CASE REPORT

Meduloblastoma Nodular Desmoplásico Localizado no Ângulo Pontocerebelar. Relato de Caso

Desmoplastic Nodular Medulloblastoma Located at the Cerebellopontine Angle. Case Report

  • Augusto Cesar Santos Esmeraldo (1,2)
  • Ivison Xavier Duarte (3)
  • Marcos Paulo Teixeira Santos (4)
  • Carlos Umberto Pereira (5,6)
  Views: 171
  Downloads: 7

Resumo

Introdução: o meduloblastoma é raro na idade adulta. A localização primária no ângulo pontocerebelar é pouco relatada na literatura. Relato do caso: paciente de 22 anos de idade, masculino, com história de vertigem e zumbidos associado a cefaleia. Submetido à ressonância magnética do crânio que demonstrou lesão expansiva no ângulo pontocerebelar direito. Resultado: submetido a craniectomia suboccipital direita e exérese total da lesão. Resultado do exame anatomopatológico foi de meduloblastoma nodular desmoplásico. Conclusão: devemos considerar a possibilidade de meduloblastoma no diagnóstico diferencial em lesões que envolvem o ângulo pontocerebelar.

Palavras-chave

Tumor; Ângulo pontocerebelar; Diagnóstico diferencial; Meduloblastoma; Fossa posterior

Abstract

Introduction: medulloblastoma is rare in adulthood. The primary location in the cerebellopontine angle has few reports in the literature. Case presentation: a 22-year-old male patient with a history of vertigo and tinnitus associated with headache is reported. He was submitted to magnetic resonance imaging of the skull and an expansive lesion at the right cerebellopontine angle was found. Results: right suboccipital craniectomy and total lesion excision were performed. Diagnosis was set as Desmoplastic/nodular medulloblastoma. Conclusion: we should consider the possibility of diagnosis of medulloblastoma in the differential diagnosis in lesions involving the cerebellopontine angle.

Keywords

Tumor; Cerebellopontine angle; Differential diagnosis; Medulloblastoma; Posterior fossa

References

1. Fallah A, Banglawala SM, Provias J, Jha NK. Extra-axial medulloblastoma in the cerebellopontine angle. Can J Surg. 2009;52(4):E101-2. PMid:19680492.

2. Akay KM, Erdogan E, Izci Y, Kaya A, Timurkaynak E. Medulloblastoma of the cerebellopontine angle: case report. Neurol Med Chir. 2003;43(11):555-8. http://dx.doi.org/10.2176/nmc.43.555. PMid:14705323.

3. Farwell JR, Flannery JT. Adult occurrence of medulloblastoma. Acta Neurochir. 1987;86(1-2):1-5. http://dx.doi.org/10.1007/BF01419496. PMid:3618300.

4. Brunori A, Scarano P, Chiappetta F. Non-acoustic neuroma tumor (NANT) of the cerebello-pontine angle: a 15-year experience. J Neurosurg Sci. 1997;41(2):159-68. PMid:9385566.

5. Moffat DA, Ballagh RH. Rare tumors of the cerebellopontine angle. Clin Oncol. 1995;7(1):28-41. http://dx.doi.org/10.1016/S0936- 6555(05)80632-6. PMid:7727303.

6. Bonneville F, Sarrazin JL, Marsot-Dupuch K, et al. Unusual lesions of the cerebellopontine angle: a segmental approach. Radiographics. 2001;21(2):419-38. http://dx.doi.org/10.1148/ radiographics.21.2.g01mr13419. PMid:11259705.

7. Akay KM, Erdogan E, Izci Y, Kaya A, Timurkaynak E. Medulloblastoma of the cerebellopontine angle: case report. Neurol Med Chir. 2003;43(11):555-8. http://dx.doi.org/10.2176/nmc.43.555. PMid:14705323.

8. Becker RL, Becker AD, Sobel DF. Adult medulloblastoma: review of 13 cases with emphasis on MRI. Neuroradiology. 1995;37(2):104-8. http://dx.doi.org/10.1007/BF00588622. PMid:7760992.

9. Mueller DP, Moore SA, Sato Y, Yuh WT. MRI spectrum of medulloblastoma. Clin Imaging. 1992;16(4):250-5. http://dx.doi. org/10.1016/0899-7071(92)90007-V. PMid:1473031.

10. Naim-ur-Rahman, Jamjoom A, al-Rayess M, Jamjoom ZA. Cerebellopontine angle medulloblastoma. Br J Neurosurg. 2000;14(3):262- 3. http://dx.doi.org/10.1080/026886900408504. PMid:10912209.

11. 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(1):25. http:// dx.doi.org/10.4103/2152-7806.77178. PMid:21427791.

