Clinical profile of Ewing’s sarcoma/ Primitive neuroectodermal tumors in a developing country, an institutional experience.
Ewing’s sarcoma is the second most common bone tumor among children. The present study is an observational study started from 2008 till 2017 at one regional cancer centre of north India. Purpose of study: To record clinical profile of EWING’S SARCOMA/PNET patients, the impact of prognostic factors on outcome, and to see overall outcome in localized, metastatic and recurrent disease. MATERIALS AND METHODS: A detailed data was recorded regarding demography, clinical characteristics, imaging details, laboratory investigations and treatment received, from Regional cancer centre registry data base. Only histologically proven Ewing sarcoma/Primitive neuroectodermal tumor patients were taken and recorded. Standard operating procedures were used in evaluation and staging of disease. RESULTS: Most common site was pelvic bones, followed by Tibia, chest wall ribs and then by femur. Sixty percent had localized disease and 40% had metastatic disease. Most common site of metastasis was lungs (31.4%), followed by bone marrow (29.6%). The optimum duration of chemotherapy received by only 24.7% of patients. 67% patients received local control treatment. Mean OS was 2.3(27.6 M) years and median OS was 2.0(24 M) years, with a range of 0.7(8.4 M) to 8.9(106.8 M) years. The 3year OS was 26.5% and 5-year OS was 7%. In univariate analysis, gander had significant impact on PFS, while as LDH, age, ESR had significant impact on OS. CONCLUSION: There are still unmet needs in our part of world, which can be looked on very low over all survivals compared to rest of world.
Key words: OS- Overall survival, DFS- Disease free survival, PFS-Progression free survival,
ES-Ewing’s sarcoma, PNET-Primitive neuroectodermal tumor, CR- complete response, PR- partial response, PD- Progressive disease, SD- stable disease.