- Email: [email protected]
Frontoethmoidal Schwannoma with Exertional Cerebrospinal Fluid Rhinorrhea: Case Report and Review of Literature
Accepted Manuscript Frontoethmoidal Schwannoma with Exertional Cerebrospinal Fluid Rhinorrhea: a Case Report and Review of the Literature Yuichiro Yoneoka, M.D., Ph.D., Katsuhiko Akiyama, M.D., Ph.D., Yasuhiro Seki, M.D., Go Hasegawa, M.D., Ph.D., Akiyoshi Kakita, M.D., Ph.D. PII:
S1878-8750(18)30054-8
DOI:
10.1016/j.wneu.2018.01.015
Reference:
WNEU 7212
To appear in:
World Neurosurgery
Received Date: 19 August 2017 Revised Date:
30 December 2017
Accepted Date: 3 January 2018
Please cite this article as: Yoneoka Y, Akiyama K, Seki Y, Hasegawa G, Kakita A, Frontoethmoidal Schwannoma with Exertional Cerebrospinal Fluid Rhinorrhea: a Case Report and Review of the Literature, World Neurosurgery (2018), doi: 10.1016/j.wneu.2018.01.015. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
1
ACCEPTED MANUSCRIPT
Frontoethmoidal schwannoma with exertional cerebrospinal fluid rhinorrhea: A case report and review of the literature
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Yuichiro Yoneoka, M.D., Ph.D.,1,4 Katsuhiko Akiyama, M.D., Ph.D.,1 Yasuhiro Seki, M.D.,1 Go Hasegawa, M.D., Ph.D.,2 Akiyoshi Kakita, M.D., Ph.D. 3
Department of Neurosurgery, Uonuma Institute of Community Medicine, Niigata University
SC
1.
Medical and Dental Hospital
Department of Pathology, Uonuma Institute of Community Medicine, Niigata University Medical and Dental Hospital
M AN U
2.
Department of Pathology, Brain Research Institute, Niigata University
4.
Department of Neurosurgery, Brain Research Institute, Niigata University
Correspondence: Yuichiro Yoneoka, M.D., Ph.D.
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3.
EP
Department of Neurosurgery, Uonuma Institute of Community Medicine, Niigata University Medical and Dental Hospital
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4132, Urasa, Minami-Uonuma, Niigata, 949-7302, Japan Tel: +81-25-777-3200, Fax: +81-25-777-5030, Email: [email protected]
Key words: frontoethomoidal schwannoma; olfactory groove; exertional cerebrospinal fluid rhinorrhea; anterior skull base
Abbreviations and Acronyms: AFP, alpha-fetoprotein; CSF, cerebrospinal fluid; CT, computed tomography; CA19-9, carbohydrate antigen 19-9; CA125, carbohydrate antigen 125; CEA, carcinoembryonic antigen; olfactory groove, MR, magnetic resonance; SCC, squamous cell carcinoma antigen 1
2
ACCEPTED MANUSCRIPT ABSTRACT
Background: Frontoethmoidal schwannomas are rare. No case manifesting exertional cerebrospinal fluid (CSF)
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rhinorrhea has ever been reported to the best of our knowledge.
Case Description:
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In this report, we describe an extremely rare case of frontoethmoidal schwannoma extending through the olfactory groove (OG) with exertional CSF rhinorrhea as the initial symptom. A
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50-year-old woman was presented to our clinic for frequent nasal discharge on exertion. A postcontrast computed tomographic scan demonstrated heterogeneously enhanced tumor from the anterior cranial fossa to the anterior ethmoid sinus. A gadolinium-enhanced T1-weighted magnetic resonance image revealed a well-defined heterogeneously enhanced tumor situated in the midline anterior cranial fossa and the anterior ethmoid sinus. After the resection, the defect of the right
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anterior skull base was reconstructed with a fascia graft and adipose tissue taken from the abdomen as well as a pedicle periosteum flap. A histological examination revealed the tumor as schwannoma.
Conclusion:
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operation.
EP
Her rhinorrhea completely resolved. She regained sense of smell and taste one month after the
According to previous reports, para/peri-OG schwannomas can be divided into four types: subfrontal; nasoethomoidal; frontoethomoidal; and ethomofrontal. Among them, a frontoethomoidal schwannoma can manifest exertional CSF rhinorrhea as an initial symptom.
