{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T00:55:13Z","timestamp":1790729713486,"version":"4.1.0"},"reference-count":20,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"13","license":[{"start":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T00:00:00Z","timestamp":1790553600000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Neurosurg Case Lessons"],"published-print":{"date-parts":[[2026,9,28]]},"abstract":"\n BACKGROUND<\/jats:title>\n \n Primary CNS sarcoma,\n DICER1<\/jats:italic>\n -mutant is almost exclusively found in the supratentorial compartment. Isolated spinal lesions are exceedingly rare.\n <\/jats:p>\n <\/jats:sec>\n \n OBSERVATIONS<\/jats:title>\n \n The authors present a case of thoracic intradural extramedullary primary CNS sarcoma,\n DICER1<\/jats:italic>\n -mutant. An adult male with a remote history of a left cerebellar lesion resection with unknown pathology presented with sensation changes extending caudally from the torso. He subsequently underwent laminectomy and tumor resection of the lesion. The patient experienced recurrence at 1.5 months requiring re-resection for palliative decompression.\n <\/jats:p>\n <\/jats:sec>\n \n LESSONS<\/jats:title>\n Molecular characterization is essential for accurate diagnosis of this rare entity given its substantial overlap with histological mimics. Following resection and tissue identification of the tumor, patients should be monitored closely for local recurrence. While no standardized treatment exists, adjuvant chemoradiation therapy may improve time to progression and overall survival in an otherwise aggressive disease.<\/jats:p>\n \n https:\/\/thejns.org\/doi\/10.3171\/CASE26660<\/jats:ext-link>\n <\/jats:p>\n <\/jats:sec>","DOI":"10.3171\/case26660","type":"journal-article","created":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T00:03:36Z","timestamp":1790121816000},"source":"Crossref","is-referenced-by-count":0,"title":["Primary CNS sarcoma, DICER1-mutant with rhabdomyoblastic differentiation arising within the thoracic spine: illustrative case"],"prefix":"10.3171","volume":"12","author":[{"given":"John T.","family":"Tsiang","sequence":"first","affiliation":[{"name":"Department of Neurological Surgery, Rush University Medical Center, Chicago"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-9173-7350","authenticated-orcid":true,"given":"Tiffany M.","family":"San","sequence":"additional","affiliation":[{"name":"Chicago Medical School, Rosalind Franklin University of Medicine and Science, North Chicago"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S. Daniel","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Neurological Surgery, Loyola University Medical Center, Maywood"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5687-6488","authenticated-orcid":true,"given":"Ronak H.","family":"Jani","sequence":"additional","affiliation":[{"name":"Department of Neurological Surgery, Loyola University Medical Center, Maywood"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heather L.","family":"Smith","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, Loyola University Medical Center, Maywood, Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul M.","family":"Arnold","sequence":"additional","affiliation":[{"name":"Department of Neurological Surgery, Loyola University Medical Center, Maywood"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"issue":"8","key":"bib1","series-title":"Neuro Oncol","first-page":"1231","article-title":"The 2021 WHO Classification of Tumors of the Central Nervous System: a summary","volume":"23","author":"Louis","year":"2021","unstructured":"Louis DNPerry AWesseling PThe 2021 WHO Classification of Tumors of the Central Nervous System: a summary. Neuro Oncol. 2021;23(8):1231-1251. doi:10.1093\/neuonc\/noab106"},{"issue":"4","key":"bib2","series-title":"Cancer","first-page":"697","article-title":"Primary central nervous system sarcoma with DICER1 mutation\u2014treatment results of a novel molecular entity in pediatric Peruvian patients","volume":"128","author":"Diaz Coronado","year":"2022","unstructured":"Diaz Coronado RYMynarek MKoelsche CPrimary central nervous system sarcoma with DICER1 mutation\u2014treatment results of a novel molecular entity in pediatric Peruvian patients. 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