Case Report

Ependymomas with extraneural metastasis to lung in children: A case report and literature review

Liang Wang*, Miao Lou, Shunnan Ge, Guodong Gao, Yan Qu, Jiarui Zhang, Min Chao and Yang Jiao

Published: 16 June, 2020 | Volume 4 - Issue 1 | Pages: 041-045

Ependymomas, which account for 10% of pediatric central nervous system (CNS) tumors, arise from the ependymal cells that line the cerebral ventricles and the central canal of the spinal cord. Extraneural metastasis to lung is rare for ependymomas primary tumors. Repeated surgeries that disrupt the blood-brain barrier may contribute to haematogenous spread, but the mechanism remains unclear. We present a case of ependymoma with extraneural metastasis to lung in a child and discuss reported cases of extracranial metastatic ependymoma with this presentation.

Read Full Article HTML DOI: 10.29328/journal.acr.1001039 Cite this Article Read Full Article PDF


Pediatric ependymoma; Extraneural metastasis; Surgery; Radiotherapy; Poor outcome


  1. Gerstner ER, Pajtler KW. Ependymoma. Semin Neurol. 2018; 38: 104-111. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/29548057
  2. Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, et al. The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol. 2016; 131: 803‐820. PubMed: https://pubmed.ncbi.nlm.nih.gov/27157931/
  3. Khalid SI, Kelly R, Adogwa O, Carlton A, Woodward J, et al. Pediatric Spinal Ependymomas: An Epidemiologic Study. World Neurosurg. 2018; 115: e119‐e128. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/29631082
  4. Lee JC, Sharifai N, Dahiya S, Kleinschmidt-DeMasters BK, Rosenblum MK, et al. Clinicopathologic features of anaplastic myxopapillary ependymomas. Brain Pathol. 2019; 29: 75‐84. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/30417460
  5. Newton HB, Henson J, Walker RW. Extraneural metastases in ependymoma. J Neurooncol. 1992; 14: 135-142. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/1432036
  6. Subramanian A, Harris A, Piggott K, Shieff C, Bradford R. Metastasis to and from the central nervous system--the 'relatively protected site'. Lancet Oncol. 2002; 3: 498-507. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/12147436
  7. Houston SC, Crocker IR, Brat DJ, Olson JJ. Extraneural metastatic glioblastoma after interstitial brachytherapy. Int J Radiat Oncol Biol Phys. 2000; 48: 831-836. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/11020581
  8. Rickert CH. Extraneural metastases of paediatric brain tumours. Acta Neuropathol. 2003; 105: 309-327. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/12624784
  9. Varan A, Sari N, Akalan N, Söylemezoğlu F, Akyüz C, et al. Extraneural metastasis in intracranial tumors in children: the experience of a single center. J Neurooncol. 2006; 79: 187-190.
  10. Wen HL. Ependymoma with extracranial metastases. J NeuropathExp Neurol. 1957; 16: 112.
  11. Breslich PJ. Ependymoma of the right occipital lobe with death from extracranial metastases. J Lancet. 1957; 77: 99-103. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/13406418
  12. Perrin EV. Extracranial metastases from intracranial gliomata; report of two cases in children. Am J Clin Pathol. 1958; 30: 244-251. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/13571191
  13. Glasauer FE, Yuan RHP. Intracranial Tumors with Extracranial Metastases. J Neurosurg. 1963; 20: 474-493. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/14193871
  14. Macmahon HE, Urista MS. The spread of an ependymoma from the brain to the lungs. Am J Surg. 1964; 107: 765-768. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/14166217
  15. Wentworth P, Birdsell DC. Intracranial ependymoma with extracranial metastases. J Neurosurg. 1966; 25: 648-651. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/5925727
  16. Hojgaard K, Johansen A. A case of ependymoma with remote metastasis. Acta Pathol Microbiol Scand Suppl 1970; 212: Suppl 212: 198. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/5267090
  17. Mavroudis C, Townsend JJ, Wilson CB. A metastasizing ependymoma of the cauda equina. Case report. J Neurosurg. 1977; 47: 771-775. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/908942
  18. Fischer C, Haque SS, Huse JT, Blochin E, Souweidane MM, et al. Extraneural ependymoma: distant bone, lung, liver, and lymph node metastases following bevacizumab. Pediatr Blood Cancer. 2013; 60: 143-145. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/22976578
  19. Alzahrani A, Alassiri A, Kashgari A, Alrehaili J, Alshaalan H, et al. Extraneural metastasis of an ependymoma: a rare occurrence. Neuroradiol J. 2014; 27: 175-178. PubMed: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4202865/
  20. Morris DM, Steinert HR, Wiernik PH. Ineffectiveness of chemotherapy in patients with metastatic ependymoma of the cauda equina. J Surg Oncol. 1983; 22: 33-36. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/6823114
  21. Weiss L. A metastasizing ependymoma of the cauda equina. Cancer. 1955; 8: 161-171. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/13231048
  22. Hoffman HJ, Duffner PK. Extraneural metastases of central nervous system tumors. Cancer. 1985; 56: 1778-1782. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/4027909
  23. Adair JC, Baldwin N, Kornfeld M, Rosenberg GA. Radiation-induced blood-brain barrier damage in astrocytoma: relation to elevated gelatinase B and urokinase. J Neurooncol. 1999; 44: 283-289. PubMed: https://www.ncbi.nlm.nih.gov/pubmed/10720208
  24. Fujimori T, Iwasaki M, Nagamoto Y, Kashii M, Sakaura H, et al. Extraneural metastasis of ependymoma in the cauda equina. Global Spine J. 2013; 3: 33-40. PubMed: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3854601/


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