referral and management guidelines for brain /cns cancers … docs/guidelines... · 2015-04-15 ·...
TRANSCRIPT
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North Trent Cancer Network
Last Updated March 2012
Produced by the North Trent Brain /CNS Cancer Group
Referral and Management Guidelines for Brain /CNS Cancers within North Trent
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CONTENTS
Page
Introduction 4
Members of the Group 5
Neuro Sciences NEUROSCIENCES PATHWAY
6
1. Low grade glioma 7 1.1 PREFACE 1.2 REFERRALS TO SERVICE 1.3 INTERVAL SCANNING 1.4 SURGICAL TREATMENT 1.5 FURTHER INVESTIGATION 1.6 FURTHER MANAGEMENT 1.7 RADIOTHERAPY 1.8 CHEMOTHERAPY 1.9 CLINICAL TRIALS APPENDIX
7 8 8 9 10 10 11 11 11 12
2. High grade glioma 14
2.1 REFERRALS TO CNS SERVICE 2.2 SURGICAL TREATMENT 2.3 FURTHER INVESTIGATIONS 2.4 FURTHER MANAGEMENT 2.5 RADIOTHERAPY 2.6 CHEMOTHERAPY 2.7 TREATMENT AT PROGRESS 2.8 CLINICAL TRIALS
14 14 15 15 15 17 18 19
3. Brain Metastases 20 3.1 PREFACE 3.2 REFERRALS TO SERVICE 3.3 MDT DISCUSSIONSURGICAL 3.4 TREATMENT 3.5 FURTHER MANAGEMENT
20 21 21 22 22
4. Non Skull base meningioma 23 4.1 REFERRAL TO SERVICE 4.2 MDT DISCUSSION 4.3 TREATMENT
23 23 24
5. Skull Base 25
5.2 MDT DISCUSSION 5.5 REFERRALS TO SKULL BASE SERVICE 5.6 ANTICIPATED IMAGING 5.7 REFERAL DEADLINE FOR MDT
26 27 27 28
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5.8 PROTOCOL FOR TAKING ACTION 5.9 INDIVIDUAL TREATMENT PLANS APPENDIX PATIENT PATHWAY
28 28 37
6.Pituitary 38 4.1 LINKS TO GUIDANCE 4.2 PATIENT PATHWAY
38 39
7. Rehabilitation 41 7.1 PATHWAY 41
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Introduction These guidelines have been produced by the North Trent Brain and Central Nervous System Cancer Group. They apply to all patients treated within North Trent and Lincoln for cancers of the Brain and Central Nervous System. They contain general guidance for the management of patients including clinical assessment, investigation and treatment principles. The more common primary sites are covered in greater detail, with site-specific guidance. Guidelines for high grade glioma, low grade glioma, meningioma and skull base are included Guidelines for Rare CNS tumours – See published DOH BNOS Guidelines Guidelines for pituitary tumours are set out in the Pituitary endocrinology handbook Management guidelines for cerebral metastases
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MEMBERS OF THE GROUP
Current Membership Group:
Mr Hesham Zaki (Chair) NSSG Chair Sheffield Teaching Hospitals
Mr David Jellinek Lead Neurosciences MDT Sheffield Teaching Hospitals
Bernadette Conwill MDT co-ordinator Sheffield Teaching Hospitals
Caroline Bridgewater Lead Network Onco MDT Sheffield Teaching Hospitals
Thomas Carroll Lead Skull base MDT Sheffield Teaching Hospitals
Carolyn Wilkie Deputy Gen Manager Sheffield Teaching Hospitals
Caroline Allsop Cancer Tracker Sheffield Teaching Hospitals
Jonathan Webster Cons Endocrinologist Sheffield Teaching Hospitals
Helen Lee CNS Brain CNS Sheffield Teaching Hospitals
Hayley Williams Cancer Manager Sheffield Teaching Hospitals
Joanne Ferraby Service Manager Sheffield Teaching Hospitals
John Newell-Price Lead Pituitary MDT Sheffield Teaching Hospitals
Claire Tooth Neuro rehab Lead Sheffield Teaching Hospitals
Fiona McKevitt Cons Neurology Sheffield Teaching Hospitals
Karen Ibbotson CNS Brain CNS Sheffield Teaching Hospitals
Charles Romanowski Lead for Imaging Sheffield Teaching Hospitals
Vicky Lee Consultant Sheffield Childrens Hospital
Dinesh Chadha Lead Doncaster Doncaster
Annette Clark Macmillan CNS Barnsley
Ruth Broadhurst Palliative Care Rotherham
Jane Thornton Speech and Language Sheffield Teaching Hospitals
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1. Management Guidelines for Low Grade Glioma in North Trent and Lincoln Date: March 2012 Date for guideline review: March 2013
1.1 PREFACE
Gliomas are divided by morphological criteria into four grades. Grade I and II tumours
are termed ‘low-grade’, III and IV ‘high-grade’ or malignant tumours. Grade I tumours
are the most indolent and rarely grow or progress into higher grade tumours.
Complete surgical excision can often represent a cure. Grade II tumours though
classed as benign have the potential to become malignant. The most common grade
II neuro-epilethial tumours include astrocytoma, oligodendroglioma and mixed
oligoastrocytoma. These are generally incurable. The differentiation of glioma grade
can be difficult on initial imaging and therefore tissue diagnosis can be important. The
most common presentation of low grade gliomas (LGG) is with a seizure disorder.
Though European guidelines on the management of LGG exist (European Journal of
Neurology 2010; 17(9): 1124–1133) practices do vary as there is currently no Class I
evidence for the best management. LGG management was recently reviewed in J
Neurosurgery (J Neurosurgery 2011; 115:948-65). Policies include ‘watch and wait’
with no initial surgical intervention and regular interval scanning assessing for
radiological evidence of early malignant transformation; early surgical intervention for
histological diagnosis or maximal possible resection. Not all gliomas are suitable for
this latter option as the location of the tumour may mean surgery carries an
unacceptable risk of permanent neurological deficit. Some case series however have
reported that near total resection can improve seizure control, progression-free and
overall survival, whilst reducing the risk of malignant transformation (Class III
evidence).
