Pediatric cerebellopontine angle and internal auditory canal tumors

Abstract

<jats:sec> <jats:title>Object</jats:title> <jats:p>The aim in this study was to describe the clinical presentation, differential diagnosis, and risk for neurofibromatosis Type 2 (NF2) in pediatric patients presenting with cerebellopontine angle (CPA) and internal auditory canal (IAC) tumors.</jats:p></jats:sec> <jats:sec> <jats:title>Methods</jats:title> <jats:p>The authors conducted a retrospective study at a tertiary care academic referral center. All patients with an age ≤ 18 years who had presented with an extraaxial CPA or IAC tumor between 1987 and 2012 were included in the study cohort. Data regarding symptoms, diagnosis, tumor characteristics, and NF2 status were collected and analyzed.</jats:p></jats:sec> <jats:sec> <jats:title>Results</jats:title> <jats:p>Sixty patients (55% female, 45% male) harboring 87 tumors were identified. The mean age at diagnosis was 12.8 years (median 14.0 years, range 0.9–18.9 years). Schwannomas were the most commonly identified lesions (57 of 87 tumors, including 52 vestibular, 3 facial, and 2 trigeminal schwannomas), followed by meningiomas (5 of 87) and epidermoid cysts (4 of 87). Six malignant tumors were diagnosed, including small-cell sarcoma, squamous cell carcinoma, malignant meningioma, atypical rhabdoid-teratoid tumor, endolymphatic sac tumor, and malignant ganglioglioma. Headache, followed by hearing loss and imbalance, was the most common presenting symptom, whereas dysphagia, otalgia, and facial pain were uncommon.</jats:p> <jats:p>Neurofibromatosis Type 2 was diagnosed in 20 (61%) of 33 patients with vestibular schwannoma (VS), while the other 13 patients (39%) had sporadic tumors. Nineteen of the 20 patients with NF2 met the diagnostic criteria for that disorder on initial presentation, and 15 of them presented with bilateral VS. At the last follow-up, 19 of the 20 patients subsequently diagnosed with NF2 demonstrated bilateral VSs, whereas 1 patient with a unilateral VS and multiple other NF2-associated tumors has yet to demonstrate a contralateral VS to date. Only 1 patient presenting with an isolated unilateral VS and no family history of NF2 demonstrated a contralateral VS on subsequent radiological screening.</jats:p></jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>Cerebellopontine angle and IAC tumors in the pediatric population are rare. There are several noteworthy differences between the adult and pediatric populations harboring these lesions. While VS is the most common pathology in both age groups, the lesion was found in only 60% of the pediatric patients in the present study. Unlike in adults, VSs in the pediatric population were associated with NF2 in over one-half of all cases. The majority of pediatric patients with NF2 fulfilled the diagnostic criteria at initial presentation; however, approximately 7% of patients presenting with a seemingly sporadic (no family history of NF2) unilateral VS will meet the criteria for NF2 later in life. Finally, malignancies account for a significantly higher percentage (10%) of cases among pediatric patients. These findings underscore the importance of early screening and close radiological follow-up and may be helpful in patient counseling.</jats:p></jats:sec>

Pediatric vestibular schwannomas: Evaluation of clinical features, treatment strategies and long-term results of 10 cases

“…In one large series [10] of pediatric CPA tumors, VSs also comprised the largest portion (60%), followed by meningioma (6%) and epidermoid cysts (5%), similar to adult population. [10,13,14] Likewise, Cunningham et al [15] reported that rate of VS is 86% on the basis of data obtained from a study with 115 pediatric lateral skull base lesions. However, in a study of Zúccaro and Sosa's [16] which consisted of 33 cases of pediatric CPA tumors, this rate was only 24%.…”

“…[8] On the other hand, significant proportion of unilateral pediatric VSs, assessed as sporadic at the time of diagnosis, has been reported to be diagnosed with NF2 at a later stage in life. [10] The rate of VSs is 96% in patients with genetically detected NF2, taking into consideration both unilateral and bilateral tumors. [17] In their study that analyzed pediatric CPA tumors, Holman et al [10] reported that 6% of children with schwannoma showed NF2 trait.…”

“…Neurofibromatosis type 2 (NF2) association is common in schwannomas, which account for approximately 2% of posterior fossa tumors in the pediatric population. [10] Treatment planning criteria pertinent to adults may not always be adequate for the pediatric group.…”

Giant cerebellopontine angle schwannoma in a child

“…There have been a limited number of case reports of spontaneous intracranial schwannomas in children 1 2. Up to 40% of children with cerebellopontine angle tumours may be negative for neurofibromatosis type 2 2.…”

Treatment decision-making for sporadic small vestibular schwannoma in a pediatric patient: A case report and literature review

“…At 36 months post-treatment, the patient exhibited no identifiable tumor enlargement. Within the literature, the treatment strategies for small VSs are varied, making it difficult for a neurosurgeon to decide the best course of action (4,7,8). In a study from the US, the strongest predictors of treatment modality were patient age and tumor size (3).…”