Epstein-Barr virus-associated smooth muscle tumor partially occluding the superior sagittal sinus: illustrative case

Abstract

Background: Venous sinus-occlusive mass lesions are infrequent and commonly include meningiomas, with Epstein-Barr virus-associated smooth muscle tumor (EBV-SMT) being much less common.

Observations: The authors present a case report of a venous sinus-occlusive EBV-SMT in an adolescent immunosuppressed male after liver transplantation.

Lessons: The case highlights the importance of considering blood flow dynamics and the use of advanced imaging modalities including incident dark field microcirculation microscopy and indocyanine green video angiography when managing venous sinus-occlusive mass lesions. To the best of the authors' knowledge, this is the first report of a primary intracranial venous sinus EBV-SMT occurring in an HIV-negative patient after liver transplantation, and it illustrates management dilemmas of a sinus-occlusive tumor at a sensitive region of the superior sagittal sinus. https://thejns.org/doi/10.3171/CASE24759.

Keywords: Epstein-Barr virus; superior sagittal sinus; tumor.

FIG. 1.

A: T2- and T1-weighted axial MRI sequences showing initial lymphoproliferative disease in the left basal ganglia and caudate regions after craniotomy. B:Axial (left) and coronal (right) T1-weighted sequences showing a right frontal Ommaya reservoir. Arrows indicate the catheter tip in the left lateral ventricle approaching the foramen of Monro.

FIG. 2.

A:Preoperative sagittal (left) and coronal (right) T1-weighted postcontrast MRI sequences showing heterogeneously enhancing tumor in the superior sagittal sinus measuring 4.5 cm in length by 1.5 cm in width, located at the junction of the anterior two-thirds and posterior one-third of the sinus. B: Preoperative sagittal (left) and coronal (right) MRV sequences showing partial occlusion of the superior sagittal sinus by the tumor. C: Repeat sagittal (left) and coronal (right) MRV sequences suggesting eventual complete occlusion of the superior sagittal sinus after several months. D: Sagittal (left) and coronal (right) digital subtraction angiograms confirming complete occlusion of the superior sagittal sinus. E: Postoperative sagittal (left) and coronal (right) T1-weighted postcontrast MRI sequences on POD1 demonstrating complete resection of the tumor. F and G: Postoperative sagittal MRV sequences showing a nonocclusive thrombus extending from the operative site to immediately proximal to the torcula (G). H: Follow-up sagittal (left) and coronal (right) T1-weighted MRI sequences 22 months after anticoagulation discontinuation showing fully resolved thrombus. I: Follow-up sagittal (left) and coronal (right) T1-weighted MRI sequences at the 5.5-year follow-up showing thrombus remained fully resolved and no lesion recurrence.

FIG. 3.

Intraoperative microscopic view showing the lesion being resected from within the superior sagittal sinus and its gross appearance (well-encapsulated, gray, rubbery mass) along with one of several focal points of adherence to the endothelial lining of the sinus.