REVIEW

Cerebellopontine Angle Hemangioblastoma Imaging Features: an illustrated systematic review

Características Imaginológicas do Hemagioblastoma Pontocerebelar: revisão sistemática ilustrada

  • Naiany Olivotto Martins    Naiany Olivotto Martins
  • Marianna Rafaelly Sforza Auerswald    Marianna Rafaelly Sforza Auerswald
  • Yeda da Silva    Yeda da Silva
  • Giocondo Sabedotti
  • Bruno Lucena Antunes Abrantes    Bruno Lucena Antunes Abrantes
  • Claudio Fróes de Freitas    Claudio Fróes de Freitas
  • Emiko Saito Arita    Emiko Saito Arita
  • Plauto Christopher Aranha Watanabe    Plauto Christopher Aranha Watanabe
  • Luciana Munhoz    Luciana Munhoz
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Resumo

O hemangioblastoma localizado no ângulo pontocerebelar (ACP) é pouco frequente e seu diagnóstico é desafiador. Assim, o objetivo desta revisão é delinear as características de imagem do hemangioblastoma de ACP usando vários exames de imagem para fornecer uma referência para os médicos ao estabelecer um diagnóstico e o tratamento. Além disso, é descrito um relato de caso. As bases de dados pesquisadas até outubro de 2023 foram incluídas utilizando palavras-chave para hemangioblastoma combinadas com palavras-chave para técnicas de diagnóstico por imagem. Foram incluídos 24 estudos que consistiram em 18 relatos de caso e 6 casos. A síndrome de Von Hippel-Lindau (BVS) associada ao hemangioblastoma de ACP foi identificada em nove estudos. As características da imagem do hemangioblastoma de ACP dependem do tipo de lesão (cística, sólida e mista cística/sólida). Os tumores por TC são principalmente hipodensos, com forte realce heterogêneo pelo contraste. O exame de ressonância magnética mostra hiperintensidade ponderada em T2 se um componente cístico estiver presente. Os principais diagnósticos diferenciais incluem PCR, neuroblastoma e meningioma. O tratamento cirúrgico deve considerar as características de imagem para escolher uma abordagem mais segura. Registro PROSPERO CRD42023408847.

Palavras-chave

Hemangioblastoma; Neoplasmas cerebelares; Ressonância magnética; Tomografia computadorizada

Abstract

Hemangioblastoma located in the cerebellopontine angle (CPA) is infrequent and its diagnosis is challenging. Hence, the objective of this review is to outline the imaging features of CPA hemangioblastoma using various imaging examinations to provide a reference for physicians when establishing diagnosis and treatment. Moreover, a case report is described. Databases searched up to October 2023 were included using keywords for hemangioblastoma combined with keywords for diagnostic imaging techniques. A total of 24 studies were included that consisted of 18 case reports and 6 cases. Von Hippel–Lindau (VHL) syndrome associated with PCA hemangioblastoma was identified in nine studies. CPA hemangioblastoma imaging features depend on lesion type (cystic, solid, and mixed cystic/solid). CT tumors are mainly hypodense, with strong heterogeneous contrast enhancement. Magnetic resonance imaging testing shows T2- weighted hyperintensity if a cystic component is present. The main differential diagnoses include CPA neuroblastoma and meningioma. Surgical treatment should consider imaging features to choose a safer approach. PROSPERO registration CRD42023408847.

