{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,24]],"date-time":"2026-07-24T11:38:12Z","timestamp":1784893092504,"version":"3.55.0"},"reference-count":50,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"16","license":[{"start":{"date-parts":[[2022,10,17]],"date-time":"2022-10-17T00:00:00Z","timestamp":1665964800000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,10,17]]},"abstract":"<jats:sec>\n                    <jats:title>BACKGROUND<\/jats:title>\n                    <jats:p>Primary extraosseous intracranial Ewing\u2019s sarcoma, also known as a peripheral primitive neuroectodermal tumor or \u201csmall round blue cell tumor,\u201d is an extremely rare entity with limited representation in the literature beyond the pediatric population.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>OBSERVATIONS<\/jats:title>\n                    <jats:p>A 67-year-old male suffering occipital headache, nausea, and gait disturbance was found to have a large, avidly contrast-enhancing cerebellopontine angle mass extending into the cervical spinal canal with associated mass effect on medulla, cerebellum, fourth ventricle, and cervical spinal cord. This mass was not present on the imaging from 8 years prior. He underwent surgical debulking and pathology results demonstrated a malignant small round cell tumor showing diffuse immunopositivity for cytokeratins, CD99 and NKX2.2 with EWRS1-FLI1 rearrangement in 84% of the nuclei confirmatory of Ewing\u2019s sarcoma. After 14 cycles of chemotherapy and 6 weeks of radiotherapy, 22 months after discovery, the patient remains in clinical and radiographic remission with complete return to his baseline functioning.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>LESSONS<\/jats:title>\n                    <jats:p>Primary skull base extraosseous Ewing\u2019s sarcoma should be considered in the differential diagnosis even in the elderly population when imaging studies demonstrate aggressive tumor growth patterns. Tumor debulking to establish a diagnosis followed by adjuvant chemoradiation therapy can result in clinical improvement with remission.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3171\/case22214","type":"journal-article","created":{"date-parts":[[2022,10,17]],"date-time":"2022-10-17T04:34:25Z","timestamp":1665981265000},"source":"Crossref","is-referenced-by-count":5,"title":["Primary intracranial extraosseous Ewing\u2019s sarcoma of the skull base in an elderly adult: illustrative case"],"prefix":"10.3171","volume":"4","author":[{"given":"Kristine","family":"Ravina","sequence":"first","affiliation":[{"name":"Departments of Neurosurgery and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sonora A.","family":"Windermere","sequence":"additional","affiliation":[{"name":"Departments of Neurosurgery and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qing","family":"Zhao","sequence":"additional","affiliation":[{"name":"Departments of Pathology & Laboratory Medicine and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adam","family":"Lerner","sequence":"additional","affiliation":[{"name":"Department of Medicine, Section of Hematology\/Oncology, Boston University School of Medicine, Boston, Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Dyer","sequence":"additional","affiliation":[{"name":"Radiation Oncology, Boston Medical Center, Boston, Massachusetts; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Urvashi","family":"Upadhyay","sequence":"additional","affiliation":[{"name":"Departments of Neurosurgery and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R. 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