All information given here is of general nature only, a tumor therapy always belongs in the hands of an experienced oncologist!
Synonyms
Bone sarcoma, PNET (primitive neuroectodermal tumor), Askin’s tumor, Ewing – Bone sarcoma English: Ewing ́s sarcoma
Definition
Ewing’s sarcoma is a bone tumor originating from the bone marrow, which can occur between the ages of 10 and 30. However, children and young people up to the age of 15 are mainly affected. Ewing’s sarcoma occurs less frequently than osteosarcoma. Ewing’s sarcoma is located in the long tubular bones (femur (thigh bone) and tibia (shin bone)), as well as in the pelvis or ribs. In principle, however, all bones of the trunk and extremity skeleton can be affected; metastasis is possible, especially in the lungs.
Frequency
The probability of developing Ewing sarcoma is < 1:1,000,000, and studies have shown that about 0.6 new Ewing sarcoma patients per million people who are accustomed to the disease each year. Compared to osteosarcoma (approx. 11 %) and chondrosarcoma (approx.
6 %), Ewing’s sarcoma ranks third as a further representative of primary malignant bone tumours. While Ewing’s sarcoma mainly occurs between the 10th and 30th year of life, a main manifestation could be observed in the 2nd decade of life (15th year of life). The main manifestation is therefore the growing skeleton, with boys (56%) being slightly more likely to develop Ewing sarcoma than girls. If we now compare the primary malignant bone tumours of childhood and adolescence, Ewing’s sarcoma is in second place: In childhood bone sarcomas, the proportion of so-called osteosarcomas is about 60 %, while the proportion of Ewing’s sarcoma is about 25 %.
Causes
As explained and explained in the summary, the cause that can be held responsible for the development of Ewing’s sarcoma is not yet fully understood. However, it has been found that Ewing’s sarcoma often occurs when there are skeletal anomalies in the family or when patients suffer from retinoblastoma (= malignant retinal tumour occurring in adolescence) from birth. Research has shown that tumour cells of the so-called family of Ewing’s sarcoma show a change on chromosome no. 22. It is assumed that this mutation is present in about 95% of all patients.
Localization
The most frequent localizations of Ewing’s sarcoma are found in the long tubular bones, here mainly in the tibia and fibula, or in flat bones. Nevertheless, as a malignant bone cancer, Ewing’s sarcoma can affect all bones. The larger bones are most commonly affected, the smaller ones rather rarely.
If the long tubular bones are affected, the tumour is usually found in the area of the so-called diaphysis, the shaft area. Preferred localizations: Due to the early strong hematogenic metastasis (see following section) a localization in the soft tissues is also conceivable. – approx.
30% femur (thigh bone)
- Approx. 12% Tibia (shin bone)
- Approx. 10% Humerus (upper arm bone)
- Approx.
9% Basins
- About 8% fibula. Ewing’s sarcoma is only localised in approximately one in five cases in the pelvic bone as the primary tumour (the tumour’s point of origin). Significantly more often, however, the primary tumour is located in a long tubular bone.
The first symptoms can be swelling, pain and overheating in the area of the pelvis. The foot is a rare localisation of a primary tumour. It is more common that primary tumours from the tibia or fibula favour a metastasis in the foot.
If an unclear, painful swelling and overheating of the foot occurs, especially in adolescence, then in addition to juvenile arthritis, Ewing’s sarcoma should be ruled out under certain circumstances. In this case, the worst does not necessarily have to be assumed. Targeted diagnostics in the form of imaging can provide initial clarity about the causes of the complaints.
