Skip to main content
Log in

Hydrocephalus in primary intradural spinal cord tumors: a systematic review of the literature in the pediatric population

  • Short Review
  • Published:
Neurosurgical Review Aims and scope Submit manuscript

Abstract

Hydrocephalus in children with primary intradural spinal cord tumors is exceedingly rare. Herewith, we performed a systematic literature review to address epidemiology, suggested pathophysiological mechanisms, prognostic factors, and treatment of such cases. We performed a systematic review with the best available evidence on cases of pediatric primary intradural tumors of the spinal cord presented with hydrocephalus. The patients were subjected to quantitative analysis on a basis of epidemiological features (age, sex, tumor type and location, clinical presentation, survival, dissemination). The possible pathophysiological theories are discussed in detail. Forty-four studies with a total of 121 patients were included in the study. Astrocytomas were the most frequent tumor (64.5%) type, while most tumors were located in cervical (31.4%) or cervicothoracic region (25.6%). About half of the cases concerned children under 6 years of age. The block of subarachnoid CSF (cerebrospinal fluid) pathways from disseminated tumor cells and the neoplastic inflammation caused by tumor elements advocated to be the major pathogenetic mechanisms. Surgical excision of the tumor and hydrocephalus treatment is usually performed. Primary intradural spinal cord tumors should be considered in children with communicative hydrocephalus of unknown etiology. Onset of hydrocephalus after tumor removal is related to higher mortality.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

  1. Schellinger A, Propp M, Villano L, Mc-Carthy J (2007) Descriptive epidemiology of primary spinal cord tumors. J Neuro-Oncol 87(2):173–179

    Article  Google Scholar 

  2. Huisman T (2009) Pediatric tumors of the spine. Cancer Imaging 9(A):S45–S48. https://doi.org/10.1102/1470-7330.2009.9012

    Article  PubMed  PubMed Central  Google Scholar 

  3. Rossi A, Gandolfo C, Morana G, Tortori-Donati P (2007) Tumors of the spine in children. Neuroimaging Clin N Am 17(1):17–35. https://doi.org/10.1016/j.nic.2006.11.004

    Article  PubMed  Google Scholar 

  4. Die KW (1931) Augenveranderungen bei den entzundlichen. Erkrankungen des Zentralnervensystem. In: Schieck F, Bruckner A (eds) Kurzes Handbuch der Ophtalmologie, Vol, vol 2, pp 779–780

    Google Scholar 

  5. Moher D, Liberati A, Tetzlaff J, Altman DG (2009) The PRISMA Group 2009, Preferred Reporting Items for Systematic Reviews and Meta-Analyses: the PRISMA statement. PLoS Med 6(7):e1000097

    Article  Google Scholar 

  6. Mirone G, Cinalli G, Spennato P, Ruggiero C, Aliberti F (2011) Hydrocephalus and spinal cord tumors: a review. Childs Nerv Syst 27(10):1741–1749. https://doi.org/10.1007/s00381-011-1543-5

    Article  PubMed  Google Scholar 

  7. Oi S, Raimondi AJ (1981) Hydrocephalus associated with intraspinal neoplasms in childhood. Am J Dis Child 135(12):1122–1124. https://doi.org/10.1001/archpedi.1981.02130360030011

    Article  CAS  PubMed  Google Scholar 

  8. Rifkinson-Mann S, Wisoff H, Epstein F (1990) The association of hydrocephalus with intramedullary spinal cord tumors: a series of 25 patients. Neurosurgery. 27(5):749–754

    Article  CAS  Google Scholar 

  9. Russell N (2000) Infantile spinal cord tumor presenting as hydrocephalus. Ann Saudi Med 20(4):244–245

    Article  CAS  Google Scholar 

  10. Caviness J, Tucker M, Pia S, Tam D (1998) Hydrocephalus as a possible early symptom in a child with a spinal cord tumor. Pediatr Neurol 18(2):169–171. https://doi.org/10.1016/S0887-8994(97)00158-6

