Ependymal Tumors Flashcards
Supratentorial ependymomas (WHO grades 2 and 3) are well-circumscribed supratentorial glioma with uniform cells having round nuclei that form focal pseudorosettes or ependymal rosettes embedded in a fibrillary matrix. These are thought to arise from radial glial cells. Which of the following statements is correct regarding treatment of these tumors?
● A. Postoperative radiotherapy has no role in these tumors
● B. WHO grade is the most important prognosticator of survival in adults and children
● C. The outcome is worse for infratentorial than for supratentorial ependymomas in adults
● D. Gross total removal is the most important prognosticator of overall survival in adults and children
● E. Gross total removal cannot be achieved in most of the cases
D. Gross total removal is the most important prognosticator of overall survival in adults and children
Supratentorial tumors with morphological and immunohistochemical features of ependymoma and ZFTA fusion positive are WHO grade 2 or 3 tumors. What is the most common location for these tumors?
● A. Frontal lobe
● B. Parietal lobe
● C. Both A and B
● D. Thalamus
● E. Hypothalamus/third ventricular region
C. Both A and B
Supratentorial tumor with morphological and immunohistochemical features of ependymoma with YAP1 fusion positive seem to be restricted to young children. What is the most common location for these tumors?
● A. Thalamus
● B. Hypothalamus
● C. Within or adjacent to the lateral ventricle
● D. Temporal lobe
● E. In spinal cord
C. Within or adjacent to the lateral ventricle
A patient presents in neurosurgical emergency with signs of raised intracranial pressure, diplopia, and facial weakness. MRI of brain was done and posterior fossa ependymoma (WHO grade 2 or 3) was diagnosed radiographically. Following statements are correct regarding this tumor except?
● A. These are mostly found in the floor of the fourth ventricle
● B. Ependymomas have the potential to spread through CSF to the entire neuraxis, a process known as seeding resulting in so-called drop mets
● C. There is no need to do imaging of the entire neuraxis
● D. Extension through the foramina of Luschka or Magendie may be seen
● E. The tumor enhances with IV contrast except for cystic portion or areas of necrosis
C. There is no need to do imaging of the entire neuraxis
Which of the following statements is incorrect regarding treatment of posterior fossa ependymoma?
● A. The goal of surgery is maximal possible resection of intracranial portion without causing neurological deficit, while gross total resection may not be possible in many cases because of extensive involvement of the floor of the fourth ventricle
● B. Lesions in the fourth ventricle region are approached via midline suboccipital craniectomy
● C. Traditional radiotherapy is 45 to 48 Gy to tumor bed, while for spinal drop mets, 30 Gy is given to the entire spinal axis with boost to regions showing drop mets
● D. There is very small risk of recurrence following subtotal resection
● E. 5-year survival in pediatric population is 20 to 30%, while it is 80% in adults
D. There is very small risk of recurrence following subtotal resection
Posterior fossa group A (PFA) ependymoma and posterior fossa group B (PFB) ependymoma are both WHO grade 2 or 3 tumors. PFA ependymomas most frequently occurs in preadolescents, while group B ependymoma mostly occurs in adults and adolescents. The prognosis of both these tumors depends on the extent of resection. Which statement is correct regarding location of these tumors?
● A. Both of these tumors arise from the floor of the fourth ventricle
● B. Group B tumors arise from the floor, while group A tumors arise from the roof or lateral wall
● C. Group B tumors arise from the roof or lateral wall, while group A tumors arise from the floor of the fourth ventricle
● D. Both tumors arise from the roof of the fourth ventricle
● E. Location of these tumors is variable in every patient
B. Group B tumors arise from the floor, while group A tumors arise from the roof or lateral wall
Spinal ependymomas are intramedullary spinal gliomas with vast majority of these tumors being grade 2 (grade 3 tumors being rare) and most common location at cervical and cervicothoracic levels. Which of the following are the imaging
characteristics of these tumors?
● A. Hypointense on T1 and T2 with no enhancement and often eccentric and poorly demarcated
● B. Hypointense on T1 and hyperintense on T2 with contrast enhancement and often eccentric and poorly demarcated
● C. Hypointense on T1 and hyperintense on T2 with contrast enhancement, and more centrally located and more readily defined
● D. Hypointense on T1 and hypointense on T2 with no contrast enhancement, and more centrally located and more readily defined
● E. Hyperintense on T1 and T2 with contrast enhancement, and more centrally located and more readily defined
C. Hypointense on T1 and hyperintense on T2 with contrast enhancement, and more centrally located and more readily defined
Myxopapillary ependymomas are WHO grade 2 tumors that essentially only occur at conus medullaris or filum terminale and are differentiated from metastatic carcinoma, paragangliomas, schwannomas, chordomas, and myxoid chondrosarcomas by using GFAP staining. Complete excision of these tumors is often not possible due to invasion of the spinal cord parenchyma and/or CSF dissemination. What is the prognosis of these
tumors?
● A. Poor prognosis with 10-year survival less than 50%
● B. Unfavorable prognosis with 10-year survival less than 90%
● C. Favorable prognosis with 10-year survival more than 50% in adults and children
● D. Poor prognosis with 10-year survival less than 10%
● E. Favorable prognosis with 10-year survival of more than 90%
E. Favorable prognosis with 10-year survival of more than 90%
Subependymomas are WHO grade 1 tumors which are mostly incidentally found as nonenhancing, well-demarcated intraventricular mass and are followed up with serial imaging if asymptomatic. What is the most common location for these tumors?
● A. Fourth ventricle
● B. Third ventricle
● C. Lateral ventricles
● D. Sylvian aqueduct
● E. Frontal horn of lateral ventricle
A. Fourth ventricle
Spinal ependymoma, MYCN-amplified, is a rare intramedullary tumor, mostly found in cervical and thoracic cord region and shows the same imaging characteristics as other ependymomas. These tumors have poor prognosis and early spread
throughout the neuraxis is frequent. What is the recurrence rate of these tumors which are reported up till now?
● A. 100%
● B. 80%
● C. 70%
● D. 60%
● E. 50%
A. 100%
Ependymomas are believed to arise from bipolar progenitor cells that are thought to be a major source of neurons in the developing nervous system. What are these cells also called?
● A. Radial glial cells
● B. Progenitor cells
● C. Axial cells
● D. Circumferential cells
● E. CD4 positive cells
A. Radial glial cells
All of the following molecular genetic studies are done to subclassify ependymomas except?
● A. ZFTA fusion
● B. YAP1 fusion
● C. P65
● D. L1CAM
● E. BRAF
E. BRAF
Posterior fossa group B ependymomas are mostly WHO grade II/III tumors but have a worse prognosis as they can often be not removed completely due to their propensity to invade which of the following?
● A. Cerebral cortex
● B. Basal cranial nerves
● C. Obex
● D. Mid brain
● E. White matter tracts
C. Obex
Ependymomas rank 2nd only to medulloblastomas in radiosensitivity. Radiotherapy is administered after surgical excision. What is the dose to tumor bed for cranial lesions?
● A. 30 Gy
● B. 35 to 38 Gy
● C. 3D conformal XRT with 59.4 Gy to tumor bed + 1 cm margin
● D. 62 Gy
● E. 65 Gy
C. 3D conformal XRT with 59.4 Gy to tumor bed + 1 cm margin
A young child was operated on for ependymoma. After 2 weeks, CSF cytology was performed that showed evidence of drop mets. What will be the radiotherapy dose to the entire spine in this case?
● A. 30 Gy
● B. 35 to 38 Gy
● C. 45 to 48 Gy
● D. 62 Gy
● E. 65 Gy
A. 30 Gy
