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DOI: 10.1055/s-0043-1768443
Surgical Management of Large (≥3 cm) Trigeminal Schwannomas: Functional Outcomes and Approach Selection in Multicompartmental Schwannomas
- Abstract
- Introduction
- Relevant Surgical Anatomy
- Classification of Trigeminal Schwannomas
- Methods
- Results
-
Surgical Approaches
- Discussion
- Conclusion
- References
Abstract
Introduction Trigeminal schwannoma surgery has shown a remarkable improvement in functional recovery and tumor resection. In the era of radiosurgery, these outcomes need to be characterized for tumors which are outside the realm of being treated with radiosurgery. We present a series of trigeminal schwannomas larger than 3 cm, surgical approaches used, and outcomes with an emphasis on functional recovery in a high-volume center with radiosurgery facilities.
Method All consecutive cases of trigeminal schwannoma from January 2012 to May 2021 which were more than 3 cm in size and underwent microsurgery were included in this series. The surgical approach, neurological outcomes, and extent of resection were defined objectively with pre/postoperative magnetic resonance imaging.
Results A total of 83 such cases (>3 cm) were found, with cranial nerve symptoms (5th most common) being the commonest. Twenty three percent cases had blindness due to secondary optic atrophy and eighteen percent had long tract motor symptoms signifying the tumor burden in our series. Radiological gross total excision was achieved in 75.9% cases.
Conclusion Large-volume schwannomas present with cranial nerve involvement and may need extensive skull base approaches. Functional outcomes need to be prioritized and can be achieved albeit with lesser gross resection rates. Hearing and facial preservation in addition to relief of trigeminal symptoms should be the goal of resection with minimal additional morbidity.
#
Introduction
Schwannomas were first described in 1910 by Verocay[1] and originate from Schwann cells, predominantly affecting the sensory cranial nerves. Vestibular schwannomas are by far the most common but among the nonvestibular ones, trigeminal schwannomas represent the majority accounting for 0.8 to 8%.[2] These lesions are benign and can present at any age but have a peak incidence in the third and fourth decade.[3] [4] [5] [6] [7]
#
Relevant Surgical Anatomy
The trigeminal nerve fibers join the brainstem around the midpoint of the ventral pons and are composed of a large sensory root and a small medial motor root. Fibers pass upward toward the petrous apex, traversing the cerebellopontine cistern, and leave the posterior fossa through the porus trigeminus. Once through the porous trigeminus, the fibers converge to form the trigeminal ganglion (Gasserian ganglion), all except the motor component. The trigeminal ganglion sits within Meckel's cave, which is formed by dura and arachnoid in a recess shaped like a “trident.” Each finger of the glove corresponds to branches of the trigeminal nerve. The ophthalmic branch (V1) passes along the lateral wall of the cavernous sinus and into the orbit. The maxillary nerve (V2) passes beneath the dura, below the point where the medial and lateral walls of the cavernous sinus dura fuse, and exits the skull through the foramen rotundum. The mandibular root (V3) passes extradurally through the foramen ovale, forming the mandibular nerve which has a sensory component and motor component that supply the muscles of mastication. The petrous and lacerum part of the internal carotid artery lie beneath Meckel's cave within the petrous bone.
#
Classification of Trigeminal Schwannomas
For an in-depth understanding of the classification, it is important to understand the surgical anatomy of the trigeminal nerve and its surrounding structures. The classification systems[3] [4] [5] [6] [7] mirror the anatomy ([Table 1]), but they can be broadly understood as middle fossa predominantly, middle and posterior fossa, and posterior fossa predominant tumors with small extensions in each compartment. The surgical approach can be divided as per the location of the majority of the tumor ([Table 2]). Recently, there has been an increase in endoscopic approaches (EAs) and the same has been shown to have low morbidity, especially in carefully selected cases. However, in the majority of the series[3] [4] [5] [6] [7] [8] [9] [10] [11] [12] [13] [14] [15] [16] [17] [18] ([Table 3]), the transcranial approaches remain the workhorse for these cases with good resection rates and minimal morbidity, especially in recent series.
Jefferson[3] |
Samii et al[4] |
Yoshida and Kawase[5] |
Ramina et al[6] |
Wanibuchi et al[7] |
---|---|---|---|---|
A |
A |
M |
C |
Ganglion |
Middle fossa |
Middle fossa |
Middle fossa |
Middle fossa |
Tumor of ganglion (middle fossa) |
B |
B |
P |
D |
Root |
Posterior fossa |
Posterior fossa |
Posterior fossa |
Posterior fossa |
Tumor of trigeminal root (posterior fossa) |
C |
C |
MP |
B |
Dumbbell |
Combination of both middle and posterior fossa |
Combination of both middle and posterior fossa |
Combination of both middle and posterior fossa |
Middle fossa with extracranial extension |
Cavernous root (middle fossa and posterior fossa |
ME |
E |
Cavernous peripheral root |
||
Combination of both middle and extracranial fossa |
Middle and posterior fossa |
Middle fossa with extracranial extension |
||
E |
||||
Extracranial tumors |
||||
D |
MPE |
A |
Peripheral |
|
Predominately extracranial tumor which has extended intracranially |
Middle, posterior, and extracranial tumor |
Extracranial tumor with extension into middle fossa |
Extracranial tumor |
|
F |
||||
Middle, posterior, and extracranial tumor |
Abbreviations: E (extracranial) in which the tumor arises from an extracranial peripheral branch of the trigeminal nerve such as the mandibular nerve; M (middle fossa):tumor arising from the Gasserian ganglion; ME (middle fossa—extracranial) which includes tumors in the middle fossa extending into either the orbit, infratemporal fossa, or pterygopalatine fossa; MP which refers to tumor spanning the middle fossa and posterior fossa; MPE which includes tumors which are in the middle, posterior fossa and extracranial compartment); P (posterior fossa) where the origin is the root of the trigeminal nerve
Abbreviations: CSF, cerebrospinal fluid; EEA, endoscopic endonasal approach.
