Journal of Clinical Neuroscience xxx (2014) xxx–xxx
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Clinical Study
Endoscopic endonasal resection of sinonasal and anterior skull base schwannomas Danielle M. Blake b, Qasim Husain b, Vivek V. Kanumuri b, Peter F. Svider b, Jean Anderson Eloy a,b,c, James K. Liu a,b,c,⇑ a b c
Department of Neurological Surgery, Rutgers New Jersey Medical School, 90 Bergen Street, Suite 8100, Newark, NJ 07103, USA Department of Otolaryngology – Head and Neck Surgery, Rutgers New Jersey Medical School, Newark, NJ, USA Center for Skull Base and Pituitary Surgery, Neurological Institute of New Jersey, Rutgers New Jersey Medical School, Newark, NJ, USA
a r t i c l e
i n f o
Article history: Received 4 March 2014 Accepted 9 March 2014 Available online xxxx Keywords: Anterior skull base tumor Endoscopic endonasal approach Endoscopic skull base surgery Olfactory groove Schwannoma Sinonasal tumors Subfrontal
a b s t r a c t Sinonasal and anterior skull base (ASB) schwannomas are rare entities. The majority of these lesions are found within the sinonasal tract, although some have intracranial extension via invasion of the ASB. Often, these tumors can be confused for other entities, especially olfactory groove meningiomas and esthesioneuroblastomas in the olfactory groove region, and juvenile nasopharyngeal angiofibromas in the infratemporal fossa. We present a single institutional series of four patients with sinonasal and ASB schwannomas that were resected purely via an endoscopic endonasal approach. A retrospective chart review was performed to identify individuals with sinonasal and/or ASB schwannomas. Demographic data, presenting symptoms, imaging, treatment, and follow-up were recorded. Two male and two female patients were included in this study. The average age was 53.5 years (range, 21 to 71). The most common presenting symptoms were facial pain and hyposmia. All tumors were treated with endoscopic endonasal resection. One patient had intradural intracranial extension and required an extended endoscopic endonasal transcribriform approach with ASB resection, while another case involving the infratemporal fossa was treated with an extended endoscopic endonasal transpterygoid approach to this region. There were no major complications at an average follow-up of 9 months (range, 0 to 16). Sinonasal and ASB schwannomas are rare entities, with often nondescript symptoms and can often mimic other types of skull base tumors. Total resection via a purely endoscopic endonasal approach can be considered for these rare neoplasms. Ó 2014 Elsevier Ltd. All rights reserved.
1. Introduction Schwannomas, also known as neurilemmomas, are benign and slow growing tumors arising from Schwann cells in the peripheral nervous system [1–3]. The neurosurgical literature has described these tumors as predominantly intracranial in location, with the ability to extend into the paranasal sinuses [4–12]. Within the head and neck region, however, only about 3–4% arise from the sinonasal tract [2,13]. Sinonasal schwannomas are believed to arise from Schwann cells associated with the ophthalmic and maxillary branches of the trigeminal nerve, as well as autonomic nerves to the septal vessels and mucosa, and therefore, these tumors can be found in the pterygopalatine fossa (PPF) and the anterior skull base (ASB) [1].
These tumors have a tendency to present with seizures [14], increased intracranial pressure [15], and frontal lobe dysfunction [16] when located intracranially, and as nasal obstruction, epistaxis, and pain when located in the sinonasal tract [17]. Due to the rare nature of this tumor, there is little literature evaluating the efficacy of surgical approaches for its treatment. In the past, lateral rhinotomy approaches, midfacial degloving, and bifrontal craniotomy were required for the effective removal of this neoplasm. With the increase in utilization of the endoscope, and its role in tumor extirpation, we describe our experience with purely endoscopic endonasal surgery for treatment of sinonasal and ASB schwannomas.
2. Materials and methods ⇑ Corresponding author. Tel.: +1 973 972 2906; fax +1 973 972 2333. E-mail address: [email protected] (J.K. Liu).
A retrospective review was performed between January 2011 and December 2012 in order to identify patients with the diagnosis
http://dx.doi.org/10.1016/j.jocn.2014.03.007 0967-5868/Ó 2014 Elsevier Ltd. All rights reserved.
