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1 Introduction to Supratentorial Adult Brain Neoplasms Jay J. Pillai, M.D. Director of Functional MRI Associate Professor Neuroradiology Division The Russell.

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Presentation on theme: "1 Introduction to Supratentorial Adult Brain Neoplasms Jay J. Pillai, M.D. Director of Functional MRI Associate Professor Neuroradiology Division The Russell."— Presentation transcript:

1 1 Introduction to Supratentorial Adult Brain Neoplasms Jay J. Pillai, M.D. Director of Functional MRI Associate Professor Neuroradiology Division The Russell H. Morgan Department of Radiology and Radiological Science Johns Hopkins Univ. School of Medicine

2 General classification Primary Intra-axial Tumors: Primary Intra-axial Tumors: Astrocytic Neuroepithelial tumors Astrocytic Neuroepithelial tumors – Diffuse Infiltrative: Fibrillary, anaplastic, GBM, Gliomatosis cerebri – Localized: Pilocytic, PXA, SGCA Non-astrocytic Neuroepithelial tumors Non-astrocytic Neuroepithelial tumors – Oligodendroglioma – Ependymal tumors (ependymoma II, subepend I), choroid plexus lesions Neuronal / mixed Neuronal / mixed – Ganglioglioma (gangliocytoma) – DIG, DNET, Central neurocytoma Embryonal tumors: PNET, medulloblastoma Embryonal tumors: PNET, medulloblastoma Lymphoma Lymphoma Primary Extra-axial tumors: Primary Extra-axial tumors: Tumors of the meninges Tumors of the meninges – Tumors of meningothelial cells: meningioma – Mesenchymal non-meningothelial tumors: sarcomas, MFH, lipomas, hemangioma – Primary melanocytic tumors: melanocytoma, melanoma Neurogenic tumors Neurogenic tumors – Neurofibromas, schwannomas Metastases Metastases

3 Gliomas Astrocytomas Astrocytomas Oligodendrogliomas Oligodendrogliomas Ependymomas Ependymomas Choroid plexus tumors Choroid plexus tumors (mixed gliomas – oligoastrocytomas) (mixed gliomas – oligoastrocytomas)

4 Astrocytomas Cell of origin: Astrocytes Cell of origin: Astrocytes Most common CNS malignancy Most common CNS malignancy 75% of the neuroepithelial tumors 75% of the neuroepithelial tumors Grades: Grades: – Low grade: Gr I & II (circumscribed vs. diffuse) – High grade: Gr III & IV (anaplastic vs glioblastoma)

5 Ast rocytic tumors Pilocytic Astrocytoma – low grade (WHO Gr I) Pilocytic Astrocytoma – low grade (WHO Gr I) – Cyst with a mural nodule – cerebellar (juvenile), hypothalamic- chiasmatic, thalamic Diffuse / Fibrillary Astrocytoma – (WHO Gr II) Diffuse / Fibrillary Astrocytoma – (WHO Gr II) – 1/3 FL, 1/3 TL; gemistocytic astro more aggressive than fibrillary, 80% conv to GB, excl supratent Anaplastic Astrocytoma (Gr III)--may prog to GB in 2yr Anaplastic Astrocytoma (Gr III)--may prog to GB in 2yr Glioblastoma (Multiforme) (Gr IV)--m.c. astro (50-60% of astro, 15% of ic tumors) Glioblastoma (Multiforme) (Gr IV)--m.c. astro (50-60% of astro, 15% of ic tumors) Subependymal Giant Cell Astrocytoma (Gr I)--TS Subependymal Giant Cell Astrocytoma (Gr I)--TS Pleomorphic xanthoastrocytoma (Gr II) Pleomorphic xanthoastrocytoma (Gr II) Gliomatosis cerebri Gliomatosis cerebri

