 30 percent among them are people suffering from cancers, especially rare ones. The study also included patients from Poland.

Taking into account 190 rare cancers, approximately 41,000 new cases are expected each year in Poland, which corresponds to 22 percent. total malignant neoplasm cases. It was also assessed that the prognosis of patients with rare neoplasms is worse than that of patients suffering from neoplasms, which are more common in the population.

The 5-year survival rate for rare cancer patients was 48%, while the 5-year survival rate for common cancer patients was 65%.

Rare cancer in Poland

Various cancers can be considered rare in each country's population. Research from the RARECARE project shows that there are 3 types of cancer considered rare in the European population, while in Poland they are one of the most common cancers.

This group included - squamous cell carcinoma of the larynx, squamous cell carcinoma of the cervix and adenocarcinoma of the ovary. In the case of laryngeal cancer, this is mainly due to a higher percentage of tobacco smokers than in Europe.

In the case of cervical and ovarian cancer, the problem is still a small percentage of women undergoing preventive examinations. The higher prevalence of cervical cancer is also due to the lower percentage of HPV vaccinated patients.

Rare neoplasms - specialized treatment

Rare neoplasms pose a great challenge for both doctors and patients. In the case of doctors, due to the rarity of these diseases, it is difficult to gain experience in the treatment and diagnosis of such cancers.

Therefore, the diagnosis of a rare cancer is often delayed, there is also a huge problem with the availability of recommended treatment methods, as well as insufficient access to information for both doctors and patients.

Patients with suspected rare neoplasms should be looked after by a highly specialized center experienced in treatment and diagnostics.

For this purpose, the ERN-EURACAN initiative was established, which brings together oncology centers specializing in the treatment of rare cancers, thanks to which it is possible to consult a patient's case online with experienced specialists from all over Europe and refer them to the appropriate center specialized in the treatment of a given cancer.

Worth knowing

Rare neoplasms are diseases with very uncharacteristic symptoms that may resemble other, more common diseases, and therefore pose a great challenge to doctors.

The care of specialists experienced in both diagnosis and treatment of patients with such neoplasms is essential in the treatment of rare cancers. An invaluable element of prophylaxis is also the patient's awareness of disturbing symptoms and reporting to a doctor as soon as possible if such symptoms occur.

Rare neoplasms - neuroendocrine tumors

Neuroendocrine tumors are a diverse group of cancers that originate in cells that are capable of producing peptide hormones and biogenic amines (e.g., adrenaline).

Such tumors can be spread throughout the body, most oftenhowever, they are found in the digestive tract.

Neuroendocrine tumors do not give characteristic symptoms, and often even imitate symptoms of other diseases. The symptoms caused by neuroendocrine tumors depend on where the tumor is located and what hormone or other biologically active substance is producing the tumor.

The most common ones are sudden drenching sweats, sudden facial erythema, sweating, diarrhea, shortness of breath or a rapid heartbeat unrelated to emotions or physical exertion.

Therefore, neuroendocrine tumors can mimic asthma, irritable bowel syndrome, menopause, and mental disorders.

The diagnosis of neuroendocrine tumors is a big challenge for doctors, but once they are diagnosed, they can be successfully treated. The basis of treatment is surgery to remove the tumor, and as an alternative, pharmacological treatment is also used, administering somatostatin analogues to the patient.

Rare cancer - lymphoma

Lymphomas are cancers that originate in the cells of the lymphatic system. All lymphomas are malignant neoplasms, however, there are lymphomas of greater or lesser malignancy. Lymphomas usually affect adolescents and young adults.

The characteristic symptoms are enlarged lymph nodes, liver and spleen. There are also night sweats, fever unrelated to infection, weight loss, and abdominal pain associated with an enlarged liver.

The diagnosis is made on the basis of the histopathological examination of the collected lymph node. Treatment consists of chemotherapy and radiation therapy.

The prognosis for lymphoma depends on the type of lymphoma. Hodgkin's lymphoma, i.e. Hodgkin's lymphoma, is a cancer with a good prognosis, B- and T-cell lymphomas have a worse prognosis, at the level of 40-50%. healings.

Rare tumors - sarcomas

Sarcomas are a diverse group of cancers. They are rare tumors only in adults, they are always malignant. Sarcomas grow as a superficial tumor on the surface of the body that is characterized by rapid, aggressive growth and infiltration of surrounding tissue, causing pain, pressure, heaviness, swelling, and difficulty moving the affected limb.

Diagnostics is based on the histopathological examination of the tumor specimen, and the treatment is based on the surgical removal of the tumor along with a margin of he althy tissue.

The prognosis depends on the location of the tumor, the stage of the disease, and whether the tumor has been completely removed.

Overall, however, the prognosis is good, current treatments are showing good results and are oftencomplete recovery.

Read also:

  • Cancer: prevention, diagnosis and treatment of cancer
  • Cancer classification
  • Causes of cancer or where does cancer come from?
About the authorBow. Agnieszka MichalakA graduate of the First Faculty of Medicine at the Medical University of Lublin. Currently a doctor during postgraduate internship. In the future, he plans to start a specialization in pediatric hematooncology. She is particularly interested in paediatrics, hematology and oncology.

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Oncology