All patients with grade II gliomas, including those who have undergone surgery, will
require continued surveillance as the vast majority will recur/transform into higher
grade tumours.
Patients will be stratified on initial presentation into prospective risk categories. This
will be based on validated multi centre population data (N=537) from the US based
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on the UCSF risk stratification criteria published 2008 & validated in 2009 . (J
Neurosurgery 2008; 109:817-824 & J Neurosurgery 2009; 111:203-210).
Tumour location – eloquent cortex 1 point
KPS ≤ 80 1 point
Age > 50 1 point
Maximum tumour diameter >4cms 1 point
Patients with a score 0-1 are ‘low risk’ 90% 5 years survival
Patients with a score of 2 are ‘low/intermediate’ risk 81% 5 years survival
Patients with a score of 3 are ‘high risk’ 53% 5 years survival
Patients with score of 4 are high risk 46% 5 year survival
1.2 REFERRALS TO SERVICE
All new cases will be discussed via the Neurosciences MDT (NMDT). Patients are
referred to the NMDT via a referral form (Appendix 1). Clinical history and radiology
will be reviewed and a decision made regarding future management. This will include
a ‘watch and wait’ policy with regular interval scanning (see below) or initial surgical
intervention (see below). Patients will be allocated a key worker and offered an
appointment in the low grade glioma clinic. Following initial discussion patients that
require on going follow up with interval imaging will be rediscussed at the brain/CNS
tumour cancer network MDT (CNMDT).
1.3 INTERVAL SCANNING
The initial interval scan for patients with a newly identified presumed LGG, who were
not deemed to need immediate surgery, will undergo an initial three month MRI scan
utilising the LGG protocol on the 3 Tesla MRI scanner.
Radiology MRI protocol for low-grade glioma
T2 axial imaging
Single voxel long TE Spectroscopy
Volume T1 pre-contrast
Diffusion weighted imaging (DWI)
Perfusion weighted imaging (PWI) - Dynamic susceptibility
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Volume FLAIR
T2 coronal imaging
Volume T1 post-contrast
If imaging is deemed stable at this point, that is, it demonstrates no adverse features
to suggest malignant transformation (see below), interval scanning will continue at a
minimum of every 6 months.
Radiological signs suggestive of transformation to high grade glioma
• Contrast enhancement
• Accelerated rate of growth (based on 3D volume calculation).
• High cerebral blood flow on PWI MR – not previously seen
• Increased Choline, decreased NAA on MR Spectroscopy
If patients have signs consistent with definite malignant transformation – new
contrast enhancement and/or markedly accelerated rate of growth on volume studies
- they are discussed in the NMDT for possible surgical treatment (see below).
If patients have signs consistent with possible malignant transformation – no contrast
enhancement, but a substantial acceleration in rate of growth on volume studies
and/or increased perfusion not previously seen, and/or change in spectroscopy –the
CNMDT will discuss surgical intervention. Surgical treatment may be considered at
this stage, alternatively repeat earlier interval scanning at 3 months maybe
recommended.
The definition of the terms “markedly accelerated” and “substantial acceleration” is
still undergoing evaluation and hence absolute values are not included in this
document. None-the-less, analysis of volume data forms an important aspect of the
radiological follow up of these patients.
1.4 SURGICAL TREATMENT
All presumed low grade intrinsic brain tumours will be discussed in the NMDT prior to
any surgical intervention. A decision will be made in this forum about the potential
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place of surgery at that point in clinical management. Furthermore, a provisional
decision will be made about the type (biopsy, limited resection, or radical resection)
of surgical procedure. This decision will only be acted upon by a core surgical
member of the NMDT.
Where appropriate radical resection with a goal of ≥90% debulking of the tumour will
be considered. This ≥90% debulking is based on currently available published data
on the impact of extent of resection on 10 year survival of LGG. Where
physiologically appropriate (proximity of eloquent cortex or subcortical fibre tracts) a
patient potentially suitable for radical surgery should be offered an awake
craniotomy. However, if patient preference is for the procedure to take place under
general anaesthetic then electrical cortical mapping with MEPs should be used.
All patients will undergo a detailed post operative scan to determine extent of surgical
resection at 3 months post surgery.
If histology confirms low grade glioma WHO grade I with complete resection then
surveillance with interval scanning may not be deemed necessary.
If post-surgery histology confirms low grade glioma WHO grade II then surveillance
with interval scanning will continue unless considered high-risk in which case early
radiotherapy maybe considered.
If histology demonstrates high grade glioma (WHO grade III or IV) then referral to the
oncology department will be made.
1.5 FURTHER INVESTIGATIONS
To aid surgical planning functional MRI and DTI sequences with 3D fibre tracking
may be indicated pre-operatively.
1.6 FURTHER MANAGEMENT
Patients with epilepsy will be referred to a neurologist.
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Patients with specific rehabilitation needs will be referred to the appropriate allied
health professional.
1.7 RADIOTHERAPY FOR LOW GRADE GLIOMA
Radiotherapy has not been shown to have a positive impact on overall survival in low
grade glioma, it merely increases time to tumour progression. Patients at high risk of
early progression however can be considered for radiotherapy. LGG patients with
medically refractory epilepsy may also be considered for radiotherapy.
Risk factors for early progression (each equal to 1 point)
• Age≥40
• Tumour diameter≥6cm
• Tumour crossing the midline
• Astrocytic rather than oligodendroglial type
• Permanent neurological deficit
High risk score equates to >2 points (J Clin Oncol 20:2076-2084).
Lack of 1p/19q co-deletion is an additional poor prognostic factor and though not in
prognostic scoring model may also be taken into account.
