J Neurol Surg B Skull Base 2024; 85(03): 267-286
DOI: 10.1055/s-0043-1768443
Original Article

Surgical Management of Large (≥3 cm) Trigeminal Schwannomas: Functional Outcomes and Approach Selection in Multicompartmental Schwannomas

Srinivas Dwarakanath
1   Department of Neurosurgery, NIMHANS, Bangalore, Karnataka, India
,
1   Department of Neurosurgery, NIMHANS, Bangalore, Karnataka, India
,
Sarthak Mehta
1   Department of Neurosurgery, NIMHANS, Bangalore, Karnataka, India
,
1   Department of Neurosurgery, NIMHANS, Bangalore, Karnataka, India
,
Arivazhagan A.
1   Department of Neurosurgery, NIMHANS, Bangalore, Karnataka, India
,
KVLN Rao
1   Department of Neurosurgery, NIMHANS, Bangalore, Karnataka, India
,
Sampath Somanna
1   Department of Neurosurgery, NIMHANS, Bangalore, Karnataka, India
› Author Affiliations
 

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.

Table 1

Classification systems for trigeminal schwannoma

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


Table 2

Approaches to trigeminal schwannomas according to location

Location

Approach

Remarks

Middle fossa

 • Extradural frontotemporal

 • Lateral basal subtemporal

Modifications depending on the extent

 1. Infratemporal-zygomatic arch

 2. Orbit-lateral orbitotomy or orbitozygomatic approach

Prefer extra-interdural approach

Posterior fossa

 • Lateral suboccipital retrosigmoid approach

 • Retrosigmoid intradural suprameatal (RIST) approach

RIST increases access to the posterior middle

fossa and paracavernous region.

Middle and posterior fossa

 • Lateral basal subtemporal extradural approach

 • Anterior transpetrosal approach.

 • Retrosigmoid intradural suprameatal (RIST) approach: suprameatal tubercle above the internal acoustic meatus drilled.

 • Presigmoid and combined petrosal approaches

Most cases do not need petrosal drilling, tumor can be followed through the mouth of Meckel's cave without drilling the petrous apex or sectioning the tentorium.

Meckel's cave

Endoscopic or extended endoscopy

 • Combine with transclival

 • Transnasal: foramen of rotundum or the inferior

  orbital fissure

 • Transorbital approach: Meckel's cave only

EEA risk

 1. CSF leak due to the skull base defect

 2. V1 neuropathy (sacrifice of the vidian nerve)

 3. Rhinosinusitis

Transorbital advantage:

 1. Low risk of corneal keratopathy.

 2. Shorter trajectory to Meckel's

 3. Direct visualization of the abducens nerve

Abbreviations: CSF, cerebrospinal fluid; EEA, endoscopic endonasal approach.


Table 3

Approaches and surgical outcomes of major microsurgical series for trigeminal schwannomas

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).

Table 4

Characteristics of the study population

Total Number of patients

83

Age

Median, IQR

33, 44–26

Gender

Males

43

Females

40

Symptoms

Seizures

1

5th Nerve

72

6th Nerve

19

7th Nerve

27

8th Nerve

24

Neurological deficits

Visual disturbances

19

5th nerve motor deficits

62

5th nerve sensory deficits

61

Motor deficits

15

Dimensions (≥3cm)

Length (median, IQR)

4, 4.9–3.5

Breadth (median, IQR)

3.5, 4.2–3.0

Consistency (n = 61)

Solid

30

Solid cystic

29

Cystic

2

Middle fossa involvement (n = 71)

Yes

65

No

6

Brain stem compression (n = 71)

Yes

58

No

13

Approach types

FTOZ

43

RMSOC

13

Subtemporal (ST)

21

Combined Petrosal (CP)

1

Endonasal (EN)

5

Follow-up

Percentage of people followed-up

66.3

Median duration

6 m

IQR

2–12 m

Trigeminal motor weakness

46

Trigeminal sensory weakness

38

Facial paralysis

31

Hearing loss

11

Residue (intraoperative impression)

Residue(postoperative MRI)

45

20

Mode of treatment of residue (n = 20)

GKRS

8

Reexploration

12

Abbreviations: FTOZ, frontotemporal-zygotomy; GKRS, Gamma Knife radiosurgery; IQR, interquartile range; MRI, magnetic resonance imaging; RMSO, retrosigmoid approach.


Table 5

Age and gender distribution

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]).

Table 6

Clinical features of study population

S.No.

