referral and management guidelines for brain /cns cancers … docs/guidelines... · 2015-04-15 ·...

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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

[email protected]

[email protected]

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.


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.


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.


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.


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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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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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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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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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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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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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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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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referral and management guidelines for brain /cns cancers … docs/guidelines... · 2015-04-15 ·...

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