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Third ventricular chordoid glioma: a distinct clinicopathologic entity
D J Brat1, B W Scheithauer, S M Staugaitis
1Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA.
Journal of Neuropathology and Experimental Neurology
|May 26, 1998
Summary
A new type of third ventricular neoplasm with a chordoid appearance has been identified in 8 patients. These rare brain tumors show distinct cellular and stromal features, with some cases requiring further investigation for optimal treatment.
Area of Science:
- Neuropathology
- Neurosurgery
- Oncology
Background:
- Third ventricular neoplasms are rare and can present diagnostic challenges.
- Understanding the clinicopathologic features of newly identified tumor entities is crucial for accurate diagnosis and patient management.
Observation:
- Eight cases of third ventricular neoplasms with a distinctive chordoid appearance were identified.
- Tumors presented as large, well-circumscribed masses, with some exhibiting cystic components.
- Histologically, tumors showed cords of epithelioid cells with eosinophilic cytoplasm and extracellular mucin, resembling chordomas.
- Immunohistochemistry revealed strong reactivity for GFAP and vimentin, with low MIB-1 labeling index.
- Tumor cells lacked desmosomes and cilia, with focal microvilli and intermediate junctions observed ultrastructurally.
Findings:
- The identified neoplasms represent a distinct clinicopathologic entity.
- Tumor cells exhibited glial differentiation markers (GFAP, vimentin).
- The stromal component showed extracellular mucin and inflammatory infiltrates, including lymphocytes and plasma cells.
- Adjacent brain tissue demonstrated reactive changes like gliosis and Rosenthal fibers.
Implications:
- This study defines a novel entity of third ventricular neoplasms, aiding in differential diagnosis.
- The findings highlight the importance of integrating imaging, histology, immunohistochemistry, and ultrastructural analysis for accurate characterization.
- Prognostic implications are suggested by outcomes related to the extent of surgical resection, emphasizing the need for complete tumor removal where possible.