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Ependymoma with extensive lipidization mimicking adipose tissue: a report of five cases
M C Sharma1, R Arora, R Lakhtakia
1All India Institute of Medical Sciences, Department of Pathology, New Delhi, 110029, India.
Abstract:
Lipomatous ependymoma is a recently described entity and only 3 cases of this variant have been reported in the literature. We report 5 cases of this rare variant of ependymoma. Patients age ranged from 4 years to 45 years and, interestingly, all of them were males. Two tumors were supratentorial in location, 2 in the fourth ventricle and 1 was intramedullary. Microscopically all of them showed the classical histology of ependymoma along with lipomatous differentiation. The lipomatous component was composed of cells with a large clear vacuole pushing the nucleus to the periphery and giving a signet ring cell appearance. This component demonstrated positivity for GFAP and S-100 protein thereby confirming its glial lineage. Three of the 5 tumors were high grade (WHO-grade III), had a high MIB-1 labelling index (MIB-1 LI) and showed recurrence on follow-up. However, 2 were low grade (WHO grade II) and patients are free of disease till the last follow up.
Insights
This study describes lipomatous ependymoma, a rare brain tumor variant. Five male cases showed distinct lipomatous features, with outcomes varying by tumor grade.
Area of Science:
- Neuro-oncology
- Pathology
- Rare tumor variants
Background:
- Lipomatous ependymoma is a rare glial tumor variant.
- Only three cases were previously reported in medical literature.
Observation:
- This study presents five new cases of lipomatous ependymoma.
- Patients were males aged 4 to 45 years.
- Tumor locations included supratentorial, fourth ventricle, and intramedullary sites.
Findings:
- Histopathology revealed classical ependymoma features with lipomatous differentiation.
- The lipomatous component showed glial lineage markers (GFAP, S-100).
- High-grade (WHO III) tumors exhibited high MIB-1 labeling index and recurrence, while low-grade (WHO II) tumors showed no recurrence.
Implications:
- This expands the understanding of lipomatous ependymoma characteristics.
- Tumor grade and MIB-1 labeling index are crucial prognostic indicators.
- Further research is needed to elucidate the behavior of this rare entity.