Clinical Neurology and Neurosurgery 119 (2014) 106–109

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Case Report

Endoscopic resection of an infraorbital nerve schwannoma夽 Pierre-Olivier Champagne a,∗ , Martin Desrosiers b , Robert Moumdjian a a b

Department of Neurosurgery, Notre-Dame Hospital, Université de Montréal, Montréal, Québec, Canada Department of Otorhinolaryngology, Notre-Dame Hospital, Université de Montréal, Montréal, Québec, Canada

a r t i c l e

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Article history: Received 7 August 2013 Received in revised form 15 December 2013 Accepted 19 January 2014 Available online 27 January 2014 Keywords: Schwannoma Endoscopic endonasal surgery Infraorbital nerve Minimally invasive surgery Trigeminal nerve

1. Introduction Schwannomas are benign tumours which originate from the Schwann cells sheathing the nerves. Their usual treatment consists of a complete surgical excision. While schwannomas of the peripheral nerves are relatively common, trigeminal nerve schwannomas originating from the extracranial portion of the nerve and extending inside the cranium are a rarity. For this precise type of tumour, the favoured way of removal is via a transcranial, infratemporal approach [1]. In this report, we present the case of a 48 year-old man with a voluminous trigeminal nerve schwannoma originating from the infraorbital nerve that extended intracranially. It was successfully treated using an endoscopic endonasal approach. 2. Case report A 48-year-old man with no significant past medical history presented with a mass in the right infraorbital region increasing in size over the past 6 years. The patient has been neglecting this mass over the years and waited before seeking medical attention. Associated symptoms were paresthesia over the right cheek and difficulty with

夽 Presented at the 2012 Paris annual encounter of the Société de Neurochirurgie de Langue Franc¸aise (SNCLF). ∗ Corresponding author at: 3824 Saint-André app3, Montréal, Québec, Canada H2L 3V8. E-mail address: pierre [email protected] (P.-O. Champagne). 0303-8467/$ – see front matter © 2014 Elsevier B.V. All rights reserved. http://dx.doi.org/10.1016/j.clineuro.2014.01.017

chewing on the right side. Nasal symptoms were notably absent. Physical exam revealed a firm, nontender right infraorbital mass, right exophthalmia and upward gaze deficit of the right eye causing a vertical binocular diplopia. Computed tomography scan and MRI imaging showed a multilobar, gadolinium enhancing mass centred on the right pterygopalatine fossa, measuring 5.6 cm × 3.1 cm and 6 cm craniocaudaly (Fig. 1). It extended anteriorly to deform the posterior wall of the maxillary sinus, inferior and laterally to the temporal fossa, and superiorly through the foramen ovale to reach the cavernous sinus. There was no evidence of bone destruction and the mass was well delineated. There was no evidence of extensive vascularisation suggesting a diagnosis such as juvenile angiofibroma. Our main diagnostic impression at that time was that of an infraorbital nerve (V2) schwannoma and the patient was scheduled for a biopsy/resection of the lesion through an endoscopic endonasal approach. Intra-operatively, the tumour was resected through an endoscopic approach using a right endoscopic maxillary meatotomy, with an extension to the pteryigomaxillary fossa created surgically through the posterior wall of the maxillary sinus (Figs. 2 and 3). In order to perform the resection of this large mass via the limited access, the tumour was initially “cored” out to reduce its size prior to final resection. Its mucoïd texture and well-circumscribed borders allowed the tumour to collapse upon itself as the surgery progressed, permitting removal of this large tumour without an external incision. The final intracranial portion was accessed by resecting the medial portion of the pterygopalatine fossa contents to improve access to the lateral aspect of the sphenoid. A connection between the mass and the infraorbital

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Fig. 1. Pre-operative MRI (T1, contrast enhanced) showing the massive enhancing lesion spanning both in the (a) extracranial and (b) intracranial compartments.

nerve found during the surgery confirmed our initial impression regarding its origin. The resection was judged near-total at the end of the surgery, with all of the apparent tumour removed. A capsule fragment in the right cavernous sinus abutting the internal carotid artery was judged non-resectable and left in place. The patient evolved well post-operatively with no complications save for a slight hypoesthesia of the right V2 distribution which was already present preoperatively in the form of paresthesia. CT scan imaging on post-operative day one showed no bleeding and no sing of residual mass. Clinically, there was no

sign of cerebro-spinal fluid leak and the patient was able to leave the hospital 2 days after surgery with no complaint about pain. Histological examination of the mass confirmed the diagnosis by showing the typical characteristics of a schwannoma (Fig. 4). 2 months follow-up showed no clinical or radiological recurrence. MRI at that time revealed macroscopic resection with some contrast enhancement in the right parasellar region (Fig. 5). The numbness in the right V2 area persisted but nevertheless regressed over the course of time.

