- Email: [email protected]
Schwannoma of the upper cervical spine--a case report
Journal Pre-proof chwannoma of the upper cervical spine--a case report Primadenny Ariesa Airlangga, Bambang Prijambodo, Aries Rakhmat Hidayat, Steesy Benedicta PII:
S1008-1275(19)30065-3
DOI:
https://doi.org/10.1016/j.cjtee.2019.07.005
Reference:
CJTEE 412
To appear in:
Chinese Journal of Traumatology
Received Date: 4 March 2019 Revised Date:
29 July 2019
Accepted Date: 18 August 2019
Please cite this article as: Airlangga PA, Prijambodo B, Hidayat AR, Benedicta S, chwannoma of the upper cervical spine--a case report, Chinese Journal of Traumatology, https://doi.org/10.1016/ j.cjtee.2019.07.005. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. 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. © 2019 Production and hosting by Elsevier B.V. on behalf of Chinese Medical Association.
Schwannoma of the Upper Cervical Spine A Case Report Primadenny Ariesa Airlangga1, Bambang Prijambodo2, Aries Rakhmat Hidayat1, Steesy Benedicta3 1
Staff of Orthopedics & Traumatology Department, Faculty of Medicine, Universitas Airlangga/Dr. Soetomo General Hospital, Surabaya, Indonesia
2
Professor of Orthopedics & Traumatology Department, Faculty of Medicine, Universitas Airlangga/Dr. Soetomo General Hospital, Surabaya, Indonesia
3
Resident of Orthopedics & Traumatology Department, Faculty of Medicine, Universitas Airlangga/Dr. Soetomo General Hospital, Surabaya, Indonesia
Article type: case report *Corresponding author: Primadenny Ariesa Airlangga Authors: 1. Name
: Primadenny Ariesa Airlangga, M.D
Address : Jl. Dharmahusada Indah Barat IIA/49, Surabaya, East Java, Indonesia Email
: [email protected]
Phone
: +6281803336666
2. Name
: Prof. Bambang Prijambodo, MD, DR(Sci), FICS, FCSRS
Email
: [email protected]
Phone
: +62811336033
3. Name
: Aries Rakhmat Hidayat, M.D
Email
: [email protected]
Phone
: +628123060286
4. Name
: Steesy Benedicta, M.D
Email
: [email protected]
Phone
: +628159033664 1
Schwannoma of the Upper Cervical Spine A Case Report
Abstract Upper Cervical Schwannomas are rare, benign tumors that is usually asymptomatic. It is accounted for only ten percent of Schwannomas cases manifesting on the fourth and fifth decades. Aggressive and total resection is the treatment of choice for this tumor and correct diagnosis found by biopsy of the mass. Preoperative diagnosis was difficult, relies on clinical suspicion, and confirmed by surgical pathology. A male 54-year-old male with chief complain of progressive weakness and numbness of his right arm since four months. He had a history of lymph node tumor in 2007 and excised in 2011. Neurological decrease was found on the right arm. The radiographic examination showed lytic lesion on the second, third, and fourth cervical spine. Computed Tomography scan showed destruction extending to the first cervical. Capsulated extradural extramedullary mass and compression to the spinal cord was found from the Magnetic Resonance Imaging. Two stage operation was performed. The first stage was posterior decompression with occipitocervical fusion and instrumentation, while the second was anterior decompression and iliac strut graft. Both operations performed using the surgical ultrasonic dissector. In the three months follow-up, it showed neurological progressif improving. Neurological deficit appears when there is compression on the spinal cord. Total resection is the treatment of choice for these tumors. Preoperative imaging should be performed to exclude malignant tumor and found tumor extension. Keywords: Upper Cervical Spine; Schwannoma; Posterior decompression instrumentation, Anterior decompression instrumentation
2
