Kawabe Keita, Nakayama Yoko, Jimbo Yasushi, Usuda Hiroyuki, Toyoshima Yasuko, Kawaguchi Tadashi
Department of Neurosurgery, Nagaoka Red Cross Hospital, Nagaoka, Niigata, Japan.
Department of Pathology, Nagaoka Red Cross Hospital, Nagaoka, Niigata, Japan.
NMC Case Rep J. 2019 Dec 18;7(1):29-34. doi: 10.2176/nmccrj.cr.2019-0058. eCollection 2020 Jan.
Primary intracranial malignant epidermoids are rare, with most cases developing from a pre-existing benign epidermoid cyst. We report a case involving a rare autopsy finding of a primary intracranial malignant epidermoid in the brainstem with cerebellopontine angle (CPA) involvement. A 53-year-old woman with double vision was diagnosed with right abducens palsy. At her visit to our hospital 3 months after the onset of the first symptom, she presented left hypoglossal nerve paralysis and truncal ataxia in addition to right abducens palsy. Magnetic resonance imaging (MRI) revealed a mass lesion (2-cm long and 3-cm thick) in the left CPA that exhibited gadolinium enhancement. Moreover, gadolinium-enhanced magnetic resonance imaging (MRI) revealed abnormal multiple brainstem and supratentorial mass lesions with partial enhancement. Whole-body computed tomography failed to identify any possible primary lesion. Following a tentative diagnosis of an epidermoid cyst with an assumption that the tumor was highly aggressive, we performed subtotal surgical resection of the CPA tumor. Histological findings revealed a malignant epidermoid in the CPA lesion. Although the patient underwent radiation and chemotherapy after the surgical resection, she died of respiratory failure 10 months after the onset of symptoms. Herein, we report the rare clinical course and autopsy data, and discuss the characteristic features of this rare condition.
原发性颅内恶性表皮样囊肿罕见，大多数病例由先前存在的良性表皮样囊肿发展而来。我们报告一例罕见的尸检病例，该原发性颅内恶性表皮样囊肿位于脑干并累及小脑脑桥角（CPA）。一名53岁出现复视的女性被诊断为右侧展神经麻痹。在首发症状出现3个月后来我院就诊时，除右侧展神经麻痹外，她还出现了左侧舌下神经麻痹和躯干共济失调。磁共振成像（MRI）显示左侧CPA有一个肿块病变（长2 cm，厚3 cm），呈钆增强。此外，钆增强磁共振成像（MRI）显示脑干和幕上有多个异常肿块病变，部分呈增强表现。全身计算机断层扫描未发现任何可能的原发病变。在初步诊断为表皮样囊肿并假设肿瘤具有高度侵袭性后，我们对CPA肿瘤进行了次全手术切除。组织学检查结果显示CPA病变为恶性表皮样囊肿。尽管患者在手术切除后接受了放疗和化疗，但在症状出现10个月后死于呼吸衰竭。在此，我们报告这一罕见的临床病程和尸检数据，并讨论这种罕见疾病的特征。