IDH mutational analysis will be performed based on immunohistochemical staining.
Radiotherapy Regime
Where appropriate, we would aim to give conformal radiotherapy, the maximum dose
for Grade II glioma 50.4Gy/26-28#
1.8 CHEMOTHERAPY
At present there are no indications for chemotherapy for LGG outside of clinical trials.
1.9 CLINICAL TRIALS
The North Trent NSSG is committed to participation in national and international
clinical trials.
There are currently no low grade glioma clinical trials open to new patients.
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Appendix 1
NEURO ONCOLOGY MDT REFERRAL PROFORMA
1. To be completed for all referrals and sent to MDT coordinator prior
to meeting
Fax no: 0114 2268795
2. Tel no: 0114 2268721
3. Date:
Consultant: Hospital:
Question to MDT:
…………………………………………………………………………………………………
…………………………………………………………………………………………………
……………………………………………………................................................................
.............
Current clinical condition (Conscious level/neurology/comorbidities):
WHO Performance status
(circle most appropriate):
Medication
Is patient currently on?
0 Normal Activity Aspirin Yes/ No
1 Capable of Light Work Warfarin Yes/No
2 Self-caring, up >50% of day Dexamethasone Yes/No - Dose
3 Limited Self-care, up <50% of day
4 In bed
……………………………………………………………………………..
.…….………………………………………………………………………
……………
……………………………………………………………………………
……………………………………………………………………………
………………
Information about CNS disease given to patient:
NHS Number: Hosp No: DoB: Patient Name: Patient Address:
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……………………………………………………………………………
….………………………………………………………………………
……………..
Has patient been told they might have cancer? Yes ���� No ����
Are dietician/physio/OT/SALT involved in the patient’s care? - (circle as necessary)
Referral completed by: …………………….. Designation/grade: ……………………
Contact Number:………………… Clinical Referral Letter attached? Yes ���� No ����
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2. Management Guidelines for High Grade Gliomas in North Trent
and Lincoln
Date: February 2012
Date for guideline review: January 2013
2.1 REFERRALS TO CNS SERVICE/IMAGING
All cases will be discussed via the Neurosciences MDT, ideally prior to
surgery unless cases present to the neurosurgeons as emergencies. Patients
are referred to the MDT via a referral form (Appendix 1).The referrer should
ensure that patients have had a CT or MRI brain with contrast.
Radiology and clinical history will be reviewed and a decision made regarding
timing and nature of surgery if appropriate. Following surgery patients will be
rediscussed with their histology results and performance status (Appendix 2).
Patients will be allocated a key worker. MDT outcomes will be communicated
to referrers by fax within 24 hours.
2.2 SURGICAL TREATMENT
Where possible (except on grounds of the need for urgent surgical
intervention) all presumed intrinsic brain tumours before any surgical
intervention will be discussed in the Neurosciences MDT. A decision will be
made in this forum about the place of surgery in clinical management. Further,
a provisional decision will be made about the type (biopsy, limited resection,
or radical resection) of surgical procedure. This information will either be
directed acted upon by a core surgical member of the Neurosciences MDT, or
if appropriate on the grounds of clinical urgency, by the duty on-call
neurosurgeon after discussion with a core surgical member of the
Neurosciences MDT.
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2.3 FURTHER INVESTIGATIONS
Grade 3 glioma
Histology of the anaplastic oligodendroglioma or mixed
astrocytic/oligodendroglioma will be forwarded for FISH testing for 1p 19q
deletions to the immnunohistochemistry laboratory at Sheffield Childrens’
Hospital.
2.4 FURTHER MANAGEMENT
Further management is dependent on the patient’s performance status and
age. Patients who are performance status 2 or better will be referred for an
opinion with the clinical oncologists regarding radiotherapy. Patients with
performance status 3 or 4 should be referred to the local palliative care
services for best supportive care.
For patients with specific needs referral to appropriate AHPs will be
recommended. Patients with epilepsy that has not been well controlled will be
referred to a neurologist.
2.5 RADIOTHERAPY
Introduction
Below is the list of referral guidelines for consideration of radiotherapy. In
addition, referral should take into consideration inclusion in clinical trials in
which other treatment options may be available.
Poor performance status, mental or physical frailty, vertigo, significant
respiratory or cardiovascular impairment may make administration of
radiotherapy inappropriate. Previous radiotherapy to the head may make
further treatment impossible.
When a decision is made for a patient to have radiotherapy a green sheet is
completed, details of previous imaging, patient positioning and which type of
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immobilisation device are required will be completed. If mri-CT fusion is
required this will be noted and whether or not the patient is in a clinical trial.
When planning radiotherapy pre- or post-operative mri image fusion will be
used wherever possible and appropriate. The dose/fractionation for
radiotherapy will take into account patient’s performance status and age.
Grade III and IV gliomas who are performance status WHO = 2 or better and
less than 70 years old.
Patients with grade IV tumours up to approx 70 years of age who are of good
performance status (WHO = 0 or 1 and where it has been agreed by the MD
team) are offered concomitant chemoradiotherapy with temozolamide.
Patients who are over 70 and of good performance status may be offered a
high dose palliative regime which is to be planned as per a radical treatment
with an immobilisation device.
Patients with PS=2 will be offered palliative radiotherapy.
All patients will be treated with 6MV photons
Doses
Grade III/IV Radical 60Gy/28-30#
High dose palliative treatment 40Gy/13-15# over 3 weeks. This dose should
mainly be for use in older (i.e.>70 years) patients who are reasonably fit.
Palliative radiotherapy 30Gy/6# over 2 weeks.
Patients will be reviewed weekly on treatment.
Patients with grade IV glioma receiving concomitant temozoalmide treatment
require height and weight and FBC when attending for their virtual simulation.
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All patients will be reviewed prior to treatment commencing for their
verification images to be checked, consent to be obtained and any
chemotherapy to be prescribed.
