Primary Ewing Sarcoma in Spinal Epidural Space: Report of Three Cases and Review of the Literature

SummaryEwing sarcoma (ES) is the most common malignant bone tumor in children younger than 10 years of age. Extraosseous ES (EES) arising from the spinal epidural space is extremely rare. We aimed to report herein three cases of primary spinal epidural EES and to review the related literature. We report our experience with three cases of primary spinal epidural EES in a single institution (aged 34, 14, and 65 years). The patients were admitted with complaints of weakness of the lower extremities and urinary retention. Magnetic resonance imaging (MRI) showed an epidural mass with cord compression at T4-T6, L2-L3 and T7-T8 levels, respectively. All three patients underwent laminectomy; total resection of the epidural mass was performed in two patients and gross total resection in one patient. Immunohistochemical examinations revealed ES. All patients underwent chemotherapy and radiotherapy after surgery. No evidence of recurrence or metastasis was detected after 18 and 16 months, respectively, for the first two cases. In the third patient, gross total resection was performed due to tumor infiltration and invasion to the surrounding tissue, and residual tumor in the surrounding tissue was noted on MRI for the 14-month follow-up period.

Key words:Ewing Sarcoma,Primary, Spinal Epidural

ÖzetEwing sarkoma (ES) 10 yaş altı çocuklarda en sık görülen malign kemik tümörüdür. Spinal epidural mesafeden kaynaklanan ekstraosseus ES (ESS) oldukça nadirdir. Biz burada primer spinal epidural ESS tanısı alan üç vakayı sunmayı ve litaretürü gözden geçirmeyi amaçladık. Tek klinikte tedavi edilen primer spinal epidural ESS tanısı alan üç vakalık deneyimimizi sunuyoruz. Yaşları sırasıyla 34, 14,65 olan hastalar alt ekstremite kuvvetsizliği ve idrar retansiyonu bulguları ile hastanemize başvurmuşlardır. Magnetik rezonans görüntülemede (MRG) sırası ile T4-T6, L2-L3 ve T-T8 seviyelerinde spinal kord kompresyonu yapan epidural kitle tespit edilmiştir. Üç hastaya da dekompresif laminektomi yapılmıştır. İki hastada epidural kitle total çıkartılırken bir hastada kitle gros total çkartılmıştır. İmmunohistokimyasal incelemelerle ES tanısı konulmuştur. Tüm vakalar cerrahi sonrası kemoterapi ve radyoterapi tedavisi görmüşlerdir. İlk iki vaka için sırasıyla 18 ve 16 aylık takiplerde rezidü yada rekürens gözlenmezken çevre dokulara tümör ünvazyonu ve infiltrasyonuna bağlı gros total rezeksiyon yapılan üçüncü hastada 14 aylık takipler sonrasında MRG'de çevre dokularda rezidü tumör dokusu saptanmıştır. Primer spinal epidural ESS vakalarında total rezeksiyon sonrası kemoterapi ve radyaterapi en etkili tedavi şekli olarak önerilmektedir

Anahtar Kelimeler:Ewing Sarkoma,Primer, Spinal Epidural

Keywords:Cranial firearm injuries, intracranial hemorrhage, morbidity and mortality.

INTRODUCTIONEwing sarcoma (ES) is the most common malignant bone tumor in children younger than 10 years of age and is characterized histologically by small round blue cells with varying degree of neuroectodermal differentiation(1-6,10,12,24,33-38,43-51). It mostly arises in the long bones and pelvis, while the hands, feet, vertebral bones, and soft tissue are affected considerably less often(1,6,7,13-22,40-44). Extraosseous ES (EES) was identified by Tefft in 1969(47). Osseous ES, EES, and peripheral primitive neuroectodermal tumors (pPNET) are generally known today as ES family tumors. Primary spinal PNET and/or spinal extraskeletal ES family tumors are rare lesions appearing in the spinal extradural space(4,5,6,9,12,13,14,25-30,32,40). EES arising from the spinal epidural space is extremely rare, and to date, only 63 cases of primary EES arising in the spinal epidural space have been reported in the literature. We aimed to report herein our experience with three cases of primary spinal epidural EES in a single institution, seen between January 2012 and July 2013. We describe the clinical presentation and therapeutic strategies, together with a brief review of the literature.