Epithelioid hemangioendothelioma of the craniocervical junction; case report and review

Abstract

Epithelioid hemangioendotheliomas are uncommon vascular neoplasms and their spinal location is even rarer. We report clinical course of a 31-year-old man with an epithelioid hemangioendothelioma at the cranio-cervical junction. A cervical magnetic resonance imaging revealed tumor that caused posterior cervical cord compression. C1,2,3 total laminectomy and surgical excision of the tumor was performed. Postoperative external beam radiation was performed on the surgical field especially around the right vertebral artery. At 2-year follow-up there was no neurological deficit and no tumor recurrence.

Keywords: Craniocervical junction; Epithelioid hemangioendothelioma; Surgical excision.

Fig. 1

Preoperative T2-weighted images demonstrated low intensity mass lesion at the craniocervical junction.

Fig. 2

After Gadolinium injection sagittal T1-weighted magnetic resonance images showed that mass lesion extends from craniocervical junction to epidural surface.

Fig. 3

After Gadolinium injection axial T1-weighted images revealed a compression to spinal cord.

Fig. 4

Preoperative sagittal computed tomography images showed a lesion located in craniocervical junction and extends to occipital bone superiorly and C3 posterior structures inferiorly.

Fig. 5

Cranio-cervical instrumentation in early postoperative period.

Fig. 6

No recurrence was detected on sagittal magnetic resonance imaging obtained in the second postoperative year.

Fig. 7

A calcified barrier originated from dural graft was seen on computed tomography obtained in the second postoperative year.

Fig. 8

An infiltration with tumor cells with eosinophilic cytoplasm was seen between muscle fibers (A), in the magnified preperate, tumor cells with large eosinophilic cytoplasm, pleomorphic and dense nucleus were seen. Cytoplasmic vacuoles were seen in some tumor cells (B). After immunohistochemical examinations, tumor cells were negative with TTF-1 and tiroglobuline. Dense positive with vimentin, CD34 and CD31 (C) and focal positive with pan-Cytokeratin (D). A- HE, ×200, B- HE, ×400, C- CD31, ×200, D- PCK, ×400.