Unusual manifestation of cerebellopontine angle medulloblastoma with tinnitus and sensorineural hearing loss

1. Xia H, Zhong D, Wu X, Li J, Yang Y, Sun X. Medulloblastomas in cerebellopontine angle: Epidemiology, clinical manifestations, imaging features, molecular analysis and surgical outcome. J Clin Neurosci. 2019;67:93-8. DOI: 10.1016/j.jocn.2019.06.013. Open DOISearch in Google Scholar

2. Merchant TE, Pollack IF, Loeffler JS. Brain tumors across the age spectrum: biology, therapy, and late effects. Semin Radiat Oncol. 2010;20(1):5866. DOI: 10.1016/j.semradnoc.2009.09.005. Open DOISearch in Google Scholar

3. Bailey P, Cushing H. Medulloblastoma cerebelli, a common type of mid-cerebellar glioma of childhood. Arch Neurol Psychiatr. 1999;14:192-224. Search in Google Scholar

4. Jaju A, Yeom KW, Ryan ME. MR imaging of pediatric brain tumors. Diagnostics (Basel). 2022;12(4):961. DOI: 10.3390/diagnostics12040961. Open DOISearch in Google Scholar

5. Dung LT, Duc NM. Medulloblastoma in the cerebellopontine angle mimicking a schwannoma. Clin Case Rep. 2021;9(4):1948-53. DOI: 10.1002/ccr3.3912. Open DOISearch in Google Scholar

6. Kadin ME, Rubinstein LJ, Nelson JS. Neonatal cerebellar medulloblastoma originating from the fetal external granular layer. J Neuropathol Exp Neurol. 1970;29(4):583-600. DOI: 10.1097/00005072-197010000-00005. Open DOISearch in Google Scholar

7. Goudihalli SR, Pathak A, Brar R, Mundi I. Reappraisal of cerebellopontine angle medulloblastomas: Report of a fatal case and lessons learned. Interdisciplinary Neurosurgery. 2018;12:20-3. Search in Google Scholar

8. Murase M, Saito K, Abiko T, Yoshida K, Tomita H. Medulloblastoma in older adults: a case report and literature review. World Neurosurg. 2018;117:25-31. DOI: 10.1016/j. wneu.2018.05.216. Open DOISearch in Google Scholar

9. Chen CC, Cheng PW, Tseng HM, Young YH. Posterior cranial fossa tumors in young adults. Laryngoscope. 2006;116(9):1678-81. DOI: 10.1097/01.mlg.0000233507.13293.d9. Open DOISearch in Google Scholar

10. Hadi I, Roengvoraphoj O, Niyazi M, Roeder F, Schuller U, Belka C, et al. Medulloblastoma in adults: A retrospective single institution analysis. Stranhlenther Onkol. 2018;194(3):225-34. DOI: 10.1007/s00066-017-1235-5. Open DOISearch in Google Scholar

11. Spina A, Boari N, Gagliardi F, Franzin A, Terreni MR, Mortini P. Review of cerebellopontine angle medulloblastoma. Br J Neurosurg. 2013;27(3):316-20. DOI: 10.3109/02688697.2012.741733. Open DOISearch in Google Scholar

12. Gil-Salu JL, Rodriguez-Pena F, Lopez-Escobar M, Palomo MJ. Medulloblastoma presenting as an extra-axial tumor in the cerebellopontine angle. Neurocirugia (Astur). 2004;15(3):285-9. DOI: 10.1016/s1130-1473(04)70485-6. Open DOISearch in Google Scholar

13. Akay KM, Erdogan E, Izci Y, Kaya A, Timurkaynak E. Medulloblastoma of the cerebellopontine angle –case report. Neurol Med Chir (Tokyo). 2003;43(11):555-8. DOI: 10.2176/nmc.43.555. Open DOISearch in Google Scholar

14. Britton BH. Adult medulloblastoma: neurotologic manifestations. Ann Otol Rhinol Laryngol. 1975;84(3 Pt 1):364-7. DOI: 10.1177/000348947508400313. Open DOISearch in Google Scholar

