Wang Chao, Kuang Pingding, Xu Fangfang, Hu Ling
Department of Radiology, the Second Affiliated Hospital, School of Medicine, Zhejiang University.
Department of Ultrasound, Hangzhou Women's Hospital, Hangzhou, Zhejiang, China.
Medicine (Baltimore). 2019 May;98(18):e15548. doi: 10.1097/MD.0000000000015548.
Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis. Petroclival RDD is extremely rare. To the best of our knowledge, only 7 cases of petroclival RDD have been reported so far. Herein, we present the 8th case of intracranial RDD with the petroclival and parasellar involvement mimicking multiple meningiomas.
A 57-year-old woman presented with a 1-year history of vision diminution and 1-month hearing loss in her right ear.
Contrast-enhanced Magnetic resonance imaging (MRI) of the brain demonstrated multiple well-defined, homogenous mass which closely related to the dura mater in the bilateral parasellar and petroclival regions range from the basement of anterior to posterior cranial fossa. The lesions were T1 isointense, T2 hypointense, and homogeneously enhanced. Initial diagnosis of multiple meningiomas was made according to MRI findings. Final diagnosis of RDD was confirmed by histopathological and immunohistochemical examinations after subtotal surgical resection.
The patient received subtotal resection because multiple lesions were extensive.
The vision diminution recovered well after the surgery but the hearing loss in her right ear was still persisted.
Although rare, a standard RDD typically are dural-based, extra-axial, well-circumscribed masses mimicking meningioma, and presenting with characterized hypo to isointense on T1-weighted images, hypo to isointense on T2-weighted images, and obvious enhancement. Resection of the intracranial lesion is the most effective treatment. In case of subtotal resection, the application of adjunctive radiotherapy and/or steroid agents should be advised. Final diagnosis of RDD should be confirmed by histopathological and immunohistochemical examinations.
罗萨伊-多夫曼病(RDD)是一种罕见的非朗格汉斯细胞组织细胞增多症。岩斜区RDD极为罕见。据我们所知,迄今为止仅报道了7例岩斜区RDD。在此,我们报告第8例累及岩斜区和鞍旁的颅内RDD,其表现类似多发脑膜瘤。
一名57岁女性,有1年视力减退病史,右耳听力丧失1个月。
脑部增强磁共振成像(MRI)显示双侧鞍旁和岩斜区有多个边界清晰、均匀的肿块,从前颅窝底部到后颅窝范围均与硬脑膜密切相关。病变在T1加权像上呈等信号,T2加权像上呈低信号,并呈均匀强化。根据MRI表现初步诊断为多发脑膜瘤。在次全手术切除后,通过组织病理学和免疫组织化学检查确诊为RDD。
由于病变广泛,患者接受了次全切除。
术后视力减退恢复良好,但右耳听力丧失仍持续存在。
尽管罕见,但典型的RDD通常是基于硬脑膜的、轴外的、边界清晰的肿块,类似脑膜瘤,在T1加权图像上呈低到等信号,在T2加权图像上呈低到等信号,并明显强化。切除颅内病变是最有效的治疗方法。如果是次全切除,建议应用辅助放疗和/或类固醇药物。RDD的最终诊断应通过组织病理学和免疫组织化学检查来证实。