International Journal of Hematology and Oncology 2023, Vol 33, Num 4 Page(s): 014-019
RETROSPECTIVE EVALUATION OF ADULTS WITH SOFT TISSUE EWING SARCOMA AND PRIMITIVE NEUROECTODERMAL TUMORS

ŞEBNEM KARAKAN1, BERNA ÖKSÜZOĞLU1, DOĞAN UNCU1, NURİYE YILDIRIM1, BURÇİN BUDAKOĞLU1, METİN KÜÇÜKAZMAN1, HULKİ GENÇ1, SAADET AKDUR1, NURULLAH ZENGİN1

Ankara Numune Eğitim ve Araştırma Hastanesi, 5. İç Hastalıkları Kliniği

Keywords: primitive neuroectodermal tumors, soft tissue tumors, ewing's sarcoma
We retrospectively evaluated 11 Ewing family (EF) tumor patients between 1998-2002 in the records of 5th Internal Medicine and Medical Oncology Department, Ankara Numune Education and Research Hospital. Median age and follow-up period were 20 (16-63) years and 14 (3-36) months, respectively. All of the patients had systemic therapy and 9 patients had additional local therapy. Local therapy modalities were surgery (n=4, 36.3%), radiotherapy (n=2, 18.1%) and combination of both (n=3, 27.2%). Complete remission, partial remission and progression were noted in 2 (20%), 2 (20%) and 6 (60%), respectively. One patient died with cranial metastasis without response evaluation. Mean progress-free survival of 4 patients who responded therapy was 5.5 (3-6) months. The mean overall survival value was 14 (2.5-36) months. Mean overall and progress-free survival of patients with and without pelvic localization were similar (p>0.05). Tumor size, localization, local treatment modality, LDH and erythrocyte sedimentation rate were not correlated with survival rates. Although statistically insignificant, our patients had unfavorable prognostic factors, such as age, bulky tumor, metastasis at presentation. These factors might contribute to the unfavorable outcome in these patients. Larger studies are needed to confirm this issue. Multidisciplinary approach and new treatment modalities might improve the prognosis of PNET patients.