15. Galvez EM, Meseguer DH, Ortega FG, Mondejar JM. Sudden deafness and cerebellar tumour. J Laryngol Otol. 1994;108(7):584-6. DOI: 10.1017/s0022215100127495. Open DOISearch in Google Scholar

16. Nishizawa S, Yokoyama T, Hinokuma K, Uemura K. Unilateral sensori-neural hearing disturbance caused by intramedullary cerebellar tumors - three case reports. Neurol Med Chir. 1997;37(9):701-7. DOI: 10.2176/nmc.37.701. Open DOISearch in Google Scholar

17. Mehta JS, Sharr MM. An unusual cause of acute labyrinthine failure. J Laryngol Otol. 1998;112(2):193-5. DOI: 10.1017/s0022215100140290. Open DOISearch in Google Scholar

18. Park SY, Kim JH, Kim KT, Kim YJ, Kim TH, Hwang K, et al. A case of medullomyoblastoma of cerebellopontine angle mimicking acoustic neuroma. Yonsei Med J. 2004;45(4):719-22. DOI: 10.3349/ymj.2004.45.4.719. Open DOISearch in Google Scholar

19. Magliulo G, Parrotto D, Pulice G, Celebrini A, Giangaspero F. Medulloblastoma of the cerebellopontine angle in adulthood. Audiol Med. 2005;3:208-11. Search in Google Scholar

20. Adeleye AO, Balogun JA. Bilateral deafness and blindness from a IVth ventricular medulloblastoma. Br J Neurosurg. 2009;23(3):315-7. DOI: 10.1080/02688690802512858. Open DOISearch in Google Scholar

21. Lobbes M, Hoeberigs MC, van Genugten J, van Oostenbrugge R, Stokroos R, Cornips EM, et al. A rare case of extra-axial medulloblastoma presenting with multiple cranial nerve thickening. Neuro Oncol. 2010;12:1-6. Search in Google Scholar

22. Terakawa Y, Tsuyuguchi N, Takami T, Ohata K. Medulloblastoma manifesting as sudden sensorineural hearing loss. J Korean Neurosurg Soc. 2011;50(1):51-3. DOI: 10.3340/jkns.2011.50.1.51. Open DOISearch in Google Scholar

23. Chou YT, Liao PW, Lin MCH, Chou JC, Lin YH, Lin MH, et al. Medulloblastoma presenting with pure word deafness: report of one case and review of literature. Pediatr Neonatol. 2011;52(5):290-3. DOI: 10.1016/j.pedneo.2011.06.009. Open DOISearch in Google Scholar

24. Amene CS, Yeh-Nayre LA, Crawford JR. Isolated sensorineural hearing loss as initial presentation of recurrent medulloblastoma: neuroimaging and audiologic correlates. Clin Neuroradiol. 2013;23(4):301-3. DOI: 10.1007/s00062-012-0186-6. Open DOISearch in Google Scholar

25. Bahrami E, Bakhti S, Fereshtehnejad SM, Parvaresh M, Khani MR. Extra-axial medulloblastoma in cerebello-pontine angle: A report of a rare case with literature review. Med J Islam Repub Iran. 2014;28:57. Search in Google Scholar

26. Alnaami I, El-Hakim H, Aronyk K, Pugh J, Lu JQ, Mehta V. A pediatric medulloblastoma presenting as an isolated unilateral sensory neural hearing loss. ZUMJ. 2016;22(5):209-13. Search in Google Scholar

27. Roy CF, Yang L, Daniel SJ. A pediatric medulloblastoma presenting as isolated sensori-neural hearing loss: Case report and review of the literature. Int J Pediatr Otorhinolaryngol. 2019;126:109640. DOI: 10.1016/j.ijporl.2019.109640. Open DOISearch in Google Scholar

28. Magg J, Nägele T, Alber M, Weichselbaum A, Ebinger M, Schuhmann MU. Unilateral hearing loss due to cochlear nerve involvement as isolated symptom of a primary medulloblastoma. Neuropediatrics. 2020;51(2):170-2. DOI: 10.1055/s-0039-3399528. Open DOISearch in Google Scholar

