European Orthopaedics and Traumatology

, Volume 5, Issue 4, pp 375–378 | Cite as

Epithelioid hemangioendothelioma of the cervical spine successfully treated with subtotal resection and radiotherapy

  • Daniel Pérez-Prieto
  • Josep Lloreta
  • Manuel Ramírez
Case Report
  • 43 Downloads

Abstract

Epithelioid hemangioendothelioma (EHE) is a mid-grade malignant neoplasm of endothelial origin with an unpredictable clinical course and prognosis, representing <1 % of malignant bone tumors. A 63-year-old man was visited for a loss of strength in the upper right limb. MRI confirmed an expansive process in C5, PET scan did not find any other tumor localizations, and the CT-guided biopsy determined it to be an endothelial tumor. C5 corpectomy along with a C2-T2 arthrodesis was performed in a two-stage surgery, and subtotal tumor excision was obtained. Then, the patient underwent 23 radiotherapy sessions. The anatomopathological result was a low-grade EHE. At a 3-year follow-up, the patient is autonomous for ADLs with a low degree of dexterity in both hands. Preoperative CT-guided biopsy is usually non-definitive as was in the present case. Therefore, an inmunohistochemical study for the detection of factor VII antigen is crucial for definitive diagnosis. Surgical ablation should be the basis for treatment as it has been proven that “en bloc” resection reduces local recurrence. Radiotherapy has demonstrated to be helpful.

Keywords

Cervical spine Tumor Epithelioid hemangioendothelioma Radiotherapy 

Notes

Conflict of interest

The authors declare that they have no conflict of interest.

References

  1. 1.
    Kerry G, Marx O, Kraus D, Vogel M, Kaiser A, Ruedinger C et al (2012) Multifocal epithelioid hemangioendothelioma derived from the spine region: case report and literature review. Case Rep Oncol 5(1):91–98PubMedCentralPubMedCrossRefGoogle Scholar
  2. 2.
    Ma J, Wang L, Mo W, Yang X, Xiao J (2011) Epithelioid hemangioendotheliomas of the spine: clinical characters with middle and long-term follow-up under surgical treatments. Eur Spine J 20(8):1371–1376PubMedCentralPubMedCrossRefGoogle Scholar
  3. 3.
    Yim KL, Sumathi VP, Spooner D (2012) Radiotherapy as an effective primary treatment for epithelioid haemangioendothelioma of the cervical spine. Anticancer Res 32(10):4597–4600PubMedGoogle Scholar
  4. 4.
    Ellis TS, Schwartz A, Starr JK, Riedel CJ (1996) Epithelioid hemangioendothelioma of the lumbar vertebral column: case report and review of literature. Neurosurgery 38(2):402–407PubMedCrossRefGoogle Scholar
  5. 5.
    Aquilina K, Lim C, Kamel MH, Marks CJ, O'Sullivan MG, Keohane C (2005) Epithelioid hemangioendothelioma of the spine. Report of two cases. J Neurosurg Spine 3(5):393–399PubMedCrossRefGoogle Scholar
  6. 6.
    Kopniczky Z, Tsimpas A, Lawson DD, King A, Pohl U, Pollock JR et al (2008) Epithelioid hemangioendothelioma of the spine: report of two cases and review of the literature. Br J Neurosurg 22(6):793–797PubMedCrossRefGoogle Scholar
  7. 7.
    Hislop H (1995) Daniels and Worthingham’s muscle testing: techniques of manual examination, 6th edn. Saunders, PhiladelphiaGoogle Scholar
  8. 8.
    Weiss SW, Enzinger FM (1982) Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer 50(5):970–981PubMedCrossRefGoogle Scholar
  9. 9.
    Themistocleous GS, Papagelopoulos PJ, Petraki KD, Stilianessi EV, Partsinevelos AA, Sapkas GS (2005) A 23-year-old woman with complete paraplegia and anesthesia below the T8 level. Clin Orthop Relat Res (430):258–265Google Scholar
  10. 10.
    Aflatoon K, Staals E, Bertoni F, Bacchini P, Donati D, Fabbri N, et al (2004) Hemangioendothelioma of the spine. Clin Orthop Relat Res (418):191–197Google Scholar
  11. 11.
    Evans HL, Raymond AK, Ayala AG (2003) Vascular tumors of bone: a study of 17 cases other than ordinary hemangioma, with an evaluation of the relationship of hemangioendothelioma of bone to epithelioid hemangioma, epithelioid hemangioendothelioma, and high-grade angiosarcoma. Hum Pathol 34(7):680–689PubMedCrossRefGoogle Scholar
  12. 12.
    Casey MC, Lim C, Hickey MC (2004) Case of the month: an unusual cause of neck pain. Br J Radiol 77(918):539–540PubMedCrossRefGoogle Scholar
  13. 13.
    Gokhan GA, Akyuz M, Gurer IE, Tuncer R (2006) Epithelioid hemangioendothelioma derived from the spine region: case report and review of the literature. Wien Klin Wochenschr 118(11–12):358–361PubMedCrossRefGoogle Scholar
  14. 14.
    Calderaro J, Guedj N, Dauzac C, Wassef M, Guigui P, Bedossa P (2011) A case of epithelioid hemangioma of the spine. Ann Pathol 31(4):312–315PubMedCrossRefGoogle Scholar
  15. 15.
    Campanacci M, Boriani S, Giunti A (1980) Hemangioendothelioma of bone: a study of 29 cases. Cancer 46(4):804–814PubMedCrossRefGoogle Scholar
  16. 16.
    Sybert DR, Steffee AD, Keppler L, Biscup RS, Enker P (1995) Seven-year follow-up of vertebral excision and reconstruction for malignant hemangioendothelioma of bone. Spine (Phila Pa 1976) 20(7):841–844CrossRefGoogle Scholar
  17. 17.
    Tountas AA, Fornasier VL, Harwood AR, Leung PM (1979) Postirradiation sarcoma of bone: a perspective. Cancer 43(1):182–187PubMedCrossRefGoogle Scholar

Copyright information

© EFORT 2014

Authors and Affiliations

  • Daniel Pérez-Prieto
    • 1
  • Josep Lloreta
    • 2
  • Manuel Ramírez
    • 1
  1. 1.Department of Orthopedic Surgery and TraumatologyHospital del Mar - Parc de Salut MARBarcelonaSpain
  2. 2.Department of PathologyHospital del Mar - Parc de Salut MARBarcelonaSpain

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