Huo C W, Caputo C, Wang Y Y
Department of Neurosurgery, St Vincent's Hospital Melbourne, Victoria, Australia.
Department of Surgery, St Vincent's Hospital, The University of Melbourne, Parkville, Victoria, Australia.
Surg Neurol Int. 2018 Jan 22;9:15. doi: 10.4103/sni.sni_269_17. eCollection 2018.
Keratinous or epidermoid cysts (ECs) are encapsulated lesions lined by squamous cell epithelium. They comprise approximately 1% of intracranial lesions. Contrary to dermoid cysts, they lack dermal elements such as sebaceous or apocrine glands and hair follicles. The sellar region is the second most common intracranial site following the cerebellopontine angle. Here, we report a case of EC in a patient who complained of endocrine disturbances. We also performed a systematic review on previously published cases to analyze clinical and radiological characteristics and report the treatment outcomes of suprasellar ECs.
A 42-year-old woman presented with a one-year history of amenorrhea, weight gain, severe headache, and visual disturbances for 6 months. Work-up identified an elevated prolactin level and a temporal field defect of the right eye. Magnetic resonance imaging (MRI) showed a cystic suprasellar lesion pushing on the optic chiasm. She underwent endoscopic trans-sphenoidal surgery, which confirmed a keratinous cyst on histology. Postoperatively, complete resection was confirmed on imaging. She did well although her hospital stay was prolonged due to diabetes insipidus and hypocortisolism.
Chronic endocrine disturbances can be the presenting complaints of a suprasellar EC, whose T1-weighted MRI appearance can be non-specific, mimicking other differential diagnoses, such as a Rathke's cleft cyst. However, the T2-weighted MRI appearances of ECs are generally hyper-intense and lesions show diffusion restriction. Treatment is surgical and yields good outcomes in most cases reported.
角质囊肿或表皮样囊肿(ECs)是由鳞状上皮细胞衬里的包膜性病变。它们约占颅内病变的1%。与皮样囊肿不同,它们缺乏皮脂腺、顶泌汗腺和毛囊等皮肤成分。鞍区是继桥小脑角之后第二常见的颅内部位。在此,我们报告一例主诉内分泌紊乱的EC患者病例。我们还对先前发表的病例进行了系统综述,以分析临床和放射学特征,并报告鞍上EC的治疗结果。
一名42岁女性,有1年闭经、体重增加病史,6个月来有严重头痛和视力障碍。检查发现催乳素水平升高,右眼颞侧视野缺损。磁共振成像(MRI)显示鞍上囊性病变压迫视交叉。她接受了内镜经蝶窦手术,组织学检查证实为角质囊肿。术后影像学检查证实完全切除。尽管因尿崩症和皮质醇减退症住院时间延长,但她恢复良好。
慢性内分泌紊乱可能是鞍上EC的主要表现,其T1加权MRI表现可能不具有特异性,可模仿其他鉴别诊断,如拉克氏裂囊肿。然而,EC的T2加权MRI表现通常为高信号,病变显示扩散受限。治疗方法为手术,在大多数报道的病例中效果良好。