Extra-axial medulloblastoma in the cerebellar hemisphere
Keywords:
medulloblastoma, cerebellum, extra-axial, pediatric neoplasms, posterior fossa tumorsAbstract
Introduction: Medulloblastoma is the most frequent malignant, invasive primary tumor of the central nervous system in children. It represents approximately 25% of all pediatric intracranial tumors and 33% of all childhood posterior fossa tumors. It is typically located in the posterior fossa presenting as a midline intraaxial mass involving the cerebellar vermis and/or the roof of the fourth ventricle. Its extra-axial location is rare, few cases are reported in the world literature.
Objective: To describe a case of medulloblastoma with an infrequent location.
Clinical case: This is a 18 years old male patient, who came to the consultation due to headache, gait instability, nausea and vomiting. An extra-axial medulloblastoma in the left cerebellar hemisphere was diagnosed. Total surgical resection of the tumor was performed, with favorable post-surgical evolution.
Conclusions: The extra-axial appearance of medulloblastoma is extremely rare, but it should be considered in the differential diagnosis of extra-axial lesions of the posterior fossa. Most extra-axial medulloblastomas occur in the cerebellar pontine angle. However, extra-axial medulloblastomas can also appear in the cerebellar hemisphere, as in this case.Downloads
References
1. Presutto E, Chappell M, Fullmer J, Ezhapilli S. Posterior fossa medulloblastoma in an atypical extra-axial location: A case report. Radiol Case Rep. 2018 Feb 3;13(2):365-370. doi: 10.1016/j.radcr.2018.01.007. PMID: 29904474; PMCID: PMC5999935.
2. Goyenechea Gutiérrez FF. Tumores de fosa posterior. En: Lesiones del Sistema Nervioso. Goyenechea Gutiérrez FF. Pereira Riverón R.. La Habana: Ciencias Médicas; 2014. p. 161-6.
3. Singh SK, Kumar N. Extraaxial Cerebellopontine Angle Medulloblastoma with Multiple Intracranial Metastases in Adult: A Rare Case Report with a Review of Literature. Asian J Neurosurg. 2020 Aug 28;15(3):695-698. doi: 10.4103/ajns.AJNS_120_20. PMID: 33145231; PMCID: PMC7591221.
4. Singh S, Israrahmed A, Verma V, Singh V. Extra-axial tentorial medulloblastoma: a rare presentation of a common posterior fossa tumour. BMJ Case Rep. 2021 Jun 28;14(6):e242865. doi: 10.1136/bcr-2021-242865. PMID: 34183313; PMCID: PMC8240586.
5. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, Scheithauer BW, Kleihues P. The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol. 2007 Aug;114(2):97-109. doi: 10.1007/s00401-007-0243-4. Epub 2007 Jul 6. Erratum in: Acta Neuropathol. 2007 Nov;114(5):547. PMID: 17618441; PMCID: PMC1929165.
6. Chung EJ, Jeun SS. Extra-axial medulloblastoma in the cerebellar hemisphere. J Korean Neurosurg Soc. 2014 Jun;55(6):362-4. doi: 10.3340/jkns.2014.55.6.362. Epub 2014 Jun 30. PMID: 25237434; PMCID: PMC4166334.
7. Igual Estellés L . Berlanga Charriel P. Cañete Nieto A. Meduloblastoma: mejoría de la supervivencia en las últimas décadas. Experiencia de un centro. An Pediatr (Barc). [Internet] 2017 [citado 29/10/21]; 86(1):4---10. Disponible en: http://dx.doi.org/10.1016/j.anpedi.2016.03.004.
8. Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK, Ohgaki H, Wiestler OD, Kleihues P, Ellison DW. The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol. 2016 Jun;131(6):803-20. doi: 10.1007/s00401-016-1545-1. Epub 2016 May 9. PMID: 27157931.
9. Louis DN, Perry A, Wesseling P, Brat DJ, Cree IA, Figarella-Branger D, Hawkins C, Ng HK, Pfister SM, Reifenberger G, Soffietti R, von Deimling A, Ellison DW. The 2021 WHO Classification of Tumors of the Central Nervous System: a summary. Neuro Oncol. 2021 Aug 2;23(8):1231-1251. doi: 10.1093/neuonc/noab106. PMID: 34185076; PMCID: PMC8328013.
10. Al-Sharydah AM, Al-Abdulwahhab AH, Al-Suhibani SS, Al-Issawi WM, Al-Zahrani F, Katbi FA, Al-Thuneyyan MA, Jallul T, Mishaal Alabbas F. Posterior fossa extra-axial variations of medulloblastoma: a pictorial review as a primer for radiologists. Insights Imaging. 2021 Apr 6;12(1):43. doi: 10.1186/s13244-021-00981-z. PMID: 33822292; PMCID: PMC8024434.
11. Pant I, Chaturvedi S, Gautam VK, Pandey P, Kumari R. Extra-axial medulloblastoma in the cerebellopontine angle: Report of a rare entity with review of literature. J Pediatr Neurosci. 2016 Oct-Dec;11(4):331-334. doi: 10.4103/1817-1745.199477. PMID: 28217158; PMCID: PMC5314849.
12. Thanh Dung L, Minh Duc N. Medulloblastoma in the cerebellopontine angle mimicking a schwannoma. Clin Case Rep. 2021 Feb 12;9(4):1948-1953. doi: 10.1002/ccr3.3912. PMID: 33936621; PMCID: PMC8077292.
13. Duc NM, Huy HQ. Magnetic Resonance Imaging Features of Common Posterior Fossa Brain Tumors in Children: A Preliminary Vietnamese Study. Open Access Maced J Med Sci. 2019 Aug 14;7(15):2413-2418. doi: 10.3889/oamjms.2019.635. PMID: 31666838; PMCID: PMC6814486.
14. Doan NB, Patel M, Nguyen HS, Janich K, Montoure A, Shabani S, Gelsomino M. A Rare Extra-Axial Midline Tentorial Adult Medulloblastoma with Dural-Tail Sign Mimicking a Meningioma. Asian J Neurosurg. 2018 Apr-Jun;13(2):475-477. doi: 10.4103/1793-5482.228563. PMID: 29682065; PMCID: PMC5898136.
15. Minh Thong P, Minh Duc N. The Role of Apparent Diffusion Coefficient in the Differentiation between Cerebellar Medulloblastoma and Brainstem Glioma. Neurol Int. 2020 Oct 29;12(3):34-40. doi: 10.3390/neurolint12030009. PMID: 33137983; PMCID: PMC7768368.
16. Thanh Dung L, Minh Duc N. Medulloblastoma in the cerebellopontine angle mimicking a schwannoma. Clin Case Rep. 2021 Feb 12;9(4):1948-1953. doi: 10.1002/ccr3.3912. PMID: 33936621; PMCID: PMC8077292.
17. Rutka JT, Kuo JS, Carter M, Ray A, Ueda S, Mainprize TG. Advances in the treatment of pediatric brain tumors. Expert Rev Neurother. 2004 Sep;4(5):879-93. doi: 10.1586/14737175.4.5.879. PMID: 15853514.
