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- Title
Primary intracranial sarcoma, DICER-1 mutant, with hemorrhagic presentation: A case report.
- Authors
Edelbach, Brandon Michael; Gospodarev, Vadim; Raghavan, Ravi; Dye, Justin
- 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.
- Subjects
MULTINUCLEATED giant cells; TUMOR growth; INTRATHECAL injections; SYMPTOMS; GENOMICS
- Publication
Surgical Neurology International, 2024, Vol 15, p1
- ISSN
2229-5097
- Publication type
Case Study
- DOI
10.25259/SNI_259_2024