Patients will be reviewed weekly on treatment.
A clinic follow up will be arranged at 4 weeks for patients on concomitant
treatment and 6 weeks for most other patients.
2.6 CHEMOTHERAPY
Concomitant Temozolamide 75mg/m2 PO daily during RT (including
weekends)
Taken 1 hr prior to RT
FBC every 2/52
PCP prophylaxis (cotrimoxazole 960mg od daily)
Antiemetics
Adjuvant temozolamide
If received temozolomide concurrently with RT, PS still 0-1 and clinically not
progressed when seen at 4/52 post RT.
Temozolomide 150mg/m2 PO, D1-5 – 1st cycle, 200mg/m2 PO, D1-5 - 2nd and
subsequent cycles if 1st cycle well tolerated.
Every 4/52, 6 cycles (rpt scan after 3 and 6 cycles)
Follow Up
For patients treated radically without concomitant chemotherapy initial mri
scan should be arranged at the 6/52 clinic visit for three months from the end
of radiotherapy. Scans will usually be requested at 3 monthly intervals
thereafter for the first year and then at 6 monthly intervals although in patients
remaining well after some time consideration may be given to increasing scan
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intervals to annually. Patients with high grade gliomas will remain under the
care of the oncology team.
For patients receiving adjuvant temozolamide mri scanning will occur at
around the time of cycle 3 and on completion of 6 cycles provided the patients
remains well.
2.7 TREATMENT AT PROGRESSION
Patients with grade III or IV gliomas who have clinically deteriorated or whose
scans show progressive disease will be discussed at the network MDT to
ensure that further surgery is not appropriate. Patients who remain well
enough (PS 0-2) and have a projected life expectancy ≥ 12/52 will be
considered for chemotherapy.
First line; PCV
Procarbazine 50mg/m2 PO, D1-10 (rounded to nearest 50mg)
Lomustine (CCNU) 100mg/m2 PO, D1 (rounded to nearest 40mg, max SA of 2
Vincristine 1.4mg/m2 IV, D1 (max dose 2mg)
Every 6/52, Response assessed clinically and with repeat scan every 3
cycles, Max 6 cycles
Second line - Temozolamide
For those who did not receive temozolamide initially or have had a long
disease free interval. Consider as first line in vegetarians due to dietary
restrictions on PCV. Doses as above for adjuvant treatment, reassess
clinically and with repeat scan every 12/52
Max no. cycles not specified – may continue if good response/stable disease
and patient choice
Third line – Carboplatin
AUC 5, IV, Every 4/52, Max 6 cycles
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Fourth Line – patients living in Yorkshire and Humber only if they remain PS0-
1 can be considered for bevacizumab.
2.8 CLINICAL TRIALS
The Central Nervous System Tumour Group is committed to participation in
national and international clinical trials.
Studies that are open as at 1st March 2011 are the EORTC CATNON (BR14)
study for patients with grade III gliomas without chromosomal deletions which
means some patients will receive temozoalmide in addition to radiotherapy for
Grade III gliomas.
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3. Management Guidelines for patients with brain metastases in North Trent and Lincoln
3.1 Preface
The National Centre for Stereotactic Radiosurgery in Sheffield has been
providing treatment with the Gamma Knife for more than two decades. With
the increasing evidence presented internationally regarding the efficacy and
safety of this technology, an increasing number of patients with brain
metastases are recognised as suitable for and referred for this treatment. This
document sets out how such patients will be selected and managed. However
the vast majority of patients with brain metastases are not suitable for focal
treatment of their brain metastases and therefore most patients continue
under the management of their treating consultants without being referred to
our service. Over the last three years the number of patients with brain
metastases being discussed has increased steadily year on year. The
number treated by stereotactic surgery for the year Apri10-Mar11 was 79.
This is only a small number of the patients discussed, as a similar number
undergo surgery and many more are discussed and treated with palliative
external beam radiotherapy or offered best supportive care only.
In order to achieve a nationally uniform access to this service, in 2008 a joint
meeting was held between the Society of British Neurological Surgeons and
the National Commissioners for Specialised Services. A consensus document
was prepared and then introduced and applied by many commissioning
bodies around the country. The consensus document states that Stereotactic
Radiosurgery will be considered if all the following criteria are met:
• Decision making for treatment is made within an IOG compliant CNS
Tumours neuroscience unit based MDT. (This will happen either at the
referring centre for patients outside our network or in the neurosciences
MDT)
• The relevant IOG compliant site specific tumour MDT has confirmed
that the life expectancy from the extracranial disease is more than 6
months.
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• Karnofsky Performance Status (KPS) ≥ 70
• Treatable peripheral disease
• Pressure symptoms which would be best relieved by surgery are
excluded
• General ability to tolerate treatment
The number of cerebral metastases or the total volume of lesions present at
the time of decision making was left by the Consensus Document for the
discretion of local multidisciplinary teams. Most internationally produced
guidelines, including the recently published systematic review and evidence-
based clinical practice guideline (J Neurooncol 96:45-68, Erratum p69-70)
refer to studies where a maximum number of four metastases were used as
an entry criterion. There is evidence for the utility and cost-effectiveness of
gamma knife radiosurgery for up to 5 lesions or more (J Clin Neurosci. 2009
May;16(5):630-4, J Neurosurg. 2000 Dec;93 Suppl 3:32-6, Technol Cancer
Res Treat. 2007 Jun;6(3):153-60.
3.2 REFERRALS TO CNS SERVICE
Patients who are felt by their treating oncologists/surgeons to be appropriate
for consideration for focal treatment of their brain metastases are referred to
the neurosciences MDT via direct contact with one of the consultant oncology
MDT members or via the usual proforma. Patients from outside of the network
are often referred directly to the stereotactic radiosurgery department and
added to the MDT list by them.
NB Cancer patients in primary care who present with symptoms suspicious of
recurrence should be referred back to the existing consultant’s secretary using
the NTCN 2WW proforma via the identified contact points.