1. Introduction Spinal Schwannomas are very rare, accounted for 25% of the intradural spinal cord in the adult with incidences around 0.3-0.4 cases/100,000 person per year, manifesting at the forth and fifth decades, on both male and female.1,2 It is a benign nerve sheath tumors from Schwann cells. Incidence in Upper Cervical showed 0.1% of all Schwannomas. Intraosseous Schwannoma accounted less than 0.2% of primary bone tumors.3 Non-intraosseous Spinal Schwannoma was most commonly found in lumbar and thoracic, while cervical was the least. Usually the patient showed no symptoms. Radiating pain, sensory deficit, motor weakness, and paresis are the signs of spinal cord compression. The tumour originated from nerve root or foramen. In 2001, Sridhar declared a classification system of benign spinal schwannoma (Fig. 1).4 In this classification, only type V showed the Intraosseous Schwannoma. Intraspinal tumor with extraspinal component >2.5cm is classified as Giant Schwannoma. Difficulty in resection is related on the tumour location, such as in Extradural Schwannoma and Giant Invasive Schwannoma. Instability is frequently found following the removal of tumour from the vertebral body.5 Macroscopically, Schwannoma is shown as an encapsulated, solid or cystic spindle cell mesenchymal tumors, while microscopically it is composed of two cellular zones: Anthony type A, which arrange with spindle-shaped Schwann cell densely and areas of palisading nuclei and Anthony B, characterized by hypocellular arrangement and large myxoid tissue.6 Giant Dumbbell Spinal Schwannoma usually showed an anteriorly extend and invade the bones and extradural location. Tumors may grow extensively out of the spinal canal, compressing the structures surrounding it, such as muscle, nerve, and artery.5
3
Fig.1. Sridhar classification of Schwannoma (source: Classification of Schwannoma from Journal of Neurosurgery Spine(4) and the figure was modified by author)
Table 1. Sridhar classification of benign nerve sheath tumors 4 Original Types Type I
Intraspinal tumor, < 2 vertebral segments in length; a : intradural; b : extradural
Type II
Intraspinal tumor, >2 vertebral segments in length (giant tumor)
Type III
Intraspinal tumor with extention into nerve root foramen
Type IV
Intraspinal tumor with extraspinal extension (dumbbell tumors); a : extraspinal component < 2.5 cm; b : extraspinal component > 2.5 cm (giant tumor)
Type V
Tumor with erosion into vertebral bodies (giant invasive tumor), lat & posterior extensions into myofascial planes
4
The symptoms may be apparent in 1- 84 months. The most common sign was radicular pain, followed by motor weakness, sensory deficit, paresis and later was bladder paresis.6 2. Case Report A 54-years-old right-handed male, working as an electronic technician, was admitted in August 2017. He complained of difficulties in writing and fine coordination when performing his job. Neurological exam was discovered at below level C5 and numbness on level C4 bilaterally. The patient first noticed his symptom 6 months prior and has been worsening slowly. Four years ago, he had a history of slow-growing anterior neck mass. A general surgeon performed an excision procedure on the mass, which histologically was found as lymph node tumor. After removal of the tumor, he had no other symptom. The plain radiograph showed lytic lesion on C2-C4 with solid margin and bone destruction on C3 and C4, with scalloping of the third cervical body (Fig. 2). Contrastenhanced CT scan of the neck showed destruction on the body of first to the fourth cervical with feeding artery originating from the vertebral artery (Fig. 3).
Fig. 2. The plain radiograph showed lytic lesion on C3 and C4. Lateral view showed collapsed of body C2-C3 (source: internal documentation)
5
a
b
Fig 3. Enhanced CT showed destruction on body of the first to fourth cervical. (a) Lateral view showed destruction of body C3 and C4, (b) AP view showed destruction of right side of C2-C4 (source: internal documentation).
MRI showed an extradural extramedullary mass on C2-C5, 39.5 x 30.9 x 45.7 mm extend to intracanal with scalloping destruction on neural foramina and extend to soft tissue posterior and anteriorly. Mass also invaded nerve root and body of vertebrae with compression of the spinal cord to the left side and cause cord edema on C4-C5 (Fig. 4a-b).
a
b
Fig. 4. (a) Sagital MRI showed a solid encapsulated mass from C2-C5 on spinal canal and extended to the bone, (b) Coronal MRI showed mass compressing the spinal canal (source: internal documentation).