(195 words)
2
3
ACCEPTED MANUSCRIPT Highlights
Para/peri-OG (olfactory groove) schwannomas can be divided into four types: subfrontal; nasoethomoidal; frontoethomoidal; and ethomofrontal.
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Among them, a frontoethomoidal schwannoma can manifest exertional CSF rhinorrhea as an initial
EP
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SC
symptom.
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3
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ACCEPTED MANUSCRIPT Introduction The growth of benign tumors does not commonly result in cerebrospinal fluid (CSF) rhinorrhea. In this report, we describe a case of frontoethmoidal schwannoma through the olfactory groove (OG) with exertional cerebrospinal fluid rhinorrhea. To the best of our knowledge, this is
RI PT
the first report of frontoethmoidal schwannoma manifesting exertional CSF rhinorrhea as the initial symptom. Since CSF rhinorrhea is the result of an abnormal communication between the sinonasal tract and the subarachnoid space, consideration of the localization of the presented frontoethmoidal
SC
schwannoma can enable us to trace the extending route of the lesion and to infer the origin of the
M AN U
schwannoma.
Case Description History and Examination
A 50-year-old woman was referred to us for evaluation of a frontobasal tumor compressing
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the right frontal lobe upward. The patient had noted rare rhinorrhea since she was 47 years of age. When she was 48 years old, she suddenly developed nasal congestion and subsequent anosmia.
EP
After that, she suffered from transient memory disturbance and abnormal behavior for two weeks. During the two weeks, she frequently blew her nose and noted associated headaches. Subsequently,
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she received psychiatric therapy and took oral antidepressants over a two-year period. Two months prior to her first visit to our hospital, she noted frequent nasal discharge which was worse on exertion. She had clear and intermittent fluid rhinorrhea from her right nostril when straining, leaning forward, or changing her position. A computed tomographic (CT) scan showed a low-density mass in the right anterior cranial fossa and the anterior ethmoid sinus (Figure 1, A, B). A postcontrast CT scan demonstrated heterogeneously enhanced tumor from the anterior cranial fossa to the anterior ethmoid sinus (Figure 1, C, D). Magnetic resonance (MR) imaging disclosed an extra-axial mass with a longer diameter of 4.3 cm in the right subfrontal region and the anterior ethmoidal region. Gadolinium-enhanced T1-weighted MR imaging revealed a well-defined 4
5
ACCEPTED MANUSCRIPT heterogeneously enhanced lesion situated in the midline anterior cranial fossa and the anterior
ethmoid sinus (Figure 2, A, B), elevating and compressing the frontal lobe with falcine midline shift from right to left. A T2-weighted MR image showed fluid-fluid levels in the tumor (Figure 2, C). The tumor had a high signal intensity appearance on a fluid attenuated inversion recovery image
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(Figure 2, D) and a low signal intensity appearance on a diffusion weighted image (Figure 2, E). A T2*-weighted image revealed hemorrhagic components in the tumor (Figure 2, F). The tumor displaced the right frontal lobe superiorly and extended into the right ethmoid sinus (Figure 3, A, B).
SC
We supposed that the CSF leaked along the tumor from the subarachnoid space to the ethmoid sinus when the patient exhibited exertion. Serum tumor markers, including carcinoembryonic antigen
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(CEA), carbohydrate antigen 19-9 (CA19-9), alpha-fetoprotein (AFP), squamous cell carcinoma antigen (SCC), carbohydrate antigen 125 (CA125), were within those reference ranges. We diagnosed the lesion as meningioma, schwannoma, olfactory neuroblastoma, olfactory ensheathing
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cell tumor [1].
Treatment
EP
A bifrontal supraorbital approach was applied by making a coronal skin incision and retracting the scalp flap forward, and bifrontal craniotomy was performed for access to the floor of
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the right anterior cranial fossa and the right superior orbit. After a right frontal dural incision, the tumor appeared in the right anterior cranial fossa, which was a well-demarcated extra-axial mass. The arachnoid adhesions to the basal frontal lobe were sharply divided. The tumor was easily separated from the right frontal lobe. Resection of the tumor progressed from the anterior cranial fossa to the anterior ethmoid sinus through the right anterior skull base. After sufficient internal debulking of the tumor was performed, sharp extracapsular dissection was used. The right olfactory bulb was not identified. The right cribriform plate was defective. The ethmoidal arteries were cauterized with the use of bipolar cauterization. Dural margins were resected. After the resection, the defect of the right anterior skull base was reconstructed with a free fascia graft and adipose 5
6
ACCEPTED MANUSCRIPT tissue taken from the abdomen as well as a pedicle pericranium flap. In the present case, we
performed a unilateral anterior cranial base resection with preservation of the contralateral olfactory cleft.