6 WHO Grade II Astrocytoma Diffuse / Low grade astrocytoma Focal or diffuse enhancing white matter mass Focal or diffuse enhancing white matter mass Supratentorial – commonly frontal or temporal in location, rare in occipital lobe Supratentorial – commonly frontal or temporal in location, rare in occipital lobe Infratentorial – typically brainstem glioma Infratentorial – typically brainstem glioma Appear circumscribed on imaging (not at surgery) Appear circumscribed on imaging (not at surgery) Low attenuation on CT wo enhanc Low attenuation on CT wo enhanc Calcification and necrosis are rare Calcification and necrosis are rare May mimic subacute infarct on CT, but MRI is definitive May mimic subacute infarct on CT, but MRI is definitive 6

7 High Grade Gliomas Highly vascular tumors with areas of necrosis and breakdown of the blood-brain barrier Highly vascular tumors with areas of necrosis and breakdown of the blood-brain barrier Significantly worse prognosis Significantly worse prognosis Tendency for recurrence Tendency for recurrence TYPES: TYPES: Anaplastic Astrocytoma (WHO Grade III) Anaplastic Astrocytoma (WHO Grade III) Glioblastoma [multiforme] (WHO Grade IV) Glioblastoma [multiforme] (WHO Grade IV) 7

8 Anaplastic Astrocytoma WHO grade III Astrocytoma WHO grade III Astrocytoma Infiltrating hemispheric mass with variable enhancement, commonly affecting white matter and located in the frontal and temporal lobes. Infiltrating hemispheric mass with variable enhancement, commonly affecting white matter and located in the frontal and temporal lobes. Brainstem or thalamic locations in children Brainstem or thalamic locations in children Ill-defined margins with edema and mass effect. Ill-defined margins with edema and mass effect. Calcification, hemorrhage and necrosis are rare. Calcification, hemorrhage and necrosis are rare. Enhancement – patchy or focal, sometimes none. Enhancement – patchy or focal, sometimes none. 8

9 Glioblastoma 15-20% of all tumors – ~50% of astrocytomas 15-20% of all tumors – ~50% of astrocytomas Frontal > temporal > parietal lobes Frontal > temporal > parietal lobes Primary GBM or secondary (dedifferentiated) GBM—prim w worse prognosis, sec in younger pts w longer clin course Primary GBM or secondary (dedifferentiated) GBM—prim w worse prognosis, sec in younger pts w longer clin course Imaging : Typically is a mass that shows a thick irregular enhancing rim around a necrotic core with moderate vasogenic edema Imaging : Typically is a mass that shows a thick irregular enhancing rim around a necrotic core with moderate vasogenic edema Butterfly configuration (extending across the CC) Butterfly configuration (extending across the CC) Multifocal or Multicentric (even wo NF1) Multifocal or Multicentric (even wo NF1) Tendency to CSF spread/drop mets/subarachnoid & subependymal seeding Tendency to CSF spread/drop mets/subarachnoid & subependymal seeding Rare variant—gliosarcoma (poor prog, all supratent) Rare variant—gliosarcoma (poor prog, all supratent)

10 Glioblastoma 15-20% of all tumors – ~50% of astrocytomas 15-20% of all tumors – ~50% of astrocytomas Frontal > temporal > parietal lobes Frontal > temporal > parietal lobes Primary GBM or secondary (dedifferentiated) GBM—prim w worse prognosis, sec in younger pts w longer clin course Primary GBM or secondary (dedifferentiated) GBM—prim w worse prognosis, sec in younger pts w longer clin course Imaging : Typically is a mass that shows a thick irregular enhancing rim around a necrotic core with moderate vasogenic edema Imaging : Typically is a mass that shows a thick irregular enhancing rim around a necrotic core with moderate vasogenic edema Butterfly configuration (extending across the CC) Butterfly configuration (extending across the CC) Multifocal or Multicentric (even wo NF1) Multifocal or Multicentric (even wo NF1) Tendency to CSF spread/drop mets/subarachnoid & subependymal seeding Tendency to CSF spread/drop mets/subarachnoid & subependymal seeding Rare variant—gliosarcoma (poor prog, all supratent) Rare variant—gliosarcoma (poor prog, all supratent)