Please cite this article in press as: Blake DM et al. Endoscopic endonasal resection of sinonasal and anterior skull base schwannomas. J Clin Neurosci (2014), http://dx.doi.org/10.1016/j.jocn.2014.03.007
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D.M. Blake et al. / Journal of Clinical Neuroscience xxx (2014) xxx–xxx
of schwannoma at our tertiary care referral center. Patients were included if the location of the schwannoma was within the sinonasal tract or ventral skull base. Patients with classic trigeminal schwannomas (those involving the Gasserian ganglion and Meckel’s cave) were excluded from this analysis since these tumors are clearly distinct from sinonasal and ASB schwannomas. A total of four patients with sinonasal and ASB schwannomas were identified. A summary of the data, including patient demographics, location, presentation, treatment, complications, outcome, and follow-up, are included in Table 1. The protocol for this study was reviewed and approved by the Institutional Review Board of Rutgers New Jersey Medical School. 3. Results 3.1. Patient 1 A 71-year-old woman with a history of asthma presented with a right nasal mass. She reported right-sided epistaxis beginning 3– 4 years before presentation that had progressed into significant nasal obstruction. Her family history was noncontributory, and her review of systems was otherwise unremarkable. On anterior
rhinoscopic examination, a right nasal mass was appreciated. Nasal endoscopy revealed deviation of the nasal septum toward the left side with a large fleshy mass in the inferior aspect of the right nasal cavity (Fig. 1A). The rest of her head and neck examination was unremarkable. CT scan showed a 3–4 cm expansile mass in the right nasal cavity suggestive of a neoplasm and deviation of the nasal septum toward the left. MRI with gadolinium enhancement showed a heterogeneous, enhancing, complex 4 cm mass in the epicenter of the right nasal cavity suspicious for a neoplasm (Fig. 1B, C). The patient underwent endoscopic endonasal resection of the right nasal mass. Intraoperative frozen sections suggested a benign lesion. The final pathologic diagnosis was consistent with a right nasal schwannoma. The patient did well postoperatively and remained disease free at 15 months without recurrence on nasal endoscopy (Fig. 1D). 3.2. Patient 2 A 62-year-old man with renal cell carcinoma presented with hyposmia and hypogeusia for 3–4 months. CT scan with contrast of the paranasal sinuses showed a large hyperdense sinonasal
Table 1 Clinical data of the four patients with sinonasal and anterior skull base schwannomas Patient
Age
Sex
Location of tumor/ Attachment
Clinical presentation
Treatment approach
Complications
Follow-up
1
71
F
Nasal cavity
Endoscopic endonasal approach
None
2
62
M
21
M
4
60
F
Nasal septum
Extended endoscopic endonasal transcribriform approach Extended endoscopic endonasal transpterygoid approach Endoscopic endonasal approach
None
3
Ethmoids, cribriform plate/ACF PPF, ITF
Facial pain, hyposmia, sneezing, eye irritation, cough Hyposmia, hypogeusia
NED, 15 months NED, 5 months NED, 16 months None
Syncope, prior episodes of epistaxis Facial pain
Mild residual numbness in V2 distribution None
ACF = anterior cranial fossa, F = female, ITF = infratemporal fossa, M = male, NED = no evidence of disease, PPF = pterygopalatine fossa.
Fig. 1. Patient 1. (A) Preoperative endoscopic view showing a right nasal schwannoma (black asterisk) prior to resection. Axial (B) and coronal (C) preoperative T1-weighted gadolinium enhanced MRI showing the schwannoma (white asterisk). (D) Delayed postoperative endoscopic endonasal view of the right nasal cavity showing absence of tumor after endoscopic resection.
Please cite this article in press as: Blake DM et al. Endoscopic endonasal resection of sinonasal and anterior skull base schwannomas. J Clin Neurosci (2014), http://dx.doi.org/10.1016/j.jocn.2014.03.007
D.M. Blake et al. / Journal of Clinical Neuroscience xxx (2014) xxx–xxx
lesion with bony destruction of the ASB and intracranial extension (Fig. 2A). MRI revealed a 4 3.3 3.8 cm sinonasal/ASB mass eroding the cribriform plate with intracranial extension (Fig. 2B). A biopsy taken at an outside hospital was suggestive of a spindle cell neoplasm consistent with peripheral nerve sheath tumor. The patient underwent complete resection of the lesion via an extended endoscopic endonasal transcribriform approach (Fig. 2C). The operative techniques and surgical nuances of this approach are demonstrated in an online surgical video [17], however, the details of the case and subsequent follow-up have not been previously published. Reconstruction was performed using a triple layer closure technique (fascia lata, acellular dermal allograft, and vascularized nasoseptal flap) [18–20]. Frozen sections of the lesion were suspicious for spindle cell neoplasm of mesenchymal origin. Final pathologic examination confirmed the diagnosis of schwannoma. The patient did well postoperatively without any cerebrospinal fluid leak or neurologic deficits. MRI at 5 months showed no evidence of disease (Fig. 2D). 3.3. Patient 3 A 21-year-old man presented with a sinonasal mass discovered incidentally during a work-up for recent syncopal episodes. The patient denied any rhinologic symptoms except for a remote history of twice-daily epistaxis. His head and neck examination, including anterior rhinoscopy, was unremarkable. Nasal endoscopy revealed significant fullness in the area between the tail of the middle and inferior turbinates on the left. CT scan and MRI obtained during the syncope workup showed a large enhancing mass expanding the left PPF and extending into the infratemporal fossa (ITF) with significant displacement of the posterior maxillary wall anteriorly (Fig. 3A, B). The lesion also abutted the inferior aspect of the orbit and temporal lobe of the brain but appeared to be extradural.
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The patient underwent an extended endoscopic endonasal transpterygoid approach to the PPF and ITF that included an endoscopic modified medial maxillectomy with resection of the mass (Fig. 3C, D). Gross total resection was achieved (Fig. 3E, F). Pathologic examination of the specimen revealed a schwannoma. Intraoperatively, the tumor appeared to be possibly arising from the vidian nerve, expanding the PPF. The patient did well postoperatively, with only mild residual numbness in the left cheek at 3 months that continued to improve at 1 year. CT scan at 14 months postoperatively showed no evidence of recurrence. 3.4. Patient 4 A 60-year-old woman with a 40 pack-year smoking history presented with a right painful nasal mass that was first discovered 10 years before presentation. The patient reported progressively increasing intermittent sharp nasal pain with radiation across the right midface and occasional light right-sided epistaxis. Her medical history was significant for diabetes, hypertension, and arthritis, and her family history and review of systems were unremarkable. Anterior rhinoscopic examination revealed patent nasal cavities bilaterally with a small papillomatous-appearing lesion over the right nasal septum. The rest of her head and neck examination was unremarkable. The patient underwent uneventful endoscopic endonasal resection of the lesion. A pedunculated anterior septal mass measuring 0.6 0.4 0.3 cm was removed. Pathologic examination was consistent with a sinonasal schwannoma. Postoperatively, the patient was without complaints and disease free. 4. Discussion Schwannomas are benign nerve sheath tumors that arise from the Schwann cells of the peripheral nervous system. Between 25–45% of schwannomas occur in the head and neck region with
Fig. 2. Patient 2. (A) Sagittal CT scan with contrast showing a large hyperdense sinonasal lesion with bony destruction of the anterior skull base and intracranial extension. (B) Coronal T1-weighted gadolinium enhanced MRI showing the schwannoma (white asterisk) involving the paranasal sinuses and anterior skull base. (C) Intraoperative endoscopic view showing the schwannoma (black asterisk) during resection. (D) Postoperative sagittal T1-weighted gadolinium enhanced MRI demonstrating complete tumor removal after an endoscopic endonasal transcribriform approach.
