Immunotherapy for Solitary Fibrous Tumor (Hemangiopericytoma)

Abstract

Introduction: Solitary fibrous tumors (SFTs) of the central nervous system represent a unique entity with limited data on best treatment practices. Case Report: Here, we present a case of multiply recurrent central nervous system SFT treated with radiation and immunotherapy. Immunotherapy was chosen based on mutations of genes encoding DNA repair enzymes detected through next-generation sequencing of the tumor, DNA polymerase epsilon catalytic subunit (POLE) and mutL homolog 1. The use of radiation and immunotherapy led to slight shrinkage and no recurrence of the tumor for over 2 years. Conclusion: The presence of somatic DNA repair enzyme gene mutations in SFT may suggest a benefit from a combination of radiotherapy and immunotherapy. This may serve as a biomarker for guiding management in patients with this rare tumor.