Series (year) |
Cases (n) |
Location |
Surgical approach |
Outcomes |
Resection rates (%) |
---|---|---|---|---|---|
Samii et al. (1995)[4] |
5 |
A Middle fossa |
Frontotemporal craniotomy intradural |
Mortality 0% |
Gross total resection 100% |
1 |
B Posterior fossa |
Retrosigmoid |
Trigeminal function |
Gross total resection 100% |
|
5 |
C Combination of middle and posterior fossa |
Subtemporal + presigmoid |
Slightly worse 75% |
Gross total resection 100% |
|
1 |
D Predominately extracranial |
Frontotemporal craniotomy, extradural |
Static 25% |
||
Gross total resection 100% |
|||||
Konovalov et al (1996)[8] |
26 |
Posterior fossa |
Suboccipital |
3% Mortality |
Gross total resection or near total resection in 77% |
Transtentorial |
|||||
42 |
Gasserian ganglion (middle fossa) |
Subtemporal |
Moderate or no V palsy 85% |
||
Frontotemporal |
Same 7% |
||||
Subfrontal |
Worsened 7% |
11.7% Surgery for symptomatic recurrence |
|||
30 |
Dumbell (middle fossa and posterior fossa) |
Temporal-suboccipital |
|||
Presigmoid |
|||||
13 |
Intraorbital |
Frontozygomatic |
|||
Yoshida and Kawase[5] |
5 |
M Middle fossa |
Frontotemporal epidural – interdural approach |
KPS outcomes |
60% Gross total resection (after introduction of skull base techniques 100%) |
> 90 92% |
|||||
< 80 8% |
|||||
5 |
P Posterior fossa |
Lateral suboccipital or transpetrosal |
Trigeminal hypesthesia |
60% Gross total resection (after introduction of skull base techniques 66%) |
|
1 |
E Extracranial |
Zygomatic infratemporal |
46.6% worse |
Gross total resection 100% |
|
53.4% same or better |
|||||
10 |
MP Middle and posterior fossa |
Anterior petrosectomy |
Gross total resection 80% (after introduction of skull base techniques 90%) |
||
Trigeminal pain |
|||||
2 |
MPE Middle and posterior fossa and extracranial extension |
Zygomatic infratemporal and Anterior petrosectomy |
Better 33% |
Gross total resection 100% |
|
4 |
ME Middle fossa and extracranial extension |
Orbitozygomatic infratemporal approach |
Gross total resection 75% |
||
Goel et al (2003)[9] |
29 |
A Middle fossa |
Lateral basal subtemporal |
Mortality 3% |
Gross total resection 69.9% |
7 |
B Posterior fossa |
Retrosigmoid |
|||
30 |
C Combination of middle and posterior fossa |
Petrosal + lateral basal subtemporal |
|||
7 |
D Extracranial |
Frontotemporal ± orbitozygomatic |
|||
Ramina et al (2008)[6] |
2 |
A Predominately extracranial with small extension into middle fossa |
Extradural (transmaxillary or transmaxillary and extradural middle fossa) |
Mortality 0% |
Gross total resection 94% |
Trigeminal hypesthesia |
|||||
2 |
B Middle fossa with extracranial extension |
Intradural middle fossa |
Worsened 41.10% |
||
Trigeminal pain |
|||||
5 |
C Middle fossa |
Intradural middle fossa or extradural middle fossa |
Improvement 88.9% |
||
Preserved motor function 41% |
|||||
2 |
D Posterior fossa |
Retrosigmoid |
|||
4 |
E Middle and posterior fossa |
Presigmoid or 2 stage retrosigmoid/middle fossa |
|||
2 |
F Middle, posterior fossa and extracranial extension |
Combination of all |
|||
Fukaya et al (2010)[10] |
8 |
M Middle fossa |
Frontotemporal/subtemporal/anterior petrosectomy/trans-zygomatic and orbitozygomatic |
Mortality 0% |
Gross total resection or near total resection in 81% |
12 |
P Posterior fossa |
Lat suboccipital/anterior petrosectomy |
CN V palsy 43.8% |
||
4 |
E Extracranial extension |
Supraorbital/subtemporal/transzygomatic/zygomatic infratemporal fossa approach |
|||
22 |
MP Middle and posterior fossa |
Subtemporal/anterior petrosectomy/zygomatic transpetrosal approach |
|||
7 |
ME Middle fossa and extracranial extension |
Frontotemporal/orbitozygomatic |
|||
4 |
MPE Middle and posterior fossa and extracranial extension |
Zygomatic transpetrosal |
|||
Srinivas et al (2011)[11] |
0 |
A. Predominately extracranial with small extension in middle fossa |
0% Mortality |
Gross total resection 85% |
|
4 |
B. Predominately middle fossa with extracranial extension |
Frontotemporal craniotomy and orbitozygomatic craniotomy |
|||
5 |
C. Middle fossa |
Frontotemporal craniotomy and epidural approach |
|||
2 CN VII palsy, 1 CN VIII |
|||||
11 |
D. Posterior fossa |
Suboccipital craniotomy |
|||
18 |
E Middle and posterior fossa |
Frontotemporal craniotomy and orbitozygomatic craniotomy, frontotemporal craniotomy and orbitozygomatic craniotomy + mandibular swing, frontotemporal craniotomy and petrosectomy, combined middle/posterior fossa approach |
|||
5 |
F. Middle fossa, posterior fossa, and extracranial extension |
||||
Wanibuchi et al (2012)[7] |
39 |
Ganglion (middle fossa) |
Extradural temporopolar |
Mortality 0% |
Gross total resection 84.6% |
Pericavernous lateral loop |
|||||
Trigeminal hypesthesia |
|||||
14 |
Peripheral (extracranial) |
Gross total resection 78.6% |
|||
5 |
V1 |
Temporopolar transorbital |
15.9% improvement |
||
72.5% static |
|||||
3 |
V2 |
Preauricular transzygomatic |
11.6% decline |