Please cite this article in press as: Blake DM et al. Endoscopic endonasal resection of sinonasal and anterior skull base schwannomas. J Clin Neurosci (2014), http://dx.doi.org/10.1016/j.jocn.2014.03.007
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D.M. Blake et al. / Journal of Clinical Neuroscience xxx (2014) xxx–xxx
Fig. 3. Patient 3. Preoperative axial (A) and coronal (B) T1-weighted gadolinium enhanced MRI showing a large schwannoma (white asterisk) involving the pterygopalatine fossa and infratemporal fossa. (C, D) Endoscopic endonasal intraoperative view of the tumor during resection. Axial (E) and coronal (F) postoperative CT scans showing complete removal without evidence of residual or recurrent tumor.
approximately 3–4% arising in the sinonasal region [2]. Schwannomas of the ASB are exceedingly rare and are often described as subfrontal or olfactory groove schwannomas [12,21,22]. Rarely, they may occur in the intracranial space and extend into the paranasal sinuses or originate in the paranasal sinuses and extend to the cribriform plate with possible intradural invasion [4–12,23–25]. The unusual locations described in the present report include the nasal cavity, nasal septum, cribriform plate, PPF and ITF. In a recent systematic analysis of patients with ASB and sinonasal schwannomas, 46.8% were exclusively sinonasal, 31.9% were exclusively intracranial, 12.8% were primarily intracranial with extension into the paranasal sinuses, and 8.5% were primarily sinonasal with intracranial extension [3]. Sinonasal and ASB schwannomas are defined histologically by the presence of Antoni type A and Antoni type B areas, as well as Verocay bodies. Immunohistochemical staining is important for accurate diagnosis. Schwannomas characteristically stain positive for S-100 which is one of the most important hallmarks for histopathologic diagnosis. The immunohistochemical profile may also reveal positive staining for neuron-specific enolase (NSE) and vimentin and negative staining for glial fibrillary acidic protein (GFAP), epithelial membrane antigen (EMA), and CD56 [26]. The
histopathology was confirmed in the present series with all patient samples staining positive for S-100. When located in the cribriform region and ASB, patients typically present with nasal obstruction [3], headache [3], and epistaxis, although facial pain and hyposmia were the most common presenting symptoms in the present series. When they involve the cribriform plate and ASB, they often mimic other tumors such as olfactory groove meningiomas (which exhibit similar patterns of location, calcification, and contrast enhancement) [27], hemangiopericytomas [28,29], and esthesioneuroblastomas [14,22,26,30]. When schwannomas arise primarily in the paranasal sinuses, they can often mimic sinonasal malignancies, such as adenoid cystic carcinoma, squamous cell carcinoma, esthesioneuroblastoma, and sinonasal melanoma. The tumor that involved the PPF and ITF in the young male with prior episodes of epistaxis (Patient 3) mimicked a juvenile nasopharyngeal angiofibroma. Primary treatment for sinonasal and ASB schwannomas is surgical resection, with the goal of curative gross total resection if safely feasible [3,17]. The most common approach reported for ASB schwannomas is bifrontal craniotomy, which allows midline access to the ASB, cribriform plate and paranasal sinuses [5,7–11,25,26,30– 34]. However, with the advent of endoscopic skull base surgery,
Please cite this article in press as: Blake DM et al. Endoscopic endonasal resection of sinonasal and anterior skull base schwannomas. J Clin Neurosci (2014), http://dx.doi.org/10.1016/j.jocn.2014.03.007
D.M. Blake et al. / Journal of Clinical Neuroscience xxx (2014) xxx–xxx
the surgical landscape has evolved to now include endoscopic endonasal approaches as well as endoscopic-assisted approaches to provide access to these tumors [4,17]. These approaches offer superior visualization while avoiding the need for craniotomy, brain retraction, and potentially disfiguring transfacial incisions [1,17,35]. Endoscopic endonasal approaches are particularly effective for schwannomas arising in the paranasal sinuses as well as those that extend into the midline ventral skull base [4]. In the present series, a purely endoscopic endonasal approach was used in all four patients. Two cases were primarily sinonasal, one case involved the cribriform and ASB, and one case was primarily located in the PPF and ITF. Patient 2 had an olfactory groove and subfrontal schwannoma involving the ASB that required an extended endoscopic endonasal transcribriform approach. In this case, intradural involvement was present and a large skull base dural defect was repaired successfully with a triple-layer reconstruction (autologous fascia lata, acellular dermal allograft, and a vascularized pedicled nasoseptal flap) that we have previously described for endoscopic reconstruction of transcribriform defects [18]. Postoperatively, there was no cerebrospinal fluid leak. Patient 3 had a schwannoma, most likely arising from the vidian nerve, involving the PPF and ITF that required an extended endoscopic endonasal transpterygoid