Age

Gender

Trigeminal symptoms

Other cranial nerves affected

Motor symptoms

Seizures

Sensory symptoms

Motor symptoms

2nd

6th

7th

8th

1

40

Female

No

No

Yes

No

No

No

No

No

2

52

Female

Yes

Yes

Yes

Yes

No

No

No

No

3

21

Male

Yes

Yes

No

No

No

Yes

No

No

4

22

Male

Yes

Yes

No

No

Yes

Yes

No

No

5

30

Male

Yes

Yes

Yes

No

Yes

Yes

No

No

6

26

Female

Yes

Yes

No

No

Yes

Yes

No

No

7

26

Female

Yes

Yes

No

No

No

Yes

No

No

8

30

Female

Yes

Yes

No

No

No

No

No

No

9

30

Female

Yes

No

No

No

Yes

No

Yes

No

10

30

Male

Yes

Yes

No

No

Yes

No

Yes

No

11

36

Male

Yes

Yes

No

No

No

No

Yes

No

12

35

Female

Yes

Yes

No

Yes

Yes

Yes

No

No

13

53

Female

Yes

Yes

No

Yes

No

No

No

No

14

35

Female

Yes

Yes

Yes

No

Yes

Yes

No

No

15

63

Male

Yes

Yes

No

No

Yes

Yes

No

No

16

43

Female

No

Yes

No

No

Yes

No

No

No

17

47

Female

Yes

No

No

No

No

No

No

No

18

45

Female

Yes

Yes

Yes

Yes

No

No

No

No

19

32

Male

Yes

Yes

Yes

Yes

No

No

No

No

20

21

Male

Yes

Yes

No

Yes

Yes

Yes

Yes

No

21

21

Male

Yes

Yes

No

Yes

Yes

Yes

Yes

No

22

45

Female

Yes

Yes

No

Yes

No

No

No

No

23

46

Female

Yes

Yes

Yes

No

Yes

Yes

Yes

No

24

48

Female

Yes

Yes

No

Yes

Yes

Yes

No

25

40

Male

Yes

Yes

No

Yes

No

No

No

No

26

43

Male

Yes

Yes

No

No

No

No

No

No

27

17

Female

Yes

No

No

No

Yes

Yes

Yes

No

28

39

Female

Yes

Yes

Yes

No

Yes

Yes

No

No

29

48

Male

Yes

Yes

No

No

No

No

No

No

30

38

Male

Yes

Yes

No

No

No

Yes

No

No

31

47

Female

No

Yes

No

No

No

No

No

No

32

30

Male

Yes

Yes

No

Yes

No

Yes

No

No

33

31

Female

Yes

Yes

No

No

No

No

No

No

34

33

Male

Yes

No

Yes

No

Yes

No

No

No

35

33

Male

Yes

No

Yes

No

Yes

No

No

No

36

26

Male

No

Yes

Yes

No

No

No

No

37

26

Male

No

Yes

Yes

Yes

No

No

No

No

38

26

Male

No

Yes

Yes

No

No

No

No

39

38

Female

No

No

No

No

No

No

No

No

40

38

Female

Yes

Yes

No

No

No

No

No

No

41

16

Female

Yes

Yes

No

Yes

Yes

No

No

No

42

32

Male

Yes

Yes

No

No

No

No

No

No

43

31

Female

No

No

No

No

No

Yes

No

No

44

38

Female

No

Yes

Yes

No

No

No

No

No

45

63

Male

Yes

No

No

No

No

No

No

No

46

18

Male

No

No

No

No

No

No

No

No

47

18

Male

Yes

Yes

Yes

No

Yes

Yes

Yes

No

48

28

Male

No

Yes

No

No

Yes

Yes

Yes

No

49

67

Female

Yes

Yes

No

No

No

No

No

No

50

67

Female

Yes

Yes

No

No

Yes

No

No

No

51

34

Male

Yes

Yes

No

No

No

No

No

No

52

32

Male

No

Yes

No

No

No

No

No

No

53

52

Female

No

No

Yes

Yes

No

No

Yes

No

54

27

Female

No

No

No

No

No

No

No

No

55

65

Female

Yes

Yes

No

Yes

No

No

No

No

56

45

Male

No

No

No

No

No

No

No

No

57

46

Male

No

No

No

No

No

No

No

No

58

41

Female

Yes

Yes

No

No

Yes

No

Yes

No

59

24

Male

Yes

Yes

No

Yes

No

No

No

No

60

49

Male

Yes

No

No

No

Yes

Yes

No

No

61

32

Female

No

No

Yes

No

No

No

No

No

62

45

Female

Yes

Yes

Yes

Yes

Yes

Yes

No

Yes

63

32

Male

No

No

No

No

