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Cystic acoustic neuromas are less frequent than solid ones and present different clinical and radiological features. Cystic schwannomas are larger, show a shorter clinical history and a different risk of postoperative complications. This study was designed to compare surgical results and complications of solid and cystic vestibular schwannomas of m...
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... first symptom presented by the 80 grade III-IV pa- tients are reported in Table 1, comparing cystic and solid tumor groups. In both groups the first symptom prompting patients to medical referral was a slowly progressing hear- Fig. 1 Axial section of a large cystic VS at MRI Fig. 2 The same tumour in a coronal section ing impairment, while vertigo and headache were less fre- quent. ...
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... the opposite, duration of symptoms (Table 1) was significantly shorter in cystic VS (P<0.001) as compared to solid tumours. Among cystic VS, five patients (19%) developed a very rapid worsening due to expansion of the cystic component of the tumour that took them to medical observation within a few months from the beginning of symptoms. ...
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... Even though the exact pathophysiology of these evolved cysts are not definitely known, its direct consequence into patient management has already been described and discussed in the past: Cystic VS can demonstrate a more rapid growth and expansion [7], enhanced peritumoral adhesion [8][9][10], worse post-operative facial nerve outcome [11]. Concordant to solid VS, both radiosurgery (SRS) and microsurgical resection (SURGERY) are possible treatment modalities. ...
... The incidence of cystic morphology in the VS is reported with considerable variability: from 11.3 to 48% [9,22,23]. This cohort shows an overall incidence of 6% of the treated primary, solitary VS similar to Fundova et al. in 2000, when 773 VS patients were retrospectively reviewed [24]. ...
... However, cystic morphology is associated with a lower rate of EOR (GTR 87% vs. 96%), when treated with SURGERY in this study cohort. A comparative study in 2005 achieved a high GTR-rate in cystic tumors of 92% (solid tumors 93%) by retrosigmoid approach, but they also report that 42% of patients with cystic VS showed unfavorable facial nerve function one year post-operatively [9]. Notably, their classification for good facial outcome included HB grade 3, which was classified as poor facial outcome in our study. ...
Some vestibular schwannoma (VS) show cystic morphology. It is known that these cystic VS bear different risk profiles compared to solid VS in surgical treatment. Still, there has not been a direct comparative study comparing both SRS and SURGERY effectiveness in cystic VS. This retrospective bi-center cohort study aims to analyze the management of cystic VS compared to solid VS in a dual center study with both microsurgery (SURGERY) and stereotactic radiosurgery (SRS). Cystic morphology was defined as presence of any T2-hyperintense and Gadolinium-contrast-negative cyst of any size in the pre-interventional MRI. A matched subgroup analysis was carried out by determining a subgroup of matched SURGERY-treated solid VS and SRS-treated solid VS. Functional status, and post-interventional tumor volume size was then compared. From 2005 to 2011, N = 901 patients with primary and solitary VS were treated in both study sites. Of these, 6% showed cystic morphology. The incidence of cystic VS increased with tumor size: 1.75% in Koos I, 4.07% in Koos II, 4.84% in Koos III, and the highest incidence with 15.43% in Koos IV. Shunt-Dependency was significantly more often in cystic VS compared to solid VS (p = 0.024) and patients with cystic VS presented with significantly worse Charlson Comorbidity Index (CCI) compared to solid VS (p < 0.001). The rate of GTR was 87% in cystic VS and therefore significantly lower, compared to 96% in solid VS (p = 0.037). The incidence of dynamic volume change (decrease and increase) after SRS was significantly more common in cystic VS compared to the matched solid VS (p = 0.042). The incidence of tumor progression with SRS in cystic VS was 25%. When comparing EOR in the SURGERY-treated cystic to solid VS, the rate for tumor recurrence was significantly lower in GTR with 4% compared to STR with 50% (p = 0.042). Tumor control in cystic VS is superior in SURGERY, when treated with a high extent of resection grade, compared to SRS. Therapeutic response of SRS was worse in cystic compared to solid VS. However, when cystic VS was treated surgically, the rate of GTR is lower compared to the overall, and solid VS cohort. The significantly higher number of patients with relevant post-operative facial palsy in cystic VS is accredited to the increased tumor size not its sole cystic morphology. Cystic VS should be surgically treated in specialized centers.
... история [4]. В нашия случай от 2 месеца пациентът имаше дясна пареза на лицевия нерв, шум в дясното ухо, промяна на вкуса и влошаване на неврологичния статус. ...