approach that included an endoscopic modified medial maxillectomy. This approach allowed lateral access to the ITF without requiring a transfacial approach. In all four presented patients gross total resection was achieved, and there were no significant postoperative complications and no evidence of recurrent disease at follow-up. It is important to consider an open approach, such as a bifrontal craniotomy, or perhaps even a combined transcranial and endonasal approach (cranionasal approach) if there is significant intracranial involvement, lateral tumor extension, or intimate involvement or encasement of major vessels [10]. Because of the rarity of this tumor, the majority of cases reported in the literature exist only as case reports. Our series of four patients, albeit a small retrospective series, represents the largest reported series from a single institution to our knowledge. Suh et al. [1] reported a series of seven patients with sinonasal schwannomas collected from two institutions, of whom six were treated endoscopically. One patient underwent a lateral rhinotomy for tumor resection. These tumors were located in the ethmoid sinuses (three patients), nasal cavity (two patients), and PPF (two patients), and none in this cohort transgressed the ASB and involved the intracranial cavity. In our experience, sinonasal and ASB schwannomas can be successfully resected through a purely endoscopic endonasal approach. Tumor size and location and the experience of the multi-disciplinary endoscopic skull base team plays an important role in patient selection. For those tumors that invade intradurally, one should be prepared to perform a multi-layered skull base reconstruction with a vascularized nasoseptal flap. Although this study is limited by its retrospective nature and small sample size, the rarity of this lesion and the dearth of information in the literature on this subject should make this series valuable to endoscopic skull base surgeons. 5. Conclusion Sinonasal and ASB schwannomas are rare lesions that often mimic other skull base neoplasms. In select patients, a gross total resection can be achieved safely using a purely endoscopic endonasal approach. Conflicts of Interest/Disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication.
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References [1] Suh JD, Ramakrishnan VR, Zhang PJ, et al. Diagnosis and endoscopic management of sinonasal schwannomas. ORL J Otorhinolaryngol Relat Spec 2011;73:308–12. [2] Younis RT, Gross CW, Lazar RH. Schwannomas of the paranasal sinuses. Case report and clinicopathologic analysis. Arch Otolaryngol Head Neck Surg 1991;117:677–80. [3] Sunaryo PL, Svider PF, Husain Q, et al. Schwannomas of the sinonasal tract and anterior skull base: a systematic review of 94 cases. Am J Rhinol Allergy 2014;28:39–49. [4] Kanaan HA, Gardner PA, Yeaney G, et al. Expanded endoscopic endonasal resection of an olfactory schwannoma. J Neurosurg Pediatr 2008;2:261–5. [5] Bezirciog˘lu H, Sucu HK, Rezanko T, et al. Nasal-subfrontal giant schwannoma. Turk Neurosurg 2008;18:412–4. [6] Carron JD, Singh RV, Karakla DW, et al. Solitary schwannoma of the olfactory groove: case report and review of the literature. Skull Base 2002;12:163–6. [7] Bando K, Obayashi M, Tsuneharu F. A case of subfrontal schwannoma. No Shinkei Geka 1992;20:1189–94. [8] Choi YS, Sung KS, Song YJ, et al. Olfactory schwannoma-case report. J Korean Neurosurg Soc 2009;45:103–6. [9] Harada T, Kawauchi M, Watanabe M, et al. Subfrontal schwannoma – case report. Neurol Med Chir (Tokyo) 1992;32:957–60. [10] Zheng K, Jiang S, Xu JG, et al. Giant cranionasal schwannoma. J Clin Neurosci 2010;17:520–2. [11] de Souza HL, Ramos AM, Ramos CC, et al. Olfactory groove schwannoma: case report. Arq Neuropsiquiatr 2003;61:125–8. [12] Mirone G, Natale M, Scuotto A, et al. Solitary olfactory groove schwannoma. J Clin Neurosci 2009;16:454–6. [13] Buob D, Wacrenier A, Chevalier D, et al. Schwannoma of the sinonasal tract: a clinicopathologic and immunohistochemical study of 5 cases. Arch Pathol Lab Med 2003;127:1196–9. [14] Ulrich J, Lévy A, Pfister C. Schwannoma of the olfactory groove. Case report and review of previous cases. Acta Neurochir (Wien) 1978;40:315–21. [15] Husain M, Mishra UK, Newton G, et al. Isolated olfactory groove neurilemmoma. Surg Neurol 1992;37:115–7. [16] Vassilouthis J, Richardson AE. Subfrontal schwannoma. Report of a case. Acta Neurochir (Wien) 1980;53:259–66. [17] Liu JK, Eloy JA. Expanded endoscopic endonasal transcribriform approach for resection of anterior skull base olfactory schwannoma. J Neurosurg 2012;32:E3. [18] Eloy JA, Patel SK, Shukla PA, et al. Triple-layer reconstruction technique for large cribriform defects after endoscopic endonasal resection of anterior skull base tumors. Int Forum Allergy Rhinol 2013;3:204–11. [19] Liu JK, Schmidt RF, Choudhry OJ, et al. Surgical nuances for nasoseptal flap reconstruction of cranial base defects with high-flow cerebrospinal fluid leaks after endoscopic skull base surgery. Neurosurg Focus 2012;32:E7. [20] Eloy JA, Shukla PA, Choudhry OJ, et al. Assessment of frontal lobe sagging after endoscopic endonasal