PRINCESS MARGARET CANCER CENTRE CLINICAL PRACTICE GUIDELINES

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1 PRINCESS MARGARET CANCER CENTRE CLINICAL PRACTICE GUIDELINES CENTRAL NERVOUS SYSTEM EPENDYMOMA Last Revision Date July

2 CNS Site Group Ependymoma Author: Dr. Norm Laperriere 1. INTRODUCTION 3 2. PREVENTION 3 3. SCREENING AND EARLY DETECTION 3 4. DIAGNOSIS AND PATHOLOGY 3 5. MANAGEMENT MANAGEMENT ALGORITHMS SURGERY CHEMOTHERAPY RADIATION THERAPY 4 6. ONCOLOGY NURSING PRACTICE 5 7. SUPPORTIVE CARE PATIENT EDUCATION PSYCHOSOCIAL CARE SYMPTOM MANAGEMENT CLINICAL NUTRITION PALLIATIVE CARE REHABILITATION 6 8. FOLLOW-UP CARE 6 Last Revision Date July

3 Ependymoma 1. Introduction ependymoma of the brain and spine in adults spine is far more common in adults, intracranial more common in children account for 5% of gliomas in adults This document is intended for use by members of the Central Nervous System site group of the Princess Margaret Hospital/University Health Network. The guidelines in this document are meant as a guide only, and are not meant to be prescriptive. There exists a multitude of individual factors, prognostic factors and peculiarities in any individual case, and for that reason the ultimate decision as to the management of any individual patient is at the discretion of the staff physician in charge of that particular patient s care. 2. Prevention genetic counseling for known NF2 carriers 3. Screening and Early Detection baseline MRI brain and spine for all identified NF2 carriers 4. Diagnosis and Pathology no TMN classification arises from the ependymal lining of the CNS rarely may present as a pelvic mass perivascular pseudorosettes are typical finding ependymoma subtypes: myxopapillary (WHO grade I) cellular (WHO grade II) anaplastic for primaries of the brain or spinal cord, MRI of the other area is recommended to rule out possible CSF spread for anaplastic primaries, a day 14 post-resection or later lumbar puncture is recommended for CSF cytology and glucose and protein to assess for CSF spread 5. Management 5.1 Management Algorithms gross total resection is an important aim of surgery and is associated with an improvement in disease free survival and survival when a GTR has been achieved, it is recommended that a repeat MRI brain or spine be done 3-4 months post-resection to assess for possible residual disease if a supratentorial or spinal primary, non-anaplastic, then observation only is recommended if a post-fossa primary, or an anaplastic tumour, then high dose focal RT is recommended in the absence of CSF spread for all residual disease, post-operative focal RT is recommended for disseminated disease, craniospinal RT to 36 Gy/20 (for CSF positive disease) or 39.4 Gy/22 (for gross disease), followed by a boost to the primary and all gross disease Last Revision Date July

4 one can always consider re-rt for recurrence, same guidelines as first line RT, always use 1.8Gy fractions for recurrent re-rt 5.2 Surgery gross total resection is always considered as main goal supratentorial tumours are generally more amenable to GTR and are approached via typical supratentorial open craniotomy approaches infratentorial post fossa tumours are much more challenging to achieve GTR, particularly if there has been cerebellar-pontine or brain stem invasion, or extension to CP angle via foramina of Lushka where the presence of the lower cranial nerves can significantly reduce the likelihood of complete resection 5.3 Chemotherapy there are currently no known effective chemotherapy or molecular therapies for ependymoma in adults 5.4 Radiation Therapy Fractionated RT (brain, focal) immobilization: thermoplastic U/S frame, CRW relocatable stereotactic frame imaging: CT, MRI T1 gad, T2 GTV: enhancing tumour CTV: 5mm PTV: 3-5 mm Dose: 59.4 Gy/33 IGRT: daily cone beam CT performed, and all displacements greater than 1 mm are corrected prior to treatment delivery, and for all angular displacements greater than 3 degrees, a repeat set up is undertaken Fractionated RT (spine, focal) immobilization: body vacuum bed imaging: CT, with reference to most recent pre and post-operative MRI GTV: T2 tumour extent, not including non-enhancing syrinx CTV: full spinal canal radially, 2 cm sup and inf PTV: 5 mm Dose: 50 Gy/25 or 54 Gy/30 IGRT: daily cone beam CT performed, and all displacements greater than 1 mm are corrected prior to treatment delivery, and for all angular displacements greater than 3 degrees, a repeat set up is undertaken Craniospinal RT immobilization: supine position, thermoplastic S frame imaging: CT brain and whole spine, MRI T1 gad and flair of brain 2D planning all intracranial contents and cervical cord via lateral opposed pair, gantry rotated at approx degrees to match divergence of posterior whole spine field single post spine field accomplished at extended SSD if necessary, divergence of superior border of field matches gantry rotation of lateral brain/cervical cord fields junction is an electronic match on a bb placed on midline anterior neck of thermoplastic S frame Last Revision Date July

5 inferior border of post spine field is cm caudal to distal thecal sac in upper sacrum, usually in region of S3 vertebrae Dose: 36 Gy/20 for CSF positive disease, 39.6 Gy/22 for cases with macroscopic CSF spread Boost to primary tumour/macroscopic CSF spread following craniospinal RT GTV: surgical cavity plus residual tumour CTV: 5 mm PTV: 3-5 mm Boost to 59.4 Gy/33 fractions to primary site in brain, 54 Gy/30 to primary in spine and to metastatic gross areas of disease (both for intracranial and spinal regions) 6. Oncology Nursing Practice Refer to general oncology nursing practices 7. Supportive Care 7.1 Patient Education Driving possible restriction Seizures education about seizures what to do when a seizure occurs how to take seizure medications possible side effects of seizure medications avoid heights, taking baths or swimming alone Raised Intracranial Pressure: Steroids symptoms of raised intracranial pressure side effects of steroids titration of steroids for optimal dose When to call multidisciplinary team change in seizure pattern new or progressive neurologic loss symptoms of raised intracranial pressure 7.2 Psychosocial Care assess family finances assess for possible disability applications assess possible depression/anxiety presence or absence of drug program, apply for provincial assistance if necessary possible need for assistive devices or services in the home 7.3 Symptom Management seizures raised intracranial pressure neurologic loss Last Revision Date July

6 visual loss depression psychosis anger issues poor memory 7.4 Clinical Nutrition recommend normal diet as per recommendations of Canadian Cancer Society diabetic diet if elevation of blood glucose secondary to steroids 7.5 Palliative Care make referral in cases of progressive disease for which there is no further active therapy recommended management of uncontrolled symptoms 7.6 Rehabilitation in cases of neurologic loss, assess for possible rehabilitation OT/PT assess for supportive devices in the home 8. Follow-up Care q6 months with MRI brain or spine depending on original extent of disease after 5 years, q12 months with MRI after 10 years, q24 months with MRI Last Revision Date July

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