Ependymoma Tumors in Extracranial Regions

Ependymomas are neoplasms derived from ependymal cells lining the ventricles of the brain and central canal of the spinal cord. Although predominantly intracranial or intraspinal, rare occurrences in extracranial sites—including the mediastinum, ovary, broad ligament, peritoneum and subcutaneous tissues—have been documented. These ectopic presentations often mimic more common soft-tissue or germ-cell tumours, complicating diagnosis. Histopathological hallmarks include perivascular pseudorosettes and immunopositivity for glial fibrillary acidic protein. Imaging modalities such as computed tomography and magnetic resonance, combined with immunohistochemistry, are critical for accurate localisation and characterisation. Surgical excision remains the cornerstone of management, with variable use of radiotherapy and chemotherapy guided by site, resection margins and molecular features. Advances in molecular profiling have begun to elucidate genetic drivers, revealing overlaps with central nervous system counterparts and highlighting potential targets for systemic therapy. Understanding these rare entities informs both surgical strategy and the development of adjuvant protocols, with implications for patient prognosis and fertility considerations in younger cohorts.

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Ependymoma Tumors in Extracranial Regions publication trend

The graph below shows the total number of articles in ependymoma tumors in extracranial regions across all publications each year (not limited to Nature Index journals).

Technical terms

Ependymoma: A tumour originating from ependymal cells of the central nervous system.

Ependymal cells: Glial cells that line ventricular cavities and the spinal canal.

Perivascular pseudorosette: A histological pattern in which tumour cells form a radial arrangement around blood vessels.

Glial fibrillary acidic protein (GFAP): An intermediate filament protein used as an immunohistochemical marker for glial differentiation.

Immature teratoma: A germ-cell tumour containing embryonic-type tissues and potential for malignant transformation.

CHEK2 p.H371Y mutation: A germline alteration in the CHEK2 gene affecting cell-cycle checkpoint control, implicated in tumour development.

References

1. Primary Ependymoma in the Posterior Mediastinum. Annals of Thoracic and Cardiovascular Surgery (2011).
2. Persistent Ependymal Tumor Arising from an Immature Ovarian Teratoma: A Rare Case. Journal of Molecular Pathology (2020).
3. Primary uterine broad ligament ependymoma with CHEK2 p.H371Y germline mutation: A CARE‐compliant case report uterine broad ligament ependymoma. Journal of Obstetrics and Gynaecology Research (2021).