Primary central nervous system histiocytic sarcoma: a systematic review of clinical presentations, radiologic findings, treatment strategies, and prognostic outcomes

Background Primary central nervous system histiocytic sarcoma (PCNSHS) is a rare malignant neoplasm of histiocytic origin. Its clinical features, optimal management, and outcomes remain poorly defined. Methods We systematically reviewed PubMed, MEDLINE, Web of Science, Scopus, and Cochrane Library. Thirty-nine studies (35 case reports, 4 case series) describing 47 patients with PCNSHS were included. Clinical presentation, imaging findings, treatment modalities, and outcomes were analyzed. Results The mean age at diagnosis was 43.6 years (range 17 months–84 years); 51% were male. Headache (38%), vomiting (23%), and gait instability (23%) were the most frequent presenting symptoms. Lesions were solitary in 55% and multifocal in 44%. Contrast enhancement was reported in 81%, and peritumoral edema in 34%. Surgery was performed in 26 patients (55%), with gross total resection (GTR) in 13 (28%). Chemotherapy and radiotherapy were administered in 51% and 53% of cases, respectively. Median overall survival (OS) was 7.5 months (range 1–70 months). GTR was associated with longer survival compared to non-GTR (median OS 16 vs. 6 months; HR 6.67, 95% CI 1.82–25.0; p = 0.004). Radiotherapy improved survival (8 vs. 4.6 months; HR 2.71, 95% CI 1.31–5.63; p = 0.007). Chemotherapy showed a nonsignificant trend toward benefit (8 vs. 5 months; HR 1.56, 95% CI 0.78–3.12; p = 0.205). Solitary lesions correlated with improved outcomes. Conclusion PCNSHS is an aggressive tumor with a poor prognosis (median OS 7.5 months). Accurate diagnosis requires comprehensive imaging and immunohistochemistry to exclude mimickers. Multimodal therapy—particularly GTR and radiotherapy- appears to prolong survival, underscoring the need for collaborative efforts and prospective studies.