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CC BY-NC-ND 4.0 · Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery 2023; 42(01): e73-e78
DOI: 10.1055/s-0042-1744113
Case Report

Intradiploic Epidermoid Cyst of the Posterior Fossa – Case Report and Review of the Literature

Cisto epidermoide intradiploico da fossa posterior – Caso clinico e revisão da literatura
Gonçalo Januário 
1   Department of Neurosurgery, Centro Hospitalar e Universitário Lisboa Central, Hospital de São José, Lisboa, Portugal
,
Bruno Cunha 
2   Department of Neuroradiology, Centro Hospitalar e Universitário Lisboa Central, Hospital de São José, Lisboa, Portugal
,
Ludovica Cellini
3   Department of Neurosurgery, University Hospital of Siena, Italy
,
Gonçalo Novais
1   Department of Neurosurgery, Centro Hospitalar e Universitário Lisboa Central, Hospital de São José, Lisboa, Portugal
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Abstract

Intracranial epidermoid cysts represent ∼ 0.2 to 1.8% of all intracranial tumors. These tumors are most frequently encountered in the cerebellar pontine angle or in the parasellar region. Rarely, they arise from the cranial diploe, being able to affect every flat bone of the cranium.

We report a case of a 63-year-old male who presented with progressively worsening headache and vertigo with 6 months of evolution. Neuroimaging identified a probable occipital intradiploic epidermoid cyst with mass effect on the cerebellar hemispheres. This lesion was approached using a suboccipital craniotomy, followed by total resection of the tumor and cranioplasty with titanium plate placement. The histological evaluation confirmed the diagnosis of intraosseous epidermoid cyst. The patient had a successful recovery, without complications or neurologic dysfunction.

Being benign lesions, commonly remaining asymptomatic and rarely presenting as a bony lump in the skull bone, it could be straightforward to assume a conservative management with planned follow-up. On the other side, a more aggressive strategy with surgical excision has been advocated, especially in lesions that tend to enlarge and erode the cranial bone with possible consequent epidural extension and mass effect symptoms. A preoperative diagnosis is extremely helpful in proper surgical planning. Diffusion weighted imaging facilitates a straightforward diagnosis.

As was observed in our case, the largest reviews on intradiploic epidermoids available in the literature mostly demonstrated a benign clinical course. However, malignant transformation can occur. Some patients develop permanent neurologic deficits from mass effect or tumor infiltration. However, surgical approach of the tumor is curative in most cases. Nonetheless, from our experience, it is important to maintain clinical and imaging follow-up with regular monitoring to prevent possible tumor recurrences.

Resumo

Os cistos epidermoides intracranianos representam cerca de 0,2 a 1,8% de todos os tumores intracranianos. Esses tumores são mais frequentemente encontrados no ângulo pontocerebeloso ou na região parasselar. Raramente surgem na diploe, no entanto podem afetar todos os ossos do crânio. Relatamos o caso de um homem de 63 anos que apresentou agravamento progressivo da cefaleia e vertigem e desequilíbrio com 6 meses de evolução. O estudo de imagem realizado identificou um provável cisto epidermoide intradiploico occipital com efeito de massa nos hemisférios cerebelosos. A lesão foi abordada por craniotomia suboccipital, seguida de ressecção total do tumor e cranioplastia com colocação de placa de titânio. A avaliação histológica confirmou o diagnóstico de cisto epidermoide intraósseo. O doente teve uma recuperação bem sucedida, sem complicações ou disfunção neurológica. Sendo lesões benignas, que geralmente permanecem assintomáticas, pode-se assumir um tratamento conservador com seguimento clínico. Por outro lado, uma estratégia mais agressiva com excisão cirúrgica tem sido preconizada, principalmente em lesões que tendem a aumentar e invadir as estruturas ósseas cranianas com possível extensão peridural e sintomas causados pelo efeito de massa. Um diagnóstico pré-operatório é extremamente útil no planeamento cirúrgico adequado. A imagem ponderada por difusão facilita um diagnóstico direto. Como observado no nosso caso, as maiores revisões sobre epidermoides intradiploicos disponíveis na literatura demonstraram, na sua maioria, um curso clínico benigno. No entanto, a transformação maligna pode ocorrer. Alguns doentes desenvolvem déficits neurológicos permanentes por efeito de massa ou infiltração tumoral. A abordagem cirúrgica do tumor é curativa na maioria dos casos. Consideramos importante manter o seguimento clínico e imagiológico regular para prevenir possíveis recidivas tumorais.

Keywords

epidermoid cyst - craniotomy - cranioplasty - titanium mesh

Palavras-chave

cisto epidermoide - craniotomia - cranioplastia - rede de titânio


Publication History

Received: 01 February 2021

Accepted: 18 January 2022

Article published online:
06 September 2022

© 2022. Sociedade Brasileira de Neurocirurgia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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