J Korean Neurosurg Soc.  2014 Jun;55(6):362-364. 10.3340/jkns.2014.55.6.362.

Extra-Axial Medulloblastoma in the Cerebellar Hemisphere

• Chung EJ
• Jeun SS

• KMID: 2191118
• DOI: http://doi.org/10.3340/jkns.2014.55.6.362

Abstract

Extra-axial medulloblastoma is a rare phenomenon. We report a case in a 5-year-old boy who presented with nausea, vomiting, and gait disturbance. He was treated with total removal of the tumor. This is the first case of an extra-axially located medulloblastoma occurring in the cerebellar hemisphere posteriolateral to the cerebellopontine angle in Korea. Although the extra-axial occurrence of medulloblastoma is rare, it should be considered in the differential diagnosis of extra-axial lesions of the posterior fossa in children.

Keyword

Medulloblastoma; Cerebellum; Cerebral hemispheres; Child

MeSH Terms

Cerebellopontine Angle
Cerebellum
Cerebrum
Child
Child, Preschool
Diagnosis, Differential
Gait
Humans
Korea
Male
Medulloblastoma*
Nausea
Vomiting

Figure

• 

  Fig. 1 A well-defined, oval mass in the left posterior fossa measuring about 5.1×3.1×4.6 cm. The mass is compressing the fourth ventricle, causing hydrocephalus. A : Gadolium-enhanced axial T1-weighted MRI. B : Gadolium-enhanced coronal T1-weighted MRI.

• 

  Fig. 2 A : A soft, well-demarcated mass is observed extra-axially, attached to the dura next to the transverse-sigmoid sinus with invasion into the cerebellar hemisphere. B : The pale nodular areas contain tumor cells having low nuclear cytoplasmic ratio and the dark cellular areas are highly cellular with a high nuclear cytoplasm ratio (H&E, ×400). C : The pale area showing immunoreactivity for neuron specific enolase (×100). D : The dark cellular areas showing high Ki67 proliferative index (×100).

Reference

1. Brackmann DE, Bartels LJ. Rare tumors of the cerebellopontine angle. Otolaryngol Head Neck Surg (1979). 1980; 88:555–559. PMID: 6969383.

2. Britton BH. Adult medulloblastoma : neurotologic manifestations. Ann Otol Rhinol Laryngol. 1975; 84(3 Pt 1):364–367. PMID: 1079441.

3. Chatty EM, Earle KM. Medulloblastoma. A report of 201 cases with emphasis on the relationship of histologic variants to survival. Cancer. 1971; 28:977–983. PMID: 5111749.

4. Ehrenberger K. Endoscopy of the cerebellopontine angle. Endoscopy. 1978; 10:260–264. PMID: 310770.

5. Fujita S, Shimada M, Nakamura T. H3-thymidine autoradiographic studies on the cell proliferation and differentiation in the external and the internal granular layers of the mouse cerebellum. J Comp Neurol. 1966; 128:191–208. PMID: 5970298.

6. House JL, Burt MR. Primary CNS tumors presenting as cerebellopontine angle tumors. Am J Otol. 1985; (Suppl):147–153. PMID: 3878087.

7. Jaiswal AK, Mahapatra AK, Sharma MC. Cerebellopointine angle medulloblastoma. J Clin Neurosci. 2004; 11:42–45. PMID: 14642364.

8. Kadin ME, Rubinstein LJ, Nelson JS. Neonatal cerebellar medulloblastoma originating from the fetal external granular layer. J Neuropathol Exp Neurol. 1970; 29:583–600. PMID: 5471923.

9. Kumar R, Achari G, Mishra A, Chhabra DK. Medulloblastomas of the cerebellopontine angle. Neurol India. 2001; 49:380–383. PMID: 11799412.

10. Mohan M, Pande A, Vasudevan MC, Ramamurthi R. Pediatric medulloblastoma : a review of 67 cases at a single institute. Asian J Neurosurg. 2008; 2:63–69.

11. Naim-ur-Rahman , Jamjoom A, al-Rayess M, Jamjoom ZA. Cerebellopontine angle medulloblastoma. Br J Neurosurg. 2000; 14:262–263. PMID: 10912209.

12. Park TS, Hoffman HJ, Hendrick EB, Humphreys RP, Becker LE. Medulloblastoma : clinical presentation and management. Experience at the hospital for sick children, toronto, 1950-1980. J Neurosurg. 1983; 58:543–552. PMID: 6827349.

13. Pramanik P, Sharma MC, Mukhopadhyay P, Singh VP, Sarkar C. A comparative study of classical vs. desmoplastic medulloblastomas. Neurol India. 2003; 51:27–34. PMID: 12865511.

14. Raaf J, Kernohan JW. Relation of abnormal collections of cells in posterior medullary velum of cerebellum to origin of medulloblastoma. Arch Neurol Psychiatry. 1944; 52:163–169.

15. Ringertz N, Tola JH. Medulloblastoma. J Neuropathol Exp Neurol. 1950; 9:354–372. PMID: 14784854.

16. Stevenson L, Echlim F. Nature and origin of some tumors of cerebellum : Medullobastoma. Arch Neurol Psychiatry. 1934; 31:93–109.

17. Weisberg LA. Computerized tomographic findings in medulloblastomas. Comput Radiol. 1982; 6:83–91. PMID: 7083845.

18. Yamada S, Aiba T, Hara M. Cerebellopontine angle medulloblastoma : case report and literature review. Br J Neurosurg. 1993; 7:91–94. PMID: 8435152.

19. Zee CS, Segall HD, Miller C, Ahmadi J, McComb JG, Han JS, et al. Less common CT features of medulloblastoma. Radiology. 1982; 144:97–102. PMID: 6979760.