多伦多病童医院脑干胶质瘤课件

1、among brainstem gliomasatectal gliomabfocal midbrain tumorcfocal intrinsic pontine gliomaddorsal/exophytic gliomaediffuse intrinsic pontine glioma*ffocal medullary gliomagcervicomedullary gliomaa few important distinctions* a form of high grade glioma, akin toanaplastic astrocytoma or glioblastoma m

2、ultiformeaugust 2001august 2006october 2014august 2000december 2001december 2001december 2002low grade glioma of the brainstem: chemotherapy with weekly vincristine and carboplatindiagnosis (11/2013)1/2015 (one year of vbl)braf v600 mutated tumourtypical dpgtypical bsg 13 year old10 month history of

3、 progressive right sided weakness, (r) cn 7 and 8grade 2 on histolology 17 year old12 month history of dizziness when lying downno cn deficit, no long tract sign, no ataxia11 year-oldjanuary 20042010 (18 years old)january 200420101 day oldpm: pnet1 day oldno pm4 month oldpilocytic astrocytomaon chem

4、odpglggfocal hggdpglgg2 year-old, 5 months history of ataxia and gaze palsybiopsy: low grade astrocytoma3 years old, nf110/20127/20133 years old mild hemiparesisbiopsy: infiltrative astrocytoma (grade 2)9/201210/2016malignant glioma of ponscanadian cases by year standard rt50-54 gy in 1.8 gydaily fr

5、actionscurrent trend to move to conformal techniqueshyperfractionation: results of prospective studiesfreeman et al, pog 9239, ijrobp199954 gy in 30 fractions versus 39 gy in 13 fractionszhagloul et alradiotherapy & oncology 2014patient died at 11 months post diagnosisage at diagnosis(months)sex

6、neurological signs at presentationinterval between onset of symptoms and diagnosis(weeks)initial treatmentsurvival (years)cranial nerve palsypyramidal deficitscerebellar signs20maleyesyesyes24 rt +temozolomide+522maleyesyesno12-24 rt+4clinical characteristics, treatment and outcome of surviving pati

7、entsmri imaging of long term survivorsoctober 2011january 2012january 2017long term survivorbiopsycohort 1 mgmt- egfr-cohort 2 mgmt- egfr+cohort 3 mgmt+ egfr-cohort 4 mgmt+egfr+rt bevacizumabrtbevacizumab erlotinibrtbevacizumabtemozolomidertbevacizumab erlotinibtemozolomide4 weeks bevacizumab4 weeks

8、 bevacizumab erlotinib4 weeks bevacizumab4 weeks bevacizumab erlotinibmaintenance bevacizumabmaintenance bevacizumab erlotinibmaintenancebevacizumab temozolomidemaintenance bevacizumab erlotinibtemozolomidemri diagnosis dipgtreatment schemaenrollmenttissue analysesboston/ucsf protocoldec 2013oct 201

9、330 gy in 17 sessions oct 2012: 54 gy in 30 sessions dipgshgas13579112468101315171921x12141618202213579111315171921x246810121416182022dipgs are genetically distinct from supratentorial high grade astrocytomasdipghga12345678910111234567891011chromosome 14chromosome 17p13p12p11.2q11.1q11.2q12q13.1q21.

10、1q21.2q21.3q23.1q22.1q23.2q23.3q24.1q24.2q24.3q31.1q31.3q32.13q32.2q32.33p13.3p13.2p13.1p11.2p12q11.2q12q21.2q21.31q21.32q21.33q22q23.2q24.1q24.2q24.3q25.1q25.3p13p12p11.2q11.1q11.2q12q13.1q21.1q21.2q21.3q23.1q22.1q23.2q23.3q24.1q24.2q24.3q31.1q31.3q32.13q32.2q32.33p13.3p13.2p13.1p11.2p12q11.2q12q21.2q21.31q21.32q21.33q22q23.2q24.1q24.2q24.3q25.1q25.3dipgs are genetically distinct from supratentorial