Thymus carcinomas occur with a frequency of less than 0.2 to 0.4 per 100,000 inhabitants (= incidence). They are therefore among the rarest cancers. At the same time, they are also the most common tumor formations in adults in the upper mediastinum. Put simply, this is the upper chest area outside the lungs.
The thymus is part of the immune system and is included in the lymphatic system. Therefore, thymomas usually consist of altered epithelial cells and some T lymphocytes as well as other cells of the immune system.
The most commonly used classification of thymic carcinoma is that of the World Health Organization (WHO). This divides the tumor into six classes based on cell origin (type A, AB, B1, B2, B3 and C). Onlythymus carcinoma types B3 and C have a malignant character with a poor prognosis.
Cancer cell
The clinical staging, which is also decisive for therapy, is based on the so-called Masaoka classification. The histological parameters and the spread of the thymus carcinoma are particularly important for this. The latter also includes the formation of metastases. In general, however, thymic carcinomas metastasize less frequently than other tumors. If metastases are present at an advanced stage, they are more likely to be found in neighboring organs and tissues.
Thymomas grow slowly. Symptoms do not appear for a long time. Thymic carcinoma is therefore very often diagnosed as an incidental finding. If tumor-related symptoms occur, they are usually non-specific. For example
- pain,
cough- ,
- hoarseness or
- breathing problems (dyspnoea)
have been described- .
have been described.
In addition, there are paraneoplastic syndromes such as myasthenia gravi, which is found in up to 45 % of patients.
As previously mentioned, thymic carcinoma is often discovered by chance as a mass on an X-ray. The exact diagnosis of the thymoma is then made using computer tomography (CT).
Computed tomography is used for diagnosis
Recently, positron emission tomography, or PET for short, has also been increasingly used for the diagnostic imaging of thymic carcinoma. Histological characterization of the thymoma is sometimes necessary.
This enables the differential diagnosis of other cancers or metastases from other tumor foci to be reliably excluded. However, such a procedure is not explicitly required by the specialist associations.
The treatment of choice ("gold standard") for thymic carcinoma is complete surgical removal of the tumor (= tumor resection). However, around 40% of thymic carcinomas are already growing invasively into the surrounding tissue at the time of diagnosis. This makes removal of the tumor impossible in some cases.
Depending on the stage of the disease, the thymoma can be completely removed (= R0 resection) or a margin with residual tumor must be left behind. In the latter case, the operation is usually followed by radiotherapy. The aim of this is to destroy the residual tumor and prevent metastasis.
The aim is to completely remove the thymus carcinoma surgically
If surgery is not possible, doctors resort to a combination of radiotherapy and chemotherapy to treat the thymus carcinoma. Reasons why thymic carcinoma may be inoperable include
- Poor general condition of the patient,
- serious concomitant diseases,
- Tumor too large or already strongly infiltrating.
The prognosis of patients with thymic carcinoma (thymoma) is closely related to the Masaoka stage of the disease at diagnosis, e.g:
- In stage I, the 5-year survival is 100% and the recurrence rate is < 0.9%.
- In stage III, 5-year survival is still 89%, but the recurrence rate rises to 28.4%.
- Finally, in stage IVa, 5-year survival is 71% and the recurrence rate is 34.3%.
Early diagnosis and treatment at an early stage of the disease therefore offer the best chances of recovery.
Doctors who treat thymus carcinoma are usually specialists in general and thoracic surgery as well as oncologists. In terms of treatment, thymomas or thymic carcinomas belong to the broad field of interdisciplinary oncology due to their diversity. They therefore require cooperation between surgeons and other specialist groups, as well as in the follow-up care of patients.