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Child's Nervous System

, Volume 35, Issue 3, pp 559–563 | Cite as

Surgical considerations in a paediatric case of a large skull-base epithelioid haemangioendothelioma

  • Suyi OoiEmail author
  • Matthew Gutman
  • Chris Xenos
  • Ronil Chandra
  • Catriona McLean
Case Report

Abstract

Intracranial epithelioid haemangioendothelioma (EHE) is a rare intermediate grade vascular tumour with heterogeneous clinical and histopathological behaviour. We present the surgical considerations of an exceptionally large skull-based EHE in an 11-year old female who presented to our institution with headaches and a protuberance over the left parietal area. Magnetic resonance imaging (MRI) demonstrated a left sided 10.5 × 6.6 × 11.1 cm extra-axial tumour arising from the parieto-temporaloccipital region which was continuous with the calvarium. An initial biopsy confirmed EHE. Staged treatment involved preoperative angiography and embolization. The patient underwent an extensive tumour excision and acrylic cranioplasty. Residual tumour persists in the petrous temporal bone. No neurological deficit was sustained. Postoperatively, we proceeded to tumour surveillance rather than adjuvant therapies, and follow-up imaging up to 36 months postoperatively has shown no tumour progression. We illustrate our surgical management of this large EHE and review the literature of this rare pathological entity with variable tumour behaviour and potential role for adjuvant therapy.

Keywords

Intracranial epithelioid haemangioendothelioma Paediatric Embolization 

Abbreviations

EHE

Epithelioid haemangioendothelioma

Notes

Compliance with ethical standards

Conflict of interest

The authors have no conflicts of interest to disclose.