11 11 Value of perfusion imaging in evaluation of biological behavior of gliomas even when findings are not consistent with initial histopathologic and standard MR imaging Law M, Oh S, Johnson G, Babb JS, Zagzag D, Golfinos J, Kelly PJ. Perfusion magnetic resonance imaging predicts patient outcome as an adjunct to histopathology: a second reference standard in the surgical and nonsurgical treatment of low-grade gliomas. Neurosurgery Jun;58(6): ; discussion Law M, Oh S, Johnson G, Babb JS, Zagzag D, Golfinos J, Kelly PJ. Perfusion magnetic resonance imaging predicts patient outcome as an adjunct to histopathology: a second reference standard in the surgical and nonsurgical treatment of low-grade gliomas. Neurosurgery Jun;58(6): ; discussion Danchaivijitr N, Waldman AD, Tozer DJ, Benton CE, Brasil Caseiras G, Tofts PS, Rees JH, Jäger HR. Low-grade gliomas: do changes in rCBV measurements at longitudinal perfusion-weighted MR imaging predict malignant transformation? Radiology Apr;247(1): Danchaivijitr N, Waldman AD, Tozer DJ, Benton CE, Brasil Caseiras G, Tofts PS, Rees JH, Jäger HR. Low-grade gliomas: do changes in rCBV measurements at longitudinal perfusion-weighted MR imaging predict malignant transformation? Radiology Apr;247(1):170-8.

12 Treatment of GBM 12 Standard treatment: complete resection of CE tumor followed by chemoradiation (External Beam RT + temozolomide) --direct placement of chemotherapeutic agents into resection cavity (carmustine[BCNU] wafers—Gliadel ) or convection-enhanced delivery – intracerebral drug delivery via small intracranial catheters w pressure gradient to control infusion rates Newer experimental therapies: 1) Therapy targeting cancer stem cells 2) Antiangiogenic therapy (bevacizumab [targets VEGF-A], cediranib [targets VEGFR1-3, PDGFR-beta, c-Kit proto-oncogene], etc.) 3) Immunotherapy (IL4-P38KDEL, IL13-PE8QQR)—interleukins; natural toxins can be conjugated to drugs that bind to receptors overexpressed by certain tumor cells Lima FR, Kahn SA, Soletti RC, et al. Glioblastoma: Therapeutic challenges, what lies ahead. Biochim Biophys Acta 2012; 1826 (12):

13 13

14 Pseudoeffects: Pseudoprogression & Pseudoresponse There is a lot of interest and some recent literature on the subject of pseudo-effects following treatment of glioblastoma. In practice, often it comes down to obtaining a follow up MR to see how findings change longitudinally. RANO criteria has replaced older McDonald (2D meas—sum of products of perpend diam/25% incr CE for prog) and RECIST (1 D meas) criteria for assessment of treatment response. New RECIST + F criteria (considering maximum diameter of both CE and T2/FLAIR regions—takes into account pseudoresponse just as RANO criteria do) also proposed, but currently RANO criteria are most widely accepted.

15 Pseudoprogression in Glioblastomas Quant and Wen. RANO. Curr Oncol Rep (2011) 13:50–56 Pseudo-progression: An increase in contrast enhancement and/or edema on MRI without true tumor progression. Pseudo-progression: An increase in contrast enhancement and/or edema on MRI without true tumor progression. May occur post XRT alone, but enhanced by the addition of temozolomide to radiotherapy (chemoradiation), particularly in GBM patients with methylated MGMT. May occur post XRT alone, but enhanced by the addition of temozolomide to radiotherapy (chemoradiation), particularly in GBM patients with methylated MGMT. Can occur in 40-50% pts on chemoradiation Can occur in 40-50% pts on chemoradiation In half of patients, the increased CE w/wo increased edema eventually subsides despite continuing temozolomide, suggesting that the CE reflects transiently increased permeability of the tumor vasculature from irradiation. In half of patients, the increased CE w/wo increased edema eventually subsides despite continuing temozolomide, suggesting that the CE reflects transiently increased permeability of the tumor vasculature from irradiation. In some cases may progress to actual radiation necrosis, but if patient is asymptomatic, then may continue treatment. In some cases may progress to actual radiation necrosis, but if patient is asymptomatic, then may continue treatment. Critical to be aware to avoid premature discontinuation of an effective therapy based on apparently worsening MRI findings alone. Critical to be aware to avoid premature discontinuation of an effective therapy based on apparently worsening MRI findings alone. 15

