Diagnostic challenges of primary central nervous system histiocytic sarcoma: case report and literature review

Histiocytic sarcoma (HS) is a rare malignant tumor that primarily affects the lymph nodes, intestines, skin, and soft tissues. Primary central nervous system histiocytic sarcoma (PCNSHS) is even rarer. We present the case of a 50-year-old Asian male with PCNSHS who was hospitalized after experiencing intermittent headaches and slurred speech for a week. During surgery, both tumor cells and purulent material were observed, and the diagnosis of PCNSHS was ultimately confirmed by immunohistochemistry. Furthermore, we conducted a literature review from 1952 to the present, screening and analyzing 49 related cases across 41 publications.