Prospective Evaluation of the Clinical Profile and Referral Pattern Differences of Vestibular Schwannomas and Other Cerebellopontine Angle Tumors

Ragab, Ahmed; Emara, Afaf; Shouker, Maged; Ebied, Osama M.

doi:10.1097/mao.0b013e318255dd59

Cited by 9 publications

(3 citation statements)

References 16 publications

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“…16 This finding was similar in the adult population, although at a significantly decreased frequency compared with the 55%-95% documented in adults. 19,20 Similar to the findings of Evans et al, approximately one-fourth of our patients with NF2 presented with hearing loss. 6 In comparison, we found that a much larger proportion of pediatric patients with nonsyndromic VSs (69%) presented with asymmetrical hearing loss.…”

Section: Discussionsupporting

confidence: 77%

“…21,22 Ragab et al demonstrated that VS-type lesions at diagnosis were significantly smaller than non-VS lesions, which was confirmed in our study. 20 Additionally, we found that the average size of VS was larger in nonsyndromic patients than in NF2-affected children. The smaller tumor size and lower prevalence of hearing loss at presentation among pediatric patients with NF2, as compared with those factors in children with sporadic VS, is primarily explained by the high number of asymptomatic patients in whom NF2 was diagnosed as a result of early screening.…”

Section: Discussionmentioning

confidence: 62%

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Pediatric cerebellopontine angle and internal auditory canal tumors

Holman¹,

Schmitt²,

Carlson³

et al. 2013

PED

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Object 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. Methods 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. Results 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. 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. Conclusions 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.

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Section: Discussionsupporting

confidence: 77%

Section: Discussionmentioning

confidence: 62%

Pediatric cerebellopontine angle and internal auditory canal tumors

Holman¹,

Schmitt²,

Carlson³

et al. 2013

PED

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“…[ 11 ] Case reports show that this variant, while rare, has appeared in various locations from the filum terminale to cervicomedullary junction as well as intracranially arising from locations in the parenchyma and intraventricularly. [ 4 6 9 14 15 ]…”

Section: Discussionmentioning

confidence: 99%

Vestibular schwannoma or tanycytic ependymoma: Immunohistologic staining reveals

et al. 2014

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Background:The cerebellopontine angle (CPA) is a common location for primary tumors, most often vestibular schwannomas, and also meningiomas, dermoids, and a host of other neoplasms. Our case report illustrates how radiologic and histopathologic presentations of an unusual variant of ependymal neoplasm can be diagnostically challenging and how accurate diagnosis can affect treatment protocols.Case History:Our patient had a CPA mass that was a variant of ependymoma known as tanycytic ependymoma that mimicked vestibular schwannoma radiologically and during intraoperative pathologic examination. Diagnosis as a World Health Organization (WHO) grade II tanycytic ependymoma was supported by its appearance on evaluation of the permanent sections, its diffuse immunoreactivity for glial fibrillary acidic protein (GFAP), and the perinuclear dot-and-ring-like staining for epithelial membrane antigen (EMA).Conclusions:Our patient's CPA mass initially believed to be a vestibular schwannoma on preoperative evaluation, surgical appearance, and intraoperative pathologic consultation was then correctly diagnosed as a WHO grade II tanycytic ependymoma on permanent histologic sections with the assistance of immunohistochemical stains, including EMA. After this definitive diagnosis, our patient's adjuvant treatment was adjusted. Earlier diagnosis could have provided guidance for goals of resection and prompt initiation of adjuvant treatment.

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Primary cerebellopontine angle melanoma: Case Report and Systematic Review

Rodríguez-Zúñiga

Effio-Iman

2017

Polish Annals of Medicine

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Prospective Evaluation of the Clinical Profile and Referral Pattern Differences of Vestibular Schwannomas and Other Cerebellopontine Angle Tumors

Cited by 9 publications

References 16 publications

Pediatric cerebellopontine angle and internal auditory canal tumors

Pediatric cerebellopontine angle and internal auditory canal tumors

Vestibular schwannoma or tanycytic ependymoma: Immunohistologic staining reveals

Primary cerebellopontine angle melanoma: Case Report and Systematic Review

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