Rare cancers

Comparatively few people suffer from a rare tumor disease: Fewer than 6 in 100.000 people are affected by the respective cancer. In this article you will learn what is important for diagnosis and treatment.

At a glance

About one fifth of all cancer patients suffer from a tumor that is rarely diagnosed.
Rare disease situations can also arise if the cancer has arisen in an unusual organ or if, for example, there are concomitant diseases or pregnancy.
For some of the rare cancers, there are treatment guidelines based on clinical studies.
For others, doctors use specialist publications as a guide for treatment.
For some patients with a rare tumor, treatment is possible as part of a trial.
Those affected by a rare tumor are advised to seek treatment at a specialized center.

Notice: The information in this article cannot and should not replace a visit to the doctor and should not be used for self-diagnosis or self-treatment.

Rare cancers: An elderly woman is lying in a bed. A nurse sits next to her and holds her hand. The two smile at each other

When do experts refer to a rare tumor?

Experts speak of a rare type of cancer when fewer than 6 out of 100.000 people are diagnosed with it each year. In total, there are about 200 different rare cancers. It is estimated that around one fifth of all cancer patients suffer from a rare tumor.

Different causes can lead to rare disease situations:

It is actually a type of tumor, or at least a particular subtype, that affects comparatively few people.

In addition, there may be other reasons why doctors refer to a tumor as rare:

The diagnosed cancer originates in an unusual organ or tissue.
The patient himself or herself is in a special situation. Examples would be cancer during pregnancy or breast cancer in men. Even a tumor that usually occurs in people in a different age group can be called rare.

It can happen that concomitant diseases limit the usual treatment options or make them impossible. Even then, doctors have to find individual solutions.

Who is affected by the designation "rare If the patient is unsure, he or she should ask exactly what is meant by this term.

There are often treatment guidelines for rare cancers and disease situations that doctors can use as a guide.

What symptoms may occur with a rare tumor?

There are no typical symptoms for a rare tumor. The symptoms depend on the region of the body where the tumor occurs. A rare cancer that grows close to the surface of the body may show up as a painless swelling or lump. If it enlarges, displaces tissue or even grows into nerves, there may be feelings of tension or pain in the affected area of the body. A rare tumor that has developed in internal organs can go unnoticed for a long time before it triggers symptoms. Only when it grows into surrounding tissues or organs, it can lead to local complaints and dysfunctions.

If there is a swelling or if symptoms last longer than 4 weeks, the affected person should go to the doctor and have this clarified. This can be done by the family doctor or- depending on the location of the tumor Being a specialist. Physicians in private practice are already in a good position to narrow down the causes of the complaints and, if necessary, to initiate further diagnostic steps.

What are the causes of rare cancers?

Cancer is caused by changes in the genetic material of a single cell. They cause the cell to multiply unchecked and form a tumor. Cancer is said to exist when the cancer cells grow into the surrounding tissue and eventually even form metastases.

Why this happens cannot be answered for most cancer patients. Probably several factors are working together. In addition to coincidence, which leads to an increased risk of cancer depending on age, lifestyle, environmental and hereditary factors may also play a role.

Important to know: It is always advisable to lead a healthy lifestyle with plenty of exercise, eat a balanced diet and avoid cancer risk factors. However, this is no guarantee that one will not develop cancer.

How rare cancers are diagnosed?

Rare tumors can occur in many different subtypes and in different parts of the body. This can complicate the diagnosis. However, a correct diagnosis is the prerequisite for optimal treatment.

If there is a suspicion of tumor disease? The patient should then present to a center that specializes in the treatment of tumors in the region of the body in question. Experts from various disciplines work together there- mainly oncologists, surgeons, pathologists, radiologists and radiotherapists. Experts refer to this as an interdisciplinary team.

Physicians have a variety of complementary diagnostic procedures at their disposal:

questioning the patient (anamnesis) about previous diseases and symptoms
physical examination
Examination of blood and / or urine in the laboratory
Imaging procedures such as computer tomography (CT), magnetic resonance imaging (MRI), ultrasound or X-rays: Radiologists use these to check how extensive the tumor is.
Removal of a tissue sample (biopsy) and fine-tissue examination: pathologists examine the removed tissue in a laboratory and thus find out the type of tumor .

If there is a suspicion of a rare tumor after the fine-tissue diagnosis has been made? Then, experts may recommend sending the tissue samples to a reference laboratory. A second opinion from an experienced pathologist can help correctly classify the tumor.

You want to know more about the diagnostic process? The website of the Cancer Information Service, German Cancer Research Center (Deutsches Krebsforschungszentrum, DKFZ) provides an initial overview.

How rare tumors are treated?

One of the most important pieces of information for treatment planning is the determination of the cancer type. In addition, physicians need to know whether:

the tumor is localized.
Has invaded neighboring tissues.
Whether it has already spread to distant organs in the body, i.e., metastasized.

This distinction is a prerequisite for determining the appropriate therapy.

How cancer is treated?

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If the tumor is limited to one region of the body or one organ? If there are no metastases yet?

