Primary Ewing’s sarcoma of the spine: case report

Abstract

-Primary malignant sarcomas of the spine are extremely rare. Because of biological heterog e n e i t y, these tumors have variable sensitivity to radiation and chemotherapy. Adequate local contro l t h rough complete tumor removal is an important therapeutic goal. However, aggressive resection of tumors in the spinal column must be coupled with restoration of spinal column stability and minimization of neural deficits. The balance of these factors makes treatment of primary sarcomas of the spine challenging, and dictates an individual approach to treatment. We report on a 18 years old man with primary Ewing´s s a rcoma of the nonsacral spine. The clinical picture and imaging characteristics were analyzed as well as the management modalities and outcome.KEY WORDS: Ewing´s sarcoma, primary spine tumor, spine stabilization. Sarcoma de Ewing primário da coluna vertebral: relato de casoRESUMO -Sarcomas malignos primários da coluna são extremamente raros. Devido sua heterogeneidade biológica, estes tumores apresentam sensibilidade variada à radioterapia e à quimioterapia.O contro l e local adequado através da ressecção tumoral completa é um objetivo terapêutico importante. Contudo, a ressecção completa dos tumores da coluna vertebral deve ser realizada com a restauração da estabilidade espinhal e minimização do déficit neurológico. O equilíbrio entre estes fatores torna o tratamento dos sarcomas da coluna desafiador, exigindo-se uma abordagem individualizada para o tratamento de cada paciente. Relatamos um caso de sarcoma de Ewing da coluna não-sacral em um homem de 18 anos. O q u a d ro clínico e características de imagem foram analisados, bem como a modalidades de manejo e o prognóstico.PALAVRAS-CHAVE: sarcoma de Ewing, tumor espinhal, estabilização espinhal. P r i m a ry malignant sarcomas of the spine are extremely rare. It has been estimated that they account for only 3.5% to 14.9% of all primary bone sarc om a s 1 -5 . In the study of primary vertebral Ewing's sarcoma (ES), the division of the spine into nonsacral a n d sacral is important and is dictated by the diff e re n t behavior of ES in these two regions in terms of re sponse therapy and survival rates 4 . Most studies on ES involving the mobile spine are limited to case re p o rt s . A few larger series evaluated patients with primary ES affecting the mobile and nonmobile spine 1 , 6 -9 . Mic roscopic examination demonstrates small, ro u n d blue cells with a uniform appearance. Electron micro sc o p y, immunohistochemistry, and cytogenetic studies help diff e rentiate ES from other small, round blue ceel tumors. The translocation t(11;22)(q24;q12) is identified in most cases of ES 1 0 . Adequate tre a t m e n t of primary malignant spine tumors is aimed at cure rather than palliation whenever possible. The definitive management of Ewing's sarcoma of the spine, as in other locations, could include three main modalities: surg e ry, radiotherapy, and combination chemotheraphy 1 . The purpose of the current study is to re p o rt the diagnosis an...

Primary Ewing's sarcoma of the cervical vertebra: one case report and literature review

“…MRI is very sensitive in detecting the early bone marrow involvement of the lesion. Whole-body radionuclide bone scan is an important step to exclude the metastatic Ewing's sarcoma [3,11].…”

“…MRI delineates the tumor extends beyond the affected vertebral body into the posterior spinal elements. The paraspinal soft-tissue masses extend above and below the involved vertebra [8], compressing the cord circumferentially through the intervertebral foramen [11].…”

Primary osseous tumors of the pediatric spinal column: review of pathology and surgical decision making

“…Salvage surgery for residual tumor can be considered based on location and prognosis. Spinal resection and reconstruction (spondylectomy) can be considered following chemotherapy to reduce the risk of local recurrence, with improved long-term survival compared with that following intralesional excision or radiation therapy; 26 cases involving the sacrum may involve pelvic fixation and reconstruction. In the case of lower-grade tumors, surgery followed by adjuvant chemotherapy and radiation is the current treatment standard.…”

Unusual Presentation of a Primary Ewing’s Sarcoma of the Spine with Paraplegia: A Case Report

“…If the patient is neurologically stable then a tissue diagnosis is required, a large bore needle biopsy may be considered. Once the diagnosis of Ewing's sarcoma is confirmed, neo adjuvant chemotherapy is started [8]. A neurological deficit warrants early surgical decompression of the cord which can provide maximum chance of recovery and also specimen for histopathology.…”

“…Following surgery post operative chemotherapy to control micrometastases and radiotherapy is very important. Neo adjuvant chemotherapy which is usually given as three or four drug regimen is more beneficial in shrinking the tumour, thereby increasing the chances of total excision, but also takes care of micrometastases and gives an idea about responsiveness of the tumour to adjuvant therapy [8].…”