Keywords

Hemagioblastoma; Cerebellopontine angle; Cerebellar neoplasms; Magnetic resonance imaging; Computed tomography

References

1. Matsushima T. The cerebellopontine angle: basic structures and the “rules of three.” In: Matsushima T, editor. Microsurgical anatomy and surgery of the posterior cranial fossa. Tokyo: Springer; 2016. pp. 101-108
2. Cheng J, Liu W, Zhang S, Lei D, Hui X. Clinical features and surgical outcomes in patients with cerebellopontine angle hemangioblastomas: retrospective series of 23 cases. World Neurosurg. 2017;103:248-56. http://doi.org/10.1016/j.wneu.2017.03.144. PMid:28400229.
3. Feletti A, Marrone F, Barresi V, Sala F. Hemangioblastoma with contrast-enhanced cystic wall: when the surgical rule must not be respected. World Neurosurg. 2021;149:190-4. http://doi.org/10.1016/j. wneu.2021.02.111. PMid:33677088.
4. Kuharic M, Jankovic D, Splavski B, Boop FA, Arnautovic KI. Hemangioblastomas of the posterior cranial fossa in adults: demographics, clinical, morphologic, pathologic, surgical features, and outcomes: a systematic review. World Neurosurg. 2018;110:e1049-62. http://doi.org/10.1016/j.wneu.2017.11.173. PMid:29229339.
5. Persad AR, Khormi YH, van Landeghem F, Chow MM. Unusual case of hemangioblastoma of the cerebellopontine angle. Surg Neurol Int. 2017;8(1):264. http://doi.org/10.4103/sni.sni_310_17. PMid:29184715.
6. Coulibaly O, Diawara S, Fatemi N, et al. Cerebellar liponeurocytoma mimicking cerebellopontine angle epidermoid cyst: a case report. Case Reports in Clinical Medicine. 2014;3(4):235-9. http://doi.org/10.4236/ crcm.2014.34054.
7. Moscovici S, Candanedo C, Spektor S, Cohen JE, Kaye AH. Solid vs. cystic predominance in posterior fossa hemangioblastomas: implications for cerebrovascular risks and patient outcome. Acta Neurochir (Wien). 2022;164(5):1357-64. http://doi.org/10.1007/s00701-021-04828-w. PMid:33811520.
8. Knobloch K, Yoon U, Vogt PM. Preferred reporting items for systematic reviews and meta-analyses (PRISMA) statement and publication bias. J Craniomaxillofac Surg. 2011;39(2):91-2. http://doi. org/10.1016/j.jcms.2010.11.001. PMid:21145753.
9. Aslam S, Emmanuel P. Formulating a researchable question: A critical step for facilitating good clinical research. Indian J Sex Transm Dis AIDS. 2010;31(1):47-50. http://doi.org/10.4103/0253-7184.69003. PMid:21808439.
10. Moola S, Munn Z, Tufanaru C, et al. Systematic reviews of etiology and risk. In Aromataris E, Munn Z, editors. JBI Manual for Evidence Synthesis. Adelaide, Australia: JBI; 2017. vol. 5, pp. 217-69.
11. Young S, Richardson AE. Solid haemangioblastomas of the posterior fossa: radiological features and results of surgery. J Neurol Neurosurg Psychiatry. 1987;50(2):155-8. http://doi.org/10.1136/jnnp.50.2.155. PMid:3494815.
12. 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://doi.org/10.1148/ radiographics.21.2.g01mr13419. PMid:11259705.
13. Kamitani H, Hirano N, Takigawa H, et al. Attenuation of vascularity by preoperative radiosurgery facilitates total removal of a hypervascular hemangioblastoma at the cerebello-pontine angle: case report. Surg Neurol. 2004;62(3):238-43, discussion 43-4. http://doi.org/10.1016/j. surneu.2003.09.030. PMid:15336869.
14. Amano T, Tokunaga S, Shono T, et al. Cerebellar hemangioblastoma manifesting as hearing disturbance. Neurol Med Chir (Tokyo). 2009;49(9):418-20. http://doi.org/10.2176/nmc.49.418. PMid:19779288.
15. Rachinger J, Buslei R, Prell J, Strauss C. Solid haemangioblastomas of the CNS: a review of 17 consecutive cases. Neurosurg Rev. 2009;32(1):37- 48. http://doi.org/10.1007/s10143-008-0166-0.
16. Ichikawa T, Hamazaki S, Sakai N, et al. Mixed germ cell tumor and hemangioblastoma in the cerebellum: report of a rare coexistence. Brain Tumor Pathol. 2011;28(3):279-84. http://doi.org/10.1007/s10014-011- 0031-7. PMid:21442239.
17. Qiao PF, Niu GM, Han XD. Hemangioblastoma originating from the right cerebellopontine angle. Neurosciences (Riyadh). 2011;16(4):372- 4. PMid:21983384.
18. Friedmann DR, Grobelny B, Golfinos JG, Roland JT Jr. Nonschwannoma tumors of the cerebellopontine angle. Otolaryngol Clin North Am. 2015;48(3):461-75. http://doi.org/10.1016/j.otc.2015.02.006. PMid:26043142.
19. Grahovac G. Solid hemangioblastoma of vestibular nerve mimicking vestibular schwannoma. Neurol Sci. 2015;36(8):1537-9. http://doi. org/10.1007/s10072-015-2216-z. PMid:25899423.
20. Kord Valeshabad A, Xiao L, Amin-Hanjani S, Alsadi A, Valyi- Nagy T, Kim J. Sporadic hemangioblastoma of the cavernous sinus and meckel’s cave. J Neurol Surg Rep. 2018;79(4):e98-102. http://doi. org/10.1055/s-0038-1676455. PMid:30574445.
21. Staudt MD, Hebb MO. Staged multi-modality treatment approaches for giant cerebellopontine angle hemangioblastomas. J Clin Neurosci. 2018;53:224-8. http://doi.org/10.1016/j.jocn.2018.04.027. PMid:29685408.
22. Shinya T, Nagamine H, Sugawara KI, Ishiuchi S. The usefulness of indocyanine green during surgery for hypervascular posterior fossa tumors. Surg Neurol Int. 2018;9(1):90. http://doi.org/10.4103/sni. sni_19_18. PMid:29770250.
23. Qian H, Shi X, Wang L. Distal outflow-related anterior inferior cerebellar artery aneurysm in hemangioblastoma. World Neurosurg. 2019;127:284-8. http://doi.org/10.1016/j.wneu.2019.04.023. PMid:30986586.
24. Mooney MA, Cavallo C, Belykh E, et al. Posterior petrosal transotic approach for cerebellopontine angle hemangioblastoma: technical case report. Oper Neurosurg (Hagerstown). 2019;17(6):E269-73. http://doi. org/10.1093/ons/opz058. PMid:31329951.
25. Burnette MS, Mann TS, Berman DJ, Nguyen TT. Brain tumor, pheochromocytoma, and pregnancy: a case report of a cesarean delivery in a patient with von hippel-lindau disease. A A Pract. 2019;13(8):289- 91. http://doi.org/10.1213/XAA.0000000000001049. PMid:31206381.
26. Dou YF, Liang ZH. Cerebellopontine angle neoplasms in four cases: intra-axial or extra-axial? Chin Med J (Engl). 2019;132(15):1870-1. http://doi.org/10.1097/CM9.0000000000000338. PMid:31283652.
27. Lan Z, Richard SA, Zhang Y. Cystic-solid hemangioblastoma at the cerebellopontine angle: a case report. Medicine (Baltimore). 2020;99(3):e18871. http://doi.org/10.1097/MD.0000000000018871. PMid:32011511.
28. Ferreira BP No, Martins Barreto Santana J, Dornellys da Silva Lapa J, Cristina de Souza Melo T, Maynart Pereira Oliveira A. Noncystic cerebellopontine angle hemangioblastoma: A case of an atypical location. Int J Surg Case Rep. 2020;74:234-7. http://doi.org/10.1016/j. ijscr.2020.08.028. PMid:32892127.
29. Kishi S, Maeda M, Kogue R, Umino M, Matsubara T, Sakuma H. Hemangioblastoma of the Cerebellopontine Angle Evaluated with Pseudocontinuous Arterial Spin Labeling. Magn Reson Med Sci. 2021;20(1):18-9. http://doi.org/10.2463/mrms.ici.2020-0011. PMid:32132312.
30. Ahmed G, Sheikh U, Masri S, Joseph J, Sonwalker H. Predominantly Solid Hemangioblastoma Presenting as an Extra-Axial Cerebellopontine Angle Lesion. Cureus. 2021;13(2):e13071. http://doi.org/10.7759/ cureus.13071. PMid:33680613.
31. Singh PR, Sharma RK, Chaturvedi J, Nayak N, Sharma AK. Surgical outcome of large solid posterior fossa hemangioblastoma without preoperative embolization. J Neurol Surg A Cent Eur Neurosurg. 2022;83(3):224-30. http://doi.org/10.1055/s-0041-1728766. PMid:34433222.
32. Boutakioute B, Zouine Y, Chehboun A, Ouali M, Ganouni NCIE. Successful preoperative embolization of a cystic-solid variant of cerebellopontine angle hemangioblastoma. Radiol Case Rep. 2022;17(12):4799-803. http://doi.org/10.1016/j.radcr.2022.09.045. PMid:36212759.
33. Wanebo JE, Lonser RR, Glenn GM, Oldfield EH. The natural history of hemangioblastomas of the central nervous system in patients with von Hippel-Lindau disease. J Neurosurg. 2003;98(1):82-94. http://doi. org/10.3171/jns.2003.98.1.0082. PMid:12546356.
34. Bush ML, Pritchett C, Packer M, Ray-Chaudhury A, Jacob A. Hemangioblastoma of the cerebellopontine angle. Arch Otolaryngol Head Neck Surg. 2010;136(7):734-8. http://doi.org/10.1001/archoto.2010.98. PMid:20644074.
35. Dow GR, Sim DW, O’Sullivan MG. Excision of large solid haemangioblastomas of the cerebellopontine angle by a skull base approach. Br J Neurosurg. 2002;16(2):168-71. http://doi. org/10.1080/02688690220131804. PMid:12046738.
36. Maślińska D, Woźniak R, Kaliszek A, Schmidt-Sidor B, Lipska A, Woolley DE. Phenotype of mast cells in the brain tumor. Capillary hemangioblastoma. Folia Neuropathol. 1999;37(3):138-42. PMid:10581846.
37. Ho VB, Smirniotopoulos JG, Murphy FM, Rushing EJ. Radiologic-pathologic correlation: hemangioblastoma. AJNR Am J Neuroradiol. 1992;13(5):1343-52. PMid:1414827.
38. Chang KC, Hsieh CT, Huang JS. Supratentorial hemangioblastoma: A rare case report and literature review. Radiol Case Rep. 2022;17(11):4156- 60. http://doi.org/10.1016/j.radcr.2022.07.014. PMid:36105844.
39. Sakamoto N, Ishikawa E, Nakai Y, et al. Preoperative endovascular embolization for hemangioblastoma in the posterior fossa. Neurol Med Chir (Tokyo). 2012;52(12):878-84. http://doi.org/10.2176/nmc.52.878. PMid:23269042.

1Faculdade de Medicina Positivo, Curitiba (PR), Brazil

2Faculdade de Medicina Pequeno Príncipe, Curitiba (PR), Brazil.

3Clinica Sabedotti, Ponta Grossa (PR), Brazil.

4Department of Stomatology, Faculdade de Odontologia, Universidade de São Paulo, São Paulo (SP), Brazil.

5Faculdade de Medicina Pequeno Príncipe, Curitiba, (PR), Brazil.

6Department of Stomatology, Public Health and Forensic Dentistry, Faculdade de Odontologia, Universidade de São Paulo, Ribeirão Preto (SP), Brazil.

 

Received June 10, 2024

Accepted Dec 2, 2024

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