6
Embolization and angiography were performed 4 days before the operation to reduce blood flow and found that feeding artery diameter was 0.3 mm with stenosis of right vertebral artery on third cervical and microcatheter was unable to pass. Angiography discovered that the tumour compressed the vertebral artery on the third cervical with the smallest diameter at 0.42 mm, which was only 33% of the normal vertebral artery. Feeding artery found on level C2-C3 with the biggest diameter at 0.34 mm as shown in Fig. 5.
Fig. 5. Angiograph showed Feeding artery (short arrow) and basilar artery compressed by mass and sized only one third of normal diameter (long arrow) (source: internal documentation)
We performed two stages procedure, the first on December 2017. We performed posterior decompression and stabilization with instrumentation and fusion. A soft capsulated greyish mass lied on C2-C5, which was removed using the ultrasonic surgical aspirator. Cavitron Ultrasonic Surgical Aspirator (CUSA) was our instrumentation we used to destroy tissue tumor by heat and cavitation. Macroscopically, all tumors invading posterior side have been removed. Spinal canal was not intruded by the tumors. No need to sacrifice any nerve. Vertebral artery compressed by the tumor, but no invasion of tumor into the artery. After the removal of tumor, we fused the occipital to seventh cervical using pedicle screws (Fig. 6).
7
a
b
c
d
Fig. 6. Tumor seen as the capsulated soft greyish mass on C2-C5 and performed decompression and occipitocervical fusion. (a) Mass on extradural C2-C5 found after opening the muscle, (b) Placing lateral mass screw on C2, C5 and C6 before performing tumor removal. Yellow circle showed macroscopic of tumor, (c) Removed the tumor using CUSA®, and (d) Performed occipitocervical fusion (source: internal documentation).
During operation, we also took a sample of the mass for pathologic examination. Histopathology confirmed as a benign Schwannoma which shown proliferation of Schwann cell, spindle core, thin chromatin. Characteristically of Anthony A and Anthony B cells also found (Fig. 7).
a
b
8
Fig. 7. Histopathology using Hematoxylin & Eosin staining with 200x magnification found the proliferation of Schwann cell with (a) some Anthony A (yellow arrow) and Anthony B area (black arrow), (b) Anthony B area with 400x magnification (source: internal documentation).
Three weeks after the first operation, we performed the anterior approach procedure. Initially, we planned mandible osteotomy to reach second and third cervical. Carotid pulse was palpated and the superior recurrent laryngeal nerve was identified and preserved. The prevertebral fascia was visible and using a fine needle, position checked using image intensifier and found the third cervical already reached (Fig. 8). Incision extended to submandibular without mandible osteotomy. Tumors located an anterior part was removed using the ultrasonic surgical aspirator. Iliac graft was taken and grafted as buttresses on C2C5 using anterior cervical plate (Fig. 9). Anterior plate was used to buttressing. Body of C2 and C3 was destroyed which no screw can hold tight.
a
b
Fig. 8. The level was checked using image intensifier and tumor was decompressed using cavitron ultrasonic aspirator. (a) second stage operation performed anterior approach using CUSA®, (b) approach already reach C2 using CUSA® (source: internal documentation)
9
b
a
Fig. 9. (a) iliac graft placed and buttress (b) using plate (source: internal documentation)
Postoperatively, the patient was put in Minerva cast for two months to provide and protect the fusion of cervical. The postoperative evaluation showed an increase on the motoric and sensoric nerve system from 3/5 on the right side to 5/5 within 2 months. Plain radiology on 2nd month evaluation found stable fixation without any loosening on screw (Fig. 10). The patient also started to work again in the third month.
a
b
c
Fig. 10. Two months radiological evaluation from lateral view. (a) 2 months evaluation, (b) 7 months evaluation, (c) one year evaluation (source: internal documentation)
We continuously followed up clinical on this patient. MRI evaluation performed one year post op to seek any recurrence of tumor. Patient already gained full motoric and sensoric nerve system and already back to work without any sequels. We performed brain 10
CT in order to find any metastatic to brain stem, but showed that head CT was clear (Fig 11). We also performed MRI of cervical, found mass on paravertebrae level VC2-3 entering the foramen canal and encased a. Vertebrae, ended at level fourth and fifth cervical. Also found destruction of body of cervical second and third (Fig. 12).