    Article  CAS  PubMed  Google Scholar 

  11. Rangwala D, Oh G, Barks L, Nikas C (2017) Intramedullary thoracic spine astrocytoma presenting as hydrocephalus in an infant: a case report. Pediatr Neurosurg 52:327–330. https://doi.org/10.1159/000479323

    Article  PubMed  Google Scholar 

  12. Schijman E, Zúccaro G, Monges JA (1981) Spinal tumors and hydrocephalus. Childs Brain 8(6):401–405

    CAS  PubMed  Google Scholar 

  13. Lazzara W, Ragland L, Smith W, Knorr R, Weiner A, Kamath V et al (1998) Basimeningeal/spinal axis primitive euroectodermal tumor without intraaxial lesion: radiological features. J Neuroimaging 8(1):49–54. https://doi.org/10.1111/jon19988149

    Article  CAS  PubMed  Google Scholar 

  14. Maurice-Williams S, Lucey J (1975) Raised intracranial pressure due to spinal tumours: 3 rare cases with a probable common mechanism. Br J Surg 62(2):92–95. https://doi.org/10.1002/bjs.1800620203

    Article  CAS  PubMed  Google Scholar 

  15. Kordás M, Czirják S, Dóczi T (1997) The spinal tumour related hydrocephalus. Acta Neurochir 139:1049–1054. https://doi.org/10.1007/BF01411559

    Article  PubMed  Google Scholar 

  16. Kushel’ V, Belova D, Tekoev R (2017) Intramedullary spinal cord tumors and hydrocephalus: an analysis of the results of surgical treatment in 541 patients. Zh Vopr Neirokhir Im N N Burdenko 81(4):56–60. https://doi.org/10.17116/neiro201781456-60

    Article  PubMed  Google Scholar 

  17. Hassan F, Paluzzi A, Kayello R, Bradey N, Strachan D (2008) Hydrocephalus as presenting feature of spinal astrocytoma in a adolescent patient. Br J Neurosurg 22(3):433–435. https://doi.org/10.1080/02688690701818893

    Article  CAS  PubMed  Google Scholar 

  18. Sublett M, Davenport C, Eisenbrock H, Dalal S, Jaffar A, Kershenovich A (2017) Pediatric primary diffuse leptomeningeal primitive neuroectodermal tumor: a case report and literature review. Pediatr Neurosurg 52(2):114–121.10.1159/000452807

    Article  Google Scholar 

  19. Morais A, Cardeal D, Ribeiro e Ribeiro, Frassetto P, Andrade G, Matushita H. (2017) Hydrocephalus: a rare initial manifestation of sporadic intramedullary hemangioblastoma. Childs Nerv Syst 33(8):1399–1403. https://doi.org/10.1007/s00381-017-3415-0

    Article  PubMed  Google Scholar 

  20. Cinalli G, Sainte-Rose C, Lellouch-Tubiana A, Sebag G, Renier D, Pierre-Kahn A (1995) Hydrocephalus associated with intramedullary low-grade gliomas. J Neurosurg 83(3):480–485. https://doi.org/10.3171/jns.1995.83.3.0480

    Article  CAS  PubMed  Google Scholar 

  21. Galarza M, Peretta P, Gazzeri R, Cinalli G, Forni M, Morra I, Ragazzi P, Sandri S (2006) Spinal cord gliomas and hydrocephalus: utility of neuroendoscopy. Minim Invasive Neurosurg 49(6):347–352. https://doi.org/10.1055/s-2006-955066

    Article  CAS  PubMed  Google Scholar 

  22. Aryan S, Ghosal N, Aziz A, Hegde S, Dadlani R (2014) Infantile holocord cellular ependymoma with communicating hydrocephalus: unusual presentation of a rare case. Brain Tumor Pathol (31):47–50. https://doi.org/10.1007/s10014-013-0145-1

  23. Kogler A, Orsolic K, Kogler V (1998) Intramedullary lipoma of dorsocervicothoracic spinal cord with intracranial extension and hydrocephalus. Pediatr Neurosurg 28:257–260. https://doi.org/10.1159/000028661

