Pediatric vestibular schwannomas: case series and a systematic review with meta-analysis

Abstract

<jats:sec><jats:title>OBJECTIVE</jats:title><jats:p>Sporadic unilateral vestibular schwannomas are rare in the pediatric population. Little has been reported in the literature on the presentation, tumor size, response to surgical treatment, and recurrence rates in these younger patients. The authors’ goal was to describe their institutional experience with pediatric sporadic vestibular schwannomas and to conduct a meta-analysis of the existing literature to provide further insight into the presentation, tumor characteristics, and surgical outcomes for these rare tumors to help direct future treatment strategies.</jats:p></jats:sec><jats:sec><jats:title>METHODS</jats:title><jats:p>The authors performed a retrospective review of all patients 21 years of age or younger with unilateral vestibular schwannomas and without neurofibromatosis type 2 who underwent resection by the senior authors between 1997 and 2019. A systematic review of the literature and meta-analysis was also performed by entering the search terms “pediatric” and “vestibular schwannoma” or “acoustic neuroma,” as well as “sporadic” into PubMed. Presentation, treatment, clinical outcomes, and follow-up were analyzed.</jats:p></jats:sec><jats:sec><jats:title>RESULTS</jats:title><jats:p>Fifteen patients were identified at the authors’ institution, ranging in age from 12 to 21 years (mean 16.5 years). Common presenting symptoms included hearing loss (87%), headache (40%), vertigo (33%), ataxia (33%), and tinnitus (33%). At the time of surgery, the mean tumor size was 3.4 cm, with four 1-cm tumors. Four patients had residual tumor following their first surgery, 3 (75%) of whom had significant radiographic regrowth that required further treatment. The literature review identified an additional 81 patients from 26 studies with patient-specific clinical data available for analysis. This resulted in a total of 96 reported patients with an overall average age at diagnosis of 12.1 years (range 6–21 years) and an average tumor size of 4.1 cm.</jats:p></jats:sec><jats:sec><jats:title>CONCLUSIONS</jats:title><jats:p>Pediatric vestibular schwannomas present similarly to those in adults, although symptoms of mass effect are more common, as these tumors tend to be larger at diagnosis. Some children are found to have small tumors and can be successfully treated surgically. Residual tumors in pediatric patients were found to have a higher rate of regrowth than those in their adult counterparts.</jats:p></jats:sec>

Evaluation and management of paediatric vertigo

“…One of the most substantial differences in the management of vertigo over the last decade has been an increased understanding of the most common causes of paediatric dizziness [1 & ,3,7,10]. A nonexhaustive list is presented in Table 1 [59][60][61][62][63][64][65][66][67][68][69][70].…”

Pediatric Skull Base Tumors

“…Vestibular schwannomas present with unilateral hearing loss, tinnitus, headache, and disequilibrium, and they can cause cerebellar ataxia and brainstem compression with significant extension into the cerebellopontine angle. [32] Pediatric vestibular schwannomas tend to be diagnosed in adolescence and the tumors tend to be larger at presentation than in adults. [32,33] Surgical resection, stereotactic radiosurgery, and observation are all valid treatment options for schwannomas, however the long life-span of pediatric patients argues for more aggressive definitive management.…”

“…[32] Pediatric vestibular schwannomas tend to be diagnosed in adolescence and the tumors tend to be larger at presentation than in adults. [32,33] Surgical resection, stereotactic radiosurgery, and observation are all valid treatment options for schwannomas, however the long life-span of pediatric patients argues for more aggressive definitive management. In one series of 148 pediatric vestibular schwannomas 82 (55.4%) patients were treated with surgery only, 45 (30.4%) with observation, 6 (4.1%) with radiation only, and 12 (8.1%) with surgery and radiation.…”

“…[33] Residual tumors after surgery may grow at a faster rate than in adults, therefore the surgeon should safely attempt gross total resection or should consider radiosurgery in cases with significant residual tumor. [32] Sporadic schwannomas are rare in children, but they are the hallmark of genetic conditions, neurofibromatosis 2 (NF2) and schwannomatosis, with bilateral vestibular schwannomas affecting 95% of individuals with NF2. [34] Overall treatment goals change in these patients due to the high lifetime number of tumors.…”

Primary pilocytic astrocytoma of the cerebellopontine angle in pediatric patient with neurofibromatosis type 1: a case report

“…Vestibular schwannoma (VS) and CPA meningioma are commonly found in pediatrics with neurocutaneous disorder such as neurofibromatosis type 2 (NF2) [ 2 ], rather than in NF1. Isolated VS may present earlier than the diagnosis of NF2 and tends to be larger (average size of 3.4 cm), more aggressive and hypervascular [ 3 , 4 ].…”