29. Aqel W, Salman A, Aldarawish A, Bakri I. Medulloblastoma at the cerebello-pontine angle resembling vestibular schwannoma: A case report and review of the literature. Int J Surg Case Rep. 2022;99:107695. DOI: 10.1016/j.ijscr.2022.107695. Open DOISearch in Google Scholar

30. Louis DN, Perry A, Wesseling P, Brat DJ, Cree IA, Figarella-Branger D, et al. The 2021 WHO Classification of Tumors of the Central Nervous System: a summary. Neuro Oncol. 2021;23(8):1231-51. DOI: 10.1093/neuonc/noab106. Open DOISearch in Google Scholar

31. Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK, et al. The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol. 2016;131(6):803-20. DOI: 10.1007/s00401-016-1545-1. Open DOISearch in Google Scholar

32. Orr BA. Pathology, diagnostics, and classification of medulloblastoma. Brain Pathol. 2020;30(3):664-78. DOI: 10.1111/bpa.12837. Open DOISearch in Google Scholar

33. Furtado SV, Venkatesh PK, Dadlani R, Reddy K, Hegde AS. Adult medulloblastoma and the “dural-tail” sign: rare mimic of a posterior petrous meningioma. Clin Neurol Neurosurg. 2009;111(6):540-3. DOI: 10.1016/j.clineuro.2009.02.002. Open DOISearch in Google Scholar

34. McManamy CS, Pears J, Weston CL, Hanzely Z, Ironside JW, Taylor RE, et al. Nodule formation and desmoplasia in medulloblastomas-defining the nodular/desmoplastic variant and its biological behavior. Brain Pathol. 2007;17(2):151-64. DOI: 10.1111/j.1750-3639.2007.00058.x. Open DOISearch in Google Scholar

35. McManamy CS, Lamont JM, Taylor RE, Cole M, Pearson AD, Clifford SC, et al. Morphophenotypic variation predicts clinical behavior in childhood non-desmoplastic medulloblastomas. J Neuropathol Exp Neurol. 2003;62(6):627-32. DOI: 10.1093/jnen/62.6.627. Open DOISearch in Google Scholar

36. Andreiuolo F, Mazeraud A, Chrétien F, Pietsch T. A global view on the availability of methods and information in the neuropathological diagnostics of CNS tumors: results of an international survey among neuropathological units. Brain Pathol. 2016;26(4):551-4. DOI: 10.1111/bpa.12383. Open DOISearch in Google Scholar

37. Remke M, Hielscher T, Northcott PA, Witt H, Ryzhova M, Wittmann A, et al. Adult medulloblastoma comprises three major molecular variants. J Clin Oncol. 2011;29(19):2717-23. DOI: 10.1200/JCO.2011.34.9373. Open DOISearch in Google Scholar

38. Hovestadt V, Ayrault O, Swartling FJ, Robinson GW, Pfister SM, Northcott PA. Medulloblastomics revisited: biological and clinical insights from thousands of patients. Nat Rev Cancer. 2020;20(1):42-56. DOI: 10.1038/s41568-019-0223-8. Open DOISearch in Google Scholar

39. Patay Z, DeSain LA, Hwang SN, Coan A, Li Y, Ellison DW. MR imaging characteristics of wingless-type-subgroup pediatric medulloblastoma. AJNR Am J Neuroradiol. 2015;36(12):2386-93. DOI: 10.3174/ajnr.A4495. Open DOISearch in Google Scholar

40. Kumar R, Liu APY, Northcott PA. Medulloblastoma genomics in the modern molecular era. Brain Pathol. 2020;30(3):679-90. DOI: 10.1111/bpa.12804. Open DOISearch in Google Scholar

41. Perreault S, Ramaswamy V, Achrol A, Chao K, Liu T, Shih D, et al. MRI surrogates for molecular subgroups of medulloblastoma. AJNR Am J Neuroradiol. 2014;35(7):1263-9. DOI: 10.3174/ajnr.A3990. Open DOISearch in Google Scholar

