Abstract Details

Title Primary Central Nervous System Histiocytic Sarcoma: A Systematic Review of Clinical Presentation, Radiologic Findings, Treatment Strategies, and Prognostic Outcomes

Topic Neuro-oncology

Presentation(s) P9 - Poster Session 9 (11:45 AM-12:45 PM)

Poster/Presentation
Number 6-009

Objective The objective of this study is to systematically analyze the clinical presentations, imaging features, treatment modalities, and outcomes of Primary Central Nervous System Histiocytic Sarcoma (PCNSHS).

Background Histiocytic Sarcoma is one of the malignant hematolymphoid neoplasm of histiocytic cell origin. Primary Central Nervous System Histiocytic Sarcoma (PCNSHS) are extremely rare neoplasm and can affect any parts of the brain and spinal cord. Due to its rarity, clinical characteristics and outcomes remain poorly understood.

Design/Methods We searched PubMed, MEDLINE, Web of Science, Scopus, and the Cochrane Library using the keywords Histiocytic Sarcoma, Central Nervous System Neoplasms, Histiocytic Neoplasms (Second, Primary), Sarcoma, Brain Neoplasms. Thirty-five relevant studies were identified including thirty -one case reports and four case series, comprising 43 reported cases of PCNSHS. Data on clinical presentation, imaging, treatment modalities, and outcomes were extracted and analyzed.

Results The mean age of the patients was 43.19 years (range: 17 months to 84 years), with 53.5% being male. The most common symptoms reported by the patients were headache (39.5%), vomiting (23.2%), gait instability (23.2%), and lower extremity weakness (23.2%). Imaging shows multifocal lesions in 44.1% and solitary lesions in 48.8%, with enhancement (81%) and peritumoral edema (40%) being common features. Kaplan-Meier analysis shows that gross total resection (p=0.018), chemotherapy (p=0.05), radiotherapy (p=0.009), and solitary lesions (p=0.043) prolong overall survival. Immunohistochemical testing reveals a predominance of histiocytic markers, including CD-68+, lysozyme, and CD-163+.

Conclusions PCNSHS is a rare, aggressive malignant tumor with a tendency for early widespread dissemination and high mortality. Its diagnosis is particularly challenging due to its rarity and the broad range of differential diagnoses. A full panel of immunohistochemical analysis along with radiological investigations needed for diagnosis. Further research is needed to enable earlier diagnosis
and to develop a multimodal treatment approach.

Authors/Disclosures
Praveen Nandha Kumar Pitchan Velammal, MBBS PRESENTER	Dr. Pitchan Velammal has nothing to disclose.
Abhilash Thatikala, MD, MBBS, DM (UAMS)	Dr. Thatikala has nothing to disclose.
Khushboo Verma, MBBS (University of Arkansas for Medical Sciences)	Dr. Verma has nothing to disclose.
Thirumalaivasan Dhasakeerthi, MBBS (UAMS)	Dr. Dhasakeerthi has nothing to disclose.
Mansi Agrawal, MBBS	Ms. Agrawal has nothing to disclose.

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