16 Pseudoresponse in GBMs Quant and Wen. RANO. Curr Oncol Rep (2011) 13:50–56 Pseudo-response: decrease in enhancement on MRI without a true anti-tumor effect Pseudo-response: decrease in enhancement on MRI without a true anti-tumor effect High permeability of HGG vasculature responsible for CE and peritumoral edema. High permeability of HGG vasculature responsible for CE and peritumoral edema. Anti-angiogenic agents targeting VEGF pathway, such as bevacizumab or cediranib, significantly reduce vascular permeability >>>high apparent radiologic response rates of 25% to 60% in GBM. Anti-angiogenic agents targeting VEGF pathway, such as bevacizumab or cediranib, significantly reduce vascular permeability >>>high apparent radiologic response rates of 25% to 60% in GBM. Bevacizumab (Avastin ®), is a monoclonal antibody targeting VEGF. Partly due to normalization of tumor vessel permeability resulting in decreased CE, not necessarily true anti-glioma effect. Partly due to normalization of tumor vessel permeability resulting in decreased CE, not necessarily true anti-glioma effect. Progression of disease while on VEGF inhibitors results in more treatment- refractory invasive nature of GBM w much worse prog—frequently seen expanding T2/FLAIR abnormality despite reduced CE, with rebound phenomenon wrt edema after discontin of Avastin ( similar to corticosteroids). Progression of disease while on VEGF inhibitors results in more treatment- refractory invasive nature of GBM w much worse prog—frequently seen expanding T2/FLAIR abnormality despite reduced CE, with rebound phenomenon wrt edema after discontin of Avastin ( similar to corticosteroids). 16

17 17 Agreement among the 4 methods was high (kappa statistic >0.75) for both determination of response and type of progression.

18 RANO Criteria--GBM 18 Quant EC, Wen PY. Response Assessment in Neuro-Oncology. Curr Oncol Rep 2011; 13:50–56.

19 19

20 RANO Surgery Task Force Recommendations : 20 Vogelbaum MA, Jost S, Aghi MK, et al., Application of novel response/progression measures for surgically delivered therapies for gliomas: Response Assessment in Neuro-Oncology (RANO) Working Group. Neurosurgery 2012; 70(1): ; discussion

21 RANO group proposed changes in terminology Complete Resection of Enhancing Tumor (CRET) and Complete Resection of Detectable Tumor (CRDT) to replace ‘GTR’ Complete Resection of Enhancing Tumor (CRET) and Complete Resection of Detectable Tumor (CRDT) to replace ‘GTR’ Partial Resection of Enhancing Tumor (PRET) and Partial Resection of Detectable Tumor (PRDT) to replace ‘Subtotal Resection’ or ‘Partial Resection’ Partial Resection of Enhancing Tumor (PRET) and Partial Resection of Detectable Tumor (PRDT) to replace ‘Subtotal Resection’ or ‘Partial Resection’ Takes into account currently available imaging technology & info needed for current clinical trials Takes into account currently available imaging technology & info needed for current clinical trials 21 Vogelbaum MA, Jost S, Aghi MK, et al., Application of novel response/progression measures for surgically delivered therapies for gliomas: Response Assessment in Neuro-Oncology (RANO) Working Group. Neurosurgery 2012; 70(1): ; discussion

22 New RANO criteria for LGG 22

23 New RANO criteria---LGG 23 Van den Bent MJ, Wefel JS, Schiff D et al., Response assessment in neuro-oncology (a report of the RANO group): assessment of outcome in trials of diffuse low- grade gliomas. Lancet Oncol 2011; 12(6):583—593.