42. Brandao LA, Poussaint TY. Posterior fossa tumors. Neuroimaging Clin N Am. 2017;27(1):1-37. DOI: 10.1016/j.nic.2016.08.001. Open DOISearch in Google Scholar

43. Franceschi E, Giannini C, Furtner J, Pajtler KW, Asioli S, Guzman R, et al. Adult medulloblastoma: updates on current management and future perspectives. Cancers (Basel). 2022;14(15):3708. DOI: 10.3390/cancers14153708. Open DOISearch in Google Scholar

44. Yeom KW, Mobley BC, Lober RM, Andre JB, Partap S, Vogel H, et al. Distinctive MRI features of pediatric medulloblastoma subtypes. AJR Am J Roentgenol. 2013;200(4):895-903. DOI: 10.2214/AJR.12.9249. Open DOISearch in Google Scholar

45. Pant I, Chaturvedi S, Gautam VKS, Sarma P, Satti DK. Extra-axial adult cerebellopontine angle medulloblastoma: Revisiting a rare entity. J Can Res Ther. 2022;18(3):770-3. DOI: 10.4103/jcrt.JCRT_675_20. Open DOISearch in Google Scholar

46. Bourgouin PM, Tampieri D, Grahovac SZ, Léger C, Del Carpio R, Melançon D. CT and MR imaging findings in adults with cerebellar medulloblastoma: Comparison with findings in children. AJR Am J Roentgenol. 1992;159(3):609-12. DOI: 10.2214/ajr.159.3.1503035. Open DOISearch in Google Scholar

47. Kohan D. Rare tumors of the cerebellopontine angle. In: Kohan D, Heman-Ackah SE, Chandrasekhar SS. Neurotology. Oxford University Press, New York; 2014, p.55-62. Search in Google Scholar

48. Eskey CJ, Belden CJ, Pastel DA, Vossough A, Yoo AJ. Neuroradiology Cases (Cases in Radiology). Oxford University Press, USA; 2012, p.187-8. Search in Google Scholar

49. Baloh RW, Honrubia V, Kerber KA. Tumors. In: Baloh RW, Honrubia V, Kerber KA. Baloh and Honrubia’s clinical neurophysiology of the vestibular system, 4 edn. Contemporary Neurology Series. Oxford University Press, New York; 2011, p.339-351. Search in Google Scholar

50. Franceschi E, Seidel C, Sahm F, Pajtler KW, Hau P. How we treat medulloblastoma in adults. ESMO Open. 2021;6(4):100173. DOI: 10.1016/j.esmoop.2021.100173. Open DOISearch in Google Scholar

51. Rutkowski S, von Hoff K, Emser A, Zwiener I, Pietsch T, Figarella-Branger D, et al. Survival and prognostic factors of early childhood medulloblastoma: an international meta-analysis. J Clin Oncol. 2010;28(33):4961-8. DOI: 10.1200/JCO.2010.30.2299. Open DOISearch in Google Scholar

52. Buglione M, Ghirardelli P, Triggiani L, Pedretti S, Pasinetti N, De Bari B, et al. Radio-therapy for adult medulloblastoma: Long term result from a single institution. A review of prognostic factors and why we do need a multi-institutional cooperative program. Rep Pract Oncol Radiother. 2015;20(4):284-91. DOI: 10.1016/j.rpor.2015.03.003. Open DOISearch in Google Scholar

53. Abidi R, Boussarsar A, Yahyaoui S, Aissaoui D, Mousli A, Kochbati L, et al. Survival and prognostic factors in adult medulloblastoma: the Salah Azaiz Institute experience. Br J Neurosur. 2021;1-4. DOI: 10.1080/02688697.2021.1895969. Open DOISearch in Google Scholar

54. Robinson GW, Rudneva VA, Buchhalter I, Billups CA, Waszak SM, Smith KS, et al. Risk-adapted therapy for young children with medulloblastoma (SJYC07): therapeutic and molecular outcomes from a multicentre, phase 2 trial. Lancet Oncol. 2018;19(6):768-84. DOI: 10.1016/S1470-2045(18)30204-3. Open DOISearch in Google Scholar

