Sporadic cerebellar hemangioblastomas and the role of cyst size in surgical outcomes – a single-surgeon experience from a single center
Authors:
O. Altunyuva 1; N. Balcin 1; A. I. Ozmarasali 1; O. Balcin 2; S. Yilmazlar 1
Authors‘ workplace:
Department of Neurosurgery, Bursa Uludag University School of Medicine, Bursa, Turkey
1; Department of Biostatistics, Bursa Uludag University School of Medicine, Bursa, Turkey
2
Published in:
Cesk Slov Neurol N 2025; 88(6): 357-364
Category:
Original Paper
doi:
https://doi.org/10.48095/cccsnn2025357
Overview
Aim: Hemangioblastomas are benign, highly vascular tumors that originate within the central nervous system. Histologically, these tumors are composed of stromal cells embedded in a dense network of blood vessels. The aim of the study was to present our surgical outcomes in sporadic cerebellar hemangioblastomas and to investigate the relationship between the cystic component size and the presence of hydrocephalus and associated clinical findings. Materials and methods: We retrospectively reviewed 29 adult patients with sporadic cerebellar hemangioblastomas operated by a single surgeon between 2010 and 2022. Tumors were classified as solid, small cystic (< 2.5 cm), or large cystic (≥ 2.5 cm). Clinical, imaging, and surgical data were analyzed, and functional outcomes were measured using the modified Rankin Scale (mRS) and Karnofsky Performance Scale (KPS) scores. The relationship between cyst size, hydrocephalus, and neurological outcome was also examined. Results: Larger cysts were significantly linked to hydrocephalus (P = 0.038) and cerebellar symptoms (P = 0.026). Regression suggested a possible association between cyst size and these findings (P = 0.062 and P = 0.053). Cyst and nodule sizes showed moderate correlation (r = 0.520; P = 0.013). Cysts were also larger in patients needing an external ventricular drain (41,00 vs. 28,78 mm; P = 0.053). No significant differences were found between groups in mRS or KPS scores before and 1 year after surgery (P > 0.6). Conclusion: Hemangioblastomas are benign, but their posterior fossa location often causes significant symptoms. En bloc resection of the nodule with wide craniotomy remains the best treatment. This study highlights the link between large cysts and hydrocephalus, suggesting early surgery may improve outcomes.
Keywords:
cyst – hydrocephalus – hemangioblastoma – posterior fossa
Sources
1. Qu L, Lv C, Ji T et al. Cerebral hemangioblastoma without von hippel-lindau syndrome: a report of 6 cases. Int J Surg Pathol 2021; 29 (2): 129–134. doi: 10.1177/106689 6920933998.
2. Sánchez-Ortega JF, Claramonte M, Martín M et al. Sporadic supratentorial hemangioblastoma with meningeal affection: a case report and literature review. Surg Neurol Int 2021; 12 : 394. doi: 10.25259/SNI_ 441_2021.
3. Lahkim M, Andour H, Laamrani FZ et al. Cerebellar hemangioblastoma: case report with review of the literature. Radiol Case Rep 2021; 16 (10): 3109–3112. doi: 10.1016/j.radcr.2021.07.027.
4. Abboud FZ, Youssoufi MA, Bouhafa T et al. A solitary hemangioblastoma of the posterior brain fossa: the role of radiotherapy. Pan Afr Med J 2020; 36 : 114. doi: 10.11604/pamj.2020.36.114.22282.
5. Splavski B, Zbytek B, Arnautovic KI. Surgical management and outcome of adult posterior cranial fossa and spinal hemangioblastoma: a 6-case series and literature review. Neurol Res 2020; 42 (12): 1010–1017. doi: 10.1080/01616412.2020.1796382.
6. Lucke-Wold B, Woolridge M, Cao DT et al. Emerging therapies of hemangioblastomas. Explor Neurosci 2023; 2 : 318–330. doi: 10.37349/en.2023.00031.