Yes

Yes

Yes

No

64

17

Male

Yes

Yes

No

No

Yes

No

Yes

No

65

19

Female

No

No

Yes

No

No

No

No

No

66

37

Male

No

Yes

No

No

Yes

Yes

No

No

67

36

Male

Yes

Yes

Yes

Yes

No

No

No

No

68

30

Male

Yes

Yes

No

No

No

No

No

No

69

18

Male

Yes

Yes

Yes

No

No

Yes

No

No

70

33

Male

Yes

No

No

No

No

No

No

No

71

66

Male

Yes

Yes

No

No

No

No

Yes

No

72

38

Male

Yes

Yes

No

No

No

No

No

No

73

38

Male

Yes

Yes

No

No

No

No

No

No

74

19

Female

Yes

Yes

No

No

No

No

No

No

75

19

Female

Yes

Yes

No

No

No

No

No

No

76

18

Male

Yes

Yes

No

No

No

No

No

No

77

31

Female

Yes

Yes

No

No

No

No

No

No

78

48

Female

Yes

No

No

No

No

No

No

No

79

20

Female

No

Yes

No

No

No

No

No

No

80

24

Male

No

Yes

No

No

No

No

No

No

81

32

Female

Yes

No

No

No

No

No

No

No

82

42

Male

Yes

Yes

No

No

No

No

No

No

83

19

Female

Yes

Yes

No

No

No

No

No

No

Table 7

Tumor characteristics in study population

S.No.

Tumor characteristics

Consistency

Length

Breadth

Height

Brainstem compression

Middle Fossa involvement

1

Solid

No

Yes

2

Solid

No

Yes

3

Solid

Yes

Yes

4

Solid

Yes

No

5

Solid

6

5.5

5

Yes

Yes

6

Solid

Yes

Yes

7

Solid

Yes

Yes

8

Solid cystic

Yes

Yes

9

Solid cystic

5.2

3.4

Yes

Yes

10

Solid

Yes

Yes

11

Solid

4

3.5

3

Yes

No

12

Solid

No

Yes

13

Solid

Yes

Yes

14

Solid

Yes

Yes

15

Solid

Yes

Yes

16

Solid

3.4

1

0.6

No

Yes

17

Solid

2

3

1.8

Yes

No

18

Yes

Yes

19

Solid

Yes

Yes

20

Solid cystic

4.5

3.5

3.5

No

Yes

21

Yes

No

22

Solid cystic

4.2

2.1

Yes

Yes

23

Solid cystic

Yes

Yes

24

Solid cystic

Yes

Yes

25

Solid cystic

No

Yes

26

Solid

No

No

27

Solid cystic

No

Yes

28

Solid cystic

Yes

Yes

29

Solid cystic

Yes

Yes

30

Solid cystic

Yes

Yes

31

Solid cystic

Yes

Yes

32

Solid cystic

Yes

Yes

33

Solid cystic

Yes

Yes

34

Solid cystic

Yes

Yes

35

Solid cystic

Yes

Yes

36

Solid cystic

Yes

Yes

37

Yes

No

38

39

40

Solid cystic

No

Yes

41

Solid cystic

Yes

Yes

42

Solid cystic

No

Yes

43

Solid cystic

Yes

Yes

44

Solid cystic

No

Yes

45

Solid

3.7

3.5

2.9

Yes

Yes

46

47

Solid cystic

Yes

Yes

48

Solid cystic

4

4

3.7

Yes

Yes

49

Cystic

4.4

2.7

2.2

Yes

Yes

50

51

Cystic

4.4

2.7

2.2

Yes

Yes

52

Solid cystic

Yes

Yes

53

Solid

No

Yes

54

Solid

4.9

3.3

Yes

Yes

55

Solid

Yes

Yes

56

Solid

3.2

3.5

4.2

Yes

Yes

57

Solid

3.2

3.5

4.2

Yes

Yes

58

Solid

4

3.3

Yes

Yes

59

Solid

Yes

Yes

60

Solid cystic

3.5

3.7

3.8

61

Solid cystic

3

2.8

Yes

Yes

62

Solid

5.2

4.5

4.5

Yes

Yes

63

Solid

Yes

Yes

64

Solid cystic

3.6

6.3

Yes

Yes

65

Solid

66

Solid

No

Yes

67

Solid

6.5

4.6

5.4

Yes

Yes

68

Solid

69

Solid

70

Solid

71

Solid cystic

5.1

3.6

4

Yes

Yes

72

Yes

Yes

73

Solid cystic

Yes

Yes

74

Solid cystic

75

Solid

Yes

Yes

76

Solid cystic

Yes

Yes

77

78

Solid cystic

Yes

Yes

79

Solid

Yes

Yes

80

Solid

Yes

Yes

81

Solid cystic

Yes

Yes

82

Solid

83

Solid cystic

Yes

Yes

Table 8

Surgery and outcomes for the cases

S.No.

