Ewing’s sarcoma: aggressive malignant tumor with a peak incidence during the second decade of life.
Ewing's sarcoma is a primary bone tumor that affects mainly children and adolescents. It's one of a group of cancers known collectively as the Ewing sarcoma family of tumors – ESFT or sometimes just EFT. It's the second most common bone cancer in children, but it's also relatively uncommon. It accounts for only 1% of all childhood cancers. Although it can occur at any age, it very rarely occurs in adults over the age of 30.
Because many illnesses can cause the same symptoms as Ewing's sarcoma, it's sometimes missed in its early stages. But early diagnosis and treatment is important. If found early enough, before it spreads to multiple organs,
The vertebral column is an infrequent site of primary involvement in Ewing sarcoma. Yet when Ewing sarcoma is found in the spine, the urge for decompression is high because of the often symptomatic compression of neural structures. It is unclear in alleviating a neurological deficit whether chemotherapy is preferred over decompressive laminectomy.
Treatment is mostly palliative: radical excision followed by RTX (very radiosensitive) and chemotherapy 1).
The use of stereotactic body radiotherapy (SBRT) is well-accepted 2).
SRS for spine metastases from Ewing sarcoma can be considered a treatment option in adolescent and young adult patients and is associated with acceptable toxicity rates. Further studies must be conducted to determine long-term local control and toxicity for this treatment modality 3).
Combined intracranial/extracranial lesions.
Spine metastases are more common than primary spine lesions.
Intramedullary Ewing's Sarcoma
see Ewing's Sarcoma peripheral primitive neuroectodermal tumor
Fifteen patients with Ewing sarcoma primarily located in the spine were treated between 1983 and 2015. Localization, neurological deficit expressed as Frankel grade, and outcome expressed as Modified Rankin scale before and after initial chemotherapy, the recurrence rate, and overall survival were evaluated.
Nine patients (60%) were female. The age at presentation was 15.0 ± 5.5 years (range: 0.9-22.8 years). Ten patients (67%) were initially treated with chemotherapy, and 1 patient (7%) was treated primarily with radiotherapy followed by chemotherapy. The remaining 4 patients (27%) were initially treated with decompressive surgery. All patients treated primarily nonsurgically improved neurologically at follow-up, showing the importance of chemotherapy as an effective initial treatment option.
Adequate and quick decompression of neural structures with similar results can be achieved by chemotherapy and radiotherapy, avoiding the local spill of malignant cells 4).
Intradural Extramedullary Ewing's Sarcoma in the Cervical Region 5).
A 21-year-old woman presenting with quadriplegia which was initially diagnosed with an epidural abscess in view of her MR scan and raised inflammatory marker levels. Histology revealed an epidural extra-osseous Ewing's sarcoma (EES). Epidural location of EES is a very rare condition which can be very challenging to diagnose. Early diagnosis and surgical excision followed by chemotherapy represent the main stem of management 6).
A case report demonstrates that hypofractionated partial-brain radiation therapy with limited margins is a reasonable approach following gross tumor resection of Ewing sarcoma metastases to the brain. The patient presented with 2 intracranial metastases treated with gross-total resection followed by radiation therapy to 30 Gy in 5 fractions. The patient experienced symptomatic treatment-related inflammatory changes with resolution after receiving dexamethasone. He remains alive at 21 months of follow-up with no evidence of disease 7).