Diagnosis and Management of a Rare Sphenoid Wing Mass with Clinical Findings Mimicking a Carotid Cavernous Sinus Fistula Melissa Chen, MD Stephen Chen, MD Vivek Sahani, DO Control #: 2805 Poster # EE-25

Disclosures None

Purpose To describe an unusual presentation of vision loss from a rare sphenoid wing hemangioendothelioma and present imaging findings that is crucial for neurosurgical planning.

History 37 year old Hispanic female from El Salvador Gradual onset of mild left eye pain and left forehead pain. Initially seen in a community clinic and prescribed eye drops for the pain. Presented to Emergency room 2 x over course of next month with severe blurring of vision, new onset tinnitus, worsening headache and sharp stabbing left eye pain.

Physical exam Left orbital proptosis and chemosis Left pupil is non-reactive to light Visual acuity 20/400 in left eye

Unenhanced CT Head On soft tissue window, a mass (red arrow) erodes the adjacent sphenoid wing. The left superior ophthalmic vein (blue arrow) is enlarged.

Enhanced CT Face Contrast enhanced CT demonstrates avid contrast enhancement of a lobulated 2.8 cm extra-axial mass (red arrow) that destroys the adjacent bone. The enlarged superior ophthalmic vein (blue arrow) enhances early, suggesting arterialization. Note that there is no enhancement of the contralateral vein.

Enhanced CT Face The left middle meningeal (blue arrow) artery is enlarged adjacent to the tumor, which suggests that it supplies the tumor.

Enhanced CT Face Coronal image demonstrates a similarly enlarged left internal maxillary artery (blue arrow) also supplying the highly vascular tumor. Atypical enlargement and early enhancement is seen in both facial veins (red arrows), indicates high flow AV shunt physiology within the mass.

External Carotid DSA Angiogram confirmed a hypertrophied middle meningeal artery (red arrow) supplied the tumor, the dominant feeder. Secondary feeder from a large posterior deep temporal artery (blue arrow). Lateral

External Carotid DSA Mass demonstrates rapid arterial venous shunt pathology (red arrow). Drainage is through the superior ophthalmic vein (blue arrow). Secondary drainage through inferior ophthalmic vein (yellow arrow). Early filling of enlarged facial vein (dark blue arrow). Lateral

MRI Head Axial T2 image demonstrates intermediate inhomogeneous signal within the mass (red). Flow voids (blue) are visible within the mass which can also be a sign of a highly vascular tumor. The left extraocular muscles (yellow) are enlarged with increased T2 signal, likely sequela of venous hypertension.

Embolization Tumor was catheterized through the middle meningeal artery (red arrow) with a 1.5 Fr. Marathon microcatheter (ev3). Lateral

Embolization Tumor was then embolized with a liquid embolic, Onyx18 (ev3) The posterior deep temporal artery was also catheterized and embolized. This tantalum opacified cast fills the tumor and feeding vessels. Lateral

Post Embolization Absent filling of the distal internal maxillary artery (red arrow) due to vasospasm. A small residual collateral (blue arrow) from the proximal middle meningeal artery remained present, but vision and proptosis improved immediately. Lateral

MRI Head Axial T1 fat saturated image with contrast demonstrates avid enhancement of the infiltrative mass (red arrow) in the sphenoid wing. Asymmetric increased enhancement is also seem in the left extra-ocular muscles (yellow arrow) Dx: Epithelioid Hemangioendothelioma

Clinical Diagnosis Vascular lesions involving the orbit are often misdiagnosed as conjunctivitis, uveitis and scleritis by clinicians. Arterial venous fistula involving the eye should be considered when patients present with:  Orbital pain with proptosis  Chemosis  Orbital bruit  Tinnitus  Vision loss Chen, SR (2016) Endovascular Approaches to Orbital Vascular Lesions and Carotid Cavernous Sinus Fistulas In Yen MT. Vascular Lesions of the Orbit and Face (pp 63-87) Switzerland, Springer International Publishing.

Epithelioid Hemangioendothelioma Rare malignant, vascular tumor Incidence 1:1,000,000 Slow growing tumors, with intermediate malignancy pattern between benign hemangioma and high grade angiosarcoma. (hematoxylin and eosin, 10x) Weiss SW, Enzinger FM (1982) Epithelioid hemangioendothelioma a vascular tumor often mistaken for a carcinoma. Cancer 50:970–981.

Epithelioid Hemangioendothelioma Accounts for only < 0.02% of intracranial tumors, where it arises from the dura or the bone. Histologically, primitive thick walled vascular channels are seen within cords and nests of spindle and epithelioid cells. (hematoxylin and eosin, 10x) Tian W-Z, Yu X-R, Wang W-W, et al (2016) Computed tomography and magnetic resonance features of intracranial hemangioendothelioma: A study of 7 cases. Oncol Lett 11:3105–3110.

Discussion Although the pathologic diagnosis and clinical presentation in this case are extremely rare, identification of key imaging findings and correlation with clinical signs are widely applicable. Severe intraoperative hemorrhage during tumor resection can be avoided if the need for pre-operative embolization is identified prospectively, avoiding intra- operative complications, shortening operative times, and reducing repeat surgery.

Diagnostic imaging clues High arterial flow  Enlargement of arteries adjacent to a tumor  Early avid enhancement  Flow voids on MR T2 weighted images Arterial venous shunting  Early venous enhancement: arterialization of the veins  Venous engorgement in draining vessels

Conclusion Optic nerve compression is not the only source of vision loss. Arterial venous shunting around the orbit can result in venous hypertension and retinal ischemia. Embolization can immediately restore and preserve vision. Radiologists should be able to recognize imaging findings of high arterial flow into a tumor and recommend pre-operative embolization to prevent excessive blood loss and surgical complications. By recognizing these imaging findings, neuroradiology can offer a more complete patient care pathway encompassing both diagnosis and therapy.