Please see Neurosciences pathway page 6 of the guidelines document which
details the process.
3.3 MDT DISCUSSION
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The neurosciences MDT has a separate subsection entitled ‘Stereo patients’
for patients with brain metastases where all of these referrals are grouped
together. This group is included weekly. This discussion only takes place once
one of the stereotactic radiosurgeons is present. The meeting is also attended
by the senior radiographer or her deputy from the stereotactic radiosurgery
team.
In order to make a decision the following information is required:
Referral letter form treating team, detailing patient’s performance status,
Mri brain with gadolinium, in the last one month,
CT C/A/P, less than 2 months old.
It is also helpful to know whether the patient is aware of the referral and
expecting to be contacted by the neurosurgical/ radiosurgical team.
Focal treatment will be offered to patients with good performance status,
treatable/stable extracranial disease and a life expectancy > six months.
It is the responsibility of the neuroscience MDT that all patients presented in
Sheffield, who fulfil the criteria for treatment with radiosurgery will be offered
an outpatient appointment with the stereotactic radiosurgery consultant team.
3.4 SURGICAL TREATMENT
Patients with treatable extracranial disease will normally be recommended
open surgery if they have an isolated large cerebellar metastasis causing
symptoms/brain stem compression and they are fit enough to go undergo
surgery.
Isolated lesions elsewhere, in similar patients will also be recommended for
surgical excision particularly if they are >3cm.
3.5 FURTHER MANAGEMENT
Once patients have been treated they will be returned back to the referring
team for all further management. If patients require retreatment in the future
they will be rediscussed in the neurosciences MDT as outlined above.
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4. Non Skull base MENINGIOMA GUIDELINES
These guidelines are intended for all non skull base meningiomas .For skull
base meningiomas please see skull base surgery operational policy.
4.1 Referral
Meningiomas are benign tumours and they are referred by 3 routes:
1. Through the emergency on call team
2. A letter of referral to a Consultant Neurosurgeon
3. Meningiomas referred directly to the Neurosciences MDT.
a) Large meningiomas presenting with mass effect should be treated
with surgical intervention within an appropriate time frame. The
patient should be assessed as soon as possible and an appropriate
treatment formulated. Surgery should be undertaken by an IOG
Compliant Consultant Neurosurgeon, with the aim of complete or
near complete excision of the meningioma if possible.
b) Small incidental meningiomas should be referred to the
Meningioma / low grade tumour surgical clinic.
4.2 MDT Discussions
All meningiomas presenting with symptomatology and mass effect should be
discussed in the MDT and an appropriate plan formulated. Small incidental
meningiomas are required to be entered on the neurosciences MDT
database. The neurosciences data manager needs to be informed and no
MDT discussion is necessary.
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4.3 Treatment
(1) WHO Grade 1 meningioma: Require treatment with surgical excision
only. If a large remnant of the meningioma has been left behind
especially if it is inside the sinus, then an appropriate serial scanning
regimen should be instituted. Initially a base line postoperative scan at
3 months followed by serial scanning typically up to 10 years. If there
is any growth of the remnant, appropriate measures should be taken
after discussion in the MDT. The options at that time would be
recurrent surgery followed by external beam radiotherapy or
stereotactic radiosurgery to the remnant.
(2) WHO Grade 2 (Atypical meningioma) there is no consensus on management
but the following guidelines are a framework. Patients will be discussed on
an individual basis in the MDT and a decision made whether it is appropriate
to give them radiotherapy following surgery or continue with serial scanning
only. Typically patients are offered an appointment with a consultant
oncologist to discuss the benefits and side effects of adjuvant radiotherapy.
The dose of radiotherapy is typically 50.4 Gy in 28 fractions.
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(3) WHO Grade 3 (Anaplastic Meningiosarcoma): Treatment consists of
full surgical excision followed by external beam radiotherapy to the pre-
op tumour bed and any residual mass.
The dose of radiotherapy is 54Gy in 30 fractions.
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5. Skull Base
5.1 All NHS patients with a suspected or newly diagnosed tumour, either
benign or cancerous, and private patients for which STH provides some
contribution to their care, will be referred into the Skull Base multidisciplinary
team, and dealt with in a skull base multidisciplinary clinic, skull base MDT
meeting, or both. Skull base tumour patients that are not formally reviewed in
the skull base MDT meeting are managed to a skull base MDT-agreed policy
as laid out in the Pathway Design document (see Appendix 1 and Appendix
6).
5.2 The following patients will be formally reviewed at the MDT Meeting
(measure 11-2K-218):
5.2.1 All patients having a known or potential malignant
neoplasm of the skull base on initial presentation.
5.2.2 All patients having a malignant neoplasm abutting the
skull base for which planned resective surgery would involve
skull base expertise for clearance.
5.2.3 All patients that have undergone surgery for benign
tumours of the skull base (e.g., meningiomas, schwannomas)
and for which histology and a baseline post-op scan available.
5.2.4 All patients that have undergone surgery for malignant
tumours involving the skull base and for which histology is
available.
5.2.5 All patients having disorders involving the skull base that
do not fit to agreed management protocols, on completion of
initial diagnostic work-up.
5.2.6 All patients undergoing interval imaging for which
subsequent issues of concern do not fit to agreed management
protocols.
5.2.7 Any other patients having disorders of the skull base as
considered appropriate by individual Skull Base MDT members.
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5.3 All patients aged 16-24 inclusive will be discussed at both the SB MDT
and the Teenage & Young Adults MDT.
5.4 All patients whose skull base cancer histological diagnosis is relevant
to other MDTs, e.g., head and neck, sarcoma, melanoma, will also be
discussed at these other MDTs both prior and following any Skull Base MDT-
led interventions.