Fig. 11. Head CT: No sign of metastatic tumor to brain (source: internal documentation)
Fig. 12. MRI of Cervical showed mass on right paravertebrae VC 2-3 and enceased of A. Vertebralis.
3. Discussion Upper Cervical Schwannoma is rare, originating from superior to middle part of the cervical chain. Schwannoma mostly occurs on the forth and fifth decade with most cases found in lumbar and thoracic. Diagnostic preoperative must be performed completely. The earlier patient showed no symptom. In the late case when patient showed a neurological 11
deficit, showed that tumor already compressed the spinal cord. On MR-imaging, a Schwannoma is generally hypointense on T1-weighted and hyperintense on T2-weighted images, depending on its cellularity. Incisional biopsy can lead to a correct diagnosis in most cervical masses. FNA-cytology can lead to correct diagnosis only in 25% of Cervical Schwannoma. The case reported, a male patient, with rare predilection of Schwannoma in upper cervical, categorized as giant schwannoma with size 39.5 x 30.9 x 45.7 mm and extend to intracanal. Difficulty in resection occured due to its location in extradural and the giant invasive schwannoma. Tumour already destroyed the vertebral body and extended from to C1 to C4. We performed triple diagnostics using clinical, radiology, and pathology examination. Clinically neurological deficit was found on the right side of trunk. Motor weakness was not obvious until the later stage. We performed plain radiograph, MRI and CT scan to evaluate the extend of destruction on the vertebral body. We found tumor already compressing the spinal cord especially on C4-C5 and destruction of the vertebral body already extrude anteriorly and extended to C1. Tumour found extradural extramedullary, while in literature 70-80% of spinal schwannomas are reported to be intradural. Preoperatively we planned on embolizing the tumor, unfortunately it was unsuccessful due to small caliber of the feeding arteries. We performed neither the FNAB nor open biopsy, instead we directly performed posterior stabilization, decompression, and fusion of occipitocervical. In order to perform complete surgical removal of mass, we perform 2 stage operation. Dissection of tumor as performed using cavitron ultrasound surgical aspirator. From the biopsy the characteristic was found of the Anthony A and Anthony B cell. Instability is frequent following removal of tumor invaded to body vertebra. To stabilize the anterior spine, we performed second stage operation anteriorly to resect the tumor anteriorly. Two-stage operation was performed to prevent postoperative instability and recurrence.
12
Complication was evaluated post-operatively, with nerve injury as the most possible complication. No sign of complication was found in this case. Postoperatively, patient put in Minerva cast for 2 months. In the course of seven months evaluation, the patient has already gained motor function and was already capable of daily activity. Fusion on C2-C4 was found radiologically (Fig. 10). The outcome of Schwannomas correlated to the preoperative neurological condition of patient. Symptoms and sign improves in 95% of patient. One year MRI evaluation we found reccurence mass with enceased of A.vertebral on level C2-3 without any symptomatic symptom.
4. Conclusion Surgery in Spinal Schwannoma usually had good prognosis. The pitfall in previous diagnostic and operation have a high possibility of recurrence. Multidiscipline and triple diagnostic are mandatory in treating tumor spine. Aggressive and total resection was the preferable option this case.
Conflict of Interest Statement The authors declare no conflict of interest in this study.
Funding/Support Statement This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
Acknowledgment None 13
References 1. 2.
3. 4.
5. 6.