... В нашия случай от 2 месеца пациентът имаше дясна пареза на лицевия нерв, шум в дясното ухо, промяна на вкуса и влошаване на неврологичния статус. Ако вътрешният слухов канал не е унищожен и няма значителна загуба на слуха при наличието на голяма кистична понтоцеребеларна туморна формация, тогава може да се подозира кистичен акустичен шваном [3][4]. Moon КС. et al. откриват, че течността на кистата има по-висока концентрация на матриксна металопротеиназа-2 (ММР 2) [3]. ...
... Според скорошни проучвания, въпреки че хирургичната резекция на кистичен шваном изглежда лесна, съществува висок риск от непреднамерено увреждане на лицевия нерв [3][4][5][6]. Според Samii et al., процентът на анатомично запазване на лицевия нерв при кистичен шваном спада от 93% при солидни тумори до 88% от тези случаи [7]. Nair S. et al. откриват в своето проучване, че хирургичните резултати при пациенти с кистичен шваном са по-лоши, с по-висока честота на субтотална ексцизия на тумор [5]. ...
Доброкачествените тумори, наречени вестибуларни шваноми, произхождат от осмия черепномозъчен нерв и имат променлива естествена история. Клинично и рентгенологично солидният вариант се отличава от формите на кистични вестибуларни шваноми, рядка доброкачествена подгрупа. Представяме клиничен случай на 67-годишен пациент, постъпил в клиниката по неврохирургия на УМБАЛ „Св. Марина” с клинична изява на пареза на десния лицев нерв, атаксия, промяна на вкуса и шум в дясното ухо от няколко месеца. ЯМР откри кистичен шваном в десния понтоцеребеларен ъгъл (Samii - 4a, Koos - IV). Беше извършена дясна субокципитална краниектомия с използване на ретросигмоиден достъп под обща анестезия. Интраоперативно беше открит жълтеникав кистичен тумор, заедно с ясно очертание от околния паренхим. Чрез микрохирургична техника, невромониторинг и невроендоскопска техника, субтотална резекция на кистите бе осъществена. Постоперативни усложнения не са наблюдавани. Следоперативно се оцени дисфункцията на лицевия нерв по скалата на Хаус-Бракман – 2. След консултациите на третия месец се подобри функцията на лицевия нерв (скала на Хаус-Бракман – 1).
... Additionally, nearly half the complications have occurred in earlier studies, whereas subsequent studies reported that complications occurred in only 7-10% of patients [44]. Obstructive hydrocephalus necessitating cerebrospinal fluid diversion is also less common in cVS, probably because cysts conform to the contour of the surrounding neural structures rather than compressing them [45]. Regarding volumetric response, it has been shown that cVS patients demonstrate approximately 2 times more shrinkage (80.2% vs. 45.9%) ...
Anecdotally, cystic vestibular schwannomas (cVSs) are regarded to have unpredictable biologic activity with poorer clinical results, and most studies showed a less favorable prognosis following surgery. While stereotactic radiosurgery (SRS) is a well-established therapeutic option for small- to medium-sized VSs, cVSs are often larger, thus making upfront SRS more complicated. The purpose of this retrospective study was to assess the efficacy and safety of upfront SRS for large cVSs. The authors reviewed the data of 54 patients who received upfront, single-session Gamma Knife radiosurgery (GKRS) with a diagnosis of large cVS (> 4 cm3). Patients with neurofibromatosis type 2, multiple VSs, or recurrent VSs and < 24 months of clinical and neuroimaging follow-up were excluded. Hearing loss (48.1%) was the primary presenting symptom. The majority of cVSs were Koos grade IV (66.7%), and the most prevalent cyst pattern was “mixed pattern of small and big cysts” (46.3%). The median time between diagnosis and GKRS was 12 months (range, 1–147 months). At GKRS, the median cVS volume was 6.95 cm³ (range, 4.1–22 cm³). The median marginal dose was 12 Gy (range, 10–12 Gy). The mean radiological and clinical follow-up periods were 62.2 ± 34.04 months (range, 24–169 months) and 94.9 ± 45.41 months (range, 24–175 months), respectively. At 2, 6, and 12 years, the tumor control rates were 100%, 95.7%, and 85.0%, respectively. Tumor shrinkage occurred in 92.6% of patients (n = 50), tumor volume remained stable in 5.6% of patients (n = 3), and tumor growth occurred in 1.9% of patients (n = 1). At a median follow-up of 53.5 months, the pre-GKRS tumor volume significantly decreased to 2.35 cm³ (p < 0.001). While Koos grade 3 patients had a greater possibility of attaining higher volume reduction, “multiple small thick-walled cyst pattern” and smaller tumor volumes decreased the likelihood of achieving higher volume reduction. Serviceable hearing (Gardner-Robertson Scale I–II) was present in 16.7% of patients prior to GKRS and it was preserved in all of these patients following GKRS. After GKRS, 1.9% of patients (n = 1) had new-onset trigeminal neuralgia. There was no new-onset facial palsy, hemifacial spasm, or hydrocephalus. Contrary to what was believed, our findings suggest that upfront GKRS seems to be a safe and effective treatment option for large cVSs.