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Postoperative Course The tumor was gross totally removed. The patient’s rhinorrhea completely resolved. A histological examination revealed the tumor to be schwannoma. The patient left our hospital without sequelae.
SC
She regained her sense of smell and taste one month after the operation. Her depression also has ameliorated since the remission of rhinorrhea. At six months after surgery, the patient continued to
M AN U
do well clinically with preservation of her sense of smell. MR imaging showed gross total resection of the tumor (Figure 3, C, D).
Discussion
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A skull base CSF leak or fistula is an abnormal communication of the sterile subarachnoid space with the sinonasal or tympanomastoid cavities, and presents clinically with clear rhinorrhea or
EP
otorrhea, caused by the presence of both an osseous and a dural defect [2]. The potential leak sites include the cribriform plate, ethmoid, sphenoid, and frontal sinus [3].
AC C
It can occur following skull-base trauma, endoscopic sinus surgery, neurosurgical procedures. Other non-traumatic causes of CSF leak include erosion of the skull base by tumors, infection, mucoceles, and irradiation. Some leaks could be spontaneous without any specific etiology [3]. The latter was traditionally considered an idiopathic cause, but more recently it has been shown to be associated with elevated intracranial pressures [4,5]. Reported presenting symptoms of OG schwannomas include headache, seizures, anosmia, frontal lobe dysfunction, and signs and symptoms related to increased intracranial pressure [6,7]. The present patient presented with CSF rhinorrhea. The growth of benign tumors does not
6
7
ACCEPTED MANUSCRIPT commonly result in CSF rhinorrhea. To the best of our knowledge, this is the first report of frontoethmoidal schwannoma manifesting exertional CSF rhinorrhea as the initial symptom. Olfactory groove (OG) schwannomas are extremely rare tumors, occurring less frequently than any other intracranial nerve schwannoma [8]. Headaches and nasal obstruction were the most
RI PT
common presenting symptoms occurring in 30.9 and 29.8% of cases, respectively [9]. Sunary et al. performed a MEDLINE/PubMed search, identifying 71 reports representing 94 cases of anterior skull base and sinonasal schwannomas [9]. In 94 patients with anterior skull
SC
base and sinonasal schwannomas, 44 (46.8%) were exclusively sinonasal, 30 (31.9%) were exclusively intracranial, 12 (12.8%) were primarily intracranial with extension into the paranasal
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sinuses, and 8 (8.5%) were primarily sinonasal with intracranial extension. Headaches and nasal obstruction were the most common presenting symptoms occurring in 30.9 and 29.8% of cases, respectively [9].
Both OG schwannomas and olfactory ensheathing cell tumors frequently demonstrate bone erosion on imaging [10]. Olfactory groove schwannomas are believed to arise from the dural
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branches of the first division of the trigeminal nerve as they pass through the anterior fossa, or from the filia olfactoria, which develops a Schwann cell layer about 0.5 mm beyond the olfactory bulb
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[11]. Our schwannoma presumably caused CSF leakage due to its upside-down teardrop shape penetrating the dural layer on the crivriform/OG from the subarachnoid space to the ethomoid sinus.
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We have classified those subfrontal, OG, anterior skull base, or nasoethmoidal/sinonasal-intracranial schwannomas schematically into four types as shown in Figure 4. Previously reported cases can be classified into one of these four types; subfrontal (olfactory groove), nasoethmoidal (sinonasal), frontoethmoidal (primarily intracranial with extension into the paranasal sinuses), or ethmofrontal (primarily sinonasal with intracranial extension). Most patients underwent surgical gross total resection via craniotomy, endoscopic endonasal approach, rhinotomy, or other sinonasal approaches [9]. Recurrence occurred in three cases (3.2 %) among 94 cases ranging from 4 months to 13 years [9]. With complete surgical resection, very low rates of tumor recurrence have been reported [12]. Overall, OG schwannomas 7
8
ACCEPTED MANUSCRIPT
have a favorable prognosis. Due to the tumor’s benign nature, complete resection of the tumor is the best treatment modality, and adjunctive therapy is not typically needed [13]. Advanced training in endonasal techniques, high-definition endoscopic equipment, dedicated instrumentation, and collaboration between otolaryngologists and neurosurgeons are
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highly recommended for extended endonasal procedures [13]. In the present case, however, we selected a transcranial approach with bifrontal craniotomy due to the need for access to the right frontal pole of the brain (Figure 3, B). The resected area of the dura and tumor was repaired with a
SC
free fascia flap and adipose tissue from the abdomen as well as a pedicle pericranium flap, resulting
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in remission of CSF rhinorrhea.