Celli P, Trillò G, Ferrante L. Spinal extradural schwannoma. J Neurosurg Spine. 2005;2(4):447-456. Seppälä MT, Haltia MJJ, Sankila RJ, Jääskeläinen JE, Heiskanen O. Long-term outcome after removal of spinal schwannoma: a clinicopathological study of 187 cases. J Neurosurg. 1995;83(4):621-626. Fawcett KJ, Dahlin DC. Neurilemmoma of bone. Am J Clin Pathol. 1967;47(6):759766. Sridhar K, Ramamurthi R, Vasudevan MC, Ramamurthi B. Giant invasive spinal schwannomas: definition and surgical management. J Neurosurg Spine. 2001;94(2):210-215. Park S, Chung S-K, Choe G, Kim H-J. Spinal intraosseous schwannoma: a case report and review. J Korean Neurosurg Soc. 2009;46(4):403. Neville BW, Damm DD, Chi AC, Allen CM. Oral and Maxillofacial Pathology. Elsevier Health Sciences; 2015.
14
S1008-1275(19)30065-3
DOI:
https://doi.org/10.1016/j.cjtee.2019.07.005
Reference:
CJTEE 412
To appear in:
Chinese Journal of Traumatology
Received Date: 4 March 2019 Revised Date:
29 July 2019
Accepted Date: 18 August 2019
Please cite this article as: Airlangga PA, Prijambodo B, Hidayat AR, Benedicta S, chwannoma of the upper cervical spine--a case report, Chinese Journal of Traumatology, https://doi.org/10.1016/ j.cjtee.2019.07.005. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. 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. © 2019 Production and hosting by Elsevier B.V. on behalf of Chinese Medical Association.
Schwannoma of the Upper Cervical Spine A Case Report Primadenny Ariesa Airlangga1, Bambang Prijambodo2, Aries Rakhmat Hidayat1, Steesy Benedicta3 1
Staff of Orthopedics & Traumatology Department, Faculty of Medicine, Universitas Airlangga/Dr. Soetomo General Hospital, Surabaya, Indonesia
2
Professor of Orthopedics & Traumatology Department, Faculty of Medicine, Universitas Airlangga/Dr. Soetomo General Hospital, Surabaya, Indonesia
3
Resident of Orthopedics & Traumatology Department, Faculty of Medicine, Universitas Airlangga/Dr. Soetomo General Hospital, Surabaya, Indonesia
Article type: case report *Corresponding author: Primadenny Ariesa Airlangga Authors: 1. Name
: Primadenny Ariesa Airlangga, M.D
Address : Jl. Dharmahusada Indah Barat IIA/49, Surabaya, East Java, Indonesia Email
: [email protected]
Phone
: +6281803336666
2. Name
: Prof. Bambang Prijambodo, MD, DR(Sci), FICS, FCSRS
: [email protected]
Phone
: +62811336033
3. Name
: Aries Rakhmat Hidayat, M.D
: [email protected]
Phone
: +628123060286
4. Name
: Steesy Benedicta, M.D
: [email protected]
Phone
: +628159033664 1
Schwannoma of the Upper Cervical Spine A Case Report
Abstract Upper Cervical Schwannomas are rare, benign tumors that is usually asymptomatic. It is accounted for only ten percent of Schwannomas cases manifesting on the fourth and fifth decades. Aggressive and total resection is the treatment of choice for this tumor and correct diagnosis found by biopsy of the mass. Preoperative diagnosis was difficult, relies on clinical suspicion, and confirmed by surgical pathology. A male 54-year-old male with chief complain of progressive weakness and numbness of his right arm since four months. He had a history of lymph node tumor in 2007 and excised in 2011. Neurological decrease was found on the right arm. The radiographic examination showed lytic lesion on the second, third, and fourth cervical spine. Computed Tomography scan showed destruction extending to the first cervical. Capsulated extradural extramedullary mass and compression to the spinal cord was found from the Magnetic Resonance Imaging. Two stage operation was performed. The first stage was posterior decompression with occipitocervical fusion and instrumentation, while the second was anterior decompression and iliac strut graft. Both operations performed using the surgical ultrasonic dissector. In the three months follow-up, it showed neurological progressif improving. Neurological deficit appears when there is compression on the spinal cord. Total resection is the treatment of choice for these tumors. Preoperative imaging should be performed to exclude malignant tumor and found tumor extension. Keywords: Upper Cervical Spine; Schwannoma; Posterior decompression instrumentation, Anterior decompression instrumentation
