primary intracranial sarcoma, DICER1-mutant

Term ID

DOID:0081316

Synonyms

Definition

A brain sarcoma that is 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. https://pubmed.ncbi.nlm.nih.gov/35789272/

References

• ICDO:9480/3
• NCI:C186610

Ontology

Human Disease   ( DOID:0081316 )