Conclusion
We report a rare case of a frontoethmoidal schwannoma with exertional CSF rhinorrhea. According to previous reports, para/peri-OG schwannomas are divided into four types: subfrontal;
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nasoethomoidal; frontoethomoidal; ethomofrontal. Among them, a frontoethomoidal schwannoma can manifest exertional CSF rhinorrhea as an initial symptom. This is the first report describing
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exertion CSF rhinorrhea as an initial symptom of frontoehmoidal schwannoma.
Compliance with ethical standards Disclosure
The manuscript has not been submitted to any other journal for simultaneous consideration. The authors have no personal financial or institutional interest in any of the drugs, materials, or devices described in this article.
Funding No funding was specifically received for this research. 8
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ACCEPTED MANUSCRIPT Conflicts of interest None.
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Informed consent
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The patient has consented to submission of this case report to the journal.
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ACCEPTED MANUSCRIPT References 1
Yasuda M, Higuchi O, Takano S, Matsumura A. Olfactory ensheathing cell tumor: a case report. J Neurooncol. 2006;76:111-113.
Reddy M, Baugnon K. Imaging of Cerebrospinal Fluid Rhinorrhea and Otorrhea. Radiol
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Clin North Am. 2017;55:167-187.
Yadav YR, Parihar V, Janakiram N, Pande S, Bajaj J, Namdev H. Endoscopic
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3
4
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management of cerebrospinal fluid rhinorrhea. Asian J Neurosurg. 2016;11:183-193.
Schlosser RJ, Wilensky EM, Grady MS, Bolger WE. Elevated intracranial pressures in spontaneous cerebrospinal fluid leaks. Am J Rhinol. 2003;17:191-195.
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Schlosser RJ, Woodworth BA, Wilensky EM, Grady MS, Bolger WE. Spontaneous
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cerebrospinal fluid leaks: a variant of benign intracranial hypertension. Ann Otol Rhinol
6
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Laryngol. 2006;115:495-500.
Adachi K, Yoshida K, Miwa T, Ikeda E, Kawase T. Olfactory schwannoma. Acta
7
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Neurochir (Wien). 2007;149:605-610.
Figueiredo EG, Soga Y, Amorim RL, Oliveira AM, Teixeira MJ. The puzzling olfactory groove schwannoma: a systematic review. Skull Base. 2011;21:31-36.
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Micovic M, Zivkovic B, Zivanovic J, Bascarevic V, Bogosavljevic V, Rasulic L. Ancient Olfactory Schwannoma - Case Report and Literature Review. Turk Neurosurg. 2017;27:656-661
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Sunaryo PL, Svider PF, Husain Q, Choudhry OJ, Eloy JA, Liu JK. Schwannomas of the sinonasal tract and anterior skull base: a systematic review of 94 cases. Am J Rhinol
10
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Allergy. 2014;28:39-49.
Darie I, Riffaud L, Saïkali S, Brassier G, Hamlat A. Olfactory ensheathing cell tumour:
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case report and literature review. J Neurooncol. 2010;100:285-289.
Strauss R, Post KD. Other schwannomas of cranial nerves. In: Kaye AH, Laeos ER, eds.
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Choi YS, Sung KS, Song YJ, Kim HD. Olfactory schwannoma -case report-. J Korean Neurosurg Soc. 2009;45:103-106.
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Pereira MC, Peris-Celda M, Qian J, Kenning TJ, Pinheiro-Neto CD. Unilateral Endoscopic Endonasal Surgery for Resection of an Olfactory Schwannoma of the Anterior
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Cranial Base in a Patient with Hereditary Hemorrhagic Telangiectasia. World Neurosurg. 2016;89:729.e15-20.
