Acute Complete Oculomotor Nerve Palsy in a Young Male Due to a Skull Base Myofibroma

A 25-year-old male patient visited the ophthalmology clinic because of upper eye lid ptosis in the right eye, binocular double vision, and light sensitivity. He was diagnosed with a complete third nerve palsy caused by a skull base myofibroma, a rare clinical entity that has not been described before in oculomotor nerve palsy

Photo and Video Essay Section Editors: Melissa W. Ko, MD Dean M. Cestari, MD Peter Quiros, MD Acute Complete Oculomotor Nerve Palsy in a Young Male Due to a Skull Base Myofibroma Tung Thanh Hoang, MD, MOphth, Thuy Van Le, MD, Dat Tran, MD, Thuong Duc Ho, MD, Nhung Hong Thi Le, MD, Prem S. Subramanian, MD, PhD Abstract: A 25-year-old male patient visited the ophthalmology clinic because of upper eye lid ptosis in the right eye, binocular double vision, and light sensitivity. He was diagnosed with a complete third nerve palsy caused by a skull base myofibroma, a rare clinical entity that has not been described before in oculomotor nerve palsy. Journal of Neuro-Ophthalmology 2021;41:e759–e760 doi: 10.1097/WNO.0000000000001177 © 2020 by North American Neuro-Ophthalmology Society O culomotor nerve palsy may have vascular, traumatic, compressive, and other causes, (1) including benign tumors, such as schwannoma (2) and neurinoma (3). In this article, we report the first case, to the best of our knowledge, of oculomotor nerve palsy caused by myofibroma. A 25-year-old male patient presented with acute right upper eye lid ptosis, binocular diplopia, and photophobia. He denied any blurry vision, ocular pain, headache, and history of trauma. MRI with contrast demonstrated a discrete, enhancing mass along the right third nerve (Fig. 1B). Time-offlight (TOF) MR angiography (MRA) showed no aneurysm. He underwent craniotomy to remove the tumor that was found intraoperatively to invade and distort the nerve Ophthalmology Department (TTH, NHTL), Hanoi Medical University, Hanoi, Vietnam; Ophthalmology Unit (TTH, NHTL), Hanoi Medical University Hospital, Hanoi, Vietnam; Save Sight Institute (TTH), the University of Sydney School of Medicine, Sydney, Australia; Neurology Unit (TVL), Hanoi Medical University Hospital, Hanoi, Vietnam; Neurosurgery Department I (DT), Viet Duc Hospital, Hanoi, Vietnam; Pathology Department (TDH), Viet Duc Hospital, Hanoi, Vietnam; and Ophthalmology Department (PSS), University of Colorado School of Medicine, Aurora, Colorado. The authors report no conflicts of interest. Address correspondence to Tung Thanh Hoang, MD, MOphth, Hanoi Medical University, No 1, Ton That Tung Street, Dong Da District, Hanoi 11518, Vietnam; E-mail: [e-mail] Hoang et al: J Neuro-Ophthalmol 2021; 41: e759-e760 FIG. 1. A. Right complete third nerve palsy. B. Tumor compressing third nerve. C. Tumor dissection. e759 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo and Video Essay FIG. 2. A. Myoid nodule (H&E, ·200). B. Smooth muscle actin (+) (SMA, ·200). C. Cellular vascular zones (H&E, ·200). D. Cellular vascular zones (CD34, ·200). (Fig. 1C). The tumor in the skull base region was morphological bland spindle cells with pale eosinophilic cytoplasm and elongated nuclei arranged in short fascicles (Fig. 2A. H&E, ·200). The lesional cells were diffusely positive for smooth muscle actin (SMA) (Fig. 2B. SMA, ·200). Prominent thin-walled branching stromal blood vessels (cellular hemangiopericytoma-like vascular zones) were nicely highlighted in a CD34 immunostain, but this marker was negative in tumor cells (Fig. 2C. H&E, ·200 and Fig. 2D. CD34, ·200). Histopathology and immunohistochemistry with SMA (+), CD34 (2), desmin (2), epithelial membrane antigen (2), and S100 (2) confirmed a myofibroma without any evidence of malignancy. MRI 1-month postoperative was clear of remaining myofibroma. Myofibroma, a rare and benign tumor involving the skin of the head and neck, seems to be more common in childhood than adulthood. Myofibromas in ocular and orbital regions have been reported, but we have not found any study describing myofibroma in the skull base affecting cranial nerves (4,5). Acute, pupil-involving oculomotor nerve palsy requires vascular neuroimaging, such as TOF MRA (1), and this case emphasizes the need to obtain structural imaging as well (i.e., MRI) to identify potential compressive lesions. e760 STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: T. T. Hoang; b. Acquisition of data: T. T. Hoang, T. Van Le, D. Tran, T. D. Ho, and N. H. Thi Le; c. Analysis and interpretation of data: T. T. Hoang, T. Van Le, D. Tran, and T. D. Ho. Category 2: a. Drafting the manuscript: T. T. Hoang and P. S. Subramanian; b. Revising it for intellectual content: T. T. Hoang and P. S. Subramanian. Category 3: a. Final approval of the completed manuscript: P. S. Subramanian. REFERENCES 1. Raza HK, Chen H, Chansysouphanthong T, Cui G. The aetiologies of the unilateral oculomotor nerve palsy: a review of the literature. Somatosens Mot Res. 2018;35:229–239. 2. Muhammad S, Niemelä M. Management of oculomotor nerve schwannoma: systematic review of literature and illustrative case. Surg Neurol Int. 2019;10:40. 3. Sharipov OI, Konovalov AN, Serova NK, Kosyr’kova AV, Kutin MA, Pitskhelauri DI, Shishkina LV, Kalinin PL. Oculomotor nerve neurinomas. Case reports and a literature review [in Russian]. Zh Vopr Neirokhir Im N N Burdenko. 2019;83:105– 111. 4. Eshraghi B, Dehghani S, Saeedi-Anari G. A rare erosive orbital mass in a child: case report of myofibroma. J Curr Ophthalmol. 2017;29:224–227. 5. Zimmermann L, Reinhard T, Lange C, Heegaard S, Auw-Haedrich C. Corneal myofibroma (keloid) in a young patient with neurofibromatosis type 2. Ocul Oncol Pathol. 2017;3:247–249. Hoang et al: J Neuro-Ophthalmol 2021; 41: e759-e760 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.