2
1. Introduction Spinal Schwannomas are very rare, accounted for 25% of the intradural spinal cord in the adult with incidences around 0.3-0.4 cases/100,000 person per year, manifesting at the forth and fifth decades, on both male and female.1,2 It is a benign nerve sheath tumors from Schwann cells. Incidence in Upper Cervical showed 0.1% of all Schwannomas. Intraosseous Schwannoma accounted less than 0.2% of primary bone tumors.3 Non-intraosseous Spinal Schwannoma was most commonly found in lumbar and thoracic, while cervical was the least. Usually the patient showed no symptoms. Radiating pain, sensory deficit, motor weakness, and paresis are the signs of spinal cord compression. The tumour originated from nerve root or foramen. In 2001, Sridhar declared a classification system of benign spinal schwannoma (Fig. 1).4 In this classification, only type V showed the Intraosseous Schwannoma. Intraspinal tumor with extraspinal component >2.5cm is classified as Giant Schwannoma. Difficulty in resection is related on the tumour location, such as in Extradural Schwannoma and Giant Invasive Schwannoma. Instability is frequently found following the removal of tumour from the vertebral body.5 Macroscopically, Schwannoma is shown as an encapsulated, solid or cystic spindle cell mesenchymal tumors, while microscopically it is composed of two cellular zones: Anthony type A, which arrange with spindle-shaped Schwann cell densely and areas of palisading nuclei and Anthony B, characterized by hypocellular arrangement and large myxoid tissue.6 Giant Dumbbell Spinal Schwannoma usually showed an anteriorly extend and invade the bones and extradural location. Tumors may grow extensively out of the spinal canal, compressing the structures surrounding it, such as muscle, nerve, and artery.5
3
Fig.1. Sridhar classification of Schwannoma (source: Classification of Schwannoma from Journal of Neurosurgery Spine(4) and the figure was modified by author)
Table 1. Sridhar classification of benign nerve sheath tumors 4 Original Types Type I
Intraspinal tumor, < 2 vertebral segments in length; a : intradural; b : extradural
Type II
Intraspinal tumor, >2 vertebral segments in length (giant tumor)
Type III
Intraspinal tumor with extention into nerve root foramen
Type IV
Intraspinal tumor with extraspinal extension (dumbbell tumors); a : extraspinal component < 2.5 cm; b : extraspinal component > 2.5 cm (giant tumor)
Type V
Tumor with erosion into vertebral bodies (giant invasive tumor), lat & posterior extensions into myofascial planes
4
The symptoms may be apparent in 1- 84 months. The most common sign was radicular pain, followed by motor weakness, sensory deficit, paresis and later was bladder paresis.6 2. Case Report A 54-years-old right-handed male, working as an electronic technician, was admitted in August 2017. He complained of difficulties in writing and fine coordination when performing his job. Neurological exam was discovered at below level C5 and numbness on level C4 bilaterally. The patient first noticed his symptom 6 months prior and has been worsening slowly. Four years ago, he had a history of slow-growing anterior neck mass. A general surgeon performed an excision procedure on the mass, which histologically was found as lymph node tumor. After removal of the tumor, he had no other symptom. The plain radiograph showed lytic lesion on C2-C4 with solid margin and bone destruction on C3 and C4, with scalloping of the third cervical body (Fig. 2). Contrastenhanced CT scan of the neck showed destruction on the body of first to the fourth cervical with feeding artery originating from the vertebral artery (Fig. 3).
Fig. 2. The plain radiograph showed lytic lesion on C3 and C4. Lateral view showed collapsed of body C2-C3 (source: internal documentation)
5
a
b
Fig 3. Enhanced CT showed destruction on body of the first to fourth cervical. (a) Lateral view showed destruction of body C3 and C4, (b) AP view showed destruction of right side of C2-C4 (source: internal documentation).