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Brain Tumors. New York, NY: Churchill Livingston; 1995:643-664
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ACCEPTED MANUSCRIPT Figure legends
Figure 1
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Computed tomography (CT) multi-planar reconstruction (MPR) shows a subfrontal low density lesion extending through the right olfactory groove into the right ethmoidal sinus. A coronal section shows bone destruction findings of the right olfactory groove (A). A sagittal section shows the
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lesion is in the right anterior skull base (B). Contrast-enhanced CT-MPR demonstrates a
heterogenously enhanced right frontoethmoidal tumor reaching the right nasal cavity (C). The
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tumor exists just behind the inner table of the frontal bone (D).
Figure 2
Magnetic resonance (MR) imaging discloses an extra-axial mass with a longer diameter of 4.3 cm in the right subfrontal region and the anterior ethmoidal region. A Gadolinium-enhanced
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T1-weighted MR image reveals a well-defined heterogeneously enhanced lesion situated in the midline anterior cranial fossa and the anterior ethmoid sinus (B), elevating and compressing the
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frontal lobe with falcine midline shift from right to left. A T2-weighted MR image shows fluid-fluid levels in the tumor (C). The tumor has high signal intensity on fluid attenuated inversion recovery
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image (D) and low signal intensity on a diffusion weighted image (E). A T2*-weighted image reveals hemorrhagic components in the tumor (F).
Figure 3
Postoperative Gadolinium-enhanced MR imaging shows gross total resection (C: coronal section, D: sagittal section) of the tumor (A: axial section, B sagittal section) with reconstruction of the right anterior skull base (C: coronal section, D: sagittal section).
12
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ACCEPTED MANUSCRIPT Figure 4
The frontoethmoidal schwannoma (A, B) is gross totally resected (C, D). The anterior skull base is reconstructed well (D).
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ACCEPTED MANUSCRIPT
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ACCEPTED MANUSCRIPT
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ACCEPTED MANUSCRIPT
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ACCEPTED MANUSCRIPT
S1878-8750(18)30054-8
DOI:
10.1016/j.wneu.2018.01.015
Reference:
WNEU 7212
To appear in:
World Neurosurgery
Received Date: 19 August 2017 Revised Date:
30 December 2017
Accepted Date: 3 January 2018
Please cite this article as: Yoneoka Y, Akiyama K, Seki Y, Hasegawa G, Kakita A, Frontoethmoidal Schwannoma with Exertional Cerebrospinal Fluid Rhinorrhea: a Case Report and Review of the Literature, World Neurosurgery (2018), doi: 10.1016/j.wneu.2018.01.015. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
1
ACCEPTED MANUSCRIPT
Frontoethmoidal schwannoma with exertional cerebrospinal fluid rhinorrhea: A case report and review of the literature
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Yuichiro Yoneoka, M.D., Ph.D.,1,4 Katsuhiko Akiyama, M.D., Ph.D.,1 Yasuhiro Seki, M.D.,1 Go Hasegawa, M.D., Ph.D.,2 Akiyoshi Kakita, M.D., Ph.D. 3
Department of Neurosurgery, Uonuma Institute of Community Medicine, Niigata University
SC
1.
Medical and Dental Hospital
Department of Pathology, Uonuma Institute of Community Medicine, Niigata University Medical and Dental Hospital
M AN U
2.
Department of Pathology, Brain Research Institute, Niigata University
4.
Department of Neurosurgery, Brain Research Institute, Niigata University
Correspondence: Yuichiro Yoneoka, M.D., Ph.D.
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3.
EP
Department of Neurosurgery, Uonuma Institute of Community Medicine, Niigata University Medical and Dental Hospital
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4132, Urasa, Minami-Uonuma, Niigata, 949-7302, Japan Tel: +81-25-777-3200, Fax: +81-25-777-5030, Email: [email protected]
Key words: frontoethomoidal schwannoma; olfactory groove; exertional cerebrospinal fluid rhinorrhea; anterior skull base
Abbreviations and Acronyms: AFP, alpha-fetoprotein; CSF, cerebrospinal fluid; CT, computed tomography; CA19-9, carbohydrate antigen 19-9; CA125, carbohydrate antigen 125; CEA, carcinoembryonic antigen; olfactory groove, MR, magnetic resonance; SCC, squamous cell carcinoma antigen 1
2
ACCEPTED MANUSCRIPT ABSTRACT
Background: Frontoethmoidal schwannomas are rare. No case manifesting exertional cerebrospinal fluid (CSF)
RI PT
rhinorrhea has ever been reported to the best of our knowledge.
