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Atypical central neurocytoma with sarcomatous differentiation
Lakshmi Vemavarapu1, Irene Czyszczon, Joseph C Parker
1From the Department of Pathology, University of Louisville, Louisville, Kentucky (Drs Vemavarapu, Czyszczon, J. C. Parker, and J. R. Parker); the Department of Hematology and Oncology, Indiana University Simon Cancer Center, Indianapolis (Dr Wagner); and the Department of Neurosurgery, Norton Neuroscience Institute, Louisville, Kentucky (Dr Vitaz).
This study details a rare intraventricular tumor in a 44-year-old woman, presenting features of atypical central neurocytoma with a sarcomatous component. This unique case highlights a novel histologic variant, expanding the understanding of central neurocytoma.
Area of Science:
- Neuro-oncology
- Surgical Pathology
- Central Nervous System Tumors
Background:
- Central neurocytoma (CNC) is a rare, typically intraventricular tumor arising from neuronal elements.
- Atypical variants of CNC can exhibit increased mitotic activity and higher proliferation indices.
- Sarcomatous transformation in CNS tumors is uncommon, posing diagnostic and therapeutic challenges.
Observation:
- A 44-year-old woman presented with symptoms suggestive of increased intracranial pressure.
- Magnetic resonance imaging identified a lobulated, enhancing intraventricular mass.
- Surgical resection revealed a biphasic tumor composed of neurocytic and sarcomatous elements.
Findings:
- Histopathological analysis demonstrated two distinct cell populations: round cells with perinuclear halos and spindle cells.
- Immunohistochemistry confirmed synaptophysin positivity in round cells and vimentin positivity in spindle cells.
- High mitotic activity (8/10 HPF) and a Ki-67 proliferation index of 15.0% were noted, consistent with atypical features and sarcomatous differentiation.
Implications:
- This case represents the first reported instance of central neurocytoma exhibiting spindle cell sarcomatous features.
- The findings expand the known spectrum of histological variations within central neurocytoma.
- Further research is warranted to understand the behavior and optimal management of this rare tumor subtype.