||
6 |
V3 |
Preauricular transzygomatic |
Pain |
||
91.7% improvement |
|||||
8.3% static |
|||||
32 |
Dumbbell |
0 worsening |
Gross total resection 78.1% |
||
30 |
Cavernous root (middle fossa + posterior fossa) |
Extended middle fossa rhomboid or combined petrosal |
|||
Dysesthesia |
|||||
Improved 80% |
|||||
2 |
Cavernous peripheral (middle fossa and extracranial extension) |
Temporopolar trans-orbital |
Static 20% |
||
/ Infratemporal fossa approach |
Weakness |
||||
Improved 33.3% |
|||||
Static 67.7% |
|||||
22 |
Root (posterior fossa) |
Retrosigmoid lateral suboccipital |
Gross total resection 77.3% |
||
Chen et al (2014)[12] |
13 |
A Middle fossa |
Transzygomatic/subtemporal approach |
Trigeminal hypesthesia |
Gross total resection 69% |
Near total resection 24% |
|||||
10 |
B Posterior fossa |
Suboccipital approach |
Improved 28% |
Subtotal resection 7% |
|
Same 72% |
|||||
21 |
C Combination of middle and posterior fossa |
Transzygomatic + extradural temporopolar |
Trigeminal pain |
||
Improved in 100% |
|||||
11 |
D Extracranial |
Transzygomatic anterior infratemporal fossa/cranio-orbital |
|||
Samii et al (2014)[13] |
8 |
A Middle fossa |
Frontotemporal ± orbitotomy |
Mortality 0% |
Gross total resection 75% |
Subtemporal |
Trigeminal hypesthesia |
||||
Frontotemporal + subtemporal |
Improvement 15% |
Near total resection 25% |
|||
Same 80% |
|||||
1 |
B Posterior fossa |
Retrosigmoid intradural suprameatal approach |
Worsens 5% |
||
Trigeminal pain |
|||||
8 |
C Combination of middle and posterior fossa |
Retrosigmoid intradural suprameatal approach + endoscope assistance |
Improved 100% |
||
Retrosigmoid intradural suprameatal approach ± subtemporal |
|||||
3 |
D Extracranial |
Infratemporal fossa approach |
|||
Infratemporal fossa + subtemporal approach |
|||||
Yang et al (2018)[14] |
18 |
Orbital apex/pterygopalatine fossa/middle cranial fossa and extension into V2 |
Medial maxillectomy approach |
Trigeminal hypesthesia |
|
Improved 62.5% |
Gross total resection or near total resection 69% |
||||
Inferior temporal fossa/pterygopalatine fossa/middle cranial fossa and cavernous sinus |
Endoscopic endonasal assisted with sublabial transmaxillary approach |
Worsened 18.8% |
|||
Same 18.8% |
|||||
9 |
Middle cranial fossa/infratemporal fossa and extension into V3 |
Endoscopic endonasal assisted with sublabial transmaxillary approach and septectomy |
Trigeminal pain |
||
Improved 45% |
|||||
Worsened 27% |
|||||
11 |
Ganglion (middle fossa) |
Same 18% |
|||
Inferior temporal fossa/middle cranial fossa/posterior cranial fossa and Meckel's cave |
Endoscopic endonasal assisted with sublabial transmaxillary approach and septectomy (+ second stage in one case with posterior fossa involvement—neurosurgery procedure but approach not specified) |
||||
1 |
Root (posterior fossa) |
Endoscopic endonasal assisted with sublabial trans-maxillary approach and septectomy + second stage (neurosurgery procedure approach not specified) |
|||
Jeong et al (2014)[15] |
9 |
M Middle fossa |
Frontotemporal craniotomy and epidural approach |
Mortality 0% |
Gross total resection 95.9% |
6 |
MP Tumor predominately in middle fossa with a posterior fossa component |
Frontotemporal craniotomy and epidural approach |
Middle fossa |
||
Frontotemporal craniotomy and epidural approach + orbitozygomatic craniotomy |
Trigeminal hypesthesia |
||||
3 |
Me3 Middle fossa and extend into intracranial V3 |
Frontotemporal craniotomy and epidural approach + Orbitotomy |
Improved 25% |
||
Same 75% |
|||||
1 |
Me1 Middle fossa and extend into intracranial V1 |
Frontotemporal craniotomy and epidural approach + orbitozygomatic craniotomy |
Worse 0% |
||
Trigeminal pain |
|||||
1 |
Mpe3 Middle fossa, posterior fossa and extend into intracranial V3 |
Frontotemporal craniotomy and epidural approach + zygomatic craniotomy |
Improved 75% |
||
Worse 25% |
|||||
16 |
Pm Tumor predominately in posterior fossa with a meddle fossa component |
Suboccipital craniotomy |
Trigeminal Weakness Improvement 60% Worsened 40% |
||
Suboccipital craniotomy + suprameatal approach |
Posterior fossa |
||||
Posterior petrosal |
Trigeminal hypesthesia |
||||
Improved 6.7% |
|||||
4 |
P Posterior fossa |
Suboccipital craniotomy |
Same 53.3% |
||
9 |
MP Middle and posterior fossa |
Frontotemporal craniotomy and epidural approach + orbitozygomatic craniotomy |
Worsened 40% |
||
Frontotemporal craniotomy and epidural approach + zygomatic craniotomy |
Trigeminal pain |
||||
Frontotemporal craniotomy and epidural approach |
Improved 100% |
||||
Posterior petrosal |
Trigeminal weakness |
||||
Staged op (suboccipital + frontotemporal craniotomy and epidural approach) |
Same 100% |
||||
Makarenko et al (2018)[16] |
2 |
M Middle fossa |
(Not specified which tumor had which approach) Frontotemporal craniotomy and orbitozygomatic osteotomy, endoscopic resection, partial labyrinthectomy and anterior petrosectomy |