5.5 How to refer to the Sheffield Skull Base MDT
The contact point for the skull base service as listed on the STH website skull
base MDT page www.sth.nhs.uk/neurosciences/neurosurgery/sheffield-skull-
base-group is:
Mr Thomas Carroll
Consultant Neurosurgeon
Department of Neurosurgery
Royal Hallamshire Hospital
Glossop Road
Sheffield
S10 2JF
Tel 0114 2712192
Fax 0114 2765925
Mr T Carroll can be contacted after-hours through the STH switchboard. In
addition to the above, an alternate means of referral, in particular if for
specifically MDT Meeting discussion, is to the Skull Base MDT Coordinator:
� Tel 0114 2712010, Miss Caroline Allsop, Skull Base MDT Coordinator
� Fax 0114 2268795, for the attention of the Skull Base MDT Coordinator
� Via the NHS.net generic account (sht-tr.Cancer-
[email protected]), for the attention of the Skull Base MDT
Coordinator.
5.6 Anticipated imaging on initial patient referral
The minimum imaging modality for referral is a CT scan of head for an
intracranial base of skull tumour or a CT of paranasal sinuses or temporal
bones for a potential malignancy involving the base of skull. In the specific
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context of asymmetric hearing loss where the concern is the possibility of an
acoustic neuroma (vestibular schwannoma) tumour, an MRI of 'IAMs' would
be expected. Any additional investigation recommendations by the Skull Base
MDT would be on a case by case basis or would be arranged directly by the
Skull Base MDT on receipt of referral.
5.7 The referral deadline for the MDT Meeting is Friday at midday.
5.8 Protocol for taking action between meetings (measure 11-2K-214)
The following applies to patients with skull base tumours for which referral to
the skull base MDT meeting is required as per the MDT-agreed Pathway
Design document. It may be necessary for patients that would normally be
expected to be discussed at the MDT meeting to have decisions made
concerning their results and/or their treatment plans prior to the next MDT
meeting. Such discussions will be subsequently endorsed at the next MDT
meeting. Such actions and discussion outside the MDT meeting are formally
recorded in the notes, e.g., the specialist MDT clinic letter copied to patient,
GP, referring clinician, and involved SB MDT members.
5.9 Individual patient’s treatment plans (measure 11-2k-227)
At the MDT Meeting an agenda of the patients discussed will be recorded
including working diagnosis, outcomes and treatment plans, inputted onto the
MDT database by the MDT Facilitator. The working diagnosis, outcomes and
treatment plans will be separately recorded in an individual patient’s notes.
For patients managed to protocol as per Pathway Design document, e.g., with
a small acoustic neuroma, the working diagnosis, outcome, and treatment
plan will be recorded in the relevant skull base multidisciplinary clinic letter
and copied to patient, GP, referring clinician, and relevant Skull Base MDT
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members
17/03/2011 Skull Base Patient Pathway
2
Skull Base Service
Structure
MDMManaged by Mr T Carroll, Clinical Lead, and Ms Caroline Allsop, Skull Base MDT Coordinator
Anterior Skull Base (TACAS/TAWAS) Clinic• Mr T Westin/Mr Showkat Mirza, Consultant ENT Surgeons• Mr T Carroll/Mr S Sinha, Consultant Neurosurgeons• Mr A Yousefpour, Consultant Maxillofacial surgeon
Lateral Skull Base (TACEN) Clinic• Mr T Carroll, Consultant Neurosurgeon• Mr M Yardley, Consultant ENT Surgeon
Ad hoc ‘Office’ Clinic(Urgent appointments)
H&N Cancer Clinic• Radiation oncologist• Mr T Westin/Mr L Durham, Consultant ENT Surgeon• Mr A Yousefpour, Consultant Maxillofacial Surgeon
Emergency Admission/Ward Review
Management to Protocol/Patient Choice
Management PlanManagement Plan
OR
Decided by:
.
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17/03/2011 Skull Base Patient Pathway
3
Cases to be discussed in Skull Base MDM
1. All patients having a known or potential malignant neoplasm involving the skull base on initial presentation.
2. All patients having a malignant neoplasm abutting the skull base for which planned resective surgery would involve skull base expertise for clearance.
3. All patients that have undergone surgery for benign tumours of the skull base (e.g., meningiomas, schwannomas) and for which histology and a baseline post-op scan available.
4. All patients that have undergone surgery for malignant tumours involving the skull base and for which histology is available.
5. All patients having disorders involving the skull base that do not fit to agreed management protocols, on completion of initial diagnosticwork-up.
6. All patients undergoing interval imaging for which subsequent issues of concern do not fit to agreed management protocols.
7. Any other patients having disorders of the skull base as considered appropriate by individual Skull Base MDT members.
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17/03/2011 Skull Base Patient Pathway
5
Care Pathway: Anterior Skull Base
Malignancy
†Surgery for cavernous sinus involvement only (1) if salvage surgery following good response
to chemoradiotherapy and with disappearance of disease from cavernous sinus or (2) if neurotropic spread in a low grade malignancy (e.g., adenoid cystic carcinoma).
Disease involving:
• Orbital apex
• Pterygopalatine fossa• Foramen ovale
• Cavernous sinus†
‘‘MeckelMeckel’’s Cave or Cavernous sinus exenterations Cave or Cavernous sinus exenteration’’
(either from below or trans(either from below or trans--cranial,cranial,
internal carotid not resected)internal carotid not resected)
Disease involving infra-/
superficial temporal fossa
Fossa ClearanceFossa Clearance
Disease involving ethmoids/nasopharyngeal roof/anterior sphenoid(e.g., ethmoidal squamous cell carcinoma)
Endoscope assisted anterior cranial fossa floor resectionEndoscope assisted anterior cranial fossa floor resection
• The appropriate skull base resection is combined with the head & neck surgical procedure relevant to the pathology, e.g., neck dissection, maxillectomy,
parotidectomy. A neck dissection, if no free flap anastamosis, will generally be delayed until a second stage.