Approach Used

Extent of resection (per-operative impression)

Radiologically defined residue present (size cm)

Follow-up duration (days)

Gamma Knife radiosurgery

Re-exploration

1

ST

STR

3.5 × 3

4,515

No

Yes

2

FTOZ

STR

1.5 × 1

71

Yes

No

3

FTOZ

STR

3.3 × 3

303

No

Yes

4

RMSOC

GTR

No

3,330

No

No

5

FTOZ

NTR

No

86

No

No

6

RMSOC

STR

2 × 1

814

No

Yes

7

ST

GTR

No

130

No

No

8

FTOZ

GTR

No

2,731

No

No

9

ST

STR

1 × 1

3,114

No

Yes

10

ST

STR

2 × 3

100

No

Yes

11

RMSOC

GTR

No

2,449

No

No

12

FTOZ

STR

0.5 ×  0.3

2,036

Yes

No

13

FTOZ

GTR

No

2,851

No

No

14

RMSOC

GTR

No

2,697

No

No

15

FTOZ

STR

1.1 ×  1.9

1,283

Yes

No

16

FTOZ

STR

0.3 ×  0.4

1,310

Yes

No

17

EN

NTR

No

1,462

No

No

18

FTOZ

GTR

No

177

No

No

19

ST

STR

No

82

No

No

20

ST

GTR

No

14

No

No

21

FTOZ

GTR

No

0

No

No

22

ST

GTR

No

258

No

No

23

ST

STR

2.4 ×  3.1

551

No

Yes

24

FTOZ

NTR

No

649

No

No

25

FTOZ

STR

3.4 ×  1.1

1,104

No

Yes

26

EN

NTR

No

0

No

No

27

EN

GTR

No

107

No

No

28

ST

GTR

No

1,444

No

No

29

FTOZ

STR

No

60

No

No

30

RMSOC

STR

4.1 ×  1.7

603

No

Yes

31

EN

STR

1.2 ×  2.4

674

Yes

No

32

FTOZ

GTR

No

169

No

No

33

FTOZ

GTR

No

44

No

No

34

FTOZ

NTR

No

153

No

No

35

RMSOC

STR

1.3 ×  0.3

369

Yes

No

36

EN

STR

No

0

No

No

37

FTOZ

STR

No

0

No

No

38

RMSOC

NTR

No

0

No

No

39

FTOZ

STR

4.1 ×  1.4

6

No

Yes

40

ST

GTR

No

649

No

No

41

FTOZ

STR

No

150

No

No

42

RMSOC

STR

3.2 ×  2.2

120

No

Yes

43

FTOZ

STR

1.3 ×  3.1

250

No

Yes

44

FTOZ

STR

0.2 ×  0.5

246

Yes

No

45

ST

STR

No

28

No

No

46

FTOZ

NTR

No

88

No

No

47

RMSOC

STR

No

88

No

No

48

FTOZ

GTR

No

391

No

No

49

FTOZ

NTR

No

241

No

No

50

RMSOC

GTR

No

139

No

No

51

FTOZ

NTR

No

106

No

No

52

ST

NTR

No

411

No

No

53

FTOZ

GTR

No

134

No

No

54

ST

GTR

No

50

No

No

55

ST

STR

No

5

No

No

56

FTOZ

GTR

No

0

No

No

57

FTOZ

GTR

No

0

No

No

58

CP

STR

1.2 ×  1.6

242

Yes

No

59

ST

GTR

No

37

No

No

60

RMSOC

NTR

No

0

No

No

61

ST

STR

No

0

No

No

62

FTOZ

GTR

No

0

No

No

63

FTOZ

GTR

No

0

No

No

64

FTOZ

NTR

No

0

No

No

65

FTOZ

GTR

No

0

No

No

66

ST

NTR

No

0

No

No

67

ST

GTR

No

0

No

No

68

FTOZ

NTR

No

27

No

No

69

ST

GTR

No

7

No

No

70

RMSOC

STR

1.2 ×  3.4

153

No

Yes

71

FTOZ

GTR

No

0

No

No

72

FTOZ

GTR

No

0

No

No

73

FTOZ

GTR

No

0

No

No

74

FTOZ

GTR

No

0

No

No

75

FTOZ

GTR

No

0

No

No

76

FTOZ

GTR

No

0

No

No

77

FTOZ

GTR

No

0

No

No

78

ST

GTR

No

0

No

No

79

FTOZ

GTR

No

0

No

No

80

ST

GTR

No

0

No

No

81

FTOZ

NTR

No

0

No

No

82

RMSOC

STR

No

0

No

No

83

FTOZ

GTR

No

0

No

No

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.