    Article  CAS  PubMed  Google Scholar 

  24. Psarros G, Swift D, Mulne F, Burns K (2015) Neurocytoma-like neoplasm of the thoracic spine in a 15-month-old child presenting with diffuse leptomeningeal dissemination and communicating hydrocephalus. J Neurosurg Pediatr 103(2):184–190. https://doi.org/10.3171/ped.2005.103.2.0184

    Article  Google Scholar 

  25. Iannelli A, Lupi G, Castagna M, Valleriani A, Bacherini F (2005) Intramedullary capillary hemangioma associated with hydrocephalus in an infant. J Neurosurg Pediatr 103(3):272–276. https://doi.org/10.3171/ped.2005.103.3.0272

    Article  Google Scholar 

  26. Lim P, Ramdas J, Miller A, Schuerch C, Wu G (2017) A rare initial presentation of primary diffuse leptomeningeal PNET in a 10-year-old-male. S D Med 70(12):543–545

    PubMed  Google Scholar 

  27. Gurkanlar D, Korkmaz E, Gurler E, Gokhan G, Kazan S (2010) Multilevel primary intraspinal PNETs in an infant associated with hydrocephalus. Turk Neurosurg 20(1):82–85

    PubMed  Google Scholar 

  28. Mock A, Levi A, Drake M (1990) Spinal hemangioblastoma, syrinx and hydrocephalus in a two-year-old child. Neurosurgery. 27:799–802. https://doi.org/10.1097/00006123-199011000-00019

    Article  CAS  PubMed  Google Scholar 

  29. Bell E, Kanodia AK, Gunaratne B, Edgar A (2018) Leptomeningeal dissemination of spinal pilocytic astrocytoma: a rare entity. BMJ Case Rep. 2018:2018 Pii: bcr-2018-226955

    Google Scholar 

  30. Tzekov C, Naydenov E, Kalev O (2007) Ependymoma of the cauda equina starting with communicating hydrocephalus: a case report. Pediatr Neurosurg 43:399–402. https://doi.org/10.1159/000106390

    Article  PubMed  Google Scholar 

  31. Miller G, Towfighi J, Page B (1990) Spinal cord ganglioglioma presenting as hydrocephalus. J Neuro-Oncol 9(2):147–152. https://doi.org/10.1007/bf02427835

    Article  CAS  Google Scholar 

  32. Cohen R, Wisoff H, Allen C, Epstein F (1989) Malignant astrocytomas of the spinal cord. J Neurosurg 70(1):50–54. https://doi.org/10.3171/jns.1989.70.1.0050

    Article  CAS  PubMed  Google Scholar 

  33. Prasad V, Basha A, Prasad M, Reddy R (1994) Intraspinal tumour presenting as hydrocephalus in childhood. Childs Nerv Syst 10(3):156–157. https://doi.org/10.1007/BF00301081

    Article  CAS  PubMed  Google Scholar 

  34. Sandalcioglu E, Gasser T, Wiedemayer H, Horsch S, Stolke D (2002) Favourable outcome after biopsy and decompression of a holocord intramedullary spinal cord astrocytoma in a newborn. Eur J Paediatr Neurol 6(3):179–182. https://doi.org/10.1053/ejpn.2002.0587

    Article  PubMed  Google Scholar 

  35. Ng K, Leung S, Boet R, Poon S (2001) Spinal cord pilocytic astrocytoma with cranial meningeal metastases. J Clin Neurosci 8(4):374–377. https://doi.org/10.1054/jocn.2000.0762

    Article  CAS  PubMed  Google Scholar 

  36. Fridley S, Chamoun B, Whitehead E, Curry J, Luerssen G, Adesina A et al (2009) Malignant rhabdoid tumor of the spine in an infant: case report and review of the literature. Pediatr Neurosurg 45:237–243. https://doi.org/10.1159/000224622

    Article  PubMed  Google Scholar 

  37. Johnson D, Schwarz S (1987) Intracranial metastases from malignant spinal-cord astrocytoma. J Neurosurg 66(4):621–625. https://doi.org/10.3171/jns.1987.66.4.0621