7. Watanabe T, Suematsu Y, Saito K et al. Selection of surgical approach for cerebellar hemangioblastomas based on venous drainage patterns. Neurosurg Rev 2021; 44 (6): 3567–3579. doi: 10.1007/s10143-021-01544-y.
8. Huntoon K, Shepard MJ, Lukas RV et al. Hemangioblastoma diagnosis and surveillance in von Hippel-Lindau disease: a consensus statement. J Neurosurg 2021; 36 (6): 1511–1516. doi: 10.3171/2021.3.JNS204203.
9. Přibáň V, Fiedler J, Rehousek P et al. Combined microsurgical and endovascular therapy of intramedullary hemangioblastoma: a case report. Cesk Slov Neurol N 2007; 70/103 (5): 580–583.
10. Vergauwen E, Steiert C, Krüger MT et al. Cumulative surgical morbidity in patients with multiple cerebellar and medullary hemangioblastomas. Clin Neurol Neurosurg 2020; 197 : 106111. doi: 10.1016/j.clineuro.2020.106111.
11. Kim EH, Moon JH, Kang SG et al. Diagnostic challenges of posterior fossa hemangioblastomas: refining current radiological classification scheme. Sci Rep 2020; 10 (1): 6267. doi: 10.1038/s41598-020-63207-0.
12. Wang Q, Cheng J, Zhang S et al. Central nervous system hemangioblastomas in the elderly (over 65 years): clinical characteristics and outcome analysis. Clin Neurol Neurosurg 2020; 189 : 105622. doi: 10.1016/ j.clineuro.2019.105622.
13. Ahadi M, Zham H, Rakhshan A et al. Hemangioblastoma of the central nervous system: a case series of patients surgically treated at Shohada-E-Tajrish Hospital, Tehran, Iran during 2004–2014. Iran J Child Neurol 2019; 13 (2): 163–169.
14. Kuharic M, Jankovic D, Splavski B et al. Hemangioblastomas of the posterior cranial fossa in adults: demographics, clinical, morphologic, pathologic, surgical features, and outcomes. a systematic review. World Neurosurg 2018; 110: e1049–e1062. doi: 10.1016/ j.wneu.2017.11.173.
15. Lee SR, Sanches J, Mark AS et al. Posterior fossa hemangioblastomas: MR imaging. Radiology 1989; 171 (2): 463–468. doi: 10.1148/radiology.171.2.2704812.
16. Başar I, Aydın Öztürk P, Tuncer MC et al. Surgical management of sporadic hemangioblastomas located in the posterior fossa of brain. Arch Ital Biol 2021; 159 (2): 51–63. doi: 10.12871/00039829202121.
17. Richard S, Graff J, Lindau J et al. Von Hippel-Lindau disease. Lancet 2004; 363 (9416): 1231–1234. doi: 10.1016/S0140-6736 (04) 15957-6.
18. Signorelli F, Piscopo G, Giraud S et al. Von Hippel-Lindau disease: when neurosurgery meets nephrology, ophthalmology and genetics. J Neurosurg Sci 2019; 63 (5): 548–565. doi: 10.23736/S0390-5616.17.04153-4.
19. Kalita O, Vaverka B, Hrabalek L et al. A strategy for diagnosis, therapy and follow-up of patients with CNS hemangioblastoma from the perspective of a neurosurgeon. Cesk Slov Neurol N 2013; 76/109 (5): 623–629.
20. Gläsker S, Vortmeyer AO, Lonser RR et al. Proteomic analysis of hemangioblastoma cyst fluid. Cancer Biol Ther 2006; 5 (5): 549–553. doi: 10.4161/cbt.5.5.2657.
21. Jeon C, Choi JW, Kong DS et al. Treatment strategy for giant solid hemangioblastomas in the posterior fossa: a retrospective review of 13 consecutive cases. World Neurosurg 2022; 158: e214–e224. doi: 10.1016/ j.wneu.2021.10.169.