MRI showed an extradural extramedullary mass on C2-C5, 39.5 x 30.9 x 45.7 mm extend to intracanal with scalloping destruction on neural foramina and extend to soft tissue posterior and anteriorly. Mass also invaded nerve root and body of vertebrae with compression of the spinal cord to the left side and cause cord edema on C4-C5 (Fig. 4a-b).
a
b
Fig. 4. (a) Sagital MRI showed a solid encapsulated mass from C2-C5 on spinal canal and extended to the bone, (b) Coronal MRI showed mass compressing the spinal canal (source: internal documentation).
6
Embolization and angiography were performed 4 days before the operation to reduce blood flow and found that feeding artery diameter was 0.3 mm with stenosis of right vertebral artery on third cervical and microcatheter was unable to pass. Angiography discovered that the tumour compressed the vertebral artery on the third cervical with the smallest diameter at 0.42 mm, which was only 33% of the normal vertebral artery. Feeding artery found on level C2-C3 with the biggest diameter at 0.34 mm as shown in Fig. 5.
Fig. 5. Angiograph showed Feeding artery (short arrow) and basilar artery compressed by mass and sized only one third of normal diameter (long arrow) (source: internal documentation)
We performed two stages procedure, the first on December 2017. We performed posterior decompression and stabilization with instrumentation and fusion. A soft capsulated greyish mass lied on C2-C5, which was removed using the ultrasonic surgical aspirator. Cavitron Ultrasonic Surgical Aspirator (CUSA) was our instrumentation we used to destroy tissue tumor by heat and cavitation. Macroscopically, all tumors invading posterior side have been removed. Spinal canal was not intruded by the tumors. No need to sacrifice any nerve. Vertebral artery compressed by the tumor, but no invasion of tumor into the artery. After the removal of tumor, we fused the occipital to seventh cervical using pedicle screws (Fig. 6).
7
a
b
c
d
Fig. 6. Tumor seen as the capsulated soft greyish mass on C2-C5 and performed decompression and occipitocervical fusion. (a) Mass on extradural C2-C5 found after opening the muscle, (b) Placing lateral mass screw on C2, C5 and C6 before performing tumor removal. Yellow circle showed macroscopic of tumor, (c) Removed the tumor using CUSA®, and (d) Performed occipitocervical fusion (source: internal documentation).
During operation, we also took a sample of the mass for pathologic examination. Histopathology confirmed as a benign Schwannoma which shown proliferation of Schwann cell, spindle core, thin chromatin. Characteristically of Anthony A and Anthony B cells also found (Fig. 7).
a
b
8
Fig. 7. Histopathology using Hematoxylin & Eosin staining with 200x magnification found the proliferation of Schwann cell with (a) some Anthony A (yellow arrow) and Anthony B area (black arrow), (b) Anthony B area with 400x magnification (source: internal documentation).
Three weeks after the first operation, we performed the anterior approach procedure. Initially, we planned mandible osteotomy to reach second and third cervical. Carotid pulse was palpated and the superior recurrent laryngeal nerve was identified and preserved. The prevertebral fascia was visible and using a fine needle, position checked using image intensifier and found the third cervical already reached (Fig. 8). Incision extended to submandibular without mandible osteotomy. Tumors located an anterior part was removed using the ultrasonic surgical aspirator. Iliac graft was taken and grafted as buttresses on C2C5 using anterior cervical plate (Fig. 9). Anterior plate was used to buttressing. Body of C2 and C3 was destroyed which no screw can hold tight.
a
b
Fig. 8. The level was checked using image intensifier and tumor was decompressed using cavitron ultrasonic aspirator. (a) second stage operation performed anterior approach using CUSA®, (b) approach already reach C2 using CUSA® (source: internal documentation)
9
b
a
Fig. 9. (a) iliac graft placed and buttress (b) using plate (source: internal documentation)
Postoperatively, the patient was put in Minerva cast for two months to provide and protect the fusion of cervical. The postoperative evaluation showed an increase on the motoric and sensoric nerve system from 3/5 on the right side to 5/5 within 2 months. Plain radiology on 2nd month evaluation found stable fixation without any loosening on screw (Fig. 10). The patient also started to work again in the third month.