... Cystic VS (described variously as more than 50% or two-thirds of the tumour showing a cyst component) have the potential for rapid growth and are considered to be an indication for MS (109)(110)(111). However, the functional outcomes for surgery in these tumors is also worse and this is attributed to the poor plane between the cyst walls and the surrounding neurovascular structures (112). ...
... Compared to SVSs, most studies reported that CVS tumour characteristics include rapid growth, large size, short clinical history of symptoms, and sudden deterioration due to unpredictable expansion of the cystic component or haemorrhage. Concerning surgical treatment, CVSs surgery is usually challenging and also has a less favourable prognosis than SVSs, especially in facial nerve function [1,24,32,39,[44][45][46]. However, some studies presented that it is unclear whether surgical outcomes for CVSs and SVSs exhibit significant differences [7,16,17,38]. ...
... Twenty-one full texts of these records were reviewed. Finally, 15 retrospective cohort studies [1,7,9,12,23,24,27,31,37,38,41,[44][45][46] and 3 prospective cohort studies [5,16,17] involving 3074 participants (including 821 patients with CVSs and 2253 patients with SVSs) were included in this meta-analysis. The search flow diagram is presented in Fig. 1. ...
... The extent of tumour resection was simply divided into two categories: gross-total resection (GTR) and non-gross-total resection (NGTR). Sixteen studies [1,5,7,9,12,16,23,24,27,31,37,38,41,[44][45][46] reported outcomes of the extent of tumour resection. In a single-arm synthesis, the pooled rates of GTR were 78.6% (95% CI, 71.2-86.0%) ...
Most studies reported that cystic vestibular schwannoma (CVS) surgery has a less favourable prognosis than solid vestibular schwannoma (SVS) surgery. However, some studies report that it is unclear whether surgical outcomes for these conditions exhibit significant differences. The aim of this meta-analysis was to pool the current literature and describe and analyse any differences in the clinical symptoms and surgical outcomes among CVS and SVS. PubMed, Embase, and Cochrane databases were searched following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines through April 22, 2020. Outcomes were analysed using a meta-analysis of the proportions. The results of the search yielded 17 studies that met the criteria for inclusion and analysis, involving 3074 participants (including 821 patients with CVSs and 2253 patients with SVSs). No significant differences in the extent of tumour resection (gross-total resection versus non-gross-total resection; RR, 0.93; 95% CI, 0.86-1.01; p = 0.096) and most other outcomes were noted between CVS and SVS cohorts. However, facial nerve function (House-Brackmann Grade I or II versus III or more) of CVS patients was worse (RR, 0.86; 95% CI, 0.78-0.93; p < 0.001) compared with SVS patients with more than 1 year of follow-up after surgery. The criteria of cystic acoustic neuroma needs to be further unified, and prospective cohort studies with larger sample sizes should be performed for further verification of these results in the future.
... In general, cystic VS have worse surgical outcomes and higher rates of subtotal resection (STR). 1,2, 6 We reviewed our series of patients with cystic VS to determine those factors linked to facial nerve preservation and tumor control at our center, and to compare our results with a similar cohort of solid VS. ...
... Recently several studies have demonstrated the efficiency of stereotactic radiosurgery (SRS) as primary treatment, even of cystic VS. 3,6,19 Frisch et al 25 observed continuous growth in 10% of irradiated cystic VS during median follow-up of 63 months. Bowden et al 26 reported tumor control in 95% for macrocystic VS at 5 years, with volumetric reduction (>20%) in 85.7%. ...
Objective Cystic vestibular schwannomas (VS) in contrast to solid VS tend to have accelerated growth, larger volume, rapid/atypical presentation, lobulated/adherent surface, and unpredictable course of the cranial nerves. Cystic VS are surgically challenging, with worse clinical outcomes and higher rate of subtotal resection (STR).