Mortality 0% |
Planned gross total resection 9/12 (75%) |
0 |
P Posterior fossa |
Trigeminal hypesthesia |
Gross total resection 100% |
||
1 |
E Extracranial |
Remaining STR was planned |
|||
4 |
MP Middle and posterior fossa |
42.8% improvement |
|||
2 |
MPE Middle and posterior fossa and extracranial extension |
Pain |
|||
3 |
ME Middle fossa and extracranial extension |
100% improvement |
|||
Park et al (2020)[17] |
9 |
M Middle fossa |
Endoscopic transorbital approach/endoscopic endonasal approach |
Trigeminal hypesthesia |
Gross total resection 48% |
Improvement 41.4% |
Near total resection 28% |
||||
8 |
E Extracranial |
Endoscopic transorbital approach/endoscopic endonasal approach |
Same 28.5% |
Gross total and near total resection 76% |
|
Endoscopic trans-orbital approach (Gross total and near total resection rate) 81.8% |
|||||
8 |
MP Middle and posterior fossa |
Endoscopic transorbital approach/endoscopic endonasal approach or endoscopic trans-orbital approach ± retrosigmoid lateral suboccipital |
|||
Endoscopic endonasal approach (Gross total and near total resection rate) 69.2% |
|||||
0 |
P Posterior fossa |
− |
|||
Li et al (2021)[18] |
8 |
M Middle fossa |
FTSA: frontotemporal subdural approach; FTEA: frontotemporal epidural approach; STAA: subtemporal epidural anterior transpetrosal approach; STTA: subtemporal transtentorial approach |
Remission rate of facial numbness 17.2% |
Gross total and near total resection rate- 90.69% |
6 |
P Posterior fossa |
SRSA: suboccipital retrosigmoid approach |
|||
19 |
MP Middle and Posterior |
FTSA: frontotemporal subdural approach; FTEA: frontotemporal epidural Approach; SRSA: suboccipital retrosigmoid approach; CSITA: combined supratentorial-infratentorial approach |
|||
3 |
ME Middle AND extracranial extension |
EEA: endoscopic endonasal approach; FTOZA: frontotemporal-orbitozygomatic approach |
|||
2 |
MPE Middle and posterior fossa and extracranial extension |
EEA: endoscopic endonasal approach |
|||
5 |
E Extracranial extension |
EEA: endoscopic endonasal approach |
#
Methods
We performed a retrospective review of all patients who underwent surgery for trigeminal schwannomas between January 2012 and May 2021 at the institute. The clinical records of patients were analyzed according to surgical approach, preoperative and postoperative neurological and cranial nerve deficit(s) status, and surgical complications documented at follow-up visits. The extent of resection was defined by comparing pre- and postoperative 3.0 T cranial magnetic resonance imaging (MRI) using T1 ± contrast agent sequences by manual volumetric segmentation.
Statistical analysis was performed using the software—R Software (Version 4.0.3). Normal distribution was assumed according to the central limit theorem. Data in text and graphs are shown as median with interquartile range or mean ± standard deviation (SD). The following p values have been considered as significant: p < 0.05.
#
Results
A total of 83 cases of large trigeminal (≥3 cm) schwannomas were operated in our center from 2012 to 2021, and the clinical details are summarized in [Table 4]. As an institute policy, any schwannomas less than 3 cm was subjected to Gamma Knife radiosurgery (GKRS) unless causing debilitating symptoms like trigeminal neuralgia, long tract signs, or cranial nerve deficits. Hence, our series differs from any previous reported ones where the percentage of large (>3 cm) schwannomas ranges from 40 to 65%.[3] [4] [5] [6] [7] [8] [9] [10] [11] [12] [13] [14] [15] [16] [17] [18] The median follow-up period was 6 months (2–12 months). The majority of our cases were young individuals (30–39 years) with median age of 33 years ([Table 5]) and an almost equal incidence among males and females (43:40).
Abbreviations: FTOZ, frontotemporal-zygotomy; GKRS, Gamma Knife radiosurgery; IQR, interquartile range; MRI, magnetic resonance imaging; RMSO, retrosigmoid approach.
Gender |
Male |
Fema le |
Total |
---|---|---|---|
Age |
|||
0–9 |
0 |
0 |
0 |
10–19 |
5 |
6 |
11 |
20–29 |
10 |
4 |
14 |
30–39 |
18 |
13 |
31 |
40–49 |
7 |
11 |
18 |
50–59 |
2 |
3 |
5 |
60–69 |
1 |
3 |
4 |
Total |
43 |
40 |
83 |
As expected the most common mode of presentation was fifth nerve symptoms (sensory and motor) followed by seventh, eighth cranial nerve, and then sixth nerve ([Table 6]). Due to the large tumor burden in most of our cases, visual disturbance due to secondary optic atrophy (postpapilledema) was seen in 19 cases (23%), and motor deficits due to long tract involvement were seen in 15 cases (18%). The follow-up was available for 71 out of 83 cases (85.5%). However, preoperative characteristics and immediate postoperative resection details were available for all ([Tables 7] and [8]).
Abbreviations: EN, endonasal; FTOZ, frontotemporal-zygotomy; GTR, gross total resection; NTR, near total resection; RMSO, retrosigmoid approach; ST, subtemporal; STR, subtotal resection.
