Department of Neurosurgery, Kanazawa University Hospital, Kanazawa, Japan.
Neurosurgery. 2011 Apr;68(4):E1144-7; discussion E1147. doi: 10.1227/NEU.0b013e31820a16d4.
We report the case of a patient with multiple neuroepithelial tumors of different histological cell types. In this patient, synchronous appearance of tumors of the central nervous system, ie, cervical ependymoma and a fourth ventricle choroid plexus papilloma, was noted.
A 36-year-old man presented with sensory disturbance of the bilateral upper extremities. Magnetic resonance imaging revealed a heterogeneously enhanced mass with syringomyelia in the upper cervical cord and a well-enhanced mass in the fourth ventricle. The patient underwent bilateral suboccipital craniotomy and laminectomy from the level of C1 to C3 for both lesions. The lesions were grossly resected. Histological and immunohistochemical examinations revealed that the cervical tumor was an ependymoma, and the fourth ventricle tumor was a choroid plexus papilloma.
The appearance of synchronous multiple neuroepithelial tumors of different histological cell types is extremely rare, and this is the first known case of ependymoma occurring synchronously with choroid plexus papilloma, both tumors having different histological cell types. Displacement and maldifferentiation of primitive, multipotent neuroepithelial cells may be a causative factor for this unique presentation.
我们报告了一例患有多种不同组织学细胞类型的神经上皮肿瘤的患者。该患者同时出现了中枢神经系统肿瘤，即颈髓室管膜瘤和第四脑室脉络丛乳头状瘤。
一名 36 岁男性出现双侧上肢感觉障碍。磁共振成像显示在上颈髓有一个不均匀增强的肿块伴脊髓空洞，以及在第四脑室有一个明显增强的肿块。患者接受了双侧枕下颅骨切开术和 C1 至 C3 的椎板切除术以切除这两个病变。病变大体上被切除。组织学和免疫组织化学检查显示颈髓肿瘤为室管膜瘤，第四脑室肿瘤为脉络丛乳头状瘤。
同时出现不同组织学细胞类型的多个神经上皮肿瘤极为罕见，这是首例已知的室管膜瘤与脉络丛乳头状瘤同时发生，且两种肿瘤具有不同的组织学细胞类型。原始、多能神经上皮细胞的移位和去分化可能是这种独特表现的一个致病因素。
