Brain Tumor Res Treat.  2025 Jan;13(1):23-28. 10.14791/btrt.2024.0042.

A Rare Case of Isolated Central Nervous System Neoplasm With Histiocytic Features

• Choi Y
• Lee Y
• Kim YI
• Yang SH

• KMID: 2565046
• DOI: http://doi.org/10.14791/btrt.2024.0042

Abstract

This paper discusses a rare case of a 49-year-old female diagnosed with a central nervous systemisolated neoplasm with histiocytic features, classified as histiocytic neoplasm, not otherwise specified (NOS). Despite comprehensive histopathological and genetic studies, accurately diagnosing the case proved challenging. Initial surgical findings and frozen biopsy suggested a low-grade glioma; however, subsequent immunohistochemistry and genetic analysis excluded known histiocytic subtypes such as ALK-positive histiocytosis and Erdheim-Chester disease. The patient’s condition improved following occupational rehabilitation therapy for right upper motor weakness, as well as anticonvulsant and radiation therapy, and her neurological condition remains stable. This case underscores the diagnostic challenges of Histiocytic Neoplasms and the necessity for interdisciplinary collaboration and sophisticated diagnostic techniques.

Keyword

Histiocytosis; Erdheim-Chester disease; Brain neoplasms; Immunohistochemistry