The large tumor volume has a reflection on the clinical results, as well as the rates of gross total removal (GTR) in the present study (75.9%) are lower compared to the previously reported series. Postoperative facial paralysis was seen in an additional four cases (27 had preoperative facial weakness) and hearing loss improved in 13 cases (24 had hearing loss preoperatively). Among the 20 cases, 12 out of 20 needed resurgery and 8 cases were amenable for GKRS.
#
Surgical Approaches
Frontotemporal-Zygotomy (± Orbitotomy) + Subtemporal Interdural Approach
Tumors with a predominant middle fossa component ([Fig. 1]) were operated on using the frontotemporal-zygotomy (FTOZ/FTZ) approach. Detailed descriptions of the same have been previously described. The patient is positioned supine with a large shoulder roll under the ipsilateral scapula, and the head is rotated to the opposite side so that the temporal region is parallel to the ground and the head is slightly tilted downward so that the zygomatic arch is at the highest point. The head is fixed using a Mayfield 3-pin. The head end of the operating table is elevated just above the level of the heart. A preoperative lumbar drain may be placed to aid in extradural retraction during the procedure, based on the surgeon's preference. We prefer a curvilinear scalp incision which begins at the region of the root of the zygoma just in front of the tragus (but within the hairline), extends superiorly for about 2 cm, and curves posteriorly gently above the upper level of the pinna of the ear till posterior margin of the pinna and then turns superiorly and then anteriorly in a gentle curve to end just at the hairline anteriorly. In the earlier years, while a single-piece FTOZ was routine, in the recent years, we have avoided the orbitotomy and just performed the FTZ. Also, the skin incision has changed and a linear (vertical) scalp incision is being used as per the surgeon's preference. The most important is that the squamous temporal should be drilled to be flush with the middle cranial fossa (MCF) base. The temporal lobe dura-mater is then retracted superiorly with gentle extradural dissection along the middle cranial fossa floor. At the foramen spinosum, the middle meningeal artery is coagulated and cut to allow for further medial extradural dissection. The extradural dissection ends at just lateral to foramen Ovale. This is followed by entering into the interdural plane.


#
Subtemporal Interdural Approach (Video Supplement)
The trigeminal nerve runs through the interdural space between the meningeal and periosteal dura and is covered with a thin membrane called the inner layer. Tumors with almost equivalent tumor presence in the middle and posterior fossa were operated on using a subtemporal (ST) interdural approach based on this anatomical feature ([Fig. 2]). Positioning and craniotomy are similar to the previous approach with the absence of fronto-orbital and zygomatic exposure ([Video 1]). In cases with infratemporal tumor extension, the lateral portion of the middle cranial base is removed until the tumor margin is exposed. The middle meningeal artery and superficial greater petrosal nerve are treated in the same manner. The periosteal dura, which is the outer layer of the dura matter and can be dissected between the periosteal dura and the meningeal dura, and the interdural space occupied by the tumor, can be entered. By peeling and tacking the meningeal dura, the tumor can be exposed without exposing the temporal lobe. The tumor, covered by only a thin membrane known as the inner layer, should then be visible. By preserving the inner layer between the cavernous sinus, the tumor can be removed without opening the cavernous sinus because this inner layer is covered around the tumor.


Video 1 A surgical video demonstrating the subtemporal interdural route for excision of large trigeminal schwannomas.
Qualität:
Generally, in trigeminal schwannomas, autopetrosectomy occurs due to the tumor growth pattern and rarely is petrosectomy required. If required, which is generally to widen the opening, drilling of the bone within this triangle constitutes anterior petrosectomy and is usually done using a diamond drill. The tumor can be followed along Meckel's cave into the posterior fossa and the tumor excised. It is important to preserve the arachnoid of the tumor as the cranial nerves in the posterior fossa lie outside this. For tumors that are adherent to the brain stem, a small portion can be left behind to avoid serious brainstem complications. The tumor is exposed and excised in a piecemeal fashion following microsurgical principles. Following tumor resection, closure of the defect is done using free fat graft and is layered with fibrin glue as closure in this region is not feasible. The convexity dura mater is approximated in the usual fashion. Care must be taken to properly wax all the bone edges for any exposed air cell. Alternatively, a pedicled muscle graft obtained by splitting the temporalis muscle can be rotated to fill the dural defect and can be loosely sutured to the adjacent subtemporal dura mater. The lumbar drain is usually continued for 2 to 3 days in the postoperative period.
The FTOZ was done to provide access to the anterior cavernous sinus and allowed greater accessibility to the structures. However, when there was no significant anterior extension, only a zygotomy was performed to provide access to the base.
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Retrosigmoid Approach
The retrosigmoid approach, which remains the workhorse for cerebellopontine angle lesion, can provide adequate access for small-to-medium tumors of the petroclival region or even large cystic lesions with Meckel's cave extension ([Fig. 3]). It has the advantage of less risk of injury to the venous structures around the petrous bone. However, it involves working through small windows between neurovascular complexes of the CP angle cistern with only one angle of visualization. For large, calcified, or highly vascular lesions, significant cerebellar retraction is usually needed for adequate exposure of the tumor. Moreover, visibility of the petroclival dura mater, the ostium of Meckel's cave, and tumor relationship with the brainstem and vessels medial to the cranial nerves remain poor. Hence, this approach is ideally limited to cases with large posterior fossa components with minimal supratentorial extension.


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Extended Endoscopic Approach
The extended EA has evolved to become a much refined minimally invasive technique; however, it is suitable for predominantly tumors restricted to Meckel's cave ([Fig. 4]) and clivus rather than those with significant lateral extension. Even with an adequate reconstruction of the defect using vascularized flaps, EA is associated with a high risk of cerebrospinal fluid (CSF) leak and meningitis.