Then the primary goal is usually to completely remove the tumor tissue and surrounding tissue. Depending on the type of tumor, chemotherapy or radiotherapy, for example, can also reduce the risk of relapse.

If metastases have already developed?

In some patients, surgeons can surgically remove not only the tumor but also individual metastases. If necessary, the patients also receive chemotherapy and/or radiation therapy. Various targeted drugs and / or a clinical trial may also be considered in this situation.

However, it is more common that treatment with the goal of a complete cure is no longer possible once metastases are present. In this case, the aim is to slow down tumor growth, prevent complications from the tumor disease and alleviate troublesome symptoms. Such treatment is called palliative therapy. Above all, it should ensure a good quality of life.

If the tumor is not confined to one region of the body, such as a rare leukemia or lymphoma?

Then, for example, chemotherapy or radiotherapy, but also targeted drugs can be used to cure or at least inhibit tumor growth and alleviate symptoms.

For some of the rare cancers, there are treatment guidelines based on clinical trials. For others, physicians base their treatment on the results of individual clinical studies or published case studies.

For some patients with a rare tumor, treatment in the context of a study is recommended.

In addition to the type and spread of the tumor, the so-called patient factors are also important for determining the treatment plan, for example, the age or concomitant diseases of a patient.

Important to know: Not every clinic is equipped to treat rare tumor diseases. Patients should clarify with their physician whether professional societies or other institutions recommend that they seek treatment at a specialized center, if possible. Such centers have a lot of experience with the respective disease pattern. One criterion: They offer participation in clinical studies for the respective type of cancer.

If you want to be treated in a specialized center, this often means an additional organizational and financial effort. This should be discussed in advance with the relevant health insurance company.

You want to know more about the treatment of a rare type of cancer and how to find a specialized center? On the website of the Cancer Information Service, German Cancer Research Center, you will find an initial overview of these options.

How to get fit again after cancer therapy?

Cancer treatment can be protracted and demand all one’s strength. To help cancer patients get back into their daily lives, they can take advantage of rehabilitation (rehab) or follow-up treatment:

A general oncology rehab helps patients regain their strength.
In addition, rehab can be tailored to the individual patient’s condition. It can have special focuses- depending on whether, for example, orthopedic, gynecological, neurological or urological limitations are present.
The rehabilitation clinics also inform patients about what they themselves can do to contribute to the success of their therapy: this may include diet, exercise and sport, or complementary therapy methods, for example.

What is meant by medical, occupational and social rehabilitation?? Who can make use of rehab? On the website of the Cancer Information Service, German Cancer Research Center (Deutsches Krebsforschungszentrum, DKFZ), you will find all the relevant facts about oncological rehabilitation.

What to consider during the follow-up or follow-up of a rare tumor?

How patients continue to be cared for after the end of therapy depends on the type and location of the tumor and the treatment carried out.

Follow-up care focuses in particular on the following:

Recognizing and treating the consequences of a therapy: these can be, for example, late effects that only become noticeable after the treatment has been completed.
Detect relapses and metastases: If a relapse occurs, experts refer to it as a recurrence.
Recognizing a second tumor.

Affected persons are therefore examined regularly after the end of therapy, initially at short intervals, then at longer intervals if necessary. The doctors ask about complaints, examine the patient and check the blood and possibly the urine. In certain situations, they use imaging examinations.

Patients can discuss how often they need to come for follow-up care and exactly which examinations are necessary with the doctors treating them.

If a rare tumor cannot be permanently cured, regular follow-up examinations help to detect troublesome symptoms at an early stage. Physician and patient can jointly discuss further therapy options and supportive measures. This should help patients maintain their quality of life for as long as possible.

What life with a rare tumor is like?

Many patients want to become active themselves in order to contribute to their recovery. There are various options for this. What can help to cope better with the disease and the consequences of therapy depends on the individual situation:

Exercise can help to overcome fatigue and weakness. It should be adapted to individual performance.
Balanced, healthy food to nutrition therapy can prevent or treat malnutrition.
Psycho-oncology counseling can help when worries and fears become overwhelming.

Where to find contacts for rare tumor types?

Doctors from different disciplines work closely together in the treatment of rare cancers. However, there is often no certification for centers with a particularly high level of experience in treating a rare type of cancer. Depending on the tumor disease, it may be that only a few centers in Germany have much experience with its treatment.

How to find a treatment center for a rare tumor?

In the care atlas for people with rare diseases you can search for care facilities and reference centers for rare diseases in Germany.

The portal is one of several partners in the "National Action Plan for People with Rare Diseases" (NAMSE) was created. However, the information is not limited to cancer.

Accessible are centers either by searching for the keyword cancer or the actual name of the disease. In addition to the centers, support groups and other contacts are given out, if available.

At the European level, patients can also find centers for rare cancers üsearch via the European network RARE CANCERS EUROPE. Apart from an overview page in German, the information is written in English.

You have further questions about how to deal with the disease in everyday life and about other support options? You can find information about this on the website of the Cancer Information Service of the German Cancer Research Center.

If you have any questions about rare cancers, you can also contact the Cancer Information Service in person by calling the toll-free number 0800 – 420 30 40 or by e-mailing [email protected].