transcribriform resection of anterior skull base tumors: is rigid structural reconstruction of the cranial base defect necessary? Laryngoscope 2012;122:2652–7. [21] Adachi K, Yoshida K, Miwa T, et al. Olfactory schwannoma. Acta Neurochir (Wien) 2007;149:605–10 [discussion 10-1]. [22] Li YP, Jiang S, Zhou PZ, et al. Solitary olfactory schwannoma without olfactory dysfunction: a new case report and literature review. Neurol Sci 2012;33:137–42. [23] Ulu EM, Cakmak O, Dönmez FY, et al. Sinonasal schwannoma of the middle turbinate. Diagn Interv Radiol 2010;16:129–31. [24] Sharma R, Tyagi I, Banerjee D, et al. Nasoethmoid schwannoma with intracranial extension. Case report and review of literature. Neurosurg Rev 1998;21:58–61. [25] Perzin KH, Panyu H, Wechter S. Nonepithelial tumors of the nasal cavity, paranasal sinuses and nasopharynx. A clinicopathologic study. XII: Schwann cell tumors (neurilemoma, neurofibroma, malignant schwannoma). Cancer 1982;50:2193–202. [26] Yako K, Morita A, Ueki K, et al. Subfrontal schwannoma. Acta Neurochir (Wien) 2005;147:655–7 [discussion 657-8]. [27] Liu JK, Christiano LD, Patel SK, et al. Surgical nuances for removal of olfactory groove meningiomas using the endoscopic endonasal transcribriform approach. Neurosurg Focus 2011;30:E3. [28] Dahodwala MQ, Husain Q, Kanumuri VV, et al. Management of sinonasal hemangiopericytomas: a systematic review. Int Forum Allergy Rhinol 2013;3:581–7. [29] Tessema B, Eloy JA, Folbe AJ, et al. Endoscopic management of sinonasal hemangiopericytoma. Otolaryngol Head Neck Surg 2012;146:483–6. [30] Amador AR, Santonja C, Del Pozo JM, et al. Olfactory schwannoma. Eur Radiol 2002;12:742–4. [31] Komoribayashi N, Arai H, Kojo T, et al. Subfrontal schwannoma: case report. No Shinkei Geka 2005;33:601–5. [32] Martínez-Soto L, Alfaro-Baca R, Torrecilla-Sardón MV, et al. A new case of ‘‘olfactory schwannoma’’; presentation and literature review. Neurocirugia (Astur) 2009;20:294–7. [33] Mey KH, Buchwald C, Daugaard S, et al. Sinonasal schwannoma–a clinicopathological analysis of five rare cases. Rhinology 2006;44:46–52. [34] Praharaj SS, Vajramani GV, Santosh V, et al. Solitary olfactory groove schwannoma: case report with review of the literature. Clin Neurol Neurosurg 1999;101:26–8. [35] Adam SI, Vining EM. Endoscopic resection of an anterior skull-base schwannoma. Int Forum of Allergy Rhinol 2012;2:264–8.
Please cite this article in press as: Blake DM et al. Endoscopic endonasal resection of sinonasal and anterior skull base schwannomas. J Clin Neurosci (2014), http://dx.doi.org/10.1016/j.jocn.2014.03.007
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Clinical Study
Endoscopic endonasal resection of sinonasal and anterior skull base schwannomas Danielle M. Blake b, Qasim Husain b, Vivek V. Kanumuri b, Peter F. Svider b, Jean Anderson Eloy a,b,c, James K. Liu a,b,c,⇑ a b c
Department of Neurological Surgery, Rutgers New Jersey Medical School, 90 Bergen Street, Suite 8100, Newark, NJ 07103, USA Department of Otolaryngology – Head and Neck Surgery, Rutgers New Jersey Medical School, Newark, NJ, USA Center for Skull Base and Pituitary Surgery, Neurological Institute of New Jersey, Rutgers New Jersey Medical School, Newark, NJ, USA
a r t i c l e
i n f o
Article history: Received 4 March 2014 Accepted 9 March 2014 Available online xxxx Keywords: Anterior skull base tumor Endoscopic endonasal approach Endoscopic skull base surgery Olfactory groove Schwannoma Sinonasal tumors Subfrontal
a b s t r a c t Sinonasal and anterior skull base (ASB) schwannomas are rare entities. The majority of these lesions are found within the sinonasal tract, although some have intracranial extension via invasion of the ASB. Often, these tumors can be confused for other entities, especially olfactory groove meningiomas and esthesioneuroblastomas in the olfactory groove region, and juvenile nasopharyngeal angiofibromas in the infratemporal fossa. We present a single institutional series of four patients with sinonasal and ASB schwannomas that were resected purely via an endoscopic endonasal approach. A retrospective chart review was performed to identify individuals with sinonasal and/or ASB schwannomas. Demographic data, presenting symptoms, imaging, treatment, and follow-up were recorded. Two male and two female patients were included in this study. The average age was 53.5 years (range, 21 to 71). The most common presenting symptoms were facial pain and hyposmia. All tumors were treated with endoscopic endonasal resection. One patient had intradural intracranial extension and required an extended endoscopic endonasal transcribriform approach with ASB resection, while another case involving the infratemporal fossa was treated with an extended endoscopic endonasal transpterygoid approach to this region. There were no major complications at an average follow-up of 9 months (range, 0 to 16). Sinonasal and ASB schwannomas are rare entities, with often nondescript symptoms and can often mimic other types of skull base tumors. Total resection via a purely endoscopic endonasal approach can be considered for these rare neoplasms. Ó 2014 Elsevier Ltd. All rights reserved.
