Population Study
(Table 1)
Table 1
Population study characteristics.
Characteristic
|
Nb
|
M/F (%)
|
5 (62.5) / 3 (37.5)
|
Mean age in years (range)
|
60 (44–69)
|
Hist. of prev. MS (delay in months)
|
1 (42)
|
Hist. of prev. GKRS
|
0
|
NF2 (%)
|
1 (12.5)
|
Clinical features (%)
balanced instability
cerebellar ataxia
headaches
hydrocephalus
Trigeminal hypoesthesia
diplopia (CN VI)
Facial weakness
hearing loss (CN VIII)
LCN (dysphagia & dysphonia)
Hypoglossal (tongue paralysis)
|
3 (37.5)
2 (25)
4 (50)
0
3 (37.5)
1 (12.5)
2 (25)
4 (50)
3 (37.5)
2 (25)
|
Radiological features
Mean diameter in mm (range) / median
Mean volume in cc (range) / median
|
33 (26–51) / 29
10 (4–25) / 7
|
Surgical Approach (%)
TL
RS
|
1 (12.5)
7 (87.5)
|
Extent of Resection (%)
GTR
NTR
PR
|
3 (37.5)
3 (37.5)
2 (25)
|
Postop vol Residue in cc
NTR group
PR group
|
0.25
2.42
|
Mean Follow-Up in months / median
|
91 (25–170) / 89
|
M: male. F: female. GTR: gross total resection. MS: microsurgery. NTR: near total resection. PR: partial resection. RS: retrosigmoid approach. TL: translabyrinthine approach. |
The study group was composed of 8 patients of which 5 were women (62.5%) and 3 men (37.5%). Median age at time of surgery was 60 years old (range 44–69).
One patient (12.5%) had been previously operated on in another center through an enlarged translabyrinthine approach (see Fig. 2). She had received a partial resection with a voluminous tumor residue. The sequential MR images showed evidence of progressive tumor growth with significant mass effect on the brainstem. She underwent salvage tumor resection 42 months after the first surgery.
One patient had clinical presentation highly suggestive of schwannomatosis. He harbored a LCN schwannoma in association with multiple schwannomas of the cauda equina. One patient had a confirmed medical history of neurofibromatosis type 2 (NF2). One other patient presented a contralateral vestibular schwannoma, but did not meet the Manchester’s criteria to conclude to NF2.
Presenting symptoms
(Table 1)
Four patients (50%) suffered from headache, but none presented symptoms related to hydrocephalus. Three (37.5%) patients reported balanced instability, 2 (25%) presented with cerebellar ataxia.
Only three patients (37.5%) presented with preoperative lower cranial nerves disturbances, clinically resulting in dysphagia and dysphonia in all of them (CN IX & X) in association with tongue paralysis (CN XII) in two patient.
Two patients (25%) reported facial hypoesthesia (CN V). Diplopia due to CN VI palsy was observed in one patient (12.5%). Two patients showed facial weakness (HB Grades 3 & 4) associated with intermedius nerve disturbances. One had been previously operated on in another center and the other one was the NF2 patient. These patients and two others (4 patients – 50%) showed preoperative non-serviceable hearing.
Radiological Features
(Table 1)
The mean largest cross sectional tumor diameter was 33mm (range 26–51/ median 29 mm); the mean tumor volume was 10 cc (range 4–25 / median 7 cc). The tumor appeared as a well-defined lesion homogeneously enhanced by Gadolinium in T1 weighted MRI in most patient. The MRI study showed evidence of jugular foramen involvement in five patients (62.5%) including two with a voluminous extracranial component, while the 3 others harbored LCN schwannomas which strictly developed in the CPA. One tumor presented a cystic component. No patient presented with radiological hydrocephalus. According to the Pellet et al. grading system [9], all 6 patients who presented with mixt nerves schwannomas harbored type A tumors (primilarly intracranial, with minimal extension to the JF). The two patients who harbored hypoglossal schwannomas presented with dumbbell-shaped tumors (with intra & extracranial extension).
Surgical Features
(Figs. 3&4 - Table 1)
Seven patients were operated on through a modified retrosigmoid approach (87.5%). The last patient had been previously operated on through an enlarged translabyrinthine approach.
The schwannoma originated from the glossopharyngeal nerve in 2 (25%), the vagus nerve in 4 (50%) and the hypoglossal nerve in 2 (25%) patients. Gross total resection (GTR) could be safely achieved in three patients (37.5%). Three patients underwent a near total resection. In those configurations, the tumor arose from CN IX & X. The glossopharyngeal nerve, of which the schwannoma was originating, could not be anatomically preserved during surgery in one case. A small tumor residue (0.16cc) was left against the choroid plexus in that case. In another case, a little tumor remnant (0.07cc) was left against CN IX because of strong adherences. A tumor remnant was left into the Jugular foramen in the last case (0.52cc). The mean postoperative tumor residue volume in the NTR group was 0.25 cc (0.07–0.52 cc). The two patients who harbored a schwannoma with a voluminous extracranial component underwent a partial tumor resection (25%). Both originated from CN XII. There was no attempt to enter into the hypoglossal canal neither to remove the extracranial part of the tumor (extracranial tumor residues: 2.18 & 2.69 cc).
Complications:
The mean length of hospital stay was 8 days (range 5–14). Three patients (37.5%) required postoperative total parental nutrition, with gradual resumption of oral feeding within a week. No postoperative hematoma, CSF leak, meningitis, or hydrocephalus occurred. The NF2 patient died seven years after surgery. Death was caused by respiratory distress syndrome related to a thoracic tumor.
Cranial Nerve Preservation: (Table 2)
Table 2: Individual patient clinical presentation and cranial nerve outcome.