a
b
c
Fig. 10. Two months radiological evaluation from lateral view. (a) 2 months evaluation, (b) 7 months evaluation, (c) one year evaluation (source: internal documentation)
We continuously followed up clinical on this patient. MRI evaluation performed one year post op to seek any recurrence of tumor. Patient already gained full motoric and sensoric nerve system and already back to work without any sequels. We performed brain 10
CT in order to find any metastatic to brain stem, but showed that head CT was clear (Fig 11). We also performed MRI of cervical, found mass on paravertebrae level VC2-3 entering the foramen canal and encased a. Vertebrae, ended at level fourth and fifth cervical. Also found destruction of body of cervical second and third (Fig. 12).
Fig. 11. Head CT: No sign of metastatic tumor to brain (source: internal documentation)
Fig. 12. MRI of Cervical showed mass on right paravertebrae VC 2-3 and enceased of A. Vertebralis.
3. Discussion Upper Cervical Schwannoma is rare, originating from superior to middle part of the cervical chain. Schwannoma mostly occurs on the forth and fifth decade with most cases found in lumbar and thoracic. Diagnostic preoperative must be performed completely. The earlier patient showed no symptom. In the late case when patient showed a neurological 11
deficit, showed that tumor already compressed the spinal cord. On MR-imaging, a Schwannoma is generally hypointense on T1-weighted and hyperintense on T2-weighted images, depending on its cellularity. Incisional biopsy can lead to a correct diagnosis in most cervical masses. FNA-cytology can lead to correct diagnosis only in 25% of Cervical Schwannoma. The case reported, a male patient, with rare predilection of Schwannoma in upper cervical, categorized as giant schwannoma with size 39.5 x 30.9 x 45.7 mm and extend to intracanal. Difficulty in resection occured due to its location in extradural and the giant invasive schwannoma. Tumour already destroyed the vertebral body and extended from to C1 to C4. We performed triple diagnostics using clinical, radiology, and pathology examination. Clinically neurological deficit was found on the right side of trunk. Motor weakness was not obvious until the later stage. We performed plain radiograph, MRI and CT scan to evaluate the extend of destruction on the vertebral body. We found tumor already compressing the spinal cord especially on C4-C5 and destruction of the vertebral body already extrude anteriorly and extended to C1. Tumour found extradural extramedullary, while in literature 70-80% of spinal schwannomas are reported to be intradural. Preoperatively we planned on embolizing the tumor, unfortunately it was unsuccessful due to small caliber of the feeding arteries. We performed neither the FNAB nor open biopsy, instead we directly performed posterior stabilization, decompression, and fusion of occipitocervical. In order to perform complete surgical removal of mass, we perform 2 stage operation. Dissection of tumor as performed using cavitron ultrasound surgical aspirator. From the biopsy the characteristic was found of the Anthony A and Anthony B cell. Instability is frequent following removal of tumor invaded to body vertebra. To stabilize the anterior spine, we performed second stage operation anteriorly to resect the tumor anteriorly. Two-stage operation was performed to prevent postoperative instability and recurrence.
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Complication was evaluated post-operatively, with nerve injury as the most possible complication. No sign of complication was found in this case. Postoperatively, patient put in Minerva cast for 2 months. In the course of seven months evaluation, the patient has already gained motor function and was already capable of daily activity. Fusion on C2-C4 was found radiologically (Fig. 10). The outcome of Schwannomas correlated to the preoperative neurological condition of patient. Symptoms and sign improves in 95% of patient. One year MRI evaluation we found reccurence mass with enceased of A.vertebral on level C2-3 without any symptomatic symptom.
4. Conclusion Surgery in Spinal Schwannoma usually had good prognosis. The pitfall in previous diagnostic and operation have a high possibility of recurrence. Multidiscipline and triple diagnostic are mandatory in treating tumor spine. Aggressive and total resection was the preferable option this case.
Conflict of Interest Statement The authors declare no conflict of interest in this study.
Funding/Support Statement This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
Acknowledgment None 13
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