Extensive craniospinal disseminated metastasis after the resection of intradural extramedullary ependymoma in the craniocervical junction: a case report and literature review

Abstract

Purpose/Aim

Intradural extramedullary (IDEM) ependymomas are very rare, and IDEM ependymomas with craniospinal disseminated metastasis are exceptionally rare; only 2 preoperative cases have been confirmed, and postoperative cases have not been reported.

Case report

We present a case of a 21-year-old female with an IDEM ependymoma of the craniocervical junction who experienced head and neck pain for more than 1 month. Magnetic resonance imaging (MRI) of the cervical spine revealed a large IDEM cystic lesion located in the medulla oblongata and the upper cervical spinal cord. The patient underwent surgery without complications, and the tumor was completely removed. Histopathological examination revealed a diagnosis of aplastic ependymoma, World Health Organization (WHO) grade III. The patient failed to follow-up with radiotherapy for one month after discharge. Nearly three months after surgery, craniospinal disseminated metastasis was found in the patient; subsequently, chemoradiotherapy was administered to prolong the survival time of the patient. Unfortunately, the patient underwent radiotherapy and chemotherapy for only 7 days; then, the patient gave up treatment and died 5 months later.

Conclusions

To the best of our knowledge, no other cases of craniocervical junction anaplastic ependymomas with craniospinal disseminated metastasis have been reported in the literature. Total resection does not completely prevent recurrence and metastasis, and MRI of the entire neuraxis and timely postoperative craniospinal radiotherapy are necessary for the treatment of this disease.

Keywords:

• Anaplastic ependymoma
• intradural extramedullary tumor
• radiation therapy
• craniocervical junction

Acknowledgments

The authors thank Gege Zhou (Department of Pathology, The Second Affiliated Hospital of Anhui Medical University) for technical assistance with the pathology.

Disclosure statement

No potential conflict of interest was reported by the author(s).

Ethics approval and consent to participate

The study was approved by the Ethics Committee of the Second Affiliated Hospital of Anhui Medical University.

Consent to publication

Informed written consent was obtained from the patient’s kin for publication of this case report and accompanying images.