Although the aforementioned approaches work fine in general for a particular type of tumor location, the choice of approach should not be purely dogmatic and preferably should be tailored based on patient age, comorbidities, performance status, the anticipation of the presence or absence of arachnoid plane between the tumor and the brainstem, the pre-operative clinical status, as well as the acquaintance and experience of the operating surgeon with the particular procedure.
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Radiosurgery
Only 8 out of 20 residual cases treated needed GKRS with a mean dose of 12 Gy. The timing of the same was on the discretion of the treating surgeon and usually was based on the volume of the residual tumor. In two cases, a large residue (>1 cm) was seen and early GKRS was planned (within 3 months of surgery) so as to prevent the increase in the volume. In the other six cases, a small residue was seen postoperatively (<1 cm) and time to recovery was given for the cranial nerves. Late GKRS (>12 months) was planned, and tumor growth was evident at the time of radiosurgery. This strategy allowed for maximizing recovery while preventing the tumor volume outpace radio-surgical safety.
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Discussion
Classification
Our series is unique as all cases included were >3 cm in dimension and not amenable for radiosurgery. Consequently, most of our cases were multicompartmental. To decide the approach, we propose going back to the 1955 classification of Jefferson. He first classified trigeminal schwannoma into three types: type A originated from GG and was located at the middle fossa; type B originated from the trigeminal root and was located at the posterior fossa; type C was dumbbell-shaped and occupied in both middle and posterior fossa. After 31 years, Lesoin et al improved the TS classification by supplementing the type of tumor originating from three peripheral branches of the trigeminal nerve and occupying the extracranial space. This classification has been used for deciding the approach to trigeminal schwannoma in our series and helps in quick and uncomplicated management in most cases. Diverse classifications have been proposed to facilitate decision-making and assess the technical difficulty ([Table 1]). However, we believe that the choice is mainly whether the tumor has to be approached via a posterior or a middle/anterior fossa approach.
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Surgical Outcomes
In our series, good clinical outcomes were achieved with visual improvement in 100% (19 out of 19), trigeminal motor improvement in 26% (16 out of 62), trigeminal sensory improvement in 38% (23 out of 61), hearing improvement in 54% (13 out of 24), and additional facial paralysis only in 4.8% cases (4 out of 83). This was achieved in large multicompartment tumors with a rate of GTR (75.9% radiological clearance) which is in line with contemporary series (vary between 48% and 100%). Our series compares very favorably with the rates of improvement in clinical symptoms in previously reported series which are generally low (7–43%).[3] [4] [5] [6] [7] [8] [9] [10] [11] [12] [13] [14] [15] [16] [17] [18] Symptoms persisted in 25 to 80% of patients and worsened in 5 to 47%. The only area where our results were in line was the improvement in trigeminal sensory or trigeminal pain which has been reported to be 33 to 100%. Other cranial nerve deficits were reported involving CN IV, VI, and VII. Konovalov et al[8] reported 5% CN III, 12% CN IV and VI, and 8% CN VII palsies. Fukaya et al[10] reported CN VI palsy (6.3%), CN VII palsy (4.2%), CN IV palsy (2.1%), and CN VIII palsy (2.1%). In the Wanibuchi et al series,[7] there was 72% improvement in CN VI function in those presenting with diplopia secondary to an abducens palsy, with the remaining 28% function remaining static. Of the three patients with preoperative CN III palsy, one improved, one remained the same, and one deteriorated. The abducens nerve seems particularly vulnerable, and this may be related to its fairly complex anatomical relationship to Meckel's cave, as previously described.
While GTR is preferable, it is preferable to target functional preservation/improvement, especially in benign diseases like schwannoma.[8] [9] [10] [11] [12] [13] [14] [15] [16] [17] [18] [19] Thus, our series shows a trend of lower rates of cranial nerve dysfunction (rather improvement) achieved using classic and well-known skull-base approaches. As good functional outcomes with GTR can be achieved and deterioration is avoidable, we emphasize a surgical strategy to prevent cranial nerve injury, especially in the case of huge schwannomas. The 48.1% resection rate was defined via per-operative impression where a part of capsule was left adherent to the vessels or nerves. However, when postoperative MRI was reviewed the rate of resection was 75.9%. This phenomenon has also been demonstrated in previous series.[20] Eventually, only 20 cases needed further treatment.
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Approach Selection
The basic division made by Samii et al[4] puts the options in a nutshell: type A, intracranial tumor predominantly in the middle fossa; type B, intracranial tumor predominantly in the posterior fossa; type C tumors in the middle and posterior fossa; and type D extracranial tumor with intracranial extensions. Type A, C, and D tumors can be targeted via a middle fossa approach, whereas type B tumors are well managed through a retrosigmoid technique; in the case of Type C tumors, a combined approach may be necessary.
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Complication Avoidance
Cranial nerve dysfunction/injury can result in either motor or sensory and contrary to popular belief both of them are equally disabling. Motor deficits can range from trigeminal nerve dysfunction in approximately 20 to 30% of cases and are persistent while sensory function invariably returns to normal in 3 to 5 months. Peeling off the tumor over the nerves and interdural dissection under nerve monitoring with careful dural incision and minimum bipolar use are some measures that can prevent these complications. Other cranial nerves can be affected depending on the tumor extension. These include trochlear nerve deficits, third nerve palsy, facial palsy, and even abducent nerve injury. Frontalis muscle weakness can result from injury of the peripheral frontal branches of the facial nerve over the temporal muscle and an interfascial method of dissection with avoidance of the fat plane can prevent these injuries.
Skull base approaches allow better exposure of these tumors, multiple working angles with minimal brain retraction, and more complete removal without increased morbidity. However, it is important to look for exuberant sinus and meticulously seal them after the drilling to avoid subsequent CSF leak, and hence, a meticulous repair is necessary to prevent this additional morbidity especially in cases with infratemporal extension.
