Skip to main content
SpringerLink
Log in

Non-chordomatous clival bony tumors: A rare experience and systematic literature review

  • Research
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Background

Non-chordomatous bony tumors of the clivus are extremely rare. Site, extent, and aggressiveness of tumor limits the extent of resection. It poses challenge to the neurosurgeons due to the complexity of anatomy. There is paucity of literature exclusively on non-chordomatous bone tumors of the clivus in young adults.

Objectives

To analyze the clinical presentation, imaging findings, surgical approach, complications, and outcome of primary clival bony tumors in young adults.

Methods

We retrospectively reviewed children and young adults with primary clival bony tumors excluding chordoma who underwent surgical resection between years 2010 and 2023 in our center. We analyzed the demographic details, imaging findings, operative variables, perioperative complications, length of stay, complications, and outcome at latest follow-up.

Results

The mean age was 17.5 ± 1.73 years (range 16 to 19 years). Headache was the presenting complaint in all four patients (100%). The mean duration of symptom was 7.25 ± 3.2 months (range 5 to 12 months). The tumor was localized in clivus in all four patients (100%). The mean length of stay in hospital was 30.5 ± 13.48 days (range 11 to 40 days). All patients underwent surgical treatment. Surgical approaches used were anterior approach in four patients (100%). Gross total excision was performed in one patient (25%), sub-total excision was performed in two patients (50%), and tumor decompression was performed in one patient (25%). Of these, three were designated as having benign tumors and one had a malignant tumor. There was no perioperative mortality. There was one mortality (25%) on 2 months follow-up due to tumor progression. Three patients (75%) had improved symptomatically at latest follow-up. Two patients (50%) received adjuvant chemoradiotherapy. The mean follow-up was 38 ± 39.29 months (range 2 to 72 months).

Conclusion

Non-chordomatous bony tumors of the clivus are rare and often underestimated. Surgery is the treatment of choice. Tumor consistency and adhesion to critical neurovascular structures precludes gross total resection. Various approaches are in the armamentarium. Approach to be decided based on the expertise of the neurosurgeon to achieve safe maximal resection. Multidisciplinary approach is mandatory for streamlined management. Adjuvant therapy is decided based on the residual tumor following surgery.

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

Access this article

Log in via an institution

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

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8
Fig. 9
Fig. 10

Similar content being viewed by others

Availability of data and materials

Datasets and imaging of patients are available.

References

  1. Gagliardi F, Spina A, Boari N, Narayanan A, Mortini P (2015) Solitary lesions of the clivus: what else besides chordomas? An extensive clinical outlook on rare pathologies. Acta Neurochir 157:597–605

    Article  PubMed  Google Scholar 

  2. Gagliardi F, Boari N, Mortini P (2013) Solitary nonchordomatous lesions of the clival bone: differential diagnosis and current therapeutic strategies. Neurosurg Rev 36:513–522

    Article  PubMed  Google Scholar 

  3. Vanhoenacker FM, De Praeter G, Kools D, Voormolen M, Parizel PM (2011) Unusual lesion of the clivus: diagnosis and discussion. Skeletal Radiol 40:243–244

    Article  PubMed  Google Scholar 

  4. Samaan NA, Vieto R, Schultz PN, Maor M, Meoz RT, Sampiere VA et al (1982) Hypothalamic, pituitary and thyroid dysfunction after radiotherapy to the head and neck. Int J Radiat Oncol Biol Phys 8(11):1857–1867

    Article  CAS  PubMed  Google Scholar 

  5. Miké V, Meadows A, D’Angio G, Group LES (1982) Incidence of second malignant neoplasms in children: results of an international study. Lancet 320(8311):1326–1331

    Article  Google Scholar 

  6. Meadows AT, Baum E, Fossati-Bellani F, Green D, Jenkin R, Marsden B et al (1985) Second malignant neoplasms in children: an update from the Late Effects Study Group. J Clin Oncol 3(4):532–538

    Article  CAS  PubMed  Google Scholar 

  7. Morita A, Sekhar LN (2000) Skull base tumors. In: Bernstein M, Berger MS (eds) Neuro-oncology: the essentials. Thieme Medical Publishers, New York, pp 419–433

