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DOI: 10.1055/s-0037-1606623
Concomitant Occurrence of Vestibular Schwannoma and Epidermoid Cyst in Neurofibromatosis: Case description and Review of the Literature
Ocorrência concomitante de schwannoma vestibular e cisto epidermoide em neurofibromatose: descrição de caso e revisão da literaturaPublication History
25 February 2017
16 August 2017
Publication Date:
26 September 2017 (online)
Abstract
We report a case of a 16-year-old female patient harboring neurofibromatosis type 2 who presented with bilateral hearing impairment, which was on the left side, as well as facial paresis (House-Brackmann grade III) and ataxic gait. A magnetic resonance imaging (MRI) exam evidenced bilateral lesions in the cerebellopontine angles (CPAs) with extension into the internal acoustic meatus, and an additional lesion in the right CPA with radiological characteristics of an epidermoid cyst. The patient was submitted to microsurgical resection, confirming a collision of a vestibular schwannoma and an epidermoid cyst in the right CPA. In the present case report, we describe the first case reported in the literature with preoperative diagnostic work-up, intraoperative findings, postoperative course of the patient, as well as a detailed literature review of these specific coinciding pathologies, denoting the importance of further genomic studies regarding multiple central nervous system (CNS) lesions.
Resumo
Relatamos o caso de uma paciente de 16 anos de idade com neurofibromatose tipo II com deficiência auditiva bilateral, pior no ouvido esquerdo, assim como paresia facial (House-Brackmann grau III) e ataxia. Estudo de ressonância magnética comprovou lesão bilateral nos ângulos cerebelopontinos (ACPs) com extensão ao meato acústico interno, e uma lesão adicional no ACP direito com características radiológicas de um cisto epidermoide. A paciente foi submetida a ressecção microcirúrgica, confirmando a colisão de um schwannoma vestibular com um cisto epidermoide no ACP direito. No presente estudo, descrevemos o primeiro caso relatado na literatura com trabalho diagnóstico pré-operatório, resultados intraoperatórios, evolução da paciente no pós-operatório, assim como revisão detalhada da literatura específica sobre essas patologias, demonstrando a importância de mais estudos genômicos sobre as múltiplas lesões do sistema nervoso central (SNC).
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References
- 1 Gardner WJ, Turner OA. Multiple Intracranial Tumors: A discussion of the relation of meningeal to acoustic tumors and a report of a case. JAMA 1939; 113: 111-113
- 2 Kuroiwa T, Ohta T, Kobata H, Yamamoto H, Kimura N. Coexistence of intracranial meningioma and primary malignant lymphoma--case report. Neurol Med Chir (Tokyo) 1990; 30 (04) 268-271
- 3 Goodman RR, Torres RA, McMurtry III JG. Acoustic schwannoma and epidermoid cyst occurring as a single cerebellopontine angle mass. Neurosurgery 1991; 28 (03) 433-436
- 4 Kleinpeter G, Matula C, Koos W. Another case of acoustic schwannoma and epidermoid cyst occurring as a single cerebellopontine angle mass: possibly not so rare?. Surg Neurol 1994; 41 (04) 310-312
- 5 Sastri BVS, Babu HVS. Concomitant Occurrence of Vestibular Schwannoma and Epidermoid Cyst. Indian J Neurosurg. 2013; 2 (03) 282-283
- 6 Tatagiba M, Roser F, Schuhmann MU, Ebner FH. Vestibular schwannoma surgery via the retrosigmoid transmeatal approach. Acta Neurochir (Wien) 2014; 156 (02) 421-425 , discussion 425
- 7 Lee KS, Seo EK. Two primary intracranial tumors of different histology: report of a case with a choroid plexus papilloma and a concurrent vestibular schwannoma in the cerebellopontine angle. Yonsei Med J 2002; 43 (01) 133-137
- 8 Muzumdar DP, Goel A, Desai KI. Pontine glioma and cerebellopontine angle epidermoid tumour occurring as collision tumours. Br J Neurosurg 2001; 15 (01) 68-71
- 9 Talacchi A, Giorgiutti F, Andrioli M, Turazzi S, Bricolo A. Intracranial coexistence of neurinoma with epidermoid cyst or cholesterol granuloma. Report of 2 cases. J Neurosurg Sci 1997; 41 (02) 179-188
- 10 Saito A, Sugawara T, Watanabe R, Akamatsu Y, Mikawa S, Seki H. Evolution of vestibular schwannoma after removal of epidermoid cyst of the same location: case report. Neurol Med Chir (Tokyo) 2009; 49 (10) 495-498
- 11 Springborg JB, Poulsgaard L, Thomsen J. Nonvestibular schwannoma tumors in the cerebellopontine angle: a structured approach and management guidelines. Skull Base 2008; 18 (04) 217-227