Fig. 2. Standard endoscopic endonasal approach for the pterygomaxillary fossa. (a) Standard endonasal initial view, arrow showing area of the middle meatus permitting access to adjacent structures. (b) View of the maxillary sinus, arrow showing the posterior wall of the maxillary sinus. (c) Removal of the posterior wall of the maxillary sinus allows exposure of the pterygomaxillary fossa.

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3. Discussion

Fig. 3. Schematic endoscopic representation of the tumour removal. (a) Tumour seen through an opening in the (b) posterior wall of the maxillary sinus, which can be seen through the maxillary meatotomy.

Fig. 4. Histopathological analysis of the tumour showing characteristic features of schwannomas, such as Antoni A areas (palisading nuclei).

Schwannomas are the most common peripheral nerve tumour. They consist of a usually benign, slow growing neoplasm originating from the Schwann cells that form a sheath around the nerves. Schwannomas of the extra-cranial aspect of the trigeminal nerve with intracranial extension are a very rare occurrence, with only 69 cases reported in literature [2]. Extra cranial schwannomas were introduced into Jefferson’s classification of trigeminal schwannomas as “type D” by Day and Fukushima in their 1998 publication [3]. They described them as schwannomas originating from either the maxillary or mandibular division of the trigeminal nerve and extending intracranially through either the foramen ovale or rotundum. Although not being true “type D” schwannomas, a few cases of infraorbital nerve schwannomas invading mainly the orbit or the pterygopalatine fossa without intracranial extension have also been reported. The current literature suggests approaching trigeminal schwannomas expanding in both the middle temporal fossa and the extracranial space via open approaches such as the extradural infratemporal [1], transmaxillary [4], transmandibular [5] and transfacial approaches. All involve risks such as facial nerve damage, and can bring major aesthetic issues. The endonasal endoscopic approach is known to allow the access to paranasal sinuses, pterygopalatine fossa and skull base. Thus, its use in treating type D schwannomas which can be by definition mostly extracranial seems acceptable. However, reports of total endoscopic removal of type D trigeminal schwannomas are rare, with only 8 cases published in the literature [2]. Factors that can make the clinicians reluctant to the endoscopic approach in such cases include (i) the size of the tumour and (ii) its intracranial invasiveness, especially those in close proximity with the cavernous sinus. In this report, we describe the successful endoscopic treatment of one of the most voluminous type D schwannoma involving the cavernous sinus. The feasibility and safety of the endoscopic approach for the resection of this type of lesion is supported by the excellent postoperative outcome reported here. The contrast enhancement seen in the parasellar region in the 2 months imaging could represent residual tumour since tumoural capsule in the right cavernous sinus was left in place at the time of surgery. Subsequent follow-up imaging will reveal in the future the significance of this contrast enhancement.

Fig. 5. Post-operative MRI (T1, contrast enhanced) showing (a) extracranial and (b) intracranial macroscopic resection with post-operatives changes and contrast enhancement in the right parasellar region.

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4. Conclusion

References

Type D trigeminal schwannoma are a rarity. Recent but sparse literature has reported the endoscopic method as a safe and minimally invasive way to treat this type of tumour. However, evidence is still lacking about the practicality of this approach, especially in cases with big and invasive schwannomas. In this report, we describe a case of a large and invasive type D schwannoma that was completely excised through a purely endoscopic approach with no complications and no recurrence on follow-up. It offers additional support that even in cases presenting with large and invasive tumours, the endoscopic approach is a valid treatment option that may be associated with less morbidity.

[1] Goel A, Shah A, Muzumdar D, Nadkarni T, Chagla A. Trigeminal neurinomas with extracranial extension: analysis of 28 surgically treated cases. J Neurosurg 2010;113(5):1079–84. [2] Zhang Q, Feng K, Ge C, Hongchuan G, Mingchu L. Endoscopic endonasal management of trigeminal schwannomas extending into the infratemporal fossa. J Clin Neurosci 2012;19(6):862–5. [3] Day JD, Fukushima T. The surgical management of trigeminal neuromas. Neurosurgery 1998;2:233–40. [4] Suenaga T, Satoh M, Fukuda S, Tanaka K. Benign schwannoma of the pterygopalatine fossa with intracranial extension: a case report. Auris Nasus Larynx 1987;14(2):115–9. [5] Kouyialis AT, Stranjalis G, Papadogiorgakis N, Papavlassopoulos F, Ziaka DS, Petsinis V, et al. Giant dumbbell-shaped middle cranial fossa trigeminal schwannoma with extension to the infratemporal and posterior fossae. Acta Neurochir (Wien) 2007;149(9):959–64.

Endoscopic resection of an infraorbital nerve schwannoma.

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