Adult primary cervical extra-osseous Ewing's sarcoma: A case report and short literature review

Primary spinal epidural extraskeletal Ewing's sarcoma (EES) is extremely rare, with a peak incidence in the second decade of life. EES in old people is challenging to treat due to the lack of specific guidelines. In this paper, I present a unique case of adult primary cervical epidural EES with a 13-month follow-up. A short literature review of the therapeutic approaches and prognosis is also presented.

I present a case of a 49-year old male patient who presented with right upper limb pain, numbness, hand grip weakness, and hyperreflexia of 3 months duration. Enhanced cervical magnetic resonance imaging showed a homogenously enhancing epidural and paravertebral soft tissue mass extending from the C6 to the T2 that appeared hypointense on T1 and hyperintense on T2. The patient underwent biopsy that confirmed EES via histopathology. Treatment with chemotherapy and radiotherapy resulted in tumor resolution and symptom relief.

EES is a type of PNET. Surgical removal is generally the treatment of choice, followed by adjunctive chemotherapy and radiotherapy. In old patients with large tumors, a more conservative approach with biopsy, adjuvant chemotherapy and radiotherapy is recommended.

Adult primary cervical epidural EES is a neurosurgical challenge due to the extension to the surrounding vital structures making the tumor not amenable for total resection as in the present case. In the absence of specific therapeutic guidelines, our case highlights the need to individualize the treatment modality according to age, tumor extension, and feasibility of total tumor resection.