1. Introduction Schwannomas, also known as neurilemmomas, are benign and slow growing tumors arising from Schwann cells in the peripheral nervous system [1–3]. The neurosurgical literature has described these tumors as predominantly intracranial in location, with the ability to extend into the paranasal sinuses [4–12]. Within the head and neck region, however, only about 3–4% arise from the sinonasal tract [2,13]. Sinonasal schwannomas are believed to arise from Schwann cells associated with the ophthalmic and maxillary branches of the trigeminal nerve, as well as autonomic nerves to the septal vessels and mucosa, and therefore, these tumors can be found in the pterygopalatine fossa (PPF) and the anterior skull base (ASB) [1].
These tumors have a tendency to present with seizures [14], increased intracranial pressure [15], and frontal lobe dysfunction [16] when located intracranially, and as nasal obstruction, epistaxis, and pain when located in the sinonasal tract [17]. Due to the rare nature of this tumor, there is little literature evaluating the efficacy of surgical approaches for its treatment. In the past, lateral rhinotomy approaches, midfacial degloving, and bifrontal craniotomy were required for the effective removal of this neoplasm. With the increase in utilization of the endoscope, and its role in tumor extirpation, we describe our experience with purely endoscopic endonasal surgery for treatment of sinonasal and ASB schwannomas.
2. Materials and methods ⇑ Corresponding author. Tel.: +1 973 972 2906; fax +1 973 972 2333. E-mail address: [email protected] (J.K. Liu).
A retrospective review was performed between January 2011 and December 2012 in order to identify patients with the diagnosis
http://dx.doi.org/10.1016/j.jocn.2014.03.007 0967-5868/Ó 2014 Elsevier Ltd. All rights reserved.
Please cite this article in press as: Blake DM et al. Endoscopic endonasal resection of sinonasal and anterior skull base schwannomas. J Clin Neurosci (2014), http://dx.doi.org/10.1016/j.jocn.2014.03.007
2
D.M. Blake et al. / Journal of Clinical Neuroscience xxx (2014) xxx–xxx
of schwannoma at our tertiary care referral center. Patients were included if the location of the schwannoma was within the sinonasal tract or ventral skull base. Patients with classic trigeminal schwannomas (those involving the Gasserian ganglion and Meckel’s cave) were excluded from this analysis since these tumors are clearly distinct from sinonasal and ASB schwannomas. A total of four patients with sinonasal and ASB schwannomas were identified. A summary of the data, including patient demographics, location, presentation, treatment, complications, outcome, and follow-up, are included in Table 1. The protocol for this study was reviewed and approved by the Institutional Review Board of Rutgers New Jersey Medical School. 3. Results 3.1. Patient 1 A 71-year-old woman with a history of asthma presented with a right nasal mass. She reported right-sided epistaxis beginning 3– 4 years before presentation that had progressed into significant nasal obstruction. Her family history was noncontributory, and her review of systems was otherwise unremarkable. On anterior
rhinoscopic examination, a right nasal mass was appreciated. Nasal endoscopy revealed deviation of the nasal septum toward the left side with a large fleshy mass in the inferior aspect of the right nasal cavity (Fig. 1A). The rest of her head and neck examination was unremarkable. CT scan showed a 3–4 cm expansile mass in the right nasal cavity suggestive of a neoplasm and deviation of the nasal septum toward the left. MRI with gadolinium enhancement showed a heterogeneous, enhancing, complex 4 cm mass in the epicenter of the right nasal cavity suspicious for a neoplasm (Fig. 1B, C). The patient underwent endoscopic endonasal resection of the right nasal mass. Intraoperative frozen sections suggested a benign lesion. The final pathologic diagnosis was consistent with a right nasal schwannoma. The patient did well postoperatively and remained disease free at 15 months without recurrence on nasal endoscopy (Fig. 1D). 3.2. Patient 2 A 62-year-old man with renal cell carcinoma presented with hyposmia and hypogeusia for 3–4 months. CT scan with contrast of the paranasal sinuses showed a large hyperdense sinonasal
Table 1 Clinical data of the four patients with sinonasal and anterior skull base schwannomas Patient
Age
Sex
Location of tumor/ Attachment
Clinical presentation
Treatment approach
Complications
Follow-up
1
71
F
Nasal cavity
Endoscopic endonasal approach
None
2
62
M
21
M
4
60
F
Nasal septum
Extended endoscopic endonasal transcribriform approach Extended endoscopic endonasal transpterygoid approach Endoscopic endonasal approach
None
3
Ethmoids, cribriform plate/ACF PPF, ITF
Facial pain, hyposmia, sneezing, eye irritation, cough Hyposmia, hypogeusia
NED, 15 months NED, 5 months NED, 16 months None
Syncope, prior episodes of epistaxis Facial pain
Mild residual numbness in V2 distribution None
ACF = anterior cranial fossa, F = female, ITF = infratemporal fossa, M = male, NED = no evidence of disease, PPF = pterygopalatine fossa.