Methods We retrospectively analyzed postoperative outcomes of 125 patients with cystic VS, operated between years 2005 and 2019 in our center. We confronted the extent of the resection and House-Brackmann (HB) grade of facial palsy with the results of comparable cohort of patients with solid VS operated in our center and literature review by Thakur et al.1
Results Translabyrinthine approach was preferred for resection of large, cystic VS (97.6%). Gross-total resection (GTR) was achieved in 78 patients (62.4%), near-total resection (NTR) with remnant (<4 × 4 × 2 mm) in 43 patients (34.4%), and STR in 4 patients (3.2%). NTR/STR were significantly associated with higher age, tumor volume >5 cm3, retrosigmoid approach, high-riding jugular bulb, tumor adherence to the brain stem, and facial nerve (p = 0.016; 0.003; 0.005; 0.025; 0.001; and <0.00001, respectively).
One year after the surgery, 76% of patients had HB grades 1 to 2, 16% had HB grades 3 to 4, and 8% had HB grades 5 to 6 palsy. Worse outcome (HB grades 3 to 6) was associated with preoperative facial palsy, tumor volume >25 cm3, and cyst over the brain stem (p = 0.045; 0.014; and 0.05, respectively). Comparable solid VS operated in our center had significantly higher HB grades 1 to 2 rate than our cystic VS (94% versus 76%; p = 0.03). Comparing our results with literature review, our HB grades 1 to 2 rate was significantly higher (76% versus 39%; p = 0.0001). Tumor control rate 5 years after surgery was 95.8%.
Conclusion Our study confirmed that microsurgery of cystic VS has worse outcomes of facial nerve preservation and extent of resection compared with solid VS. Greater attention should be paid to the above-mentioned risk factors.
... In literature, incidence variate mainly between 6%-24% (19,20), we had an incidence of 21%. Earlier literature described more aggressive, shorter symptomatic period (19,21), but no objectivation of hearing loss seems to be investigated. Our results showed no correlation between the presence of cystic components and hearing levels. ...
Objective: The aim of this study was to delineate characteristics of patients diagnosed with unilateral sporadic vestibular schwannoma. Symptomatology and MR imaging were correlated to hearing levels, tumour size, age, and radiologic features. Methods: Retrospective study in a tertiary referral centre for neurotology and skull base surgery on 384 patients with a unilateral sporadic vestibular schwannoma in the cerebellopontine angle. Patients with intralabyrinthine schwannoma, neurofibromatosis type 2, meningiomas, and patients already treated elsewhere, were excluded from analyses. Results: Age ranged from 11 to 92 years (mean age of 52 years). At presentation, 75% complained of ipsilateral subjective hearing loss, 56% of tinnitus, 41% of vertigo, 19% of ear fullness. A cystic component was observed in 21%, brainstem compression in 33%. Tumour size included 31% intracanalicular, 20% small, 28% medium, 13% moderately large, 6% large and 2% giant tumours. A weak positive correlation between tumour size and hearing loss was observed. We also noticed a weak negative correlation between age and tumour size. Conclusion: Our series confirms subjective hearing loss being the main presenting complaint of sporadic vestibular schwannoma at any age. Nevertheless, more than half of our cases presented initially with tinnitus. This emphasizes the importance of imaging for tinnitus and hearing loss in patients. Although all sizes of tumour can present with all types of hearing loss, there is a weak positive correlation between size and hearing loss. Furthermore, we observed a significant negative correlation between age and tumour size. The presence of cystic components is associated with larger tumour size but not with hearing loss.
... Vestibular schwannomas arising from the superior vestibular nerve also have more favorable prognoses in terms of hearing preservation. Cystic tumors, which are composed primarily of large coalescent cysts, are generally faster growing with a shorter clinical history and often treated as more aggressive lesions (26). ...
MRI is firmly established as an essential modality in the imaging of the temporal bone and lateral skull base. It is used to evaluate normal anatomic structures, evaluate for vestibular schwannomas, assess for inflammatory and/or infectious processes, and detect residual and/or recurrent cholesteatoma. It is also extensively used in pre- and postoperative evaluations, particularly in patients with vestibular schwannomas and candidates for cochlear implantation. Nevertheless, despite the widespread use of MRI for these purposes, many radiologists remain unfamiliar with the complex anatomy and expected imaging findings with such examinations. The purpose of this review is to provide an overview of the most useful MRI sequences for internal auditory canal and labyrinthine imaging, review the relevant anatomy, and discuss the expected appearances of the most commonly encountered pathologic entities. In addition, the features at pre- and postprocedural MRI will be discussed to help ensure that diagnostic radiologists may be of greatest use to the ordering physicians. © RSNA, 2020.