References

  1. 1.
    Weiss SW, Enzinger FM (1982) Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer 50(5):970–981PubMedGoogle Scholar
  2. 2.
    Goldblum, J.R., Folpe A.L., Weiss S.W., ed. Enzinger and Weiss’s Soft Tissue Tumurs. 6th ed., ed J.R. Goldblum. 2014, Elsevier Saunders: PhiladelphiaGoogle Scholar
  3. 3.
    Zhang J, Wang Y, Geng D Intracranial epithelioid hemangioendothelioma: an unusual CTA finding in one case. Br J Neurosurg 24(3):294–295Google Scholar
  4. 4.
    Amit A et al (2012) Malignant hemangioendothelioma of occipital bone. Chin J Cancer Res 24(2):161–163PubMedPubMedCentralGoogle Scholar
  5. 5.
    Zheng J, Liu L, Wang J, Wang S, Cao Y, Zhao J (2012) Primary intracranial epithelioid hemangioendothelioma: a low-proliferation tumor exhibiting clinically malignant behavior. J Neuro-Oncol 110(1):119–127Google Scholar
  6. 6.
    Oliveira PCR, Alcantara FP, Souza-Vianna PE, Brito AP (2012) Cerebral epithelioid hemangioendothelioma with thoracic simultaneous involvement: advanced MRI features. Arq Neuropsiquiatr 70:637–638Google Scholar
  7. 7.
    Aniba K et al (2012) A tragical paediatric case history of intraorbital and intracranial epithelioid hemangioendothelioma. Case Rep Neurol Med 2012:396097PubMedPubMedCentralGoogle Scholar
  8. 8.
    Merikas E, Grapsa D, Dikoudi E, Gkiozos I, Boura P, Charpidou A, Kainis E, Syrigos K (2015) Epithelioid hemangioendothelioma treated with bevacizumab: a case series. Cancer Treatment Communications 4:59–64Google Scholar
  9. 9.
    Raheja A, Suri A, Singh S, Kumar R, Kumar R, Nambirajan A, Sharma MC (2015) Multimodality management of a giant skull base hemangioendothelioma of the sphenopetroclival region. J Clin Neurosci 22(9):1495–1498PubMedGoogle Scholar
  10. 10.
    Pacheco JM, Goodman JC, Mandel J (2015) Intracranial epithelioid hemangioendothelioma causing subacute loss of vision. Neurology 85(8):735–736PubMedGoogle Scholar
  11. 11.
    Chen TC, Gonzalez-Gomez I, Gilles FH, McComb JG (1997) Pediatric intracranial hemangioendotheliomas: case report. Neurosurgery 40(2):410–414PubMedGoogle Scholar
  12. 12.
    Rushing EJ et al (1998) Primary epithelioid hemangioendothelioma of the clivus. Clin Neuropathol 17(2):110–114PubMedGoogle Scholar
  13. 13.
    Koh Y-C, Yoo H (2001) Epithelioid haemangioendothelioma of the sphenoid bone. J Clin Neurosci 8(4, Part A):63–66PubMedGoogle Scholar
  14. 14.
    Fernandes AL, Ratilal B, Mafra M, Magalhaes C (2006) Aggressive intracranial and extra-cranial epithelioid hemangioendothelioma: a case report and review of the literature. Neuropathology 26(3):201–205PubMedGoogle Scholar
  15. 15.
    Taratuto AL, Zurbriggen G, Sevlever G, Saccoliti M (1988) Epithelioid hemangioendothelioma of the central nervous system. Pediatr Neurosurg 14(1):11–14Google Scholar
  16. 16.
    Kubota T, Sato K, Takeuchi H, Handa Y (2004) Successful removal after radiotherapy and vascular embolization in a huge tentorial epithelioid hemangioendothelioma: a case report. J Neuro-Oncol 68(2):177–183Google Scholar
  17. 17.
    Phookan G, Davis AT, Holmes B (1998) Hemangioendothelioma of the cavernous sinus: case report. Neurosurgery 42(5):1153–1155PubMedGoogle Scholar
  18. 18.
    Zhu Y, Fan M, Pandey S, Liang W, Chang D (2016) Multiple epithelioid hemangioedothelioma of the skull in a child. Medicine 95(30):e4081PubMedPubMedCentralGoogle Scholar
  19. 19.
    Tammam AG, Lewis PD, Crockard HA (1997) Cerebello-pontine angle epithelioid haemangioendothelioma in a 4-year-old boy. Childs Nerv Syst 13(11–12):648–650PubMedGoogle Scholar
  20. 20.
    Carlotti C, Jay V, Rulka J (Jan 2000) Infantile haemangioendothelioma of the pericranium presenting as an occipital mass lesion. J Neurosurg 92(1):156–160PubMedGoogle Scholar
  21. 21.
    Puca A, Meglio M, Rollo M, Zannoni GF (1996) Intracranial epithelioid hemangioendothelioma: case report. Neurosurgery 38(2):399–401PubMedGoogle Scholar
  22. 22.
    Yeo SK et al (2007) Intracranial epithelioid hemangioendothelioma. J Korean Neurosurg Soc 42(2):129–131Google Scholar
  23. 23.
    Baehring JM, Dickey PS, Bannykh SI (2004) Epithelioid hemangioendothelioma of the suprasellar area. Arch Pathol Lab Med 128:1289–1293PubMedGoogle Scholar
  24. 24.
    Mohan SM et al (2008) Intracranial epithelioid hemangioendothelioma. Childs Nerv Syst 24(7):863–868Google Scholar
  25. 25.
    Rubin, R., Strayer, D. , ed. Rubin’s pathology: clinicopathologic foundations of medicine. 6th ed. 2012, Lippincott Williams and Wilkins: Baltimore. 1450Google Scholar
  26. 26.
    Unni KK, Ivins JC, Beabout JW, Dahlin DC (1971) Haemangioma, haemangiopericytoma, and haemangioendothelioma (angiosarcoma) of bone. Cancer 27:1403–1414PubMedGoogle Scholar
  27. 27.
    Chen Y, Chen JQ, Katz RL (2015) Epithelioid hemangioendothelioma: a study of 14 cytopathology cases. Journal of the American Society of Cytopathology 4(3):148–159Google Scholar
  28. 28.
    Chow LT, Chow W, Fong DT (1992) Epithelioid hemangioendothelioma of the brain. Am J Surg Pathol 16(6):619–625PubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Suyi Ooi
    • 1
    Email author
  • Matthew Gutman
    • 2
  • Chris Xenos
    • 1
  • Ronil Chandra
    • 1
  • Catriona McLean
    • 2
  1. 1.Monash HealthMelbourneAustralia
  2. 2.Alfred HealthMelbourneAustralia

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