Epithelioid hemangioendothelioma, an aggressive vascular tumor has the rarity of morbidity that arises in the spine. There were few cases reported in literatures in recent years, and little was known about this disease. A review study of the patient files in our constitutions between 1996 and 2006 showed that five patients were treated for spinal epithelioid hemangioendothelioma. Although only five patients, this study attempts to bring more informations about this rare lesion. This patient group included two males and three females. The lesions located in the cervical (case 1) or thoracic (case 2–4) or lumbar spine (case 5). Treatments included: laminectomy and cytoreductive surgery followed by external beam irradiation (one patient), expanded resection in piece meal with postoperative external beam irradiation (three patients), and total en bloc resection alone (one patient). Reconstruction of the spinal stability was performed in four patients. Follow-up period ranged from 25 to 72 months, averaged 47.4 months. The neurologic function of patients got a satisfactory progress except the paraplegic patient at diagnosis. The patient who received laminectomy and cytoreductive surgery followed by external beam irradiation still presented with tumor local progress, metastasis, and she died at 34 months after operation. No local recurrence or distant metastasis was detected in the other four patients. Epithelioid hemangioendothelioma of the spine is so rare in clinic as a primary aggressive vascular tumor. Based on our experience, a valid expanded resection of the tumor with adjunct radiation therapy or total en bloc excision may present with acceptable results.
Epithelioid hemangioendothelioma Spine Clinical character Outcomes Surgical treatment
This is a preview of subscription content, log in to check access.
No funds were received in support of this work. No benefits in any form have been or will be received from a commercial party related directly or indirectly to the subject of this manuscript.
Conflict of interest
Aquilina K, Lim C, Kamel MH et al (2005) Epithelioid hemangioendothelioma of the spine. Report of two cases. J Neurosurg Spine 3(5):393–399PubMedCrossRefGoogle Scholar
Arbelaez A, Castillo M, Williams L et al (1999) Vertebral hemangioendothelial sarcoma: MR findings. J Comput Assist Tomogr 23:981–983PubMedCrossRefGoogle Scholar
Bollinger BK, Laskin WB, Knight CB (1994) Epithelioid hemangioendothelioma with multiple site involvement Literature review and observations. Cancer 73(3):610–615PubMedCrossRefGoogle Scholar
Boutin RD, Spaeth HJ, Mangalik A et al (1996) Epithelioid hemangioendothelioma of bone. Skeletal Radiol 25:391–395PubMedCrossRefGoogle Scholar
Brennan JW, Midha R, Ang LC et al (2001) Epithelioid hemangioendothelioma of the spine presenting as cervical myelopathy: case report. Neurosurgery 48(5):1166–1169PubMedCrossRefGoogle Scholar
Chan YL, Ng HK, Poon WS et al (2001) Epithelioid hemangioendothelioma of the brain: a case report. Neuroradiology 43(10):848–850PubMedCrossRefGoogle Scholar
Christodoulou A, Symeonidis PD, Kapoutsis D et al (2008) Primary epithelioid hemangioendothelioma of the lumbar spine. Spine 8(2):385–390CrossRefGoogle Scholar
Cronin P, Arenberg D (2004) Pulmonary epithelioid hemangioendothelioma: an unusual case a review of the literature. Chest 125(2):789–793PubMedCrossRefGoogle Scholar
Di Girolamo A, Giacomini PG, Coli A et al (2003) Epithelioid hemangioendothelioma arising in the nasal cavity. J Laryngol Otol 117(1):75–77PubMedGoogle Scholar
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. Human Pathol 34(7):680–689CrossRefGoogle Scholar
Faust J, Schmidt M, Eysel P et al (2001) Epithelioid hemangioendothelioma of the spine. Med Klin (Munich) 96(12):740–744CrossRefGoogle Scholar
Fletcher CDM (2006) The evolving classification of soft tissue tumours: an update based on the new WHO classification. Histopathology 48:3–12 (Review)PubMedCrossRefGoogle Scholar
Gokhan GA, Akyuz M, Gurer IE et al (2006) Epithelioid hemangioendothelioma derived from the spine region: case report and review of the literature. Wien Klin Wochenschr 118(11–12):358–361PubMedCrossRefGoogle Scholar
Miettinen M. (2003) Epithelioid hemangioendothelioma. In: Diagnostic soft tissue pathology. Armed Forces Institute of Pathology, editor. Churchill Livingtone, New York, pp 323–324Google Scholar