Treatment of nonmetastatic Ewing’s sarcoma family tumors of the spine and sacrum: the experience from a single institution

Abstract

The objective of this study is to determine the best local treatment combined with neoadjuvant chemotherapy for ESFT of the spine and sacrum, for the best local treatment for Ewing sarcoma family tumors (ESFT) according to the primary site is still unclear. Nowadays surgery is used in local treatment of ESFT, but literature is scarce on the best local treatment in sites where surgery is problematic, such as the spine. This study evaluates the outcome and the rate of local recurrence of ESFT in the spine and sacrum when treated with neoadjuvant chemotherapy, and locally by radiotherapy alone or surgery, followed by reduced doses of radiotherapy. Forty-three patients with nonmetastatic ESFT located in the spine and sacrum were treated at our institution between 1983 and 2000 with neoadjuvant chemotherapy, and locally by radiotherapy alone in 26 cases, and surgery followed by radiotherapy at reduced doses in 17. The 5-and 10-year event-free survival (EFS) was 37 and 30%, and the 5-and 10-year overall survival was (OS) 42 and 32%. The prognosis was unrelated to gender and age, tumor volume, chemotherapy protocol, and local treatment. The outcome seemed worse for patients with primary tumors located in the sacrum than for patients with tumors located in the rest of the spine (5-year EFS = 23 vs. 46%). For these patients the results were significantly worse than for those we achieved with neoadjuvant treatment for ESFT located in other sites. However, no differences were observed between patients locally treated with radiotherapy alone and those treated by radiotherapy followed by surgery. We concluded that regardless of the type of local treatment even when associated with neoadjuvant therapy, ESFT in the spine and sacrum has a poor outcome and prognosis is significantly worse than that of primary ESFT in other sites.

Association of surgical resection and survival in patients with malignant primary osseous spinal neoplasms from the Surveillance, Epidemiology, and End Results (SEER) database

“…These tumors also are known for having high recurrence rates. The repeated 5-year progression-free survival has ranged from 0 to 25 % for osteosarcoma [25,30,34], 50-70 % for chondrosarcoma [4,7,16], 30-60 % for Ewing's sarcoma [2,3,15,20], and 45-65 % for chordomas [13,17]. The high recurrence rates, limited survival duration, and functional morbidity associated with these tumors have supported the need for aggressive multi-modality strategies for these tumors.…”

“…Surgical resection is typically reserved for cases in which the primary tumor can be completely removed, which is often difficult in the spine due to anatomical limitations [29]. Bacci et al [3] evaluated 43 patients with spine tumors over an approximate 20-year time span at a single institution and found no difference in survival between patients who were treated locally with radiation and those treated by radiation and surgery. Likewise, Paulino and colleagues [26] evaluated 76 patients with localized Ewing's sarcoma (only 11 of which had spine involvement) and found there was no difference in survival for patients with radiation, surgery, and radiation with surgery.…”

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

“…For instance, lesions that present in the sacrum are often large prior to diagnosis, making en bloc resection technically challenging. 9 Lesions of the mobile spine, however, often present earlier and are more amenable to resection. For Ewing sarcoma, subtotal or partial resection should be avoided; en bloc resection with negative tumor margins should be the surgical goal.…”

“…For Ewing sarcoma, subtotal or partial resection should be avoided; en bloc resection with negative tumor margins should be the surgical goal. 9,15 Unfortunately, metastatic disease can be present in 25% of patients at presentation. The most common locations for metastatic spread include the lungs, other osseous sites, the lymphatic system, the brain, and the abdominal viscera.…”

Imaging of presacral masses—a multidisciplinary approach

“…2 In fact, sacrococcygeal vs non-sacral ewing sarcomas have long-term survival rates of 25% vs 86%, respectively. 2,34,35 The mainstay of therapy is chemoradiation, with local surgical debulking. Unfortunately, therapeutic options do not appear to improve survival rates.…”