Vestibular Schwannoma pseudoprogression

Stereotactic gamma knife radiosurgery (GKS) may induce a transient enlargement of vestibular schwannomas (VS). This phenomenon, known as pseudoprogression or swelling, starts at about 3 months following GKS, peaks at about 6 months, and typically subsides thereafter, usually without significant neurological deterioration.

Although tumor growth with increasing symptoms or neurological deficit may herald treatment failure, the concept of pseudoprogression, or a transient increase in size followed by stability or regression, has been increasingly recognized following radiosurgical treatment of VS and should not be reported as treatment failure. However, although becoming recognized as a true phenomenon, we have a limited understanding of the natural history and predictors of pseudoprogression. In a landmark article in 2006, Pollock described 3 patterns of tumor enlargement after radiosurgery based on linear measurements of VS diameter 1).

Other case series have also supported the concept of pseudoprogression 2), 3) 4) 5) 6) 7) 8) using a variety of methods of tumor measurement, including volumetric analysis.


Following GKS, 43 patients (18.30%) showed Vestibular Schwannoma pseudoprogression, 15 (6.38%) exhibited hydrocephalus, 22 (9.36%) showed trigeminal neuropathy, 14 (5.96%) showed vertigo, and 25 (10.64%) showed facial myokymia. According to multivariate analysis, solid tumor nature was significantly associated with pseudoprogression and patient age was significantly associated with hydrocephalus. Patients receiving margin doses ≥13 Gy had a significantly higher probability of loss of serviceable hearing. Patients with smaller tumors had a trigeminal nerve preservation rate comparable with patients harboring larger tumors. Patients receiving margin doses <13 Gy or older patients had a significantly higher probability of vestibular nerve dysfunction 9).

Case reports

A 34-year-old female developed an aggressive enlargement of a VS 1 month after GKS. The patient was treated with an immediate external ventricular drainage and surgical resection via retrosigmoid approach for an acute neurological deterioration due to hydrocephalus and brainstem compression. Histopathological examination revealed a VS with abundant intratumoral thrombosis and necrosis, suggesting that its rapid expansion could be related to massive radiation-induced tumor necrosis. The present case indicated that rapid life-threating enlargement of a VS may occur as an early complication following GKS 10).

1)
Pollock BE. Management of vestibular schwannomas that enlarge after stereotactic radiosurgery: treatment recommendations based on a 15 year experience. Neurosurgery. 2006 Feb;58(2):241-8; discussion 241-8. doi: 10.1227/01.NEU.0000194833.66593.8B. PMID: 16462477.
2)
Prasad D, Steiner M, Steiner L. Gamma surgery for vestibular schwannoma. J Neurosurg. 2000 May;92(5):745-59. doi: 10.3171/jns.2000.92.5.0745. PMID: 10794287.
3)
Noren G. The Gamma Knife and acoustic tumors. Med Health R I. 1996;79(6):232–235.
4)
Yu CP, Cheung JY, Leung S, Ho R. Sequential volume mapping for confirmation of negative growth in vestibular schwannomas treated by gamma knife radiosurgery. J Neurosurg. 2000;93(Suppl 3):82–89.
5)
Okunaga T, Matsuo T, Hayashi N, et al. Linear accelerator radiosurgery for vestibular schwannoma: measuring tumor volume changes on serial three-dimensional spoiled gradient-echo magnetic resonance images. J Neurosurg. 2005;103(1):53–58. doi:10.3171/jns.2005.103.1.0053.
6)
Nagano O, Higuchi Y, Serizawa T, et al. Transient expansion of vestibular schwannoma following stereotactic radiosurgery. J Neurosurg. 2008;109(5):811–816. doi:10.3171/JNS/2008/109/11/0811.
7)
Hasegawa T, Kida Y, Yoshimoto M, Koike J, Goto K. Evaluation of tumor expansion after stereotactic radiosurgery in patients harboring vestibular schwannomas. Neurosurgery. 2006;58(6):1119–1128. discussion 1119–1128 doi:10.1227/01.NEU.0000215947.35646.DD.
8)
Nakamura H, Jokura H, Takahashi K, Boku N, Akabane A, Yoshimoto T. Serial follow-up MR imaging after gamma knife radiosurgery for vestibular schwannoma. AJNR Am J Neuroradiol. 2000;21(8):1540–1546.
9)
Kim JH, Jung HH, Chang JH, Chang JW, Park YG, Chang WS. Predictive Factors of Unfavorable Events After Gamma Knife Radiosurgery for Vestibular Schwannoma. World Neurosurg. 2017 Nov;107:175-184. doi: 10.1016/j.wneu.2017.07.139. Epub 2017 Aug 5. PMID: 28826715.
10)
Nasi D, Zunarelli E, Puzzolante A, Moriconi E, Pavesi G. Early life-threating enlargement of a vestibular schwannoma after gamma knife radiosurgery. Acta Neurochir (Wien). 2020 Aug;162(8):1977-1982. doi: 10.1007/s00701-020-04434-2. Epub 2020 Jun 6. PMID: 32504117.