What are thymoma and thymic carcinoma?
Thymoma and thymic carcinoma are rare tumors arising from the anterior mediastinum. Thymoma is a low-grade tumor that tends to invade surrounding organs, but rarely spreads. In contrast, thymic carcinoma is highly aggressive, and may arise from malignant transformation of a pre-existing thymoma.
Who gets thymoma or thymic carcinoma and why?
Because of the role of the thymus in the immune system, these tumors are associated with conditions, including gastrointestinal disorders (chronic ulcerative colitis), collagen and autoimmune disorders (myasthenia gravis, Sjögren’s syndrome, sclerodermia, polymyositis), hypogammaglobulinemia, , endocrine disorders (Cushing’s syndrome), nephrotic syndrome, and hematologic syndromes (red cell aplasia, agranulocytosis).
Is there a risk of brothers / sisters developing the same cancer or other types of cancer?
If any autoimmune or paraneoplastic disease exist, strict observation is required.
What are the typical symptoms and signs of thymoma and thymic carcinoma?
The commonest signs are cough, breathlessness, chest pain and bulging veins in the neck. A third of children have no symptoms, and it is a chance finding on x-rays.
Which investigations are necessary for a child with thymoma and thymic carcinoma?
A radiological assessment (CT scan chest or MRI scan, and PET- CT scan). Other tests maybe done, such as blood markers to exclude other tumours.
Surgical resection or open biopsy of the tumor is necessary to establish diagnosis.
Are there different stages of the disease?
Thymoma is usually limited to the thymus. Thymic carcinomas can spread to other parts of the body such as lymph nodes or the lungs.
What about the thymoma and thymic carcinoma treatment?
Complete removal of the tumour is the gold standard for treating all thymic tumours. When a tumor is inoperable an open biopsy or core needle biopsy is usually performed and then delayed surgery is recommened after initial chemotherapy.
Some patients require chemotherapy if the tumour is inoperable.
This type of treatment may be used for patients with disease that has not been removed by surgery or is very invasive.
What are the results of treatment?
The prognosis of thymoma is generally good.
It is much more difficult to treat thymic carcinoma so the results are not as good.
What research is happening for thymoma and thymic carcinoma ?
This is mainly happening in adult patients with thymic tumours and involves testing new drugs which target the thymic cancer specifically, after standard treatment has failed.
What EXPert is doing for children with thymoma and thymic carcinoma?
The main goals of EXPeRT group for children with thymoma and thymic carcinoma, contain:
- Collection of data of children from European countries
- Creation of guidelines for diagnosis and treatment
- Establishment of treatment centers with an advisory service
- Collaboration in international network with other specialists to provide multidisciplinary care