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[Intracranial ependymoma with extraneural metastases]
D Schreiber1, J Schneider, T Heller
1Institut für Pathologische Anatomie, Medizinischen Akademie Erfurt, DDR.
Abstract:
A report is given on a 14-year-old boy with an ependymoma of the brain which was 3 times operated upon. At autopsy, a widespread leptomeningeal and intraventricular dissemination was found. Extraneural metastases developed in the soft tissues of the neck as well as in the pleura and lung on the left side. Immunohistochemically, the cells of the brain tumor and extraneural metastases of the neck showed a positive GFAP-reaction. According to Tables 17 to 19 of Jänisch and co-workers (1976; 1988) and Table 1 of this paper, 48 intracranial, intraspinal and ectopic (subcutaneous sacrococcygeal) ependymomas with extraneural metastases were collected from the literature.
Insights
This case report details a 14-year-old boy with brain ependymoma that metastasized to extraneural sites. The study highlights rare instances of ependymoma with extraneural spread, confirmed by GFAP-positive cells.
Area of Science:
- Neuro-oncology
- Pathology
- Medical case reporting
Background:
- Ependymomas are tumors arising from ependymal cells, typically within the central nervous system.
- While intracranial and intraspinal ependymomas are well-documented, extraneural metastases are exceptionally rare.
- Understanding the metastatic potential of ependymomas is crucial for diagnosis and treatment.
Observation:
- A 14-year-old male presented with a brain ependymoma, undergoing multiple surgical interventions.
- Autopsy revealed widespread leptomeningeal and intraventricular dissemination of the tumor.
- Extraneural metastases were identified in the neck soft tissues, left pleura, and left lung.
Findings:
- Immunohistochemical analysis confirmed glial fibrillary acidic protein (GFAP)-positive cells in both the primary brain tumor and the extraneural metastases.
- A literature review identified 48 cases of ependymomas (intracranial, intraspinal, and ectopic) with extraneural metastases.
- This case adds to the scarce evidence of ependymoma's capacity for distant spread.
Implications:
- The findings underscore the importance of considering extraneural metastasis in ependymoma cases, even in young patients.
- GFAP positivity in extraneural sites supports a common origin from the primary central nervous system tumor.
- Further research into the mechanisms and clinical significance of ependymoma extraneural metastasis is warranted.