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Limitations of Microsurgery and Role of Radiosurgery
In the current era of onco-functional utopia, leaving a radio-surgical residue at the cost of preventing a new deficit is advisable. Anterior approaches provide good exposure above the internal acoustic meatus, it may not be suitable for more inferior lesions and those stuck to the brainstem/ premedullary cistern and other cranial nerves. Leaving a small residue to be later managed by radiosurgery or a second posterior approach would be desirable. These approaches initially provide a rather narrow corridor allowing limited degrees of freedom which expands as decompression is continued. Moreover, there can be a risk of injury to the vein of Labbe secondary undue temporal lobe retraction, sigmoid sinus, and jugular bulb in posterior approaches. This can be avoided by a preoperative lumbar drain placement or careful drilling respectively.
In a recent retrospective review[19] on the role of radiosurgery, the mean tumor volume was 5.5 cm3, time to follow-up was 56 months, the chance of clinical improvement was 48%, tumor control was 91%, and clinical worsening or new symptoms was 12% (most commonly trigeminal neuropathy/pain). Any comparison to surgical series has to take into consideration a larger tumor volume, the chance of a quicker and longer-lasting relief in trigeminal pain and higher preoperative dysfunction in presurgical cases when compared to radiosurgery cases. Microsurgery versus radiosurgery, in comparison, microsurgery has reduced tumor control (78 vs. 91%), and greater morbidity (35 vs. 12%), including postoperative mortality (1%) when compared to radiosurgery. While many patients had preoperative trigeminal neuropathy, 11.6 to 33% of patients experienced worsened function in the form of facial pain or hypesthesia after initial surgical removal. Radiosurgery led to a 92% tumor control rate and 94% clinical improvement or stabilization rate. Microsurgical decompression and salvage/adjuvant radiosurgery for critical areas remain the best course of action for large multicompartmental lesions.
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Conclusion
Large (>3 cm) multicompartmental trigeminal schwannomas are benign lesions and are best managed by a single skull base approach which aims at excision. At the same time, functional preservation or rather improvement should be the primary goal rather than gross total excision. Since these lesions have a benign course, carefully respecting anatomy and using epidural/interdural approaches that do not violate the pial plane and aiming for maximum resection remain the dictum. Radiosurgery either upfront or on signs of growth for residual lesion can prevent morbidity.
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Conflict of Interest
None declared.
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References
- 1 Joshi R. Learning from eponyms: Jose Verocay and Verocay bodies, Antoni A and B areas, Nils Antoni and Schwannomas. Indian Dermatol Online J 2012; 3 (03) 215-219
- 2 Deora H, Srinivas D, Beniwal M, Vikas V, Rao KVLN, Somanna S. Rare cranial nerve schwannomas: a retrospective review of nontrigeminal, nonvestibular cranial nerve schwannomas. J Neurosci Rural Pract 2018; 9 (02) 258-263
- 3 Jefferson G. The trigeminal neurinomas with some remarks on malignant invasion of the Gasserian ganglion. Clin Neurosurg 1953; 1: 11-54
- 4 Samii M, Migliori MM, Tatagiba M, Babu R. Surgical treatment of trigeminal schwannomas. J Neurosurg 1995; 82 (05) 711-718
- 5 Yoshida K, Kawase T. Trigeminal neurinomas extending into multiple fossae: surgical methods and review of the literature. J Neurosurg 1999; 91 (02) 202-211
- 6 Ramina R, Mattei TA, Sória MG. et al. Surgical management of trigeminal schwannomas. Neurosurg Focus 2008; 25 (06) E6 , discussion E6
- 7 Wanibuchi M, Fukushima T, Zomordi AR, Nonaka Y, Friedman AH. Trigeminal schwannomas: skull base approaches and operative results in 105 patients. Neurosurgery 2012;70(1, Suppl Operative)132–143, discussion 143–144
- 8 Konovalov AN, Spallone A, Mukhamedjanov DJ, Tcherekajev VA, Makhmudov UB. Trigeminal neurinomas. A series of 111 surgical cases from a single institution. Acta Neurochir (Wien) 1996; 138 (09) 1027-1035
- 9 Goel A, Muzumdar D, Raman C. Trigeminal neuroma: analysis of surgical experience with 73 cases. Neurosurgery 2003; 52 (04) 783-790 , discussion 790
- 10 Fukaya R, Yoshida K, Ohira T, Kawase T. Trigeminal schwannomas: experience with 57 cases and a review of the literature. Neurosurg Rev 2010; 34 (02) 159-171
- 11 Srinivas D, Somanna S, Ashwathnarayana CB, Bhagavatula ID. Multicompartmental trigeminal schwannomas: management strategies and outcome. Skull Base 2011; 21 (06) 351-358
- 12 Chen LF, Yang Y, Yu XG. et al. Operative management of trigeminal neuromas: an analysis of a surgical experience with 55 cases. Acta Neurochir (Wien) 2014; 156 (06) 1105-1114
- 13 Samii M, Alimohamadi M, Gerganov V. Endoscope-assisted retrosigmoid intradural suprameatal approach for surgical treatment of trigeminal schwannomas. Neurosurgery 2014;10(Suppl 4):565–575, discussion 575
- 14 Yang W, Zhao J, Han Y. et al. Long-term outcomes of facial nerve schwannomas with favorable facial nerve function: tumor growth rate is correlated with initial tumor size. Am J Otolaryngol 2015; 36 (02) 163-165
- 15 Jeong SK, Lee EJ, Hue YH, Cho YH, Kim JH, Kim CJ. A suggestion of modified classification of trigeminal schwannomas according to location, shape, and extension. Brain Tumor Res Treat 2014; 2 (02) 62-68
- 16 Makarenko S, Ye V, Akagami R. Natural history, multimodal management, and quality of life outcomes of trigeminal schwannomas. J Neurol Surg B Skull Base 2018; 79 (06) 586-592
- 17 Park HH, Hong SD, Kim YH. et al. Endoscopic transorbital and endonasal approach for trigeminal schwannomas: a retrospective multicenter analysis (KOSEN-005). J Neurosurg 2020; 133 (02) 467-476
- 18 Li M, Wang X, Chen G. et al. Trigeminal schwannoma: a single-center experience with 43 cases and review of literature. Br J Neurosurg 2021; 35 (01) 49-56
- 19 Niranjan A, Raju SS, Kano H, Flickinger JC, Lunsford LD. Clinical and imaging response to trigeminal schwannoma radiosurgery: a retrospective analysis of a 28-year experience. J Neurol Surg B Skull Base 2021; 82 (05) 491-499
- 20 Brors D, Schäfers M, Bodmer D, Draf W, Kahle G, Schick B. Postoperative magnetic resonance imaging findings after transtemporal and translabyrinthine vestibular schwannoma resection. Laryngoscope 2003; 113 (03) 420-426
Address for correspondence
Publikationsverlauf
Eingereicht: 21. Dezember 2022
Angenommen: 27. März 2023
Artikel online veröffentlicht:
17. Mai 2023
© 2023. Thieme. All rights reserved.
Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany
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References
- 1 Joshi R. Learning from eponyms: Jose Verocay and Verocay bodies, Antoni A and B areas, Nils Antoni and Schwannomas. Indian Dermatol Online J 2012; 3 (03) 215-219
- 2 Deora H, Srinivas D, Beniwal M, Vikas V, Rao KVLN, Somanna S. Rare cranial nerve schwannomas: a retrospective review of nontrigeminal, nonvestibular cranial nerve schwannomas. J Neurosci Rural Pract 2018; 9 (02) 258-263
- 3 Jefferson G. The trigeminal neurinomas with some remarks on malignant invasion of the Gasserian ganglion. Clin Neurosurg 1953; 1: 11-54
- 4 Samii M, Migliori MM, Tatagiba M, Babu R. Surgical treatment of trigeminal schwannomas. J Neurosurg 1995; 82 (05) 711-718
- 5 Yoshida K, Kawase T. Trigeminal neurinomas extending into multiple fossae: surgical methods and review of the literature. J Neurosurg 1999; 91 (02) 202-211
- 6 Ramina R, Mattei TA, Sória MG. et al. Surgical management of trigeminal schwannomas. Neurosurg Focus 2008; 25 (06) E6 , discussion E6
- 7 Wanibuchi M, Fukushima T, Zomordi AR, Nonaka Y, Friedman AH. Trigeminal schwannomas: skull base approaches and operative results in 105 patients. Neurosurgery 2012;70(1, Suppl Operative)132–143, discussion 143–144
- 8 Konovalov AN, Spallone A, Mukhamedjanov DJ, Tcherekajev VA, Makhmudov UB. Trigeminal neurinomas. A series of 111 surgical cases from a single institution. Acta Neurochir (Wien) 1996; 138 (09) 1027-1035
- 9 Goel A, Muzumdar D, Raman C. Trigeminal neuroma: analysis of surgical experience with 73 cases. Neurosurgery 2003; 52 (04) 783-790 , discussion 790
- 10 Fukaya R, Yoshida K, Ohira T, Kawase T. Trigeminal schwannomas: experience with 57 cases and a review of the literature. Neurosurg Rev 2010; 34 (02) 159-171
- 11 Srinivas D, Somanna S, Ashwathnarayana CB, Bhagavatula ID. Multicompartmental trigeminal schwannomas: management strategies and outcome. Skull Base 2011; 21 (06) 351-358
- 12 Chen LF, Yang Y, Yu XG. et al. Operative management of trigeminal neuromas: an analysis of a surgical experience with 55 cases. Acta Neurochir (Wien) 2014; 156 (06) 1105-1114
- 13 Samii M, Alimohamadi M, Gerganov V. Endoscope-assisted retrosigmoid intradural suprameatal approach for surgical treatment of trigeminal schwannomas. Neurosurgery 2014;10(Suppl 4):565–575, discussion 575
- 14 Yang W, Zhao J, Han Y. et al. Long-term outcomes of facial nerve schwannomas with favorable facial nerve function: tumor growth rate is correlated with initial tumor size. Am J Otolaryngol 2015; 36 (02) 163-165
- 15 Jeong SK, Lee EJ, Hue YH, Cho YH, Kim JH, Kim CJ. A suggestion of modified classification of trigeminal schwannomas according to location, shape, and extension. Brain Tumor Res Treat 2014; 2 (02) 62-68
- 16 Makarenko S, Ye V, Akagami R. Natural history, multimodal management, and quality of life outcomes of trigeminal schwannomas. J Neurol Surg B Skull Base 2018; 79 (06) 586-592
- 17 Park HH, Hong SD, Kim YH. et al. Endoscopic transorbital and endonasal approach for trigeminal schwannomas: a retrospective multicenter analysis (KOSEN-005). J Neurosurg 2020; 133 (02) 467-476
- 18 Li M, Wang X, Chen G. et al. Trigeminal schwannoma: a single-center experience with 43 cases and review of literature. Br J Neurosurg 2021; 35 (01) 49-56
- 19 Niranjan A, Raju SS, Kano H, Flickinger JC, Lunsford LD. Clinical and imaging response to trigeminal schwannoma radiosurgery: a retrospective analysis of a 28-year experience. J Neurol Surg B Skull Base 2021; 82 (05) 491-499
- 20 Brors D, Schäfers M, Bodmer D, Draf W, Kahle G, Schick B. Postoperative magnetic resonance imaging findings after transtemporal and translabyrinthine vestibular schwannoma resection. Laryngoscope 2003; 113 (03) 420-426







