Successful Treatment of Central Nervous System Histiocytic Sarcoma With Craniectomy and Adjuvant Radiotherapy

Histiocytic sarcoma (HS) is a rare, aggressive non-Langerhans histiocytic cell neoplasm of hematopoietic origin. Histiocytic sarcoma is prone to early systemic metastasis, rendering early diagnosis and treatment critical determinants for patient outcome. Primary HS originating from the central nervous system (CNS) is exceptionally rare and portends a poor prognosis. This grim clinical course is further complicated by the challenging diagnosis and the lack of standard treatment guidelines for the disease. This is due to the exceptionally rare nature of primary CNS histiocytic sarcoma and the limited data available on the successful management of the disease, prompting the therapeutic approach to be guided by retrospective data from case reports or single-institutional studies with a limited number of patients. Here, we report a case of a young Middle Eastern male who was diagnosed with primary CNS histiocytic sarcoma, successfully treated with frontotemporal craniotomy and adjuvant radiation therapy. We also elucidate the role of the CD163 biomarker in diagnosing HS and using surgery and adjuvant radiotherapy (RT) as a successful treatment approach for primary CNS histiocytic sarcoma.