• A lumbar drain is used intra-operatively but not used post-operatively because of
risk of ‘brain sag’ unless a B2-transferring positive CSF leak is demonstrated beyond
48hrs post-operatively.
• Cranial compartment closure (i.e., regional or free flap) is determined on an
individual patient basis by size and location of defect, previous local radiotherapy, intact local vascular circulation, and age/general health of patient. Pedicled
pericranium is always mobilised. Midline nasal roof defects are closed with
pericranium/galea, sutured in place. Mobilised temporalis, e.g., if more extended skull base resection and orbital exenteration, is preferred to a rectus free flap unless, e.g.,
resection specimen includes infra-/superficial temporal fossae or maxillary artery.
Disease involving orbit
Orbital exenterationOrbital exenteration
+/-
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17/03/2011 Skull Base Patient Pathway
6
Lateral Skull Base Malignancy
• All temporal bone squamous cell carcinomas should be worked up for petrosectomy, irrespective of whether apparently initially confined to external auditory canal or extending beyond temporal bone to involve neck or internal carotid/jugular vessels (the only contraindications are systemic metastasis, brain invasion as manifested by high signal in brain on T2 MRI, patient age and general health issues).
• Defect reconstruction preference is for a rectus abdominis free flap, unless smaller defect suitable for mobilised temporalis. Note a pedicled pericranial flap is always mobilised.
• Surgery is generally in two stages:– Tues/Stage 1 …tracheostomy/parotidectomy/neck dissection
– Thurs/Stage 2 …neuronavigation-assisted petrosectomy with rectus free flap
• Extended lumbar CSF drainage and neck wound drainage to minimise CSFoma and seroma respectively.
• Facial palsy care issues. Subsequent eye lubricants, gold-weight insertion, and facial sling for obligate facial palsy.
• Radiotherapy unless free margins obtained in en bloc specimen.
• Titanium screw implants/ear prosthesis if required.
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17/03/2011 Skull Base Patient Pathway
7
Care Pathway: Acoustic Neuromas• Lateral Skull Base Clinic (TACEN). All patients are followed-up in a joint lateral skull base clinic,
that occurs alternate weeks, by Mr T Carroll, consultant neurosurgeon, and Mr M Yardley, consultant ENT surgeon, with on-site access to audiology and audiovestibular rehabilitation.
• Small acoustic neuromas. All patients with small acoustic neuromas, i.e., intracanalicular +/- CP-angle component ≤1.5cm, to be given choice of interval imaging (usually MRI), gamma knife radiosurgery, or open surgery. A specific information sheet is provided to the patient to facilitate patient choice. These patients are managed to protocol as per specific information sheet and are not routinely discussed in the skull base MDM.
• Medium acoustic neuromas. Treatment is recommended for all patients with an acoustic neuroma of size in the CP-angle between 1.5 and 3cm. Treatment choices are gamma knife radiosurgery or open surgery as per patient choice.
• Large/giant acoustic neuromas. All patients with acoustic neuroma tumours 3cm are more are ingeneral considered for surgery rather than radiosurgery (occasional exceptions depend on patient age and general health).
• Specific other surgical indications. Surgery may be specifically recommended for patients with acoustic neuromas less than 3cm CP-angle diameter if refractive severe vertigo (specifically a trans-lab approach) or refractive severe trigeminal neuralgia. Surgery is not offered for the purposes of hearing preservation.
• Surgical approaches are either trans-lab or retromastoid and depend on individual patient/tumour anatomy.
• Facial nerve-associated tumour remnants. Although open surgery aims for a gross total resection, this is not to be at the expense of facial nerve function. In general, our preference is for a tumour remnant to be left on the facial nerve where the facial nerve is otherwise considered to be at risk. Such a facial nerve-related tumour remnant is followed with annual MR imaging and subject to gamma knife radiosurgery in the event of radiologic progression. Early radiosurgical treatment of the remnant may be indicated in specific clinical circumstances or on the basis of patient choice.
• Urgency. All patients listed for surgery with tumours ≥3cm CP-angle diameter, hydrocephalus/tonsillar descent, or refractive severe trigeminal neuralgia should be considered ‘urgent’. All patients, unless designated with a level of emergency to warrant direct admission or ad hoc office attendance, are seen in the next TACEN clinic following receipt of referral
• Imaging follow-up. All patients, irrespective of management choice, are subject to a minimum imaging monitoring period of ten years, but with the periods between interval imaging dependent on clinical circumstance. All patients undergoing surgery undergo a post-operative baseline MRI scan approximately three months following their surgery.
• Specific rehabilitation resources, dependent on patient choice and treatment outcome, are insertion of bone anchored hearing aid and facial reanimation (eyelid gold weight, static oral sling, cross-facial nerve graft).
• NF2 patients are managed as per National Commissioning Group recommendations, including in a local periodic multidisciplinary NF clinic.