Case Description:
SC
In this report, we describe an extremely rare case of frontoethmoidal schwannoma extending through the olfactory groove (OG) with exertional CSF rhinorrhea as the initial symptom. A
M AN U
50-year-old woman was presented to our clinic for frequent nasal discharge on exertion. A postcontrast computed tomographic scan demonstrated heterogeneously enhanced tumor from the anterior cranial fossa to the anterior ethmoid sinus. A gadolinium-enhanced T1-weighted magnetic resonance image revealed a well-defined heterogeneously enhanced tumor situated in the midline anterior cranial fossa and the anterior ethmoid sinus. After the resection, the defect of the right
TE D
anterior skull base was reconstructed with a fascia graft and adipose tissue taken from the abdomen as well as a pedicle periosteum flap. A histological examination revealed the tumor as schwannoma.
Conclusion:
AC C
operation.
EP
Her rhinorrhea completely resolved. She regained sense of smell and taste one month after the
According to previous reports, para/peri-OG schwannomas can be divided into four types: subfrontal; nasoethomoidal; frontoethomoidal; and ethomofrontal. Among them, a frontoethomoidal schwannoma can manifest exertional CSF rhinorrhea as an initial symptom.
(195 words)
2
3
ACCEPTED MANUSCRIPT Highlights
Para/peri-OG (olfactory groove) schwannomas can be divided into four types: subfrontal; nasoethomoidal; frontoethomoidal; and ethomofrontal.
RI PT
Among them, a frontoethomoidal schwannoma can manifest exertional CSF rhinorrhea as an initial
EP
TE D
M AN U
SC
symptom.
AC C
3
4
ACCEPTED MANUSCRIPT Introduction The growth of benign tumors does not commonly result in cerebrospinal fluid (CSF) rhinorrhea. In this report, we describe a case of frontoethmoidal schwannoma through the olfactory groove (OG) with exertional cerebrospinal fluid rhinorrhea. To the best of our knowledge, this is
RI PT
the first report of frontoethmoidal schwannoma manifesting exertional CSF rhinorrhea as the initial symptom. Since CSF rhinorrhea is the result of an abnormal communication between the sinonasal tract and the subarachnoid space, consideration of the localization of the presented frontoethmoidal
SC
schwannoma can enable us to trace the extending route of the lesion and to infer the origin of the
M AN U
schwannoma.
Case Description History and Examination
A 50-year-old woman was referred to us for evaluation of a frontobasal tumor compressing
TE D
the right frontal lobe upward. The patient had noted rare rhinorrhea since she was 47 years of age. When she was 48 years old, she suddenly developed nasal congestion and subsequent anosmia.
EP
After that, she suffered from transient memory disturbance and abnormal behavior for two weeks. During the two weeks, she frequently blew her nose and noted associated headaches. Subsequently,
AC C
she received psychiatric therapy and took oral antidepressants over a two-year period. Two months prior to her first visit to our hospital, she noted frequent nasal discharge which was worse on exertion. She had clear and intermittent fluid rhinorrhea from her right nostril when straining, leaning forward, or changing her position. A computed tomographic (CT) scan showed a low-density mass in the right anterior cranial fossa and the anterior ethmoid sinus (Figure 1, A, B). A postcontrast CT scan demonstrated heterogeneously enhanced tumor from the anterior cranial fossa to the anterior ethmoid sinus (Figure 1, C, D). Magnetic resonance (MR) imaging disclosed an extra-axial mass with a longer diameter of 4.3 cm in the right subfrontal region and the anterior ethmoidal region. Gadolinium-enhanced T1-weighted MR imaging revealed a well-defined 4
5
ACCEPTED MANUSCRIPT heterogeneously enhanced lesion situated in the midline anterior cranial fossa and the anterior
ethmoid sinus (Figure 2, A, B), elevating and compressing the frontal lobe with falcine midline shift from right to left. A T2-weighted MR image showed fluid-fluid levels in the tumor (Figure 2, C). The tumor had a high signal intensity appearance on a fluid attenuated inversion recovery image
RI PT
(Figure 2, D) and a low signal intensity appearance on a diffusion weighted image (Figure 2, E). A T2*-weighted image revealed hemorrhagic components in the tumor (Figure 2, F). The tumor displaced the right frontal lobe superiorly and extended into the right ethmoid sinus (Figure 3, A, B).