55. Ashley DM, Merchant TE, Strother D, Zhou T, Duffner P, Burger PC, et al. Induction chemotherapy and conformal radiation therapy for very young children with nonmetastatic medulloblastoma: Children’s Oncology Group study P9934. J Clin Oncol. 2012;30(26):3181-6. DOI: 10.1200/JCO.2010.34.4341. Open DOISearch in Google Scholar

56. Dufour C, Beaugrand A, Pizer B, Micheli J, Aubelle MS, Fourcade A, et al. Metastatic medulloblastoma in childhood: Chang’s classification revisited. Int J Surg Oncol. 2012;2012:245385. DOI: 10.1155/2012/245385. Open DOISearch in Google Scholar

57. Mohan M, Pande A, Vasudevan MC, Ramamurthi R. Pediatric medulloblastoma: A review of 67 cases at a single institute. Asian J Neurosurgery. 2008;2:63-69. Search in Google Scholar

58. Singh M, Cugati G, Symss NP, Pande A, Vasudevan MC, Ramamurthi R. Extra axial adult cerebellopontine angle medulloblastoma: An extremely rare site of tumor with metastasis. Surg Neurol Int. 2011;2:25 DOI: 10.4103/2152-7806.77178. Open DOISearch in Google Scholar

59. Vigneron C, Antoni D, Coca A, Niederst C, Jarnet D, Meyer P, et al. Adult medulloblastoma: Retrospective series of 21 patients. Cancer Radiother. 2016;20(1):14-7. DOI: 10.1016/j.canrad.2015.07.156. Open DOISearch in Google Scholar

60. Zhang ZY, Xu J, Ren Y, Li KKW, Ng HK, Mao Y, et al. Medulloblastoma in China: clinicopathologic analyses of SHH, WNT, and non-SHH/WNT molecular subgroups reveal different therapeutic responses to adjuvant chemotherapy. PLoS One. 2014;9(6):e99490. DOI: 10.1371/journal.pone.0099490. Open DOISearch in Google Scholar

61. Cavalli FMG, Remke M, Rampasek L, Peacock J, Shih DJH, Luu B, et al. Intertumoral heterogeneity within medulloblastoma subgroups. Cancer Cell. 2017;31(6):737-54.e6. DOI: 10.1016/j.ccell.2017.05.005. Open DOISearch in Google Scholar

62. Clifford SC, Lusher ME, Lindsey JC, Langdon JA, Gilbertson RJ, Straughton D, et al. Wnt/Wingless pathway activation and chromosome 6 loss characterize a distinct molecular sub-group of medulloblastomas associated with a favorable prognosis. Cell Cycle. 2006;5(22):2666-70. DOI: 10.4161/cc.5.22.3446. Open DOISearch in Google Scholar

63. Kool M, Jones DTW, Jäger N, Northcott PA, Pugh TJ, Hovestadt V, et al. Genome sequencing of SHH medulloblastoma predicts genotype-related response to smoothened inhibition. Cancer Cell. 2014;25(3):393-405. DOI: 10.1016/j.ccr.2014.02.004. Open DOISearch in Google Scholar

64. Zhukova N, Ramaswamy V, Remke M, Pfaff E, Shih DJ, Martin DC, et al. Subgroup-specific prognostic implications of TP53 mutation in medulloblastoma. J Clin Oncol. 2013;31(23):2927-35. DOI: 10.1200/JCO.2012.48.5052. Open DOISearch in Google Scholar

65. Remke M, Ramaswamy V, Peacock J, Shih DJH, Koelsche C, Northcott PA, et al. TERT promoter mutations are highly recurrent in SHH subgroup medulloblastoma. Acta Neuropathol. 2013;126(6):917-29. DOI: 10.1007/s00401-013-1198-2. Open DOISearch in Google Scholar

66. Northcott PA, Lee C, Zichner T, Stütz AM, Erkek S, Kawauchi D, et al. Enhancer hijacking activates GFI1 family oncogenes in medulloblastoma. Nature. 2014;511(7510):428-34. DOI: 10.1038/nature13379. Open DOISearch in Google Scholar

