Page 1 NF2 acoustic neuromas and the tumor neurosurgeon Fred G. Barker II, M.D. Dept Neurosurgery Mass General Hospital UCSF update - Jul 29 2021 1 no financial conflicts NCI, PCORI support use of bevacizumab for acoustic neuromas and auditory brainstem implants (ABI) in non - NF2 patients are not FDA labelled indications 2 Asthagiri AR et al Lancet ‘09 Manchester: BVS or NF fam hx + UVS or 2 secondary criteria AD high penetrance one third with no family history 12,000 in US 3 Source: NIS 1996-2000 Age at surgery for acoustic neuroma NF2 vs sporadic 4
Transcript
Page 1
NF2 acoustic neuromas and the tumor neurosurgeon
Fred G. Barker II, M.D. Dept Neurosurgery
Mass General HospitalUCSF update - Jul 29 2021
1
no financial conflictsNCI, PCORI supportuse of bevacizumab for acoustic
neuromas and auditory brainstem implants (ABI) in non-NF2 patients are not FDA labelled indications
2
Asthagiri AR et al Lancet ‘09
Manchester: BVS or NF fam hx+ UVS or 2 secondary criteriaAD high penetranceone third with no family history12,000 in US
3
Source: NIS 1996-2000
Age at surgery for acoustic neuroma NF2 vs sporadic
4
Page 2
NF2 and survivalMedian survival 62 yr (now 69 yr)Markers of severity:
age at onset (younger is worse)presence of meningiomasexperience in affected family members
Most patients will die of disease (bilatCN X palsies, compl of quadriplegia)
Evans et al., Q J Med, 304, 603-618, 1992
5
54 F new R hearing loss x 4 weeks, then mild L HL x 1 wkWRS 17% AD, 75% AS + HB2 R facial weakness
“galloping” (pseudo)-NF2
6
What’s new in NF2?QOL as top goal of treatment – often
avoiding radiation and surgery when possible as treatment has high risk of deficit
No targeted treatment as of yetRapidly improving rehabilitation of facial
paralysis and hearing loss including surgical and medical treatment (bevacizumab)
Facial reanimation -> sporadic VS as well
7
NF2 vs sporadic acousticsCompared to unilateral acoustics:NF2 patients have worse results with
observation, radiation, and surgery
Stakes for every decision are higher because bilateral deficits are devastating (CN5, 7, 8, lower nerves)
At each decision point, both past history and likely future developments deserve consideration
Multidisciplinary collaboration is essential
8
Page 3
Observation: NF2 vs sporadic
NF2 - nat hx consortiumsporadic – modified from Stangerup et al
extrameatal
intrameatal
Plotkin SR et al Otol Neurotol 2014; Stangerup et al., Otol Neurotol 2006
9
Hearing Loss – Word Recognition Score (WRS)
Surv
ival
Dist
ribu
tion
Func
tion
0.00
0.25
0.50
0.75
1.00
Months
0 10 20 30 40 50 60 70 80
STRATA: Tumor < 1cc Tumor ≥ 1ccAll Tumors
NF2 - nat hx consortium; Plotkin SR et al Otol Neurotol 2014
> 1cc
10
Radiation for NF2 VS
Radiation results for NF2 are poor compared to sporadic VS both in efficacy (tumor control) and safety (facial and trigeminal neuropathies, loss of hearing, postradiationmalignancy)
*5, 7 figures include temporary and permanent deficits
Sources: Mathieu D et al., Neurosurg 07; Kondziolka IJROBP 07
12
Page 4
Post-XRT malignancy
half of reported cases of post-XRT malignancy are NF cases (about 5% of patients being radiated) -> 20-fold elevation in risk
Baser et al estimated 14-fold elevation in CP angle malignancy after XRT based on survey of 1348 NF2 pts (10% in irradiated vs 0.7% non-irradiated)
Sources: Tanbouzi Husseini S et al., Laryngoscope 2011
Baser ME et al., Br J Cancer 2000
13
Facial results are worse in NF2
Samii et al. Neurosurg 199715% loss of anatomical loss of continuity of facial nerve76% HB grade 1 preop maintained HB 1 postop