... VSs account for approximately 8 to 10% of primary intracranial tumors and 80% of cerebellopontine angle tumors. 1 These tumors may undergo cystic changes, frequently as microcysts, caused by degenerative changes within the tumors. Occasionally, these areas may coalesce to form larger cysts. ...
Cystic vestibular schwannomas (VS) form a rare subgroup that differs from the solid variant clinically and radiologically. Certain unique features reported in cystic VS are larger size, presentation with atypical initial symptoms such as dysgeusia, vertigo, facial pain and gait unsteadiness, short duration of symptoms, rapid progression of symptoms or sudden deterioration and frequent involvement of facial nerve. Gamma Knife radiosurgery (GKRS) is a safe and effective treatment for VS, achieving tumor control and resulting in a very low morbidity rate. Here, we described two cases of cystic vestibular schwannomas prior to GKRS and their radiological appearances post-GKRS.
... In most studies, reported FN outcomes after tumor resection in cystic VS have been less favorable in comparison to results after removal of solid tumors of comparable size. 2, 21,22,23,24,25,26 While our institutional series showed no statistically significant difference in FN results between solid and cystic tumors, we attribute this to our protocol of leaving behind tumor around the FN (near-total resection) if necessary in cystic tumors. 27 ...
A progressive evolution in diagnostic testing and microsurgical techniques has made it possible to reduce the perioperative mortality rate of vestibular schwannoma (VS) microsurgery to less than 1%, especially at high-volume centers. 1,2,3,4 This, in part, has led to a shift in the surgeon's attention to the preservation of cranial nerve function, in particular that of the facial nerve (FN) and the cochlear nerve. 5,6,7,8 Due to the intimate association between the FN and VS capsule, surgery for this tumor is fraught with risk of FN injury. Although the anatomy of the FN is fairly consistent in the temporal bone and the cerebello-pontine angle (CPA), the tumor itself may distort or displace the nerve, which may be neither evident by way of preoperative FN deficits nor discernible on preoperative imaging. In fact, the FN is very resistant to tumor stretch and compression, and more often than not, preoperative FN function is found preserved even in most giant VS. The surgeon must be prepared to deal with difficult situations wherein the nerve is thinned and stretched, is enveloped, or is found positioned between the surgeon and the tumor. Less commonly, there are intraoperative situations that may lead to interruption of FN continuity, either intentionally or otherwise. Once a nerve is interrupted, reconstruction should be performed immediately in order to obtain the best results, either by means of a primary end-to-end coap-tation or by a cable nerve graft interposition. 9 In this chapter, we will discuss various pre-and intraoperative situations with regard to the FN and how to manage them. 32.2 Clinical Onset of Facial Nerve Paralysis As discussed previously, the FN can withstand stretching imposed by tumor growth to a great deal and hence leave the patient without clinically apparent deterioration of function. The FN, as most motor nerves, is quite resistant to tumor infiltration and nerve dysfunction typically occurs very late in the course of tumor growth. Axon and Ramsden 10 found that House-Brack-mann grade I FN function can be maintained with only 10% of motor neurons functioning. Neuronal degeneration and axonal demyelination is counterbalanced with the collateral sprouting and hypertrophy of the innervated muscle fibers. Furthermore, the absence of epineurium around the CPA and internal auditory canal (IAC) segments of the FN allows effacement of the nerve fibers and spreading of the nerve fascicles over large tumors. 10,11 In slow-growing pathologies such as VS and meningi-omas, FN fibers are stretched slowly over many months leading to splaying of fibers over the tumor capsule (▶ Fig. 32.1). 12 Conversely , early-onset FN paralysis in cases of smaller IAC or CPA tumors should signal clinicians to a suspect FN schwannoma, cavernous hemangioma, or, much less commonly, a malignant lesion. The incidence of FN dysfunction associated with untreated VS is generally less than 10% and correlates with tumor size. FN paralysis is often preceded by facial twitch or spasm. Fig. 32.1 (A) The FN is stretched and splayed over the tumor capsule in slow-growing tumors like VS. (B) Intraoperative picture of the FN after tumor removal wherein the nerve is seen to be markedly thinned and transparent.
