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Published on: January 14, 2014
Suprasellar monomorphous pilomyxoid neoplasm: an ultastructural analysis
C E Fuller1, B Frankel, M Smith
1Department of Anatomic Pathology, SUNY Upstate Medical University, Syracuse, New York, USA. fullercny@hotmail.com
Abstract:
The authors report 3 patients, 2 children and 1 adult, each of whom presented with an unusual myxoid lesion reminiscent of pilocytic astrocytoma but with other features of myxopapillary ependymoma. The magnetic resonance imaging findings in all cases showed a diffusely contrast-enhancing suprasellar mass focally extending into the third ventricle. Involvement of adjacent structures was more extensive in both infants. By light microscopy, all were composed of a monotonous population of cells with delicate piloid-like processes, loosely arranged within a prominent myxoid background. Focally, the neoplastic cells converged upon small blood vessels in pseudorosette-like formations. These histomorphologic features are identical to those of the recently described astrocytoma with monomorphous pilomyxoid features. In addition, the individual tumor cells showed strong cytoplasmic immunoreactivity with antibodies to glial fibrillary acidic protein (GFAP) and vimentin, as well as nuclear and cytoplasmic staining with S-100. All stained positive for synaptophysin and negative for chromogranin. By electron microscopy, the tumor cells were bipolar with elongated processes and apical surfaces displaying microvilli, cytoplasmic blebs and rare cilia. Vesicles and coated pits were seen, as were occasional synaptoid complexes. The current study serves to expand our clincopathologic experience with this rare and enigmatic lesion, with particular attention given to the ultrastructural characteristics.
Insights
This study details a rare brain tumor in 3 patients, presenting as a myxoid lesion with features of both pilocytic astrocytoma and myxopapillary ependymoma. Further research is needed to understand this enigmatic glial neoplasm.
Area of Science:
- Neuropathology
- Pediatric Oncology
- Neuro-oncology
Background:
- This study investigates a rare glial neoplasm with overlapping features of pilocytic astrocytoma and myxopapillary ependymoma.
- The lesion presents as a diffusely contrast-enhancing suprasellar mass, particularly in pediatric patients.
Observation:
- Three patients (2 children, 1 adult) exhibited unusual myxoid lesions with distinct histomorphologic and ultrastructural characteristics.
- Magnetic resonance imaging revealed suprasellar masses with extension into the third ventricle, showing more extensive involvement in infants.
Findings:
- Microscopic examination showed monotonous cells with piloid-like processes in a myxoid background, forming pseudorosettes around blood vessels.
- Immunohistochemistry revealed glial fibrillary acidic protein (GFAP), vimentin, and S-100 positivity, with synaptophysin positivity and chromogranin negativity.
- Electron microscopy demonstrated bipolar cells with microvilli, cytoplasmic blebs, cilia, and synaptoid complexes, consistent with a glial origin.
Implications:
- This research expands the clinicopathologic understanding of this rare and enigmatic glial tumor.
- The findings highlight the importance of detailed histomorphologic and ultrastructural analysis for accurate diagnosis.
- Further investigation is warranted to elucidate the precise nature and behavior of this neoplasm.