12. Yamada S, Aiba T, Hara M. Cerebellopontine angle medulloblastoma: case report and literature review. Br J Neurosurg. 1993;7(1):91-4. http:// dx.doi.org/10.3109/02688699308995062. PMid:8435152.

13. Yadav P, Jantre M, Thakkar D. Magnetic resonance imaging of cerebellopontine angle lesions. Med J DY Patil Univ. 2015;8(6):751-9. http://dx.doi.org/10.4103/0975-2870.169917.

14. Bourgouin PM, Tampieri D, Grahovac SZ, Léger C, Del Carpio R, Melançon DCT. CT and MR imaging findings in adults with cerebellar medulloblastoma: comparison with findings in children. AJR Am J Roentgenol. 1992;159(3):609-12. http://dx.doi.org/10.2214/ ajr.159.3.1503035. PMid:1503035.

15. Ahn MS, Jackler RK. Exophytic brain tumors mimicking primary lesions of the cerebellopontine angle. Laryngoscope. 1997;107(4):466-71. http://dx.doi.org/10.1097/00005537-199704000-00008. PMid:9111375.

16. Nagai M, Nakayama S, Kusunoki H, Sasaki M, Yamada R. Medulloblastoma originating in the pons. No Shinkei Geka. 1999;27(3):281-5. PMid:10190165.

17. Kumar R, Achari G, Mishra A, Chhabra DK. Medulloblastoma of the cerebellopontine angle. Neurol India. 2001;49(4):380-3. PMid:11799412.

18. Jaiswal AK, Mahapatra AK, Sharma MC. Cerebellopontine angle medulloblastoma. J Clin Neurosci. 2004;11(1):42-5. http://dx.doi. org/10.1016/j.jocn.2003.02.005. PMid:14642364.

19. Brackmann DE, Bartels LJ. Rare tumors of the cerebellopontine angle. Otolaryngol Head Neck Surg. 1980;88(5):555-9. http://dx.doi. org/10.1177/019459988008800508. PMid:6969383.

20. House JL, Burt MR. Primary CNS tumors presenting as cerebellopontine angle tumors. Am J Otol. 1985;6(2, Suppl.):147-53. PMid:3878087.

21. Nyanaveelan M, Azmi A, Saffari M, Banu SK, Suryati MY, Jeyaledchumy M. Cerebellopontine angle medulloblastoma. Med J Malaysia. 2007;62(2):173-4. PMid:18705459.

22. Kool M, Jones DTW, Jäger N, et al. Genone sequencing of SHH medulloblastoma predicts genotype-related response to smoothened inhibition. Cancer Cell. 2014;25(3):393-405. http://dx.doi.org/10.1016/j. ccr.2014.02.004. PMid:24651015.

23. Ellison DW, Dalton J, Kocak M, et al. Medulloblastoma: clinicopathological correlates of SHH, WNT, and non-SHH/WNT molecular subgroups. Acta Neuropathol. 2011;121(3):381-96. http:// dx.doi.org/10.1007/s00401-011-0800-8. PMid:21267586.

24. Roberts RO, Lynch CF, Jones MP, Hart MN. Medulloblastoma: a population based study of 532 cases. J Neuropathol Exp Neurol. 1991;50(2):134-44. http://dx.doi.org/10.1097/00005072-199103000- 00005. PMid:2010773.


(1) MD, Head of the Neurosurgery Medical Residency, Fundação Beneficência Hospital de Cirurgia, Aracaju (SE), Brazil.

(2) Neurosurgeon, Hospital de Urgência de Sergipe – HUSE, Aracaju (SE), Brazil.

(3) MD, Pathogist, Fundação Beneficência Hospital Cirurgia, Aracaju (SE), Brazil.

(4) MD, MR, Medical Residency in Neurosurgery, Fundação Beneficência Hospital de Cirurgia, Aracaju (SE), Brazil.

(5) MD, PhD, Preceptor at Neurosurgery Service, Fundação Beneficência Hospital de Cirurgia, Aracaju (SE), Brazil.

(6) Neurosurgeon, Hospital de Urgência de Sergipe – HUSE, Aracaju (SE), Brazil.

 

Received Mar 7, 2019
Accepted Mar 31, 2022

JBNC  Brazilian Journal of Neurosurgery

  •   ISSN (print version): 0103-5118
  •   e-ISSN (online version): 2446-6786

Contact

Social Media

   

ABNc  Academia Brasileira de Neurocirurgia

  •   Rua da Quitanda 159 – 10º andar - Centro - CEP 20091-005 - Rio de Janeiro - RJ
  •   +55 21 2233.0323
  •    abnc@abnc.org.br