SC
We supposed that the CSF leaked along the tumor from the subarachnoid space to the ethmoid sinus when the patient exhibited exertion. Serum tumor markers, including carcinoembryonic antigen
M AN U
(CEA), carbohydrate antigen 19-9 (CA19-9), alpha-fetoprotein (AFP), squamous cell carcinoma antigen (SCC), carbohydrate antigen 125 (CA125), were within those reference ranges. We diagnosed the lesion as meningioma, schwannoma, olfactory neuroblastoma, olfactory ensheathing
TE D
cell tumor [1].
Treatment
EP
A bifrontal supraorbital approach was applied by making a coronal skin incision and retracting the scalp flap forward, and bifrontal craniotomy was performed for access to the floor of
AC C
the right anterior cranial fossa and the right superior orbit. After a right frontal dural incision, the tumor appeared in the right anterior cranial fossa, which was a well-demarcated extra-axial mass. The arachnoid adhesions to the basal frontal lobe were sharply divided. The tumor was easily separated from the right frontal lobe. Resection of the tumor progressed from the anterior cranial fossa to the anterior ethmoid sinus through the right anterior skull base. After sufficient internal debulking of the tumor was performed, sharp extracapsular dissection was used. The right olfactory bulb was not identified. The right cribriform plate was defective. The ethmoidal arteries were cauterized with the use of bipolar cauterization. Dural margins were resected. After the resection, the defect of the right anterior skull base was reconstructed with a free fascia graft and adipose 5
6
ACCEPTED MANUSCRIPT tissue taken from the abdomen as well as a pedicle pericranium flap. In the present case, we
performed a unilateral anterior cranial base resection with preservation of the contralateral olfactory cleft.
RI PT
Postoperative Course The tumor was gross totally removed. The patient’s rhinorrhea completely resolved. A histological examination revealed the tumor to be schwannoma. The patient left our hospital without sequelae.
SC
She regained her sense of smell and taste one month after the operation. Her depression also has ameliorated since the remission of rhinorrhea. At six months after surgery, the patient continued to
M AN U
do well clinically with preservation of her sense of smell. MR imaging showed gross total resection of the tumor (Figure 3, C, D).
Discussion
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A skull base CSF leak or fistula is an abnormal communication of the sterile subarachnoid space with the sinonasal or tympanomastoid cavities, and presents clinically with clear rhinorrhea or
EP
otorrhea, caused by the presence of both an osseous and a dural defect [2]. The potential leak sites include the cribriform plate, ethmoid, sphenoid, and frontal sinus [3].
AC C
It can occur following skull-base trauma, endoscopic sinus surgery, neurosurgical procedures. Other non-traumatic causes of CSF leak include erosion of the skull base by tumors, infection, mucoceles, and irradiation. Some leaks could be spontaneous without any specific etiology [3]. The latter was traditionally considered an idiopathic cause, but more recently it has been shown to be associated with elevated intracranial pressures [4,5]. Reported presenting symptoms of OG schwannomas include headache, seizures, anosmia, frontal lobe dysfunction, and signs and symptoms related to increased intracranial pressure [6,7]. The present patient presented with CSF rhinorrhea. The growth of benign tumors does not
6
7
ACCEPTED MANUSCRIPT commonly result in CSF rhinorrhea. To the best of our knowledge, this is the first report of frontoethmoidal schwannoma manifesting exertional CSF rhinorrhea as the initial symptom. Olfactory groove (OG) schwannomas are extremely rare tumors, occurring less frequently than any other intracranial nerve schwannoma [8]. Headaches and nasal obstruction were the most
RI PT
common presenting symptoms occurring in 30.9 and 29.8% of cases, respectively [9]. Sunary et al. performed a MEDLINE/PubMed search, identifying 71 reports representing 94 cases of anterior skull base and sinonasal schwannomas [9]. In 94 patients with anterior skull
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base and sinonasal schwannomas, 44 (46.8%) were exclusively sinonasal, 30 (31.9%) were exclusively intracranial, 12 (12.8%) were primarily intracranial with extension into the paranasal
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sinuses, and 8 (8.5%) were primarily sinonasal with intracranial extension. Headaches and nasal obstruction were the most common presenting symptoms occurring in 30.9 and 29.8% of cases, respectively [9].
