PRINCESS MARGARET CANCER CENTRE CLINICAL PRACTICE GUIDELINES

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1 PRINCESS MARGARET CANCER CENTRE CLINICAL PRACTICE GUIDELINES CENTRAL NERVOUS SYSTEM GERM CELL TUMOURS

2 CNS Site Group Germ Cell Tumours Author: Dr. Norm Laperriere Date: February 20, 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 February

3 Germ Cell Tumours 1. Introduction very rare tumours in young adults occur in midline in pineal or suprasellar areas 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 No specific prevention available 3. Screening and Early Detection No screening available 4. Diagnosis and Pathology no TNM staging 2 distinct categories: germinoma and malignant non-germinoma germ cell tumours main pathologies: germinoma 65% germinoma with mature and/or immature teratoma 15% mixed malignant germ cell tumors 20% yolk sac tumour (endodermal sinus tumour) choriocarcinoma embryonal carcinoma mature teratoma immature teratoma typical MRI findings: supra-sellar or pineal enhancing tumour with hydrocephalus tumour cell markers done both in CSF and blood alpha-fetoprotein (AFP) considered diagnostic of yolk sac tumor (but also seen at low levels in some cases of Immature teratoma and embryonal carcinoma) human chorionic gonadotropin Beta (HCG-ß) Once considered diagnostic of choriocarcinoma, but now recognized to be produced at low levels in pure germinoma, as well as some cases of immature teratoma and embryonal carcinoma (usually < 50 mg/dl in germinomas) MRI spine and a lumbar puncture CSF for cytology, protein, sugar no sooner than 14 days post-operatively for staging purposes 5. Management 5.1 Management Algorithms Germinoma surgical biopsy, CSF diversion if necessary Last Revision Date February

4 craniospinal RT 25 Gy/20 with concurrent boost to primary 10 Gy/20, total 35 Gy/20 Non-germinoma malignant germ cell tumour biopsy, CSF diversion if necessary Cisplatin based chemotherapy for 4-6 cycles focal high dose RT 60 Gy/30 for localized tumours craniospinal RT 36 Gy/20 with boost to primary to total 60 Gy/30 and boost to gross CSF spread to 54 Gy/ Surgery initial approach to a pineal mass with hydrocephalus would include an endoscopic biopsy and 3 rd ventriculostomy no benefit from attempting a GTR in cases of germinoma in cases containing mature or immature teratoma, these elements are not particularly chemo or radiosensitive, and resection of these elements can be critical in the successful management of these sub-types of germ cell tumours, generally done after courses of chemotherapy and radiotherapy have shrunk down other elements sometimes, the mature or immature teratoma component of the tumor is so substantial, that the tumor actually increases in size with initial chemotherapy: this uncommon but well recognized scenario is known as The Growing Teratoma Syndrome, and is an indication for immediate surgical resection 5.3 Chemotherapy as we only see one of these patients every few years, the chemotherapy has not been standardized, and is given as per recent developments in the literature our current approach is to use 4 cycles of VIP: Etoposide (VP-16), Ifosfomide, Platinol (cisplatin) x q3weekly for 4 cycles 5.4 Radiation Therapy Craniospinal RT - Germinoma 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 inferior border of post spine field is cm caudal to distal thecal sac in upper sacrum, usually in region of S3 vertebrae Dose: 25 Gy/20 spread Boost to primary tumour CONCURRENT with craniospinal RT GTV: surgical cavity plus residual tumour CTV: 5 mm PTV: 3-5 mm Boost 15 Gy/20 fractions to primary site in brain, total 40 Gy/20 to primary in spine and to metastatic gross areas of disease (both for intracranial and spinal regions) Fractionated focal RT - Malignant non-germinoma germ cell tumours Last Revision Date February

5 immobilization: thermoplastic U/S frame, CRW relocatable stereotactic frame imaging: CT, MRI T1 gad, T2 GTV: enhancing tumour CTV: 5mm PTV: 3 mm Dose: 60 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 - CSF spread malignant non-germinoma germ cell tumours 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 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 60 Gy/30 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 Last Revision Date February

6 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 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 q3-4 months with MRI brain for 1 st year q6 month with MRI brain for years 2-5 q12 month with MRI brain for years 5-10 Last Revision Date February

PRINCESS MARGARET CANCER CENTRE CLINICAL PRACTICE GUIDELINES

PRINCESS MARGARET CANCER CENTRE CLINICAL PRACTICE GUIDELINES CENTRAL NERVOUS SYSTEM EPENDYMOMA Last Revision Date July 2015 1 CNS Site Group Ependymoma Author: Dr. Norm Laperriere 1. INTRODUCTION 3 2.