the term primitive neuroectodermal tumors ( pnets ) were coined by hart and earle in 1973 . these are a group of malignant neoplasms derived from the primitive neural crest and are highly malignant and mainly exist in the central nervous system ( cns ) , chest wall , lower extremities , trunk , kidney , orbit , and rarely in the spine . in contrast , primary spinal cord tumors are very rare among children with an overall incidence rate of 0.26 per 100 person - years . population - based data indicate that the most common histological types were meningiomas ( 29% ) , nerve sheath tumors ( 24% ) , and ependymomas ( 23% ) in this age group . although multimodality treatments are available , the standard treatment for pnet is complete surgical excision followed by craniospinal radiotherapy . in this article , we present a patient with primary intraspinal extradural pnet and describe its treatment regimen and outcome . an 8-year - old female child had been experiencing back pain and weakness of both lower limbs for 1 month . fifteen days before admission , she also noticed difficulty in standing . within days , the weakness progressed , and the patient was bedridden . magnetic resonance imaging ( mri ) of the dorsal spine revealed a well - defined oval dorsal epidural lesion of size 6 cm  1 cm at d7d10 level with anterior compression of the cord . it was hypointense on t1-weighted image ( t1-wi ) and t2-wi and no obvious contrast enhancement [ figure 1 ] . magnetic resonance imaging study of the dorsal spine and paravertebral region showed well - defined oval epidural lesion of size 6 cm  1 cm from d7 to d10 levels along posterior aspect of cord with compression over the cord . it was hypointense on t1/t2 with no obvious contrast enhancement in view of her symptoms , the patient was taken up for surgery , and d6d11 laminectomy was done . a grayish - white , friable , soft , nonvascular tumor was seen extradurally which was adherent to the surrounding arachnoid and pia mater of cord . histopathology revealed tumor composed of small round cells having scant cytoplasm forming diffuse sheets and islands separated by thin fibrous bands . few pseudorosettes and occasional rosette formation seen along with areas of necrosis and vascular proliferation [ figure 3 ] . on immunohistochemical ( ihc ) staining , we found diffuse , finely granular cytoplasmic material staining positive for synaptophysin throughout the tumor . the final pathologic diagnosis of pnet was made along with some neuronal differentiation . in the absence of a primary brain tumor ( d ) tumor tissue removed en bloc is seen ( a ) tissue sections show densely packed sheets of primitive appearing cells ( h and e , 10 ) . ( b ) histopathological examination shows a highly cellular tumor , consisting mainly of small round - to - oval cells with hyperchromatic nuclei and remarkably scanty cytoplasm along with the presence of homer - wright pseudorosettes suggesting a round cell tumor . note : highly cellular and poorly differentiated cells with mitosis figures and areas of necrosis with karyorrhexis ( h and e , 40 ) the patient was planned for postoperative radiotherapy and was referred to radiation oncology for adjuvant radiotherapy . a treatment plan offering best therapeutic benefit both in terms of optimum dose to target tissue and maximum sparing of the uninvolved organs was selected . the patient was given a total of 30 gy to the brain and spinal cord along with 8 g boost to involved area on dmx varian linear accelerator . the patient tolerated the adjuvant radiotherapy well , and there was a marked improvement of pain and weakness symptoms 1 month after the radiotherapy . in 1973 , hart and earle were the first to propose the term  primitive neuroectodermal tumor  for describing a neoplasm which was composed of 90%95% of undifferentiated cells which did not fulfill the diagnostic criteria for other entities . rorke and becker and hinton in 1983 proved that all cns tumors composed of undifferentiated neuroepithelial cells are to be called pnets . pnets are more common in children as in our case and can also occur in adults . the mean age is between 5 and 77 years , and 80% of tumors occur in < 15 years of age . there is a male predominance , being 1.44.8 times more common in males than in females . pnet appears histologically as predominantly undifferentiated small , blue , round cell tumor with hyperchromatic nuclei , scanty cytoplasm , and frequent mitotic figures as was found in our case . on ihc , variable positivity may be noted depending on differentiation ( neuronal , glial , or myogenic ) . in our case , it was positive for synaptophysin . the tumor usually presents with nonspecific symptoms such as paraparesis , paresthesias , gait disturbance , and low back pain as in our case . mri features of pnet are also usually nonspecific , with most of them being hyperintense on t2-wi and iso- to hypointense on t1-wi , with heterogeneous enhancement on postcontrast sequences . in our case , it was hypointense on t1-wi and t2-wi and had no obvious contrast enhancement . suggest that although imaging findings are not specific , the diagnosis could be suggested when mri shows extradural large , well - circumscribed mass extending out from the intervertebral foramen and invading paraspinal soft tissues or vertebral bones in a young patient . after adjuvant chemotherapy , survival rates may be as high as 82% at 5 years . the use of chemotherapy for treating patients with pnets outside the cerebellum , particularly spinal pnets , has been rather inconsistent apart from the standard use of surgical resection and radiotherapy . it is probably because such tumors are rare , and there is a lack of prospective protocol to address the benefits of specific chemotherapeutic regimens . survival in spinal pnet ranges from 3 months to more than 3 years from the time of diagnosis . pnet should be included in differential diagnosis of spinal tumors , especially in children and young adults having an intraspinal mass . as there is no standard protocol for the management of spinal pnets , we advise complete resection with postoperative chemoradiation . primary intraspinal pnet subtyping after histopathological and ihc studies helps us plan an optimal treatment strategy for these aggressive tumors .