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17/03/2011 Skull Base Patient Pathway
9
Care Pathway: Skull Base Meningiomas/Symptomatic
Meningioma location†
Suprasellar (visual loss)
Orbital roof osteotomyOrbital roof osteotomy
or transcranial endoscopicor transcranial endoscopic
Anterior clinoid
OrbitoOrbito--zygomaticzygomatic
Planum sphenoidale
Subfrontal osteotomySubfrontal osteotomy
Sphenoid wing
FrontoFronto--temporaltemporal
Clival/petroclival
Staged/combined approachesStaged/combined approachesVenous/CP-angle
RetromastoidRetromastoid
Tumour clearance not at expense of neurovascular structures
Interval imaging for tumour remnant progression or for tumour recurrence
Radiosurgery for recurrence or growing remnant
Radiosurgery to post-op tumour remnant if:• WHO grade 1 and residual tumour component can be demonstrated to have shown radiological progression• WHO grade 2• WHO grade 3
Radiotherapy if:• WHO grade 2, tumour remnant is demonstrated, and is not suitable for radiosurgery• WHO grade 3 irrespective of whether tumour remnant is demonstrated or not
Baseline post-op MRI scan at three months post-surgery
Skull base-specific rehabilitatory issues• Superficial temporal fossa fat injection• Paralytic squint (prisms, squint surgery)• Facial nerve (lubricants, eyelid gold weight, static oral sling, cross-facial nerve graft)• Lower cranial nerve (swallow assessment, tracheostomy, PEG, vocal cord injection)
Orbitosphenoid
Orbital margin osteotomyOrbital margin osteotomy
with lateral orbital wallwith lateral orbital wall
reconstructionreconstruction
†Tumour location
Surgical approachSurgical approach
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17/03/2011 Skull Base Patient Pathway
11
Care Pathway: Chordoma & Chondrosarcoma
Skull base petroclival or clival bony-involving tumour
Blood prolactin level to exclude atypical prolactinoma
Consider biopsy if accessible through sphenoid sinus
Skull base endoscopic resection
Endoscopic-assistedwith appropriate trans-cranial approach
(depending on extent/location of intracranial disease)
Chondrosarcoma Chordoma
Remnant/recurrence Remnant/recurrence
Radiosurgery Radiosurgery +/- proton therapy
Baseline post-op MRI at two monthsfollowed by interval MR imaging
Or
Or
Skull base-specific rehabilitatory issues• Paralytic squint (prisms, squint surgery)• Facial nerve (lubricants, eyelid gold
weight, static oral sling, cross-facial nerve graft)• Lower cranial nerve (swallow assessment, tracheostomy, PEG, vocal cord injection)
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17/03/2011 Skull Base Patient Pathway
12
Additional General Principals of Care• Patient/Carer access to information. All patients are copied into clinic letters and discharge
summaries, with objective of any operation and associated risks clearly stated. Information sheets are provided where appropriate (e.g., Craniotomy Information Sheet, Large Acoustic Neuroma Information Sheet, Small Acoustic Neuroma Information Sheet).
• Communication of interval scan results. Patients can request to have interval imaging reports posted or emailed to them or their GP in advance of any clinician-dictated letter or clinic appointment detailing results. Their imaging results and the significance of their results are communicated in either a clinician-dictated letter if there are no specific concerns or at an early clinic appointment (next relevant specialist clinic appointment) if there are concerns.
• Option for non-clinic attendance during interval imaging follow-up. Where there is no specific concerns, patients are given the option of next interval scan without the requirement for clinic attendance.
• Contact points for patients undergoing skull base surgery. All skull base surgery patients to receive consultant neurosurgeon mobile phone contact prior to discharge. All skull base surgery patients to receive neurosurgery ward contact details prior to discharge. All skull base surgery patients will have also received appropriate specialist nurse contact details.
• Initial imaging work-up to generally include skull base CT and post-contrast MRI (+/- fat suppression). In particular, the specific question should be asked of any probable benign skull base pathology (e.g., meningioma, schwannoma), to what extent does the tumour extend outside the cranial compartment.
• Angiography and embolisation may be required pre-operatively for some skull base pathologies (juvenile angiofibromas, glomus tumours, some meningiomas).
• Specific neuroendocrine assessment should be performed for parasellar/sphenoid/clival tumours (prolactin, pituitary function) depending on clinical circumstances, e.g., post-op, and for all jugular foramen tumours presumed to be neuro-endocrine in origin, i.e., glomus (urinary cathecholamines).
• Surgical dissection to be avoided for benign tumours in cavernous sinus and jugular foramen. Radiosurgery is treatment of choice in these areas.
• Reconstruction of the CSF cranial compartment is done using vascularised tissue (e.g., pericranium, mucosa, temporalis, free flaps). Dural substitutes are not used.
• Craniofacial skeleton reconstruction may a consideration for some skull base operations.Stereolithographic model generation from volume CT scans with custom-made titanium prostheses by anaplastology may be required to surgically complete the craniofacial skeleton.
• Role of adjuvant radiotherapy is not clear for benign spectrum tumours of skull base.– MDT consensus is that radiotherapy is not to be used at all for WHO grade 1/benign
meningiomas.– MDT consensus is that radiotherapy is not to be routinely used in WHO grade 2/atypical
meningiomas. For grossly resected WHO grade 2/atypical meningiomas, radiosurgery only for any imaging-demonstrated local tumour recurrences. For subtotally resected WHO grade 2/atypical meningiomas, radiosurgery to tumour remnant, then radiosurgery to any further imaging-demonstrated local tumour recurrences (radiotherapy only if post-op remnant is unsuitable for radiosurgery).
– Radiotherapy may have a role in optic nerve sheath meningiomas and large glomus tumours not amenable to radiosurgery.
• Role of proton therapy is as per national guidance (see http://www.specialisedservices.nhs.uk/document/guidance-referral-patients-abroad-nhs-proton), i.e., for chordoma and paediatric rhabdomyosarcoma and Ewing’s sarcoma.
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Appendix 1 Skull Base Patient Pathway
Process
Trigger
Alternative process
Decision
Document
Predefined process
Data
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6 Pituitary
Guidance for pituitary follows the National guidance which can be accessed visa the internet links set out below
Pituitary incidentaloma:
Guidelines: http://www.ncbi.nlm.nih.gov/pubmed/21474686
Editorial: http://www.ncbi.nlm.nih.gov/pubmed/21474690
Aploplexy:
Guidelines: http://www.ncbi.nlm.nih.gov/pubmed/21044119
Cushing’s:
Diagnosis: http://www.ncbi.nlm.nih.gov/pubmed/18334580
Treatment: http://www.ncbi.nlm.nih.gov/pubmed/18413427
Acromegaly:
Diagnosis for cure: http://www.ncbi.nlm.nih.gov/pubmed/20410227
Prolactinoma:
http://www.ncbi.nlm.nih.gov/pubmed/21296991
Growth Hormone:
http://guidance.nice.org.uk/TA64
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