Glioneuronal and Neuronal Tumors Flashcards
An 11-year-old boy presents with history of seizures. His CT scan shows calcified lesion in temporal lobe. MRI confirms space occupying lesion (SOL) in temporal lobe. The patient underwent surgery and histopathological diagnosis came out to be ganglioglioma. What is the preferred treatment in case of recurrence?
● A. Re-resection
● B. Radiotherapy
● C. Chemotherapy
● D. Observation
A. Re-resection
Desmoplastic infantile astrocytoma may recur with anaplastic transformation how long after initial resection?
● A. 3 to 5 years
● B. 8 to 10 years
● C. 12 to15 years
● D. 18 to 20 years
B. 8 to 10 years
What is the most common location for dysembryoplastic neuroepithelial tumor (DNET)?
● A. Temporal lobe
● B. Parietal lobe
● C. Occipital lobe
● D. Basal ganglia
● E. Cerebellum
A. Temporal lobe
Which type of rosettes is demonstrated in rosette forming glioneural tumor?
● A. Homer Wright rosettes
● B. Flexner Wintersteiner rosettes
● C. Ependymal rosettes
● D. Neurocyte forming rosette
D. Neurocyte forming rosette
Which of the following is not an essential diagnostic criterion for central neurocytoma?
● A. Intraventricular localization
● B. ODG like monomorphic cells
● C. Synaptophysin expression
● D. No sign of malignancy
● E. Methylation profile
D. No sign of malignancy
Dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease) is a cerebellar lesion with features of both a malformation and a low-grade neoplasm, and causes diffuse enlargement of cerebellar folia. It has the propensity to progress and recur after surgery and is strongly associated with which of the
following?
● A. Down syndrome
● B. Klinefelter syndrome
● C. Neurofibromatosis I
● D. Li Fraumeni syndrome
● E. Cowden syndrome
E. Cowden syndrome
A patient 16 years of age presents with seizure. CT imaging of brain shows hypodense area in temporal lobe with calcifications. T1WI MRI showed hypointense area while T2WI showed hyperintense area. Maximal safe resection of this tumor was done. On histopathology an intra-axial low-grade glioneuronal tumor was noted in combination with neoplastic ganglion and glial cells. Which of the following statements is incorrect regarding ganglioglioma?
● A. The most common location of this tumor is temporal lobe
● B. These are benign tumors with 97% recurrence-free survival of 7.5 years
● C. Radiotherapy is recommended in all postoperative cases
● D. These are slow-growing well-differentiated tumor that primarily affects children and young adults
● E. It is a WHO grade 1 tumor
C. Radiotherapy is recommended in all postoperative cases
On histopathology of a tumor, irregular groups of large, mature ganglion cells were noted WITH matrix resembling normal neuropil sometimes more coarsely fibrillar or vacuolated. Diagnosis of gangliocytoma was made. What is the most common location of this tumor?
● A. Frontal lobe
● B. Hypothalamus
● C. Third ventricle
● D. Temporal lobe
● E. Parietal lobe
D. Temporal lobe
Histopathology of a tumor showed biphasic morphology with dominant desmoplastic leptomeningeal component admixed with a neuroepithelial component containing astrocytic
cells only or containing astrocytes or neuronal cells. This lesion is mostly found in cerebral hemisphere of infants often as large cystic lesions frequently attached to the dura. What is the mostly likely diagnosis in this patient?
● A. Gangliocytoma
● B. Desmoplastic infantile ganglioglioma
● C. Desmoplastic infantile astrocytoma
● D. Both A and C
● E. Dysembryoplastic neuroepithelial tumor
D. Both A and C
A patient 15 years old presents with seizures that are not controlled with medication. CT of brain shows hypodense lesion with distinct margins in the temporal lobe of brain. MRI shows hypointense lesion on T1WI and hyperintense lesion on T2WI with nodular enhancement. Histopathology shows cortical glioneuronal tumor with presence of specific glioneuronal elements. What is the WHO grade of dysembryoplastic neuroepithelial tumor (DNT)?
● A. Grade 1
● B. Grade 2
● C. Grade 3
● D. Grade 4
● E. Grade N/A
A. Grade 1
Following are essential criteria for diffuse glioneuronal tumor with oligodendroglioma-like features and nuclear clusters (DGONC) (WHO grade N/A) except?
● A. Methylation profile of DGONC
● B. Nuclear clusters of small to medium sized cells exhibiting oligodendroglioma like morphology
● C. Monosomy of chromosome 14
● D. Strong expression of both OLIG2 and synaptophysin
● E. Absence of widespread GFAP expression
C. Monosomy of chromosome 14
A patient presents with dysplastic cerebellar gangliocytoma with gangliocytic lesion enlarging cerebellar folia and densely packed ganglion cells of various sizes and matrix resembling normal neuropil. What can be the likely presentation of such
tumors?
● A. Signs and symptoms of cerebellar mass
● B. Dysmetria
● C. Hydrocephalus
● D. Cranial nerve deficits
● E. All of the above
E. All of the above
A patient presents in neurosurgical OPD with complaints of seizure and headache. Biopsy of the tumor showed multinodularity with absence of mitotic activity with tumor cell/matrix vacuolation with tumor cell immunoreactivity for synaptophysin. What is the most common location of multinodular and vacuolating neuronal tumor?
● A. Frontal lobe
● B. Temporal lobe
● C. Parietal lobe
● D. Spinal cord
● E. Occipital lobe
Temporal lobe
A patient presents in emergency with signs and symptoms of increased intracranial pressure. On CT of brain an intraventricular lesion was found with calcifications and cystic degeneration, heterogeneously isointense on T1 and hyperintense on T2. MR spectroscopy shows high glycine peak. Which of the following is a recommended treatment for central neurocytoma?
● A. Total resection is often curative with no need of postoperative radiotherapy
● B. Subtotal resection can be followed by stereotactic radiosurgery especially if MIB 1 labelling is more than 2 to 4%
● C. Chemotherapy with alkylating agents, platinum-based agents, or vincristine can be done for recurrent or inoperable tumors
● D. After treatment the patient should have long-term followup imaging surveillance for recurrence
● E. All of the above
E. All of the above
Extraventricular neurocytoma is a variant of central neurocytoma which can be found in all of the following locations except?
● A. Cerebral parenchyma
● B. Brainstem
● C. Thalamus
● D. Corpus callosum
● E. Cerebellum
D. Corpus callosum