Patient
|
Age
|
Medical History
|
CN Origin
|
Clinical Presentation
|
Early postoperative new CN deficit
|
6 months postoperative CN outcome
|
1
|
44
|
NF2
|
X
|
CN V, VII (Grade 4), VIIbis, VIII, Cereb ataxia
|
CN X (dysphagia/dysphonia)
|
Total recovery CN V, CN X
Persistant CN VII, VIIbis, VIII
|
2
|
60
|
|
XII
|
CN V, VI, VIII,
IX, X, XII, Cereb ataxia
|
CN VII (Grade 5)
|
Total recovery CN VI, IX, X
Persistant CN V, VIII, XII
|
3
|
61
|
VS Koos 3 (contralateral)
|
IX
|
Headaches
|
CN X (dysphagia)
|
Total recovery CN X
|
4
|
55
|
|
X
|
CN IX, X
|
Worsening CN X (dysphagia/dysphonia)
|
Total recovery CN IX, X
|
5
|
56
|
Surgery 2011
|
XII
|
CN VII (Grade 3), VIIbis, VIII, X, XII
|
|
Improvement CN X,
Persistant CN VII, VIIbis, XII
|
6
|
68
|
|
X
|
Cereb ataxia
|
CN VIII, CN X (dysphagia/dysphonia)
|
Persistant CN X (slight dysphagia with solids)
|
7
|
69
|
|
IX
|
CN VIII, Cereb ataxia
|
CN X (dysphagia/dysphonia)
|
Persistant CN VIII,
CN X (gastrostomy)
|
8
|
68
|
Schwannomatosis
|
X
|
Asymptomatic
|
CN X (dysphonia)
|
Total recovery CN X
|
Cereb: cerebellar. CN: cranial nerve. VS: vestibular schwannoma.
Table 3: Individual patient surgical findings and tumor control outcome.
Patient
|
Age
|
Medical History
|
Tumor diameter (in mm)
|
Surgical Approach
|
CN Origin
|
Extent of Resection
|
Tumor Residue (volume)
|
Adjuvant Treatment
|
Follow-up (months)
|
Tumor Residue Last FU
|
1
|
44
|
NF2
|
28
|
RS
|
X
|
NTR
|
0,075
|
|
84
|
0,059
|
2
|
60
|
|
40
|
RS
|
XII
|
PR
|
2,808
|
GKRS
|
170
|
2,690
|
3
|
61
|
VS Koos 3
|
26
|
RS
|
IX
|
GTR
|
0
|
|
131
|
0
|
4
|
55
|
|
30
|
RS
|
X
|
GTR
|
0
|
|
140
|
0
|
5
|
56
|
Surgery 2011
|
51
|
TL
|
XII
|
PR
|
2,136
|
GKRS
|
94
|
1,593
|
6
|
68
|
|
27
|
RS
|
X
|
GTR
|
0
|
|
34
|
0
|
7
|
69
|
|
35
|
RS
|
IX
|
NTR
|
0,518
|
|
32
|
0,449
|
8
|
68
|
Schwannomatosis
|
28
|
RS
|
X
|
NTR
|
0,161
|
|
14
|
0,136
|
CN: cranial nerve. GTR: gross total resection. NTR: near total resection. PR: partial resection.
Lower cranial nerves:
Of the three patients who presented with preoperative CN IX & X injuries, one totally recovered in the early postoperative period, while the 2 others experienced transient worsening of the symptoms in the early postoperative period. Both recovered within 6 months after surgery.
All of the five patients freed from any preoperative CNs IX & X symptoms experienced transient (80%) or definitive (one patient − 20%) disturbances after surgery. They all improved within 3 to 6 months but one who retained long term laryngeal and pharyngeal paralysis in association with hypertonia of the upper esophageal sphincter. This patient harbored a schwannoma originating from the glossopharyngeal nerve, which could not be anatomically preserved during surgery. He required long term gastrostomy feeding tube.
The two patients who presented with preoperative hypoglossal nerve palsy harbored schwannomas originating from CN XII. The anatomical preservation of the nerve could not be achieved during surgery. They retained hemi-tongue paralysis. No patient experienced new postoperative hypoglossal palsy.
Cochlear nerve:
One patient experienced postoperative cophosis. None of the 4 patients who presented with hearing loss recovered serviceable hearing after surgery. As a reminder, one had been previously operated through an enlarged translabyrinthine approach, and one other was a NF2 patient.
Facial nerve:
Immediately after surgery, among the 6 patients with normal preoperative FN function, 5 (83%) retained a HB Grade I FN function, while 1 patient (16%) harbored a severe HB Grade V postoperative FN palsy. At last follow-up examination, he retained long-term intermediate HB Grade III FN function and developed a crocodile tears syndrome.
None of the 2 patients who presented with preoperative FN palsy recovered after surgery. One of these patients developed postoperative hemifacial spasm that required specialized treatment with botulinum toxin.
Other cranial nerves:
The patient suffering from preoperative diplopia due to CN VI palsy was released from this symptom 6 months after surgery. Of the two patients who reported facial hypoesthesia, one recovered while the other one improved after surgery. None of the patients freed from this symptom before surgery have developed a postoperative facial hypoesthesia.
Tumor Control: (Table 3)
The mean duration of radiological follow-up was 91 months (range 25–170 / median 89 months). Of the three patients who had received GTR, none showed evidence of tumor recurrence after surgery. The 3 patients who underwent NTR had been allocated into a Wait-&-rescan policy. Two patients had received additional GKRS of the extracranial part of the tumor residue (respectively 2.14 and 2.69cc) 6 months after surgery. Both experienced tumor volume decreasing after GKRS. Tumor control was achieved in 100% of cases. No patient presented evidence of regrowth of the tumor residue during the follow-up period.