Friedman et al. Otol Neurotol 201176% of middle fossa cases had HB1 postop
Blomstedt et al. Neurosurg 1994After facial nerve graft, 1/8 NF patients recovered to HB 3 (c/w 13/22 sporadic pts)
14
NF2 “tumors” are collision lesions
Source: Dewan R…Asthagiri A, Neuro-Oncology 2015
15
In NF2, tumor / nerve geometric relations can be unfavorable
Source: Kutz JW et al.,
Skull Base 09; MGH/MEEI
VSmeningioma
facial nervebetween tumor nodules
at surgery
16
Page 5
Facial nerve fibers within NF2 VS
embedded fibers: NF sporadicHamada et al. 4/5 0/17Jääskeläinen et al. 6/9 5/15
Source: Hamada Y et al., Surg Neurol 1997, Jääskeläinen J et al. JNS 94
17
Source: Sasaki T et al., J Neurosurg 2009
tumor
nerve
100μ
18
Facial reanimation / rehabEye care: Eyelid weights (gold or platinum)
TarsorrhaphyPunctum plug
Synkinesis, crocodile tears: botox(XII -> VII)(Temporalis “sling” – static only)(Direct repair or grafting of facial nerve)Cross-face nerve grafts, V3 motor to VIIGracilis free transfer innervated by V3 / crossed VII
L VS tumor progression s/p L gracilis flapnow at risk: L trigeminal motor (animating gracilis)
-> record EMG in gracilis and stimulate V
24
Page 7
Hearing results are worse in NF2
Friedman et al. Otol Neurotol 2011NF: 56% of those with class A
hearing preop maintained class A or B immediately postop;* 41% class D
c/w 68% class A or B, 31% class D for sporadic VS at same center
*4/17 with initial postop class A hearing had class D at last followup
25
Hearing durability in NF2Postop maintenance of hearing is worse in
NF2 and tumor recurrence is frequentOf 47 cases after middle fossa resection at
HEI, 57% had tumor recurrence (facial, cochlear) within the surgical field by average 60 mo postop24% of pts with postop class A hearing had class D at last followup (about 6 yr)
Source: Friedman et al, Otol Neurotol 2011
26
Auditory rehabilitation in NF2
Cochlear implant (if nerve intact --even after translab)
Auditory brainstem implant (ABI)Medical therapy for acoustic
neuroma (bevacizumab)
27
Cochlear implant in NF2
Pts with cochlear nerve not surgically absent are candidates; prior suboccip and concurrent translab are eligible – but op note must address this
About two-thirds of pts will achieve open set speech recognition and some can use telephone
Cochlear nerve remains source of future tumors
28
Page 8
DJL
29
FDA criteria for ABI surgery• Diagnosis of NF2• 12 years of age or older• Medically / psychologically suitable• ABI placed during 1st or 2nd tumor removal or after both tumors have been removed• No audiologic criteria• Hx of radiation – possible relative
contraindication to ABIDJL
30
Friedland et al, 1999
Left retrosigmoid approach
3 6 9 12 15 18 214 7 10 13 16 19 22
2 5 8 11 14 17 20
DJL
31
R side ABI – 10 mo old cochlear aplasia
<- rostralIAC
IX
cerebellum
Luschka ->
Typically adds 1-2 hr to tumor resection
32
Page 9
Cochlear™ ABI clinical trial92 patients - NF2 and ABI
translab approach
85-93% with sound perception, better speechreading12% with open speech set recognition with no visual cues – clearly inferior to CI-> “sleeper” ABI implantation at first side surgery (if not eligible for CI??)
DJL
33 34
Surgery - other endpoints
less frequent complete resection: 88% total resection in NF2 VS vs. 99% in sporadics (Samii)
more frequent death from surgery:higher in-hospital mortality for NF2 VS resection in US population-based study (odds ratio 14) – bilateral lower nerve palsies
Laryngoscopy before & after every NF2 post fossa craniotomy (before eating)
