Kategorie: Nádory dětského a adolescentního věku
Číslo abstraktu: 203p
Ewing’s sarcoma, a highly malignant primary bone tumour, is most frequently observed in children and adolescents aged 4–15 years. 90% of patients are younger than 20 years. Ewing’s sarcomas are rare in patients over the age of 40 years.
Prognosis is poor with 5-year overall survival of 55% to 65% in localized patients and not exceeding 25% in primarily metastatic disease.
Patients with primary bone cancers should be evaluted by a multidisciplinary team with demonstrated expertise in the management of such patients.
Treatment is divided into induction therapy (3-6 courses) followed by local therapy and consolidation chemotherapy. Local therapy is surgery, radiotherapy or a combination of both. Despite Ewing’s sarcoma being a radiosensitive tumour, surgery is preferred treatment for local control. Radiotherapy should be limited to patients with marginal or intralesional surgery or with inoperable tumours.
With adequate multimodal therapy, the results in terms of survival in patients over 40 years are comparable to those in adolescence. Patients should be enrolled in international trials, and if necessary treatment should be adjusted for lower tolerance and comorbidity.
Since 2003 our Ewing’s sarcoma patients were treated according to the international Clinical Trials EURO-E.W.I.N.G.99, from which a new protocol EWING 2008 is coming out.
A 53 years old man was found to have localized Ewing’s tumour of femur at the beginning of year 2003. After 3 courses of induction chemotherapy VIDE, a large clinical and CT/MRI progression was reported. The patient refused an amputation (limb sparing surgery was not possible).
In the end of local radiotherapy, another progression was reported. These after, patient has agreed with the amputation. His disease was still localised. After the amputation (50% necrosis only) the patient refused the next chemotherapy according to protocol. The only thing he agreed with, was a low dose chemotherapy of etoposid (35mg/m2) for the next 12 months.
In February 2011 is the patient alive free from disease (8 years from the time of diagnosis). Exception proves the rule. Probably.
Datum přednesení příspěvku: 21. 4. 2011