Therapy

Therapeutic approaches are usually applied on several levels. On the one hand, the so-called therapy plan preoperatively usually provides for chemotherapeutic treatment (= neoadjuvant chemotherapy). Even after the surgical removal of the Ewing sarcoma, the patient is treated therapeutically by radiation therapy and, if necessary, renewed chemotherapy.

This is where a difference to osteosarcoma becomes noticeable: Compared to the Ewing sarcoma, the osteosarcoma has a lower radiation sensitivity. Therapeutic goals: A so-called curative (healing) therapy approach is particularly given for patients whose Ewing sarcoma is localized and does not have any metastases. Meanwhile, the so-called neoadjuvant chemotherapy in combination with surgery and radiation therapy opens up further opportunities.

If the Ewing sarcoma metastasizes outside the lung (= generalized tumor disease; extrapulmonary metastases), the therapy usually has a palliative (life-prolonging) character (see below). Therapy modalities:local:

Preoperative chemotherapy
Surgical therapy (wide or radical resection according to Enneking)
Radiotherapy

Systemic:antineoplastic chemotherapy Curative therapy: Palliative (life-prolonging) therapy:Patients who have a generalized tumor disease (= extrapulmonary metastases), the primary tumor is located at the trunk of the body and/or the primary tumor proves to be inoperable. In such cases only palliative therapy is usually possible.

In such cases, the focus is usually on maintaining the quality of life, so that the therapy focuses on pain relief and preservation of function. – Combination therapy (first line: doxorubicin, ifosfamide, methotrexate/leukovorin, cisplatin; second line: etoposide and carboplatin) (protocols may change at short notice)

Aggressive multi-substance chemotherapy pre- and postoperatively
Local treatment in the form of surgical tumour resection or radiation alone
Supplementation of the therapy by pre-irradiation (for example in the case of inoperable tumours, non-responders) or by post-irradiation
It is important to mention in the context of surgical therapy that, not least due to the further development of surgical methods, limb preservation surgery is possible in many cases. However, the prospect of healing always has the highest priority, so that the focus should always be on radicality (= oncological quality) and not on possible loss of function.
Chemotherapy can then be continued (see above). This is then referred to as consolidation. – Patients with lung metastases may require additional interventions in the lung area, such as partial removal of the lung wings.

Forecast

Whether or not recurrences occur is strongly dependent on the extent of metastasis formation, the response to preoperative chemotherapy and the “radicality” of tumor removal. It is currently assumed that the five-year survival probability is about 50%. In particular, surgical improvements over the past 25 years have made it possible to improve the probability of survival.

Here the survival rate is about 35% . The chances of recovery from Ewing’s sarcoma are initially, as with other cancers, to be regarded as individually different, because statistics only show average recovery and survival rates. The chances of recovery are increased if the tumour can be completely removed by surgery.

Chemotherapy should be carried out beforehand to reduce the size of the tumour. After the surgical removal of the tumour, a further chemotherapy should be carried out to kill off any remaining tumour cells. If the tumour cannot be removed completely by surgery, the chances of recovery are much lower.

Here, too, a follow-up treatment with chemotherapy should be carried out. A tumour that cannot be operated on should be irradiated in any case. In general, it can be said that the chances of healing Ewing’s sarcoma are worse if metastases are already present at the time of diagnosis. This means that the tumour has spread and continues to grow elsewhere in the body.