Fig. 1. Patient 1. (A) Preoperative endoscopic view showing a right nasal schwannoma (black asterisk) prior to resection. Axial (B) and coronal (C) preoperative T1-weighted gadolinium enhanced MRI showing the schwannoma (white asterisk). (D) Delayed postoperative endoscopic endonasal view of the right nasal cavity showing absence of tumor after endoscopic resection.
Please cite this article in press as: Blake DM et al. Endoscopic endonasal resection of sinonasal and anterior skull base schwannomas. J Clin Neurosci (2014), http://dx.doi.org/10.1016/j.jocn.2014.03.007
D.M. Blake et al. / Journal of Clinical Neuroscience xxx (2014) xxx–xxx
lesion with bony destruction of the ASB and intracranial extension (Fig. 2A). MRI revealed a 4 3.3 3.8 cm sinonasal/ASB mass eroding the cribriform plate with intracranial extension (Fig. 2B). A biopsy taken at an outside hospital was suggestive of a spindle cell neoplasm consistent with peripheral nerve sheath tumor. The patient underwent complete resection of the lesion via an extended endoscopic endonasal transcribriform approach (Fig. 2C). The operative techniques and surgical nuances of this approach are demonstrated in an online surgical video [17], however, the details of the case and subsequent follow-up have not been previously published. Reconstruction was performed using a triple layer closure technique (fascia lata, acellular dermal allograft, and vascularized nasoseptal flap) [18–20]. Frozen sections of the lesion were suspicious for spindle cell neoplasm of mesenchymal origin. Final pathologic examination confirmed the diagnosis of schwannoma. The patient did well postoperatively without any cerebrospinal fluid leak or neurologic deficits. MRI at 5 months showed no evidence of disease (Fig. 2D). 3.3. Patient 3 A 21-year-old man presented with a sinonasal mass discovered incidentally during a work-up for recent syncopal episodes. The patient denied any rhinologic symptoms except for a remote history of twice-daily epistaxis. His head and neck examination, including anterior rhinoscopy, was unremarkable. Nasal endoscopy revealed significant fullness in the area between the tail of the middle and inferior turbinates on the left. CT scan and MRI obtained during the syncope workup showed a large enhancing mass expanding the left PPF and extending into the infratemporal fossa (ITF) with significant displacement of the posterior maxillary wall anteriorly (Fig. 3A, B). The lesion also abutted the inferior aspect of the orbit and temporal lobe of the brain but appeared to be extradural.
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The patient underwent an extended endoscopic endonasal transpterygoid approach to the PPF and ITF that included an endoscopic modified medial maxillectomy with resection of the mass (Fig. 3C, D). Gross total resection was achieved (Fig. 3E, F). Pathologic examination of the specimen revealed a schwannoma. Intraoperatively, the tumor appeared to be possibly arising from the vidian nerve, expanding the PPF. The patient did well postoperatively, with only mild residual numbness in the left cheek at 3 months that continued to improve at 1 year. CT scan at 14 months postoperatively showed no evidence of recurrence. 3.4. Patient 4 A 60-year-old woman with a 40 pack-year smoking history presented with a right painful nasal mass that was first discovered 10 years before presentation. The patient reported progressively increasing intermittent sharp nasal pain with radiation across the right midface and occasional light right-sided epistaxis. Her medical history was significant for diabetes, hypertension, and arthritis, and her family history and review of systems were unremarkable. Anterior rhinoscopic examination revealed patent nasal cavities bilaterally with a small papillomatous-appearing lesion over the right nasal septum. The rest of her head and neck examination was unremarkable. The patient underwent uneventful endoscopic endonasal resection of the lesion. A pedunculated anterior septal mass measuring 0.6 0.4 0.3 cm was removed. Pathologic examination was consistent with a sinonasal schwannoma. Postoperatively, the patient was without complaints and disease free. 4. Discussion Schwannomas are benign nerve sheath tumors that arise from the Schwann cells of the peripheral nervous system. Between 25–45% of schwannomas occur in the head and neck region with
Fig. 2. Patient 2. (A) Sagittal CT scan with contrast showing a large hyperdense sinonasal lesion with bony destruction of the anterior skull base and intracranial extension. (B) Coronal T1-weighted gadolinium enhanced MRI showing the schwannoma (white asterisk) involving the paranasal sinuses and anterior skull base. (C) Intraoperative endoscopic view showing the schwannoma (black asterisk) during resection. (D) Postoperative sagittal T1-weighted gadolinium enhanced MRI demonstrating complete tumor removal after an endoscopic endonasal transcribriform approach.
Please cite this article in press as: Blake DM et al. Endoscopic endonasal resection of sinonasal and anterior skull base schwannomas. J Clin Neurosci (2014), http://dx.doi.org/10.1016/j.jocn.2014.03.007
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D.M. Blake et al. / Journal of Clinical Neuroscience xxx (2014) xxx–xxx
Fig. 3. Patient 3. Preoperative axial (A) and coronal (B) T1-weighted gadolinium enhanced MRI showing a large schwannoma (white asterisk) involving the pterygopalatine fossa and infratemporal fossa. (C, D) Endoscopic endonasal intraoperative view of the tumor during resection. Axial (E) and coronal (F) postoperative CT scans showing complete removal without evidence of residual or recurrent tumor.
