J.W. Van Goethem C. Venstermans S. Dekeyzer neuronal tumors including DNET, ganglioglioma, and S. Vanden Bossche neurocytoma S. Nicolay P. M . Pa r i ze l L. van den Hauwe
B. Goraj Antwerp University Hospital - University of Antwerp, Antwerp,/BE K. Kamphuis-van Ulzen AZ KLINA, Brasschaat/BE (University Medical Center St Radboud, Nijmegen/NL)
suggested reading
• 14 entities • uncommon • 0.4%-2% of all CNS tumors
Multinodular and vacuolating neuronal tumor: MVNT MVNT • 14 entities • multinodular and vacuolating neuronal tumor • uncommon • 1st described in 2013 • 0.4%-2% of all • considered malformative or dysplastic rather than a true neoplasm CNS tumors • seizures, headache, incidental finding • ĂĚƵůƚƐшϯϬLJĞĂƌƐ • parietal and temporal lobes • MRI: – juxtacortical clusters of tiny, well-defined, round or oval-shaped nodules Multinodular and vacuolating neuronal tumor: MVNT – hyperintense on T2 and FLAIR!!! (DD with PVS) – no mass effect, no edema Diffuse glioneuronal tumor with oligodendroglial features – no enhancement (3D TSE black-blood sequence) Myxoid glioneuronal tumor Polymorphous low-grade neuroepithelial tumor of the young: PLNTY • ‘leave me alone’ lesions
MVNT 45-year-old woman, tinnitus, MR post fossa
• multinodular and vacuolating neuronal tumor • 1st described in 2013 • considered malformative or dysplastic rather than a true neoplasm • seizures, headache, incidental finding • ĂĚƵůƚƐшϯϬLJĞĂƌƐ • parietal and temporal lobes • MRI: – juxtacortical clusters of tiny, well-defined, round or oval-shaped nodules – hyperintense on T2 and FLAIR!!! (DD with PVS) – no mass effect, no edema – no enhancement (3D TSE black-blood sequence) • ‘leave me alone’ lesions
patientpa t ooff 882,2, dysphagiadysph overview
• introduction • classification • dysembryoplastic neuroepithelial tumor (DNET) • ganglioglioma (GG) • central neurocytoma • potpourri… introduction classification
neuronal & mixed neuronal-glial tumors • supratentorial parenchymal, incidentally infratentorial • aka glioneuronal tumors – dysembryoplastic neuroepithelial tumor (DNET) • varying morphologic patterns and biological behavior – ganglioglioma (GG), gangliocytoma • less common than pure glial neoplasms, accounting for 0.5%-2% of all – desmoplastic infantile ganglioglioma (DIG) primary brain tumors – papillary glioneural tumor • often seizures-associated • less biologically aggressive than most glial tumors • supratentorial intraventricular, incidentally extra-ventricular • have a more favorable prognosis – central neurocytoma & extraventricular neurocytoma remember: • WHO grade 1 or 2 -location -location -location Osborn AG. Osborn’s brain Goraj B. The Neuroradiology Journal 2008
classification
• infratentorial intraventricular – rosette-forming glioneuronal tumor of the 4th ventricle dysembryoplastic neuroepithelial tumor • infratentorial parenchymal – dysplastic gangliocytoma of the cerebellum: WHO, grade 1 = Lhermitte – Duclos disease
remember: – cerebellar liponeurocytoma -location -location
Goraj B. The Neuroradiology Journal 2008 -location
dysembryoplastic neuroepithelial tumor : DNET dysembryoplastic neuroepithelial tumor : DNET imaging • benign masses of the cerebral cortex or deep gray matter: temporal lobe (62%) and frontal lobe (31%) • wedge-shaped cortical mass, “points" toward ventricle • children and young adults: majority of patients < 20 y • multicystic/septated "bubbly" appearance • man > woman • hyperintense on T2-wi • patients present with partial complex seizures • rim of hyperintensity on FLAIR: “bright rim sign” – in 20% of cases of medically refractory epilepsy • no enhancement; 1/3 enhance – 2nd most common tumor-associated TLE • no surrounding vasogenic edema • grows slowly, surgery usually curative • remodelling the inner table of the skull • often associated with cortical dysplasia • a congenital malformation rather than a true neoplastic lesion?
