the incidence of ewing s sarcoma of bone ( esb ) is 1.7 to 2.1 per million people in the united states ( about 200 new cases / year ) they usually occur in the skeleton ( arms , legs , pelvis , chest wall ) and may spread to the lungs or other bony sites . primary ees of the spine are even more rare and less than 32 cases ( mostly adolescents ) have been reported . the lumbosacral region was the most common location ( 50% ) , followed by the thoracic region ( 25% ) . for the cervical spine ( including the cervico - thoracic junction ) only 8 cases have been reported . at present , the 5 year survival rate is about 41% . due to advancements in care survival for ewing sarcoma of the spine has significantly increased ( nearly 2-fold ) since the 1980s . nevertheless , prognosis is still dismal ( 3- to 4-fold reduced survival ) , if distant metastasis occurs . in general ewing tumours the role of surgery , especially with regard to local tumor control , is still under discussion . unlike in extremities , ewing sarcoma in the spinal epidural space or the spinal column can not be resected with wide margins due to relevant structures such as major vessels , oesophagus , and the myelon . however , decompressive surgery - even multiple - may preserve neurological function and therefore maintain quality of life although it may not prolong overall survival . based on an illustrative case , we discuss the relevant literature and try to work out a management algorithm based on preservation of neurological function and maintenance of quality of life . a 29-year - old man presented with a history of right c7-radiculopathy progressing over 3 months . then a hemiparesis occurred . magnetic resonance imaging ( mri ) demonstrated a bright enhancing intraforaminal cervical tumour with intra- and extraspinal components . during surgery an intra- and extradural gross total resection was performed . histology demonstrated a uniform population of small round blue cells with extensive mitoses and scattered necrosis . immunohistochemistry showed bright expression for mic-2 , and partial expression for vimentin , cytokeratin and synaptophysin . the fluorescent in situ hybridization ( fish ) detected the translocation of the ews gene on chromosome 22q12 . 4 weeks later , just before starting the adjuvant therapy according to euro - e.w.i.n.g . 99 protocol , a massive recurrence of the tumour lead to acute re - deterioration . postoperatively , the patient recovered and was able to walk with minor assistance , however the arm remained paretic . histology confirmed recurrence of the ewing sarcoma with a high ki-67 labelling index ( 30% ) . the planned chemotherapy was started shortly after surgery ( vincristine , ifosfamide , doxorubicin , etoposide ; six cycles ) . during the cycles the patient responded well . but after termination of the last one , the tumour progressed again . at this time the interdisciplinary tumour - board decided to administer a salvage chemotherapy ( topotecan and cyclophosphamide , two cycles ) combined with radiotherapy ( local boost and the whole spinal canal , 36 gy ) . afterwards he received highdose busulfan and mephalan and autologous stem cell transplantation . besides the dysfunction of fine motor skills by a distal paresis of the upper extremity and some degree of spinal ataxia he was able to walk with little assistance . eighteen months after treatment , the patient had no worsening of his neurological symptoms and is free of local and distant recurrence ( figures 1 - 3 ) . commonly it is thought that prognosis of ewing sarcoma is mainly dependent on the presence of metastases at initial diagnosis and not by tumour site , volume or local therapy modality . for ewing sarcoma of the spine there are only limited data about survival , especially for the primary ones . in their analysis mukherjee and colleagues found a 5-year survival rate of 41% in ewing sarcoma s patients with primary spine involvement . for pnet / ees in the spinal canal a 5-year survival rate between 0% and 37.5% was reported . for instance , bacci et al . concluded that surgery with adequate surgical margins is better than rt in cases of extremity es , but patients are better treated with full - dose rt from the start , when inadequate surgical margins are expected . ozaki et al . concluded that surgery adds to the safety of local control in patients with ewing sarcoma . interestingly , however , they reviewed also the 10-year overall survival and found a not significant relationship concerning the amount of surgical resection ( radical , wide , marginal , and intralesional ) . in comparison , treatment of ( primary ) ewing sarcomas of the spine has special characteristics . firstly , radiotherapy is limited by the tolerance of the spinal cord ( 55gy ) . secondly , achieving tumour free surgical margins or en bloc- resection may be difficult to obtain due to essential anatomic structures . however , decompressive surgery may be well indicated for spinal cord decompression and maintenance or restoration of neurological function . as in the presented case , the treatment may become very aggressive with multiple surgeries , chemo-/radiotherapy and peripheral blood stem cell transplantation ( pbsct ) . harimaya described a similar case in a 30- year - old woman , who died of intramedullary dissemination 14 months after the surgery . after subtotal resection the patient was treated by chemo- , and radiotherapy ( vaia , 50gy ) . in contrast kogawa reported a still complete remission 60 months after surgery for his patient , a 7-year old child with a primary epidural ewing sarcoma . others reported that consolidation with high - dose chemotherapy combined pbsct failed to improve overall survivalrates in patients with high - risk es . in the english literature , 8 cases of ees arising primarily within the cervical spinal canal have been reported ( table 1 ) . the mean age of these patients at diagnosis was 26 years ( range 7 - 30 years ) . most patients ( 75% ) were between 20 and 30 years old , 12.5% even in the first decade of life . it is difficult to draw strong conclusions from the presented case and the small number of cases reported in the literature . nevertheless , we believe that in case of spinal primary ees an aggressive surgical resection may improve local tumor control , maintain neurological function , preserve quality of life and may result in prolonged overall survival . the therapy is based on a multimodal concept of chemo-/radiotherapy and surgery . in cases with primary ees with spinal involvement , the role of surgery is important in order to maintain neurological function and quality of live .
primary extraosseous ewing sarcomas ( eess ) are an extremely rare pathological entity . 
 less than 32 cases have been reported in the literature . here 
 we report an uncommon case with very rapid progression in the cervical region with extra- and intradural involvement . 
 we present a thorough review of the literature and discuss possible treatment modalities . 
 the medline database was searched using the search terms : ewing sarcoma , extraosseus tumour , treatment , management , cervical spine . 
 a previously healthy 29-year - old man complained of right - sided radiculopathy ( c7 ) . 
 magnetic resonance imaging showed an enhancing foraminal , sandglass shaped neurinoma - like lesion . 
 surgery revealed an intraand extra - dural lesion , which was histologically diagnosed as ewing sarcoma . despite gross total resection 
 , there was a massive symptomatic tumor recurrence within 6 weeks . 
 a second gross total resection was realized . 
 the patient was treated according to the euro e.w.i.n.g .- protocol ( vide ) and recovered very well ( progression - free interval during therapy ) . 
 several decompressive re - surgeries were realized with adjuvant radio - chemotherapy . at the last follow - up 
 ( 17 months after initial surgery ) the patient was in remission with a good quality of live . 
 this case is to illustrate that despite extensive therapeutic efforts , the progression - free survival in case of primary ees may be very short . to maintain neurological function and good quality of live as long as possible , a multimodal strategy seems to be adequate . 
 like in the present case this implies several surgeries and adjuvant chemo - and radiotherapy . 
 whether this improves overall survival remains unclear .