Internal jugular vein vascular malformation presenting as mass at root of neck: a case report

Abstract

Background: We report a case of vascular malformation arising from internal jugular vein presenting as mass at root of neck with no clinical stigmata which to the best of our knowledge is the first reported case of an intrinsic vascular malformation arising from the internal jugular vein. Magnetic resonance imaging features of this new entity have been described.

Case presentation: A 27 year male presented with a gradually enlarging, asymptomatic swelling on left supraclavicular region with normal overlying skin. A soft mass, about 7 x 7 cm with restricted mobility was found with normal cranial nerve function. Fine needle aspiration cytology showed a hemorrhagic aspirate. Doppler showed a mass displacing left carotid artery posteriorly while left internal jugular vein was not visualized. Magnetic resonance imaging showed a well defined mass isointense to hypointense on T1 weighted and hyperintense on T2 weighted and STIR images with fluid-fluid levels. On exploration, a vascular mass arising from left internal jugular vein was found with good tissue planes, which was excised after ligating the patent internal jugular vein above and below the lesion. Histopathologic examination confirmed the diagnosis of vascular malformation.

Conclusion: The diagnosis of intrinsic vascular malformation arising from internal jugular vein should be kept in differential while dealing with masses at root of neck and magnetic resonance imaging features may help in the pre-operative diagnosis of this entity.

Figure 1

T1 W magnetic resonance image of the neck (coronal section) showing a mass in infrahyoid neck which is isointense to hypointense to muscle.

Figure 2

T2 W magnetic resonance image of the neck (sagital section) showing hyperintense mass with fluid-fluid levels (arrow head, pointing upwards).

Figure 3

T2 W magnetic resonance image of the neck (axial section) showing hyperintense mass with fluid-fluid levels.

Figure 4

intra-operative photograph showing left internal carotid artery, vagus nerve and vascular mass involving the left internal jugular vein.

Figure 5

gross cut section of the specimen showing multiple vascular channels.

Figure 6

Histological slide of the mass showing dilated vascular channels of vascular caliber with thick muscular walls and focal calcification.

Figure 7

Elastin vangeison (EVG) stained section showing lack of elastic fibers reinforcing venous origin.