24 Non Astrocytic Neuroepithelial tumors Oligodendrogliomas Oligodendrogliomas – Low grade (WHO grade II) – High Grade or Anaplastic (WHO grade III) Neuronal tumors – Ganglioglioma Neuronal tumors – Ganglioglioma Ependymal Tumors Ependymal Tumors – Subependymoma – Ependymoma Choroid Plexus tumors Choroid Plexus tumors – Papilloma and carcinoma 24

25 Oligodendrogliomas 4-5 th decade of life—mean age 35 y.o., sz, HA, half low grade 4-5 th decade of life—mean age 35 y.o., sz, HA, half low grade Slow growing tumors that typically involve the cortex and subcortical white matter—can cause scalloping of inner table Slow growing tumors that typically involve the cortex and subcortical white matter—can cause scalloping of inner table Calcifications in a large number ( ~ 80%) Calcifications in a large number ( ~ 80%) Enhancement may be mild or none Enhancement may be mild or none Frontal lobe more common other lobes, rare in posterior fossa or spine. Frontal lobe more common other lobes, rare in posterior fossa or spine. Mixed forms and high grade forms common Mixed forms and high grade forms common Typically heterogeneous in signal intensity, frequently with cystic components, possibly hemorrhage Typically heterogeneous in signal intensity, frequently with cystic components, possibly hemorrhage 1p 19q co-deletion or LOH (loss of heterozygosity), i.e., combined allelic loss of chr arms 1p and 19q ---predictor of both chemosensitivity and prolonged overall and recurrence-free survival. 1p 19q co-deletion or LOH (loss of heterozygosity), i.e., combined allelic loss of chr arms 1p and 19q ---predictor of both chemosensitivity and prolonged overall and recurrence-free survival. 10q LOH (correlated with anaplastic grade), however, predicted a survival disadvantage. 10q LOH (correlated with anaplastic grade), however, predicted a survival disadvantage. Ramirez C, Bowman C, Maurage C-A, Dubois F, Blond S, Porchet N, Escande F. Loss of 1p, 19w, and 10q heterozygosity prospectively predicts prognosis of oligodendroglial tumors---towards individualized tumor treatment? Neuro-Oncology 2010; 12(5):

26 Neuronal and mixed neuronal-glial tumors Gangliocytoma (cerebellar – LD disease)-- no risk of malignant degen; cerebral ctx or cbl; hyper NCCT w minim or no enhanc; iso T1, T2, hyper FLAIR Gangliocytoma (cerebellar – LD disease)-- no risk of malignant degen; cerebral ctx or cbl; hyper NCCT w minim or no enhanc; iso T1, T2, hyper FLAIR Ganglioglioma (anaplastic ganglioglioma)- -peds & young adults FL, ant 3 rd vent, cbl, cord, o.n.; cystic in 30-50%;1/3 calcif; 50% faint enhanc; cysts m.c. in younger [ FL, ant 3 rd vent, cbl, cord, o.n.; cystic in 30-50%;1/3 calcif; 50% faint enhanc; cysts m.c. in younger [<10y.o.];mural nodule poss; may dediff into anaplastic ganglioglioma Desmoplastic Infantile Ganglioglioma- -1 st 2 yrs Desmoplastic Infantile Ganglioglioma- -1 st 2 yrs DNET - WHO gr I;TL(50-60%)>FL(30%);simil to ganglioglioma;2 nd,3 rd dec w sz; mult cysts, T2 hyper, enh 50%, rarely recur, may arise from septum pelluc, rarely infratent DNET - WHO gr I;TL(50-60%)>FL(30%);simil to ganglioglioma;2 nd,3 rd dec w sz; mult cysts, T2 hyper, enh 50%, rarely recur, may arise from septum pelluc, rarely infratent Central neurocytoma- - calcif, may be cystic, lat or 3 rd v, often attached to septum pellucidum, peak in 3 rd decade, mean 29 y.o., mild to mod enhanc, flow voids---ddx intrav oligo Central neurocytoma- - calcif, may be cystic, lat or 3 rd v, often attached to septum pellucidum, peak in 3 rd decade, mean 29 y.o., mild to mod enhanc, flow voids---ddx intrav oligo


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