67. Seidel C, Heider S, Hau P, Glasow A, Dietzsch S, Kortmann RD. Radiotherapy in medulloblastoma—evolution of treatment, current concepts and future perspectives. Cancers. 2021;13(23):5945. DOI: 10.3390/cancers13235945. Open DOISearch in Google Scholar

68. Thompson EM, Hielscher T, Bouffet E, Remke M, Luu B, Gururangan S, et al. Prognostic value of medulloblastoma extent of resection after accounting for molecular subgroup: a retrospective integrated clinical and molecular analysis. Lancet Oncol. 2016;17(4):484-95. DOI: 10.1016/S1470-2045(15)00581-1. Open DOISearch in Google Scholar

69. Packer RJ, Sutton LN, Elterman R, Lange B, Goldwein J, Nicholson HS, et al. Outcome for children with medulloblastoma treated with radiation and cisplatin, CCNU, and vincristine chemotherapy. J Neurosurg. 1994;81(5):690-8. DOI: 10.3171/jns.1994.81.50690. Open DOISearch in Google Scholar

70. Yock TI, Yeap BY, Ebb DH, Weyman E, Eaton BR, Sherry NA, et al. Long-term toxic effects of proton radiotherapy for paediatric medulloblastoma: a phase 2 single-arm study. Lancet Oncol. 2016;17(3):287-98. DOI: 10.1016/S1470-2045(15)00167-9. Open DOISearch in Google Scholar

71. Liu KX, Ioakeim-Ioannidou M, Susko MS, Rao AD, Yeap BY, Snijders AM, et al. A multi-institutional comparative analysis of proton and photon therapy-induced hematologic toxicity in patients with medulloblastoma. Int J Radiat Oncol Biol Phys. 2021;109(3):726-35. DOI: 10.1016/j.ijrobp.2020.09.049. Open DOISearch in Google Scholar

72. Zhang R, Howell RM, Homann K, Giebeler A, Taddei PJ, Mahajan A, et al. Predicted risks of radiogenic cardiac toxicity in two pediatric patients undergoing photon or proton radiotherapy. Radiat Oncol. 2013;8(1):184. DOI: 10.1186/1748-717X-8-184. Open DOISearch in Google Scholar

73. Eaton BR, Esiashvili N, Kim S, Patterson B, Weyman EA, Thornton LT, et al. Endocrine outcomes with proton and photon radiotherapy for standard risk medulloblastoma. Neuro Oncol. 2016;18(6):881-7. DOI: 10.1093/neuonc/nov302. Open DOISearch in Google Scholar

74. Germanwala AV, Mai JC, Tomycz ND, Niranjan A, Flickinger JC, Kondziolka D, et al. Boost Gamma knife surgery during multimodality management of adult medulloblastoma. J Neurosurg. 2008;108(2):204-9. DOI: 10.3171/JNS/2008/2/0204. Open DOISearch in Google Scholar

75. Flannery T, Kondziolka D, Lunsford LD. Boost Gamma Knife surgery during multimodality management of medulloblastoma/PNET tumors. In: Hayat M. (eds.) Paediatric Cancer, Volume 3. Springer, Dordrecht; 2012, p.159-71. DOI: 10.1007/978-94-007-4528-5_18. Open DOISearch in Google Scholar

76. Packer RJ, Goldwein J, Nicholson HS, et al. Treatment of children with medulloblastomas with reduced-dose craniospinal radiation therapy and adjuvant chemotherapy: a Children’s Cancer Group Study. J Clin Oncol. 1999;17(7):2127-36. Search in Google Scholar

77. Baryawno N, Vseinbjornsson B, Eksborg S, Orrego A, Segerstrom L, Oqvist CO, et al. Tumor-growth-promoting cyclooxygenase-2 prostaglandin E2 pathway provides medulloblastoma therapeutic targets. Neuro Oncol. 2008;10(5):661-74. DOI: 10.1215/15228517-2008-035. Open DOISearch in Google Scholar