Both OG schwannomas and olfactory ensheathing cell tumors frequently demonstrate bone erosion on imaging [10]. Olfactory groove schwannomas are believed to arise from the dural
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branches of the first division of the trigeminal nerve as they pass through the anterior fossa, or from the filia olfactoria, which develops a Schwann cell layer about 0.5 mm beyond the olfactory bulb
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[11]. Our schwannoma presumably caused CSF leakage due to its upside-down teardrop shape penetrating the dural layer on the crivriform/OG from the subarachnoid space to the ethomoid sinus.
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We have classified those subfrontal, OG, anterior skull base, or nasoethmoidal/sinonasal-intracranial schwannomas schematically into four types as shown in Figure 4. Previously reported cases can be classified into one of these four types; subfrontal (olfactory groove), nasoethmoidal (sinonasal), frontoethmoidal (primarily intracranial with extension into the paranasal sinuses), or ethmofrontal (primarily sinonasal with intracranial extension). Most patients underwent surgical gross total resection via craniotomy, endoscopic endonasal approach, rhinotomy, or other sinonasal approaches [9]. Recurrence occurred in three cases (3.2 %) among 94 cases ranging from 4 months to 13 years [9]. With complete surgical resection, very low rates of tumor recurrence have been reported [12]. Overall, OG schwannomas 7
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have a favorable prognosis. Due to the tumor’s benign nature, complete resection of the tumor is the best treatment modality, and adjunctive therapy is not typically needed [13]. Advanced training in endonasal techniques, high-definition endoscopic equipment, dedicated instrumentation, and collaboration between otolaryngologists and neurosurgeons are
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highly recommended for extended endonasal procedures [13]. In the present case, however, we selected a transcranial approach with bifrontal craniotomy due to the need for access to the right frontal pole of the brain (Figure 3, B). The resected area of the dura and tumor was repaired with a
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free fascia flap and adipose tissue from the abdomen as well as a pedicle pericranium flap, resulting
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in remission of CSF rhinorrhea.
Conclusion
We report a rare case of a frontoethmoidal schwannoma with exertional CSF rhinorrhea. According to previous reports, para/peri-OG schwannomas are divided into four types: subfrontal;
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nasoethomoidal; frontoethomoidal; ethomofrontal. Among them, a frontoethomoidal schwannoma can manifest exertional CSF rhinorrhea as an initial symptom. This is the first report describing
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exertion CSF rhinorrhea as an initial symptom of frontoehmoidal schwannoma.
Compliance with ethical standards Disclosure
The manuscript has not been submitted to any other journal for simultaneous consideration. The authors have no personal financial or institutional interest in any of the drugs, materials, or devices described in this article.
Funding No funding was specifically received for this research. 8
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Informed consent
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The patient has consented to submission of this case report to the journal.
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ACCEPTED MANUSCRIPT References 1
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ACCEPTED MANUSCRIPT Figure legends
Figure 1
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Computed tomography (CT) multi-planar reconstruction (MPR) shows a subfrontal low density lesion extending through the right olfactory groove into the right ethmoidal sinus. A coronal section shows bone destruction findings of the right olfactory groove (A). A sagittal section shows the
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lesion is in the right anterior skull base (B). Contrast-enhanced CT-MPR demonstrates a
heterogenously enhanced right frontoethmoidal tumor reaching the right nasal cavity (C). The
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tumor exists just behind the inner table of the frontal bone (D).
Figure 2
Magnetic resonance (MR) imaging discloses an extra-axial mass with a longer diameter of 4.3 cm in the right subfrontal region and the anterior ethmoidal region. A Gadolinium-enhanced
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T1-weighted MR image reveals a well-defined heterogeneously enhanced lesion situated in the midline anterior cranial fossa and the anterior ethmoid sinus (B), elevating and compressing the
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frontal lobe with falcine midline shift from right to left. A T2-weighted MR image shows fluid-fluid levels in the tumor (C). The tumor has high signal intensity on fluid attenuated inversion recovery
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image (D) and low signal intensity on a diffusion weighted image (E). A T2*-weighted image reveals hemorrhagic components in the tumor (F).
Figure 3
Postoperative Gadolinium-enhanced MR imaging shows gross total resection (C: coronal section, D: sagittal section) of the tumor (A: axial section, B sagittal section) with reconstruction of the right anterior skull base (C: coronal section, D: sagittal section).
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The frontoethmoidal schwannoma (A, B) is gross totally resected (C, D). The anterior skull base is reconstructed well (D).
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