• Jingwei Cao, Wenzhe Xu, Zhenhui Du, Bin Sun, Feng Li, and Yuguang Liu.
• Department of Neurosurgery, Qilu Hospital and Brain Science Research Institute of Shandong University, Shandong, People's Republic of China; Department of Neurosurgery, Qilu Children's Hospital of Shandong University, Shandong, People's Republic of China.
• World Neurosurg. 2017 Oct 1; 106: 1054.e13-1054.e17.

BackgroundPrimary intracranial neuroendocrine carcinomas (NECs) are extremely rare malignant tumors with no previous reports of multiple ones in the literatures.Case DescriptionThe clinical presentation, preoperative and reexamined magnetic resonance imaging findings, as well as histopathologic studies of a 56-year-old female subject with multiple