    Google Scholar 

  8. Kunimatsu A, Kunimatsu N (2017) Skull base tumors and tumor-like lesions: a pictorial review. Pol J Radiol 82:398

    Article  PubMed  PubMed Central  Google Scholar 

  9. Casselman JW (2005) The skull base: tumoral lesions. Eur Radiol 15:534–542

    Article  PubMed  Google Scholar 

  10. Kashiwagi N, Hirabuki N, Andou K, Yoshifumi N, Tanaka H, Morino H et al (2006) MRI and CT findings of the giant cell tumors of the skull; five cases and a review of the literature. Eur J Radiol 58(3):435–443

    Article  PubMed  Google Scholar 

  11. Brackmann DE, Teufert KB (2006) Chondrosarcoma of the skull base: long-term follow-up. Otol Neurotol 27(7):981–991

    Article  PubMed  Google Scholar 

  12. Moscote-Salazar LR, Dolachee AA, Narvaez-Rojas A, Al–Saadi HA, Najim AA, Abdulreda AK, et al (2019) Preoperative embolization of skull–base tumors: indications, utility, and concerns. J Acute Dis 8(3):89–94

    Article  Google Scholar 

  13. Yaprak Bayrak B, Özcan E, Vural Ç, Emengen A, Çabuk B, Ceylan S (2021) A single-center experience with giant cell tumors of sphenoid bone and clivus. Tumori J 107(6):NP94–NP100

    Article  CAS  Google Scholar 

  14. Gil Z, Constantini S, Spektor S, Abergel A, Khafif A, Beni-Adani L et al (2005) Skull base approaches in the pediatric population. Head & Neck: Journal for the Sciences and Specialties of the Head and Neck 27(8):682–689

    Article  Google Scholar 

  15. Behbahani M, Rastatter JC, Eide J, Karras C, Walz P, Suresh K et al (2023) Pediatric endoscopic endonasal skull base surgery: a retrospective review over 11 years. World Neurosurg 170:e70–e78

    Article  PubMed  Google Scholar 

  16. Inoue A, Ohnishi T, Kohno S, Nishikawa M, Nishida N, Ohue S (2016) Role of denosumab in endoscopic endonasal treatment for juvenile clival giant cell tumor: a case report and review of the literature. World Neurosurg 91:674.e1–e6

    Article  Google Scholar 

  17. Goto Y, Furuno Y, Kawabe T, Ohwada K, Tatsuzawa K, Sasajima H et al (2017) Treatment of a skull-base giant cell tumor with endoscopic endonasal resection and denosumab: case report. J Neurosurg 126(2):431–434

    Article  PubMed  Google Scholar 

  18. Huh A, Villelli N, Martinez D, Ting J, Birhiray R, Payner TD et al (2018) Denosumab treatment for a residual giant cell tumor of the clivus: a case report and review of the literature. World Neurosurgery 118:98–101

    Article  PubMed  Google Scholar 

  19. Nolte K (1979) Malignant intracranial chordoma and sarcoma of the clivus in infancy. Pediatr Radiol 8:1–6

    Article  CAS  PubMed  Google Scholar 

  20. Korten AG, ter Berg HJ, Spincemaille GH, van der Laan RT, Van de Wel AM (1998) Intracranial chondrosarcoma: review of the literature and report of 15 cases. J Neurol Neurosurg Psychiatry 65(1):88–92

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Noel G, Feuvret L, Calugaru V, Hadadi K, Baillet F, Mazeron J-J et al (2004) Chondrosarcomas of the base of the skull in Ollier’s disease or Maffucci’s syndrome Three Case Reports and Review of the Literature. Acta Oncol 43(8):705–710

    Article  PubMed  Google Scholar 

  22. Zorlu F, Selek U, Soylemezoglu F, Oge K (2006) Malignant giant cell tumor of the skull base originating from clivus and sphenoid bone. J Neurooncol 76(2):149

    Article  PubMed  Google Scholar 

  23. Gupta R, Mohindra S, Mahore A, Mathuriya S, Radotra B (2008) Giant cell tumour of the clivus. Br J Neurosurg 22(3):447–449

