Primary Central Nervous System Histiocytic Sarcoma: A Systematic Review of Clinical Presentation, Radiologic Findings, Treatment Strategies, and Prognostic Outcomes
Praveen Nandha Kumar Pitchan Velammal1, Abhilash Thatikala2, Khushboo Verma2, Thirumalaivasan Dhasakeerthi2, Mansi Agrawal3
1Tirunelveli Medical College, India, 2UAMS, 3MGM Medical College, India
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.
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