Physiopathological Aspects of Sellar Epidermoid Cyst Determining Endocrine Disturbances: A Case Report

Abstract

Epidermoid cysts (ECs) of the central nervous system (CNS) constitute benign circumscribed lesions that are more common in lateral than in midline sites. Epidermoid cysts of the CNS arise more frequently in the cerebellopontine angle, around the pons, near the sella, within the temporal lobe, in the diploe, and in the spinal canal. Most common tumoral lesion of sellar region is pituitary adenoma, and sellar cystic epithelial masses may be difficult to differentiate based only on clinical and imaging findings. Epidermoid cysts are covered by keratinized squamous epithelium and are usually filled with keratin lamellae. The process is, for the most part, maldevelopmental in origin, presumably arising from trapped surface ectodermal elements in association with the developing CNS during the closure of the neural groove or formation of the secondary cerebral vesicles. In the present study, the authors describe a case of sellar epidermoid cyst producing endocrine alterations and visual disturbance in a 35 years woman, and review the physiopathological and diagnostic criteria of this lesion.

Resumo

Os Cistos Epidermoides (CE) do sistema nervoso central (SNC) constituem lesões benignas circunscritas, que são mais comuns na linha media do que em regiões laterais. Os CE do SNC ocorrem com maior frequência no ângulo pontocerebelar, ao redor da ponte, próximo à sela, no lobo temporal, na diploe e no canal espinhal. A lesão tumoral mais comum da região selar é o adenoma hipofisário, e as massas epiteliais císticas selares podem ser difíceis de diferenciar baseando-se apenas nos achados clínicos e de imagem. Os CE são cobertos por epitélio escamoso queratinizado e geralmente são preenchidos por lamelas de queratina. O processo é, em sua maior parte, de origem malformativa, presumivelmente em decorrência de elementos ectodérmicos de superfície aprisionados no desenvolvimento do SNC durante o fechamento do sulco neural ou na formação das vesículas cerebrais secundárias. Neste trabalho, os autores descrevem um caso de cisto epidermoide selar, que determinou alterações endócrinas e distúrbios visuais em uma mulher de 35 anos, e revisam os critérios fisiopatológicos e diagnósticos dessa lesão.

Keywords

epidermoid cyst - sellar region - pathology - central nervous system cysts - brain neoplasms

Palavras-chave

cisto epidermoide - região sellar - patologia - cistos do sistema nervoso central - neoplasias cerebrais

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