approximately 3–4% arising in the sinonasal region [2]. Schwannomas of the ASB are exceedingly rare and are often described as subfrontal or olfactory groove schwannomas [12,21,22]. Rarely, they may occur in the intracranial space and extend into the paranasal sinuses or originate in the paranasal sinuses and extend to the cribriform plate with possible intradural invasion [4–12,23–25]. The unusual locations described in the present report include the nasal cavity, nasal septum, cribriform plate, PPF and ITF. In a recent systematic analysis of patients with ASB and sinonasal schwannomas, 46.8% were exclusively sinonasal, 31.9% were exclusively intracranial, 12.8% were primarily intracranial with extension into the paranasal sinuses, and 8.5% were primarily sinonasal with intracranial extension [3]. Sinonasal and ASB schwannomas are defined histologically by the presence of Antoni type A and Antoni type B areas, as well as Verocay bodies. Immunohistochemical staining is important for accurate diagnosis. Schwannomas characteristically stain positive for S-100 which is one of the most important hallmarks for histopathologic diagnosis. The immunohistochemical profile may also reveal positive staining for neuron-specific enolase (NSE) and vimentin and negative staining for glial fibrillary acidic protein (GFAP), epithelial membrane antigen (EMA), and CD56 [26]. The
histopathology was confirmed in the present series with all patient samples staining positive for S-100. When located in the cribriform region and ASB, patients typically present with nasal obstruction [3], headache [3], and epistaxis, although facial pain and hyposmia were the most common presenting symptoms in the present series. When they involve the cribriform plate and ASB, they often mimic other tumors such as olfactory groove meningiomas (which exhibit similar patterns of location, calcification, and contrast enhancement) [27], hemangiopericytomas [28,29], and esthesioneuroblastomas [14,22,26,30]. When schwannomas arise primarily in the paranasal sinuses, they can often mimic sinonasal malignancies, such as adenoid cystic carcinoma, squamous cell carcinoma, esthesioneuroblastoma, and sinonasal melanoma. The tumor that involved the PPF and ITF in the young male with prior episodes of epistaxis (Patient 3) mimicked a juvenile nasopharyngeal angiofibroma. Primary treatment for sinonasal and ASB schwannomas is surgical resection, with the goal of curative gross total resection if safely feasible [3,17]. The most common approach reported for ASB schwannomas is bifrontal craniotomy, which allows midline access to the ASB, cribriform plate and paranasal sinuses [5,7–11,25,26,30– 34]. However, with the advent of endoscopic skull base surgery,
Please cite this article in press as: Blake DM et al. Endoscopic endonasal resection of sinonasal and anterior skull base schwannomas. J Clin Neurosci (2014), http://dx.doi.org/10.1016/j.jocn.2014.03.007
D.M. Blake et al. / Journal of Clinical Neuroscience xxx (2014) xxx–xxx
the surgical landscape has evolved to now include endoscopic endonasal approaches as well as endoscopic-assisted approaches to provide access to these tumors [4,17]. These approaches offer superior visualization while avoiding the need for craniotomy, brain retraction, and potentially disfiguring transfacial incisions [1,17,35]. Endoscopic endonasal approaches are particularly effective for schwannomas arising in the paranasal sinuses as well as those that extend into the midline ventral skull base [4]. In the present series, a purely endoscopic endonasal approach was used in all four patients. Two cases were primarily sinonasal, one case involved the cribriform and ASB, and one case was primarily located in the PPF and ITF. Patient 2 had an olfactory groove and subfrontal schwannoma involving the ASB that required an extended endoscopic endonasal transcribriform approach. In this case, intradural involvement was present and a large skull base dural defect was repaired successfully with a triple-layer reconstruction (autologous fascia lata, acellular dermal allograft, and a vascularized pedicled nasoseptal flap) that we have previously described for endoscopic reconstruction of transcribriform defects [18]. Postoperatively, there was no cerebrospinal fluid leak. Patient 3 had a schwannoma, most likely arising from the vidian nerve, involving the PPF and ITF that required an extended endoscopic endonasal transpterygoid approach that included an endoscopic modified medial maxillectomy. This approach allowed lateral access to the ITF without requiring a transfacial approach. In all four presented patients gross total resection was achieved, and there were no significant postoperative complications and no evidence of recurrent disease at follow-up. It is important to consider an open approach, such as a bifrontal craniotomy, or perhaps even a combined transcranial and endonasal approach (cranionasal approach) if there is significant intracranial involvement, lateral tumor extension, or intimate involvement or encasement of major vessels [10]. Because of the rarity of this tumor, the majority of cases reported in the literature exist only as case reports. Our series of four patients, albeit a small retrospective series, represents the largest reported series from a single institution to our knowledge. Suh et al. [1] reported a series of seven patients with sinonasal schwannomas collected from two institutions, of whom six were treated endoscopically. One patient underwent a lateral rhinotomy for tumor resection. These tumors were located in the ethmoid sinuses (three patients), nasal cavity (two patients), and PPF (two patients), and none in this cohort transgressed the ASB and involved the intracranial cavity. In our experience, sinonasal and ASB schwannomas can be successfully resected through a purely endoscopic endonasal approach. Tumor size and location and the experience of the multi-disciplinary endoscopic skull base team plays an important role in patient selection. For those tumors that invade intradurally, one should be prepared to perform a multi-layered skull base reconstruction with a vascularized nasoseptal flap. Although this study is limited by its retrospective nature and small sample size, the rarity of this lesion and the dearth of information in the literature on this subject should make this series valuable to endoscopic skull base surgeons. 5. Conclusion Sinonasal and ASB schwannomas are rare lesions that often mimic other skull base neoplasms. In select patients, a gross total resection can be achieved safely using a purely endoscopic endonasal approach. Conflicts of Interest/Disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication.
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