Koeller KK and Henry JM. RadioGraphics 2001 Koeller KK and Henry JM. RadioGraphics 2001 th Barkovich AJ and Raybaud C. Pediatric Neuroimaging, 5 ed. Osborn AG. Osborn’s brain T1-wi T2-wi FLAIR
GRE T2* rCBV
T1-wi + Gd
a 22-year-old woman T1-wi T2-wi FLAIR
seizures
T1-wi + Gd DWI ADC
FLAIR hyperintense rim sign
T1-wi T2-wi FLAIR T1-wi + Gd
T1-wi + Gd DWI ADC DD
• other LGG: – diffuse astrocytoma – oligodendroglioma a 15-year-old girl – pleomorphic xanthoastrocytoma – ganglioglioma seizures
T1-wi T2-wi FLAIR T1-wi + Gd
T1-wi + Gd
a 15-year-old girl
a 41-year-old man
ER: generalized seizures, loss of consciousness
T2-wi T1-wi + Gd pathology: – histology: oligodendroglioma vs DNET – FISH 1p/19q: no codeletion DNET rCBV = 1.2 T1-wi T2-wi FLAIR
T1-wi + Gd DWI ADC
a 50-year-old man
headache oligodendroglioma, WHO, grade 2 IDH status unknown, 1p/19q codeleted
FLAIR T2-wi T1-wi + Gd
T1-wi T2-wi ganglion cell tumors ganglioglioma - gangliocytoma
WHO, grade 1 WHO, grade 2
ganglion cell tumors ganglioglioma
• well-differentiated neoplasms • contain both ganglion cells and glial elements • contain mature but dysmorphic neurons • immunoreactivity for these 2 cellular populations • most ganglion cell tumors are histologically mixed tumors that – glial fibrillary acidic protein (GFAP) + contain both ganglion cell and glial elements: gangliogliomas – synaptophysin and neurofilament protein + • glial component: – the most common glioneuronal neoplasm in the CNS – astrocytes in varying states of differentiation – are the most common cause of tumor-related temporal lobe epilepsy, – affects the biologic behavior of the tumor accounting for 40% of all cases – usually has histologic features of pilocytic astrocytoma • tumors that demonstrate exclusive ganglion cell composition are – if less differentiated (as in HGG), more aggressive behavior very rare: gangliocytomas – malignant degeneration • glial component is absent in gangliocytoma Osborn AG. Osborn’s brain Koeller KK and Henry JM. RadioGraphics 2001
ganglioglioma, WHO grade 1 or 2 ganglioglioma: GG
• 0.4%-0.9% of all intracranial tumors imaging: • young patients: 80% < 30 years; peak age 10-20 years • CT: – well-circumscribed mass of low density • predilection for cerebral hemispheres: – located in the cortical gray matter – temporal lobe 75% – little mass effect or edema – frontal lobe – solid portions: • superficial location • isodense, hypodense, mixed • expansive growth; well-defined margins • variable contrast enhancement • calcification: 35% of cases – peritumoral edema is rare – erosion of the inner table of the calvarium Barkovich AJ and Raybaud C. Pediatric Neuroimaging, 5th ed. Koeller KK and Henry JM. RadioGraphics 2001 NCCT ganglioglioma: GG ganglioglioma: GG
imaging: • MRI: variable appearance – peripheral location within the cerebral hemispheres – sharply or poorly defined margins – solid, cystic, “cyst with a mural nodule”, cluster of small cysts – variable SI on T1-wi (mixed), hyperintense ĨŽĐƵƐьĐĂůĐŝĨŝĐĂƚŝŽŶ – hyperintense on T2-wi – variable enhancement of solid portions
Barkovich AJ and Raybaud C. Pediatric Neuroimaging, 5th ed. From: Koeller KK and Henry JM. RadioGraphics 2001
a 9-year-old boy
T1-wi T2-wi FLAIR
seizures
T1-wi + Gd DWI ADC
a 14-year-old boy
• November 2019: – ambulance: seizures • Prev: deja-vues Alice in Wonderland Syndrome T2-wi T1-wi + Gd • difficulties in finding his words • August 2020
T1-wi + Gd fMRI-motor a 14-year-old boy a 14-year-oldy boyy
NCCT 1 NCCT 1 November 2019 November 2019
2019
a 14-year-old boy a 14-year-old boy
NCCT 2 August 2020
2020
a 14-year-old boy
a 14-year-old boy: seizures a 11-year-old girl T1-wi T2-wi FLAIR
complex partial seizures
T1-wi + Gd DWI ADC
a 16-year-old boy
• previous medical history: CO intoxication • complex partial seizures for 3 months • clinical neurological examination: normal • EEG: normal • R/depakine 500-1000mg/day • MRI
pathology: ganglioglioma vs pilocytic astrocytoma T2-wi T1-wi + Gd T2-wi FLAIR T1-wi + Gd
T1-wi T1-wi + Gd GRE T2*