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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.

Zoom Image
Fig. 1 (A) Preoperative axial contrast-enhanced magnetic resonance images of a large dumbbell shaped trigeminal schwannoma extending through Meckel's cave. (B,C) Preoperative sagittal and coronal T2 magnetic resonance images showing the majority of the schwannoma in the middle fossa with preoperative displacement of the Basilar artery and posterior cerebral arteries. (D) Postoperative axial contrast-enhanced magnetic resonance images showing complete excision of the lesion via a left fronto-orbital approach. (E,F) Postoperative sagittal and coronal T2 magnetic resonance images showing complete excision of the lesion.

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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.

Zoom Image
Fig. 2 (A,B, and C) Preoperative axial, sagittal, and coronal contrast-enhanced magnetic resonance images of a large trigeminal schwannoma with avid contrast enhancement and brainstem compression. (D,E, and F) Postoperative axial, sagittal, and coronal contrast-enhanced magnetic resonance images showing decompression via a combined petrosal route and a small residue over the brain stem which was later irradiated with Gamma Knife

Video 1 A surgical video demonstrating the subtemporal interdural route for excision of large trigeminal schwannomas.


Quality:

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.

Zoom Image
Fig. 3 (A,B, and C) Preoperative axial, sagittal, and coronal contrast-enhanced magnetic resonance images of a large trigeminal schwannoma with maximum bulk in the posterior fossa and extension to middle fossa. (D,E, and F) Postoperative axial, sagittal, and coronal contrast-enhanced magnetic resonance images showing excision via a retro mastoid route with decompression of the middle fossa component via Meckel's cave and a small residue on the trigeminal nerve which remained indolent on follow-up.

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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.

Zoom Image
Fig. 4 (A,B, and C) Preoperative axial, sagittal, and coronal contrast-enhanced magnetic resonance images of a large trigeminal schwannoma with predominant component in the midline in Meckel's cave. (D,E, and F) Postoperative axial, sagittal, and coronal contrast-enhanced magnetic resonance images showing decompression via an endoscopic endonasal route with no residue.

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.

  • 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

Srinivas Dwarakanath, MCh, FACS, IFAANS
Department of Neurosurgery
NIMHANS, Bangalore, Karnataka 560029
India   

Publication History

Received: 21 December 2022

Accepted: 27 March 2023

Article published online:
17 May 2023

© 2023. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

  • 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

Zoom Image
Fig. 1 (A) Preoperative axial contrast-enhanced magnetic resonance images of a large dumbbell shaped trigeminal schwannoma extending through Meckel's cave. (B,C) Preoperative sagittal and coronal T2 magnetic resonance images showing the majority of the schwannoma in the middle fossa with preoperative displacement of the Basilar artery and posterior cerebral arteries. (D) Postoperative axial contrast-enhanced magnetic resonance images showing complete excision of the lesion via a left fronto-orbital approach. (E,F) Postoperative sagittal and coronal T2 magnetic resonance images showing complete excision of the lesion.
Zoom Image
Fig. 2 (A,B, and C) Preoperative axial, sagittal, and coronal contrast-enhanced magnetic resonance images of a large trigeminal schwannoma with avid contrast enhancement and brainstem compression. (D,E, and F) Postoperative axial, sagittal, and coronal contrast-enhanced magnetic resonance images showing decompression via a combined petrosal route and a small residue over the brain stem which was later irradiated with Gamma Knife
Zoom Image
Fig. 3 (A,B, and C) Preoperative axial, sagittal, and coronal contrast-enhanced magnetic resonance images of a large trigeminal schwannoma with maximum bulk in the posterior fossa and extension to middle fossa. (D,E, and F) Postoperative axial, sagittal, and coronal contrast-enhanced magnetic resonance images showing excision via a retro mastoid route with decompression of the middle fossa component via Meckel's cave and a small residue on the trigeminal nerve which remained indolent on follow-up.
Zoom Image
Fig. 4 (A,B, and C) Preoperative axial, sagittal, and coronal contrast-enhanced magnetic resonance images of a large trigeminal schwannoma with predominant component in the midline in Meckel's cave. (D,E, and F) Postoperative axial, sagittal, and coronal contrast-enhanced magnetic resonance images showing decompression via an endoscopic endonasal route with no residue.