Outcomes in different age groups with primary Ewing sarcoma of the spine: a systematic review of the literature

Abstract

<jats:sec><jats:title>OBJECTIVE</jats:title><jats:p>Ewing sarcoma (ES) is among the most prevalent of bone sarcomas in young people. Less often, it presents as a primary lesion of the spine (5%–15% of patients with ES).</jats:p></jats:sec><jats:sec><jats:title>METHODS</jats:title><jats:p>A systematic literature search was performed, querying several scientific databases per PRISMA guidelines. Inclusion criteria specified all studies of patients with surgically treated ES located in the spine. Patient age was categorized into three groups: 0–13 years (age group 1), 14–20 years (age group 2), and &gt; 21 (age group 3).</jats:p></jats:sec><jats:sec><jats:title>RESULTS</jats:title><jats:p>Eighteen studies were included, yielding 28 patients with ES of the spine. Sixty-seven percent of patients experienced a favorable outcome, with laminectomies representing the most common (46%) of surgical interventions. One-, 2-, and 5-year survival rates were 82% (n = 23), 75% (n = 21), and 57% (n = 16), respectively. Patients in age group 2 experienced the greatest mortality rate (75%) compared to age group 1 (9%) and age group 3 (22%). The calculated relative risk score indicated patients in age group 2 were 7.5 times more likely to die than other age groups combined (p = 0.02).</jats:p></jats:sec><jats:sec><jats:title>CONCLUSIONS</jats:title><jats:p>Primary ES of the spine is a rare, debilitating disease in which the role of surgery and its impact on one’s quality of life and independence status has not been well described. This study found the majority of patients experienced a favorable outcome with respect to independence status following surgery and adjunctive treatment. An increased risk of recurrence and death was also present among the adolescent age group (14–20 years).</jats:p></jats:sec>

Primary Ewing sarcoma/peripheral primitive neuroectodermal tumors in the cranial bone and mobile spine: what is the difference?

“…Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumors (pPNETs) are the second most common primary sarcoma of bone [ 1 ]. ES/pPNETs occur primarily in children and adolescents, and they exhibit an aggressive nature with a high tendency to form rapid growths and distant metastases [ 2 , 3 ]. Most primary ES/pPNETs occur in the pelvis and extremities [ 4 ].…”

Effects of different treatments and other factors on the prognosis of patients with ewing sarcoma

“…Ewing sarcoma is a small round cell tumor of bone or soft tissue originating from the neuroectoderm ( 1 ). Aggressive and highly malignant are the main pathological features of the tumor ( 1 ).…”

“…Ewing sarcoma is a small round cell tumor of bone or soft tissue originating from the neuroectoderm ( 1 ). Aggressive and highly malignant are the main pathological features of the tumor ( 1 ). Mainly due to the translocation of chromosome 11 and chromosome 22, the ews-fli1 fusion gene was formed, which led to the formation of ewing sarcoma ( 2 , 3 ).…”

“…In primary tumors, ewing sarcoma accounts for 6–8%, and occurs in the pelvis and limbs. It is rare in the spine ( 1 , 5 ). The disease has a high degree of malignancy, short course of disease, rapid metastasis, simple surgery, radiotherapy (RT), single-agent chemotherapy (CT), the effect is not very satisfactory, the vast majority of patients died within 2 years, 5 years survival rate does not exceed 10% ( 1 , 5 ).…”

“…Because most of the disease occurs within 2 years, it is generally recommended that CT should last for 2 years. Combined with a variety of treatments, the disease-free survival rate of ewing sarcoma patients has risen from less than 20% to 70–75% ( 1 ).…”