Subependymoma - atypical appearance
Six-month history of headache, dizziness, and loss of balance.
Loading Stack -
0 images remaining
The MRI with gadolinium shows a cystic mass with mural nodules with heterogeneous enhancement of the solid area with compressive effect on the fourth ventricle.
No diffusion imaging abnormalities were revealed.
Loading Stack -
0 images remaining
Clusters of isomorphic nuclei embedded in a dense, fine, glial fibrillary background, occasional calcification, no necrosis or hemorrhage. GFAP positive, P53 negative, Ki 67 proliferation index < 1%.
Final diagnosis: Subependymoma WHO grade I
Cortesy Fernando Velandia Hurtado MD.
1 case question available
This is an unusual case in that the appearance of this tumor is quite atypical for a subependymoma.
Subependymomas are slow-growing and uncommon neoplasms representing 0, 51% of all CNS tumors that arise within the ventricular system, and the spinal cord and are frequently found in the lateral wall of the fourth ventricular in about 50 to 60%.
Subependymomas, previously known as subependymal astrocytomas, are low-grade noninvasive gliomas corresponding to WHO grade I with favorable prognosis and the origin is unknown. Still, some findings suggest that it may arrive from multipotent glial progenitors in the subventricular zone.
Histopathologic are formed by clusters of isomorphic nuclei embedded in a dense, fine glial fibrillary background, occasional calcification. No necrosis or hemorrhage, also GFAP positive, KI 67 proliferation index < 1, and P53 negative.
DNA methylation divided subependymomas into three molecular subgroups: supratentorial(ST), posterior fossa(PF), and spinal (SP).
On MRI, the subependymomas usually are a well-defined solid or mixed solid and cystic intraventricular mass. The solid component is hypointense or isointense in T1WI and hyperintense in T2WI and FLAIR. They usually have focal or minimal enhancement. Hydrocephalus is very common until 94%. Hemorrhage or calcification may also be present. Cystic degeneration is seen in 80% of the patients.
Differential diagnoses include other intraventricular neoplasms such as central neurocytomas, choroid plexus papilloma, meningiomas, low-grade astrocytoma, ependymomas, medulloblastomas and others like hemangioblastoma or pilocytic astrocytoma.
The treatment included surveillance, gross total surgical resection, subtotal resection with or without radiotherapy with no significant role for radiation concerning overall survival in the SEER analysis. Immunotherapeutic treatment is potentially beneficial as an adjunct to surgery or who are not surgical candidates. There is no benefit from chemotherapy.
- Kong LY, Wei J, Haider AS, Liebelt BD, Ling X, Conrad CA, Fuller GN, Levine NB, Priebe W, Sawaya R, Heimberger AB. Therapeutic targets in subependymoma. (2014) Journal of neuroimmunology. 277 (1-2): 168-75. doi:10.1016/j.jneuroim.2014.10.008 - Pubmed
- D'Amico RS, Praver M, Zanazzi GJ, Englander ZK, Sims JS, Samanamud JL, Ogden AT, McCormick PC, Feldstein NA, McKhann GM, Sisti MB, Canoll P, Bruce JN. Subependymomas Are Low-Grade Heterogeneous Glial Neoplasms Defined by Subventricular Zone Lineage Markers. (2017) World neurosurgery. 107: 451-463. doi:10.1016/j.wneu.2017.08.009 - Pubmed
- Takako Tokumitsu, Yuichiro Sato, Tsuyoshi Fukushima, Hideo Takeshima, Shinya Sato, Yujiro Asada. Squash cytology findings of subependymomas: A report of three cases and differential diagnosis. (2018) Diagnostic Cytopathology. 46 (3): 258. doi:10.1002/dc.23840 - Pubmed
- Abdel-Aal AK, Hamed MF, Al Naief NS, Vattoth S, Bag A. Unusual appearance and presentation of supratentorial subependymoma in an adult patient. (2012) Journal of radiology case reports. 6 (8): 8-16. doi:10.3941/jrcr.v6i8.999 - Pubmed
- Ehrhart IC, Parker PE, Weidner WJ, Dabney JM, Scott JB, Haddy FJ. Coronary vascular and myocardial responses to carotid body stimulation in the dog. (1975) The American journal of physiology. 229 (3): 754-60. doi:10.1152/ajplegacy.19184.108.40.2064 - Pubmed
- Heather Leeper, Michelle M. Felicella, Tobias Walbert. Recent Advances in the Classification and Treatment of Ependymomas. (2017) Current Treatment Options in Oncology. 18 (9): 55. doi:10.1007/s11864-017-0496-7 - Pubmed
- Kim Y, Lee SY, Yi KS, Cha SH, Gang MH, Cho BS, Lee YM. Infratentorial and intraparenchymal subependymoma in the cerebellum: case report. (2014) Korean journal of radiology. 15 (1): 151-5. doi:10.3348/kjr.2014.15.1.151 - Pubmed