Primary Intracranial Sarcoma, DICER1-Mutant

**Semantic type:** Neoplastic Process

**Definition:** A primary intracranial sarcoma composed of malignant pleomorphic or spindle neoplastic cells typically demonstrating myogenic and/or chondroid differentiation. Cytoplasmic eosinophilic globules and myxoid stroma formation are usually present. It is associated with mutations in the DICER1 gene.