A case of giant Ewing's sarcoma (EES)/primitive neuroectodermal tumor (PNET) of the cervicothoracic junction in children with incomplete paralysis of both lower limbs: Case report and literature review

Background Extraosseous Ewing's sarcoma/primary neuroectodermal tumor (EES/PNET) is a rare, malignant, small round blue cell tumor, which usually involves the larynx, kidneys, and esophagus. The most common metastatic sites are lung and bone. The incidence of epidural EES/PNET was 0.9%, and a detailed search of the PubMed literature found only 7 case reports of epidural ESS/PNET at the cervicothoracic junction in children. Case description We report a case of epidural ESS/PNET at the cervicothoracic junction in a child with chest and back pain as the first symptom, which worsened after half a year and developed incomplete paralysis of both lower extremities and urinary incontinence. She underwent emergency surgery, chemotherapy and radiotherapy, and died of lung metastases 8 months after surgery. Conclusion Primary epidural tumors are mostly benign, such as spinal meningiomas and neuromas. Contrary to what has been previously thought, we report a case of malignant epidural EES/PNET at the cervicothoracic junction without bone destruction; The rarity of epidural EES/PNET at the cervicothoracic junction in children has led to a lack of data, particularly on prognostic factors and recurrence patterns. Due to the difficulty of early diagnosis and high mortality, spine surgeons must explore and increase their awareness of this disease.

[1]  Jiong Cai,et al.  Case report: Primary intracranial EWs/PNET in adults: Clinical experience and literature review , 2022, Frontiers in Oncology.

[2]  Raja K. Kutty,et al.  Ewing Sarcoma of the Cervical Epidural Space Presenting with Tetraplegia: Case Report and Review of Literature. , 2017, World neurosurgery.

[3]  G. Rosen,et al.  Adult Primary Spinal Epidural Extraosseous Ewing's Sarcoma: A Case Report and Review of the Literature , 2016, Case reports in neurological medicine.

[4]  Jun Yang,et al.  Comparison of Primary Spinal Central and Peripheral Primitive Neuroectodermal Tumors in Clinical and Imaging Characteristics and Long-Term Outcome. , 2016, World neurosurgery.

[5]  K. Karabatsou,et al.  Cervical epidural extra-osseous Ewing sarcoma mimicking an epidural abscess , 2016, British Journal of Neurosurgery.

[6]  A. Seoparjoo,et al.  Primary extraskeletal Ewing's sarcoma/primitive neuroectodermal tumour of breast , 2013, BMJ Case Reports.

[7]  H. Kato,et al.  Ewing sarcoma of the thoracic epidural space in a young child , 2013, European Spine Journal.

[8]  A. Amirjamshidi,et al.  Primary spinal extradural Ewing's sarcoma (primitive neuroectodermal tumor): Report of a case and meta-analysis of the reported cases in the literature , 2012, Surgical neurology international.

[9]  Jun Shen,et al.  Intraspinal primitive neuroectodermal tumor: imaging findings in six cases. , 2011, European journal of radiology.

[10]  J. Bridge,et al.  Extraskeletal Ewing's sarcoma of the thoracic epidural space: case report and review of the literature. , 2011, Oncology reports.

[11]  D. Indelicato,et al.  Spinal and paraspinal Ewing tumors. , 2010, International journal of radiation oncology, biology, physics.

[12]  L. Goumnerova,et al.  Primary pediatric intraspinal sarcomas. Report of 3 cases. , 2009, Journal of neurosurgery. Pediatrics.

[13]  H. Buyukhatipoglu,et al.  PRIMARY SPINAL EPIDURAL EXTRAOSSEOUS EWING'S SARCOMA MIMICKING A SPINAL ABSCESS , 2007, Pediatric hematology and oncology.

[14]  M. Lequin,et al.  Primitive neuroectodermal tumours (PNETs) located in the spinal canal; the relevance of classification as central or peripheral PNET , 2006, Journal of Neuro-Oncology.

[15]  E. Pignotti,et al.  Second Malignancy in 597 Patients With Ewing Sarcoma of Bone Treated at a Single Institution With Adjuvant and Neoadjuvant Chemotherapy Between 1972 and 1999 , 2005, Journal of pediatric hematology/oncology.

[16]  J. Goldblum,et al.  Morphologic and Immunophenotypic Diversity in Ewing Family Tumors: A Study of 66 Genetically Confirmed Cases , 2005, The American journal of surgical pathology.

[17]  S. Aida,et al.  Primary cervical spinal epidural Extra-osseous Ewing’s sarcoma , 2004, Acta Neurochirurgica.

[18]  K. Harimaya,et al.  Primitive Neuroectodermal Tumor and Extraskeletal Ewing Sarcoma Arising Primarily Around the Spinal Column: Report of Four Cases and a Review of the Literature , 2003, Spine.

[19]  S. Jain,et al.  Primary intraspinal primitive neuroectodermal tumor (PNET): a rare occurrence. , 2002, Neurology India.

[20]  M. Debiec‐Rychter,et al.  Peripheral primitive neuroectodermal tumor within the spinal epidural space , 2001, Neuropathology : official journal of the Japanese Society of Neuropathology.

[21]  S. Eustace,et al.  Extraskeletal Ewing’s Sarcoma: A Case Report and Review of the Literature , 2000, Spine.

[22]  K. Hongo,et al.  A case report and review of the literature , 2006, Journal of Neuro-Oncology.

[23]  S. Rick Miller,et al.  A Rare Occurrence , 2001 .

[24]  S. Ferrari,et al.  Prognostic factors in nonmetastatic Ewing's sarcoma of bone treated with adjuvant chemotherapy: analysis of 359 patients at the Istituto Ortopedico Rizzoli. , 2000, Journal of clinical oncology : official journal of the American Society of Clinical Oncology.

[25]  P. Pizzo,et al.  Principles and Practice of Pediatric Oncology , 1989 .