{"id":"0466ab36-2f91-4af8-b4e8-0d20a2bd13aa","slug":"primary-intracranial-sarcoma-dicer-1-mutant-with-hemorrhagic-presentation-a-case-report","title":"Primary intracranial sarcoma, DICER-1 mutant, with hemorrhagic presentation: A case report","authors":["Brandon Michael Edelbach","Vadim Gospodarev","Ravi Raghavan","Justin Dye"],"abstract":"Background: Primary intracranial sarcomas (PIS) are rare tumors with mesenchymal origins. These tumors have a heterogeneous clinical presentation and are associated with a poor prognosis. Case Description: This report highlights the complexities associated with PIS by focusing on a 26-year-old male with recurrent tumor growth facing unique challenges regarding diagnosis and treatment options . A high-grade spindle-celled neoplasm with sarcomatous features characterized the patient’s tumor. There were additional morphologic changes, including multinucleated giant cells and rare foci with eosinophilic spheroids. Genomic analysis revealed a DICER1-associated PIS. Treatment involved endovascular embolization, multiple surgical interventions, intrathecal etoposide injections, and oral pazopanib with adjuvant radiation therapy. Conclusion: This case additionally highlights an unusual association between PIS and anomalous hypervascularity, refractory hemorrhage, and subdural effusions, a presentation that is increasingly being reported in this type of tumor.","thumbnailUrl":"https://sni-digital-videos.s3.amazonaws.com/articles/0466ab36-2f91-4af8-b4e8-0d20a2bd13aa/featured/hero-1781559355475.png","publishDate":"2024-07-26T00:00:00.000Z","doi":"10.25259/SNI_259_2024","categories":["Unique Case Observations","Case Report"],"fullTextUrl":"https://surgicalneurologyint.com/wp-content/uploads/2024/07/13017/SNI-15-253.pdf"}