    Article  CAS  PubMed  Google Scholar 

  24. Balasubramaniam S, Nadkarni T, Menon R, Goel A, Rajashekaran P (2008) Primary Ewing’s sarcoma of the petroclival bone. J Clin Neurosci 15(6):712–714

    Article  PubMed  Google Scholar 

  25. Chennupati SK, Norris R, Dunham B, Kazahaya K (2008) Osteosarcoma of the skull base: case report and review of literature. Int J Pediatr Otorhinolaryngol 72(1):115–119

    Article  PubMed  Google Scholar 

  26. Salunke P, Sinha R, Khandelwal N, Kumar A, Gupta K, Mukherjee KK (2010) Primary intraosseus cavernous hemangioma of the skull base. Br J Neurosurg 24(1):84–85

    Article  PubMed  Google Scholar 

  27. Roy S, Joshi NP, Sigamani E, Malik A, Sharma MC, Mohanti BK et al (2013) Clival giant cell tumor presenting with isolated trigeminal nerve involvement. Eur Arch Otorhinolaryngol 270:1167–1171

    Article  PubMed  Google Scholar 

  28. Hadley C, Gressot LV, Patel AJ, Wang LL, Flores RJ, Whitehead WE et al (2014) Osteosarcoma of the cranial vault and skull base in pediatric patients: Report of 3 cases. J Neurosurg Pediatr 13(4):380–387

    Article  PubMed  Google Scholar 

  29. Campbell JI, Mural M, Rubino F, Lopez ES, Cervio A, Olvi L (2019) Clivus hemangioma in a pediatric patient: case report. World Neurosurg 130:512–515

    Article  PubMed  Google Scholar 

  30. Ariamanesh M, Tafrishi R, Dehghani M, Bakhshai M, Javadinia SA, Hosseini SM et al (2021) Ten years of battle with multiple recurrences of pediatric skull base chondrosarcoma: a case report. Clin Case Rep 9(10):e04904

    Article  PubMed  PubMed Central  Google Scholar 

  31. Saifuddin MSAH, Ng CY, Abdullah MS (2021) Skull base primary Ewing sarcoma: a radiological experience of a rare disease in an atypical location. The American journal of case reports 22:e930384–e930391

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Author notes

  1. Sivaraman Kumarasamy and Amandeep Kumar contributed equally and are first author.

Authors and Affiliations

  1. Department of Neurosurgery, Neurosciences Centre, All India Institute of Medical Sciences, New Delhi, 110029, India

    Sivaraman Kumarasamy, Amandeep Kumar, Pankaj Kumar Singh, Ashish Suri, Poodipedi Sarat Chandra & Shashank Sharad Kale

Authors
  1. Sivaraman Kumarasamy
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Amandeep Kumar
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Pankaj Kumar Singh
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Ashish Suri
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Poodipedi Sarat Chandra
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Shashank Sharad Kale
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

SK: conception and design of work, acquisition, analysis, interpretation of data and drafted the work. AJ: interpretation of data and revised the work. PKS: design of work, analysis, interpretation of data and drafted the work. AS: interpretation of data and revised the work. PSC: interpretation of data and revised the work. SSK: interpretation of data and revised the work. All authors have approved the submitted version (and any substantially modified version that involves the author's contribution to the study) and agreed both to be personally accountable for the contributions and to ensure that questions related to the accuracy or integrity of any part of the work.

Corresponding author

Correspondence to Pankaj Kumar Singh.

Ethics declarations

Ethics approval and consent to participate

Consent to participate and consent for publication was taken from the patient using written informed consent to publish from the parent. Ethics committee approval was obtained.

Conflict of interest

The authors declare no competing interests.

Disclaimer

The manuscript was never presented as part at a meeting, the organization, place, and exact date on which it was read.

Additional information

Publisher's Note

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

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Kumarasamy, S., Kumar, A., Singh, P.K. et al. Non-chordomatous clival bony tumors: A rare experience and systematic literature review. Childs Nerv Syst 40, 1065–1077 (2024). https://doi.org/10.1007/s00381-023-06237-z

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-023-06237-z

Keywords

Search

Navigation