    Article  CAS  PubMed  Google Scholar 

  38. Wu Y, Xu B, Lu L, Li H, Tian S, Li J et al (2018) Imaging features of spinal atypical teratoid rhabdoid tumors in children. Medicine. 97(52):e13808. https://doi.org/10.1097/MD.0000000000013808

    Article  PubMed  PubMed Central  Google Scholar 

  39. Severino M, Consales A, Doglio M, Tortora D, Morana G, Barra S, Nozza P, Garrè ML (2015) Intradural extramedullary ependymoma with leptomeningeal dissemination:the first case report in a child and literature review. World Neurosurg 84(3):865–865.e19. https://doi.org/10.1016/j.wneu.2015.04.002

    Article  PubMed  Google Scholar 

  40. Alexiou G, Siozos G, Stefanaki K, Moschovi M, Prodromou N (2013) Intramedullary spinal cord primitive neuroectodermal tumor presenting with hydrocephalus. J Child Neurol 28(2):246–250. https://doi.org/10.1177/0883073812441061

    Article  PubMed  Google Scholar 

  41. Serra S, Dabdoub C, H. da Cunha A, Salazar B, Lima T, Azevedo-Filho H (2013) Disseminated glioneuronal tumor with neuropil-like islands of the spinal cord: a distinctive entity. World Neurosurg 80(5):655–655.e5. https://doi.org/10.1016/j.wneu.2013.02.029

    Article  PubMed  Google Scholar 

  42. Baharvahdat H, Ganjeifar B, Roshan M, Baradaran A (2018) Spinal intradural primary malignant peripheral nerve sheath tumor with leptomeningeal seeding: case report and literature review. Turkish Neurosurg 28(2):317–322. https://doi.org/10.5137/1019-5149.JTN.16782-15.1

    Article  Google Scholar 

  43. Poussaint T, Yousuf N, Barnes P, Anthony D, Zurakowski D, Scott R et al (1999) Cervicomedullary astrocytomas of childhood: clinical and imaging follow-up. Pediatric radiology 29(9):662–668. https://doi.org/10.1007/s002470050671

    Article  Google Scholar 

  44. Vassilyadi M, Michaud J (2005) Hydrocephalus as the initial presentation of a spinal cord astrocytoma associated with leptomeningealspread. Pediatr Neurosurg 41(1):29–34. https://doi.org/10.1159/000084862

    Article  CAS  PubMed  Google Scholar 

  45. Ucar S, Florez G, Garcia J (1976) Increased intracranial pressure associated with spinal cord tumours. Neurochirurgia. 19(6):265–268

    CAS  PubMed  Google Scholar 

  46. Abel J, Chowdhary A, Thapa M, Rutledge C (2006) Spinal cord pilocytic astrocytoma with leptomeningeal dissemination to the brain. J Neurosurg 105(6 Suppl):508–514. https://doi.org/10.3171/ped.2006.105.6.508

    Article  PubMed  Google Scholar 

  47. Chamoun B, Whitehead E, Dause RC, Luerssen G, Okcu F, Adesina M et al (2009) Primary disseminated intradural malignant peripheral nerve sheath tumor of the spine in a child: case report and review of the literature. Pediatr Neurosurg 45(3):230–236. https://doi.org/10.1159/000224621

    Article  PubMed  Google Scholar 

  48. Ebner H, Schittenhelm J, Roser F, Scheel-Walter H, Tatagiba M, Schuhmann U (2013) Management of holocord pilocytic astrocytomas in children and adolescents: an update. Pediatr Neurosurg 48(3):133–140. https://doi.org/10.1159/000345593

    Article  Google Scholar 

  49. Inagawa T, Kamiya K, Nagasako R (1995) A case of spinal low-grade astrocytoma with exophytic and intracranial extension. Surg Neurol 43(3):261–264. https://doi.org/10.1016/0090-3019(95)80010-e

    Article  CAS  PubMed  Google Scholar 

  50. Cushing H (1914) Studies on the cerebrospinal fluid. I Introduction. J Med Res 31:1–19

    CAS  PubMed  PubMed Central  Google Scholar 

  51. Dandy W (1918) Experimental hydrocephalus. Ann Surg 70:129–142

    Article  Google Scholar 

  52. Dandy W (1918) Extirpation of the choroid plexus of the lateral ventricles in communicating hydrocephalus. Ann Surg 68:569–579

