Balkan Journal of Medical Genetics

UNUSUAL MANIFESTATION OF EXTRAOSSEOUS EWING SARCOMA: REPORT OF 3 CASES
Ioannidou M.1,*, Tsotridou E.1, Samoladas E.2, Tragiannidis A.1, Kouskouras K.3, Sfougaris D.4, Spyridakis I.5, Foroulis C.6, Galli-Tsinopoulou A.1, Hatzipantelis E.1
*Corresponding Author: MD Maria Ioannidou, St Kiriakidi 1, Children’s and Adolescent’s Hematology- Oncology Unit of 2nd Department of Pediatrics, AUTh, AHEPA University General Hospital, Thessaloniki, Greece. Zip code: 54621. Tel.: 00306942067923. E-mail: ioannidou@auth.gr
page: 77

INTRODUCTION

Extraosseous Ewing Sarcoma (EES) is a highly aggressive, poorly differentiated small round cell tumor accounting for 20-30% of Ewing Sarcoma (ES) cases. EES belongs to the histological group of Ewing Sarcoma Family Tumors (ESFT) originating from neuroectodermal and mesenchymal cells along with classical osseous ES, small-cell tumor of the thoracopulmonary region (Askin tumor) and soft- tissue-based primitive neuroectodermal tumors (PNETs) (1–4). In accordance with findings from adult studies (5), the characteristics of patients, as well as prognosis, differ between ES and EES. In the EES group a higher mean age and lower percentage of males and Caucasians were observed. Extraskeletal origin was a favorable prognostic factor. Importantly, no significant differences in genomic features were noted (6). The clinical manifestation varies largely depending on the site of origin, with a rapidly growing mass causing pain being the most common symptom. Commonly affected extraskeletal sites include the paravertebral spaces, lower extremities, head, neck and pelvis, while distant metastases are detected in up to 25% of cases, most commonly in the lungs (1,7). The recommended treatment is local therapy (surgery and/or radiotherapy) plus chemotherapy (1–3). Surgery appears to play a more vital role in EES compared to osseous ES, since complete resection is a prognostic factor for survival (8, 9). Chemotherapy is a key element in the treatment of EES, with both neoadjuvant and adjuvant therapy showing comparable results in patients with localized disease (10–12). EES is radiosensitive, but the use of radiotherapy has been reduced over the years mainly due to the advances in surgical technique and the radiationassociated risks (secondary malignancies) (13). We describe 3 patients with an uncommon presentation of EES, who were successfully treated in our Unit.

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