Prognosis and Outcome of Cervical Primary Extraosseous Intradural Extramedullary Ewing Sarcoma: A Systematic Review

Abstract

Primary spinal extraosseous Ewing sarcoma (ES) is a rare mesenchymal tumor characterized by high malignancy, occurring in a few patients with ES. The occurrence of this tumor in the intradural extramedullary spinal region is infrequent. This systematic review examines primary extraosseous intradural extramedullary ES in the cervical region to provide specific outcomes and evaluate the role of adjuvant chemoradiation in overall prognosis. A systematic review was conducted to identify all cervical primary extraosseous intradural extramedullary ES reported in the literature. The search included the databases of PubMed, Google Scholar, Medline, Embase, and Scopus from inception to June 2021. Inclusion criteria include a reported death outcome or a minimum one-year follow-up.Our search retrieved 21 articles that involved the cervical spine, but only 11 cases met the inclusion criteria. Of the nine patients who demonstrated disease progression, six experienced local failure, two had distant craniospinal axis failure, and one had systemic metastases. Five patients died of the disease at a median of 11 months after diagnosis. Our analysis showed a one-, two-, and five-year progression-free survival (PFS) of 36.4%, 36.4%, and 12.1%, respectively. The one-, two-, and five-year overall survival rates were 72.7%, 62.3%, and 46.8%, respectively. Three of the five (60%) patients who died received craniospinal radiotherapy. Of the six patients who survived, two received craniospinal radiotherapy (33%), and one received whole spine radiotherapy (17%). This review showed that patients with cervical primary extraosseous intradural extramedullary ES had poor progression-free survival and overall survival rates. The addition of adjunct craniospinal radiotherapy did not improve the prognosis of these patients.

Adult Intradural Extramedullary Ewing’s Sarcoma of the Lumbar Spine and Involving the Intervertebral Foramen: A Case Report

“…Reviewing the literature, nearly 50 cases of IEES has been reported from 1997 to 2022 [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21]. IEES usually affects the lumbar/ sacral region as in our case [12].…”

Post-Radiotherapy Complications in Ewing Sarcoma: A Case Report and Literature Review

“…Subsequently, the finding of the t(11; 22) translocation led to widespread suspicions of a neural origin. Finally, an epithelial origin was inferred from the erratic expression of cytokeratin and tight junction proteins [3,4,[7][8][9][10][11][12][13]. Additionally, spinal cord compression-related neurological impairments may cause patients to present later than expected.…”

A Rare Presentation of Extraosseous Ewing Sarcoma Manifesting as a Dumbbell Tumor on the Nape of the Neck

“…EES is considered to be a poor prognostic factor to survival when compared to skeletal Ewing sarcoma, due to its propensity for invasion of local adjacent organs and metastasizing to the lungs [ 4 , 5 ]. According to a recent review from Carballo Cuello et al, the survival for EES remains dismal: their one-, two-, and five-year progression-free survivals are 36.4%, 36.4%, and 12.1%, respectively, whereas the one-, two-, and five-year overall survival rates are 72.7%, 62.3%, and 46.8%, respectively [ 15 ]. Treatment of ES and EES usually involves the use of neoadjuvant chemo- or radiotherapy and en bloc resection of the localized tumor [ 4 , 14 ].…”

“…Upon diagnosis of ESFT, the initial treatment involves induction chemotherapy with Vincristine, Ifosfamide, Doxoroubicin, and Etoposide (VIDE) regimen for 6 cycles at 2–4-week intervals [ 14 , 15 ]. After induction chemotherapy, patients undergo local therapy with either surgical resection if the tumor can be resected or radiotherapy if it cannot, followed by consolidation therapy with Vincristine, Actinomycin D, and Cyclophosphamide (VAC) or Vincristine, Actinomycin D, and Ifosfamide (VAI) regimen [ 15 , 16 ]. High-risk patients will undergo high-dose chemotherapy with Busulphan and Melphalan followed by stem cell rescue options [ 14 , 16 ].…”