    Article  CAS  Google Scholar 

  53. Weed L (1917) An anatomical consideration of the cerebrospinal fluid. Anat Rec 12:461–496

    Article  Google Scholar 

  54. Weed L (1935) Forces concerned in the absorption of cerebrospinal fluid. Am J Phys 114:40–45

    Article  Google Scholar 

  55. Maroulis H, Maixner W, Leone E, Cinalli G (2005) Hydrocephalus and spinal tumors. In: Cinalli G, Sainte-Rose C, Maixner W (eds) J. Pediatric Hydrocephalus, pp 187–199

    Chapter  Google Scholar 

  56. Kahan H, Sklar E, Post J, Bruce J (1996) MR characteristics of Histopathologic subtypes of spinal ependymoma. AJNR Am J Neuroradiol 17:143–150

    CAS  PubMed  Google Scholar 

  57. Goy M, Pinto S, Raghavendra N, Epstein J, Kricheff I (1986) Intramedullary spinal cord tumors: MR imaging, with emphasis on associated cysts. Radiology. 161(2):381–386

    Article  CAS  Google Scholar 

  58. Bloomfield G, Johnston H, Bilston E (1998) Effects of proteins, blood cells and glucose on the viscosity of cerebrospinal fluid. Pediatr Neurosurg 28(5):246–251. https://doi.org/10.1159/000028659

    Article  CAS  PubMed  Google Scholar 

  59. Bamford R, Labadie L (1976) Reversal of dementia in normotensive hydrocephalus after removal of a cauda equina tumor. Case report. J Neurosurg 45:104–107

    Article  CAS  Google Scholar 

  60. Madsen R, Egnor M, Zou R (2006) Cerebrospinal fluid pulsatility and hydrocephalus: the fourth circulation. Clin Neurosurg 53:48–52

    PubMed  Google Scholar 

  61. Klarica M, Jukić T, Miše B, Kudelić N, Radoš M, Orešković D (2015) Experimental spinal stenosis in cats: new insight in mechanisms of hydrocephalus development. Brain Pathol 26(6):701–712. https://doi.org/10.1111/bpa.12337

    Article  PubMed  Google Scholar 

  62. Dorsey J, Hollander A, Alonso-Basanta M, Macyszyn L, Bohman E, Judy (2016) Cancer of the central nervous system. In: Niederhuber JE, Armitage JO, Doroshow JH, Kastan MB, Tepper JE (eds) Abeloff's Clinical Oncology, 5th edn, pp 938–1001

    Google Scholar 

  63. Orešković D, Klarica M (2011) Development of hydrocephalus and classical hypothesis of cerebrospinal fluid hydrodynamics: facts and illusions. Prog Neurobiol 94(3):238–258. https://doi.org/10.1016/j.pneurobio.2011.05.005

    Article  PubMed  Google Scholar 

Download references

Authors’ contributions

All authors (Marios Lampros., George Alexiou., Spyridon Voulgaris) conceived the study, co-wrote the final manuscript, and analyzed the data. Spyridon Voulgaris and George Alexiou supervised the study and gave the final approval.

Availability of data

Not applicable in this study type (Review).

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to George A. Alexiou.

Ethics declarations

Conflict of interest

The authors declare that they have no conflict of interest.

Consent to participate and consent for publication

Informed consent was not required due to the type of the study (review). Patients or animals were not subjected to clinical trials during this review.

Ethics approval

Approval from an ethics committee was not required. We performed a systematic review of the literature. Humans or animals were not subjected to clinical trials during this review. The data of all patients included in this study were obtained from the literature search and not from our institution. Personal information involving name, surname, and patients’ photos were not included in our review.

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Lampros, M., Voulgaris, S. & Alexiou, G.A. Hydrocephalus in primary intradural spinal cord tumors: a systematic review of the literature in the pediatric population. Neurosurg Rev 44, 2079–2084 (2021). https://doi.org/10.1007/s10143-020-01386-0

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10143-020-01386-0

Keywords

Navigation