• fMRI: Broca: OK • partial resection – ingrowth insula and lentiform nucleus T2-wi FLAIR T1-wi + Gd • DD: – ganglioglioma – pleomorphic xanthoastrocytoma (PXA) – (pilocytic astrocytoma)
neuropathology differential diagnostic problem: • histological features of both – pleomorphic xanthoastrocytoma (PXA) – ganglioglioma
• presence of ganglion cells • strong GFAP immunoreactivity: +++ • absence of reticuline network • increased proliferative activity: grade II
• final diagnosis: ganglioglioma, grade II causes of temporal lobe epilepsy
• most common = mesial temporal sclerosis • tumor-associated temporal lobe epilepsy – ganglioglioma (40%) central neurocytoma – DNET (20%) – diffuse low-grade astrocytoma (20%) – other (20%) WHO, grade 2 • pilocytic astrocytoma • pleomorphic xanthoastrocytoma • oligodendroglioma
Osborn AG. Osborn’s brain
central neurocytoma, WHO grade 2 central neurocytoma
NECT NECT • young adults • WHO, grade 2 lesions • body of lateral ventricles rd • 3 and lateral ventricles • lesion attached to septum pellucidum, extending through foramen of Monro in contralateral – may arise extraventricular ventricle • young and middle-aged adults (< 40 years of age) • attached to the septum pellucidum FLAIR T2 • calcification is common • CT: - mixed density, heterogeneous cystic appearance • previously called (can be confused with) intraventricular - hydrocephalus common oligodendroglioma - calcification in 50-70%, hemorrhage rare T1 + gd T1 + gd - moderate heterogeneous enhancement
• MRI: - heterogeneous mass, isointense on T1-wi - bubbly appearance on T2-wi - heterogenously hyperintense on FLAIR
FLAIR T2-wi
T1-wi + Gd subependymoma
subependymoma
SEGA potpourri T1-wi T2-wi T1-wi + Gd
dysplastic cerebellar gangliocytoma
dysplastic cerebellar gangliocytoma T2-wi
Lhermitte-Duclos WHO, grade 1
striated pattern: alternating bands of high signal intensity and normal signal intensity relative to gray matter
Spaargaren L et al. Neuroradiology 2003 contrast enhancement in LDD is due to venous proliferation within the lesion in the outer portions of the cerebellar cortex and to prominent draining veins
Spaargaren L et al. Neuroradiology 2003
dysplastic cerebellar gangliocytoma
• aka Lhermitte-Duclos disease (LDD), cerebellar hamartoma, granular cell hypertrophy, … • a rare benign cerebellar mass, characterized by overgrowth of the desmoplastic infantile tumors cerebellar cortex • usually involves the cerebellar hemisphere or the vermis • large lesions may involve both; brainstem is a rarely affected desmoplastic infantile ganglioglioma • probably a hamartoma and not a true neoplasm desmoplastic infantile astrocytoma • sporadic vs part of a multiple hamartoma syndrome: Cowden syndrome WHO, grade 1 (40%)
desmoplastic infantile ganglioglioma: DIG
• 1st described by Vandenberg et al. in 1987: 11 cases • very large brain tumor in the 1st year of life – frontal and parietal lobes a 1-day-old baby – both cystic and solid portions – intense desmoplastic reaction at the periphery of the mass with attachment to the dura macrocephaly • M:F = 2:1 tense bulging fontanelles • rapid growth • surgery • overall good prognosis Koeller KK and Henry JM. RadioGraphics 2001 T1-wi T2-wi T1-wi T1-wi T2-wi FLAIR
T2-wi T1-wi T1-wi + Gd T1-wi + Gd
DD
• variaty of more malignant tumors: – teratomas – choroid plexus papilloma – atypical teratoid/rhabdoid tumors (AT/RT) – …
paraganglioma
• < filum terminale paraganglioma • neuroendocrine tumors; WHO, grade 1 • uncommon: 300 cases reported • adults • M:F = ± 1.5:1 WHO, grade 1 • usually encapsulated • recurrences, late (30 years) • increased vascularity: feeding vessels! a 57-year-old man
paraganglioma
myxopapillary ependymomas • typical tumor < glial cells of the filum terminale • relatively common: 83% of prim. tumors in the region • usually solitary; multiple in 43% of reported cases • in children & young adults • WHO, grade 2! according to the new 2021 update of the WHO classification A. Osborn, ESNR webinar 2020 myxopapillary ependymoma