22. Xia H, Li J, Xia Y et al. Sporadic solid/cystic hemangioblastomas in the cerebellum: retrospective study of more than ten years of experience in a single center. World Neurosurg 2020; 144: e908–e915. doi: 10.1016/ j.wneu.2020.09.104.
23. Mooney MA, Cavallo C, Belykh E et al. Posterior petrosal transotic approach for cerebellopontine angle hemangioblastoma: technical case report. Oper Neurosurg 2019; 17 (6): E269–E273. doi: 10.1093/ons/opz058.
24. Pan J, Jabarkheel R, Huang Y et al. Stereotactic radiosurgery for central nervous system hemangioblastoma: systematic review and meta-analysis. J Neurooncol 2018; 137 (1): 11–22. doi: 10.1007/s11060-017-2697-0.
25. Valchář R, Liščák R, Šimonová et al. Hemangioblastoma and its treatment using Leksell gamma knife. Cesk Slov Neurol N 2008; 71/104 (2): 216–222..
26. Štoková M, Musilova B, Grubhoffer B et al. Progression of hemangioblastomas in pregnancy in von Hippel-Lindau syndrome. Cesk Slov Neurol N 2023; 86/119 (5): 333–335. doi: 10.48095/cccsnn2023333.
27. Jin N, Sun C, Hua Y et al. Anlotinib for the treatment of multiple recurrent lumbar and sacral cord hemangioblastomas: a case report. Front Oncol 2022; 12 (27): 859157. doi: 10.3389/fonc.2022.859157.
28. Sizdahkhani S, Feldman MJ, Piazza MG et al. Somatostatin receptor expression on von Hippel-Lindau-associated hemangioblastomas offers novel therapeutic target. Sci Rep 2017; 7 : 40822. doi: 10.1038/srep 40822.
29. Sokol Z, Hoeft A, Kung D et al. Intra-arterial bevacizumab for posterior fossa hemangioblastoma. Cureus 2022; 14 (12): e32624. doi: 10.7759/cureus.32624.
30. Courtney KD, Infante JR, Lam ET et al. Phase i dose-escalation trial of PT2385, a first-in-class hypoxia-inducible factor-2a antagonist in patients with previously treated advanced clear cell renal cell carcinoma. J Clin Oncol 2018; 36 (9): 867–874. doi: 10.1200/JCO.2017. 74.2627.
31. Curry L, Soleimani M. Belzutifan: a novel therapeutic for the management of von Hippel-Lindau disease and beyond. Future Oncol 2024; 20 : 1251–1266. doi: 10.2217/fon–2023–0679.
32. Dhawan A, Peereboom DM, Stevens GH. First clinical experience with belzutifan in von Hippel-Lindau disease associated CNS hemangioblastoma. CNS Oncol 2022; 11 (3): CNS91. doi: 10.2217/cns-2022-0008.
33. Odia Y, Gutierrez AN, Kotecha R. Surgically targeted radiation therapy (start) trials for brain neoplasms: a comprehensive review. Neuro Oncol 2022; 24 (Suppl 6): S16–S24. doi: 10.1093/neuonc/noac130.
34. Niu L, Zhang Y, Li Q et al. The analysis of correlative factors affecting long-term outcomes in patients with solid cerebellar hemangioblastomas. Clin Neurol Neurosurg 2016; 150 : 59–66. doi: 10.1016/j.clineuro.2016. 08.028.
35. Cervio A, Villalonga JF, Mormandi R et al. Surgical treatment of cerebellar hemangioblastomas. Surg Neurol Int 2017; 8 : 163. doi: 10.4103/sni.sni_490_16.
36. Jagannathan J, Lonser RR, Smith R et al. Surgical management of cerebellar hemangioblastomas in patients with von Hippel-Lindau disease. J Neurosurg 2008; 108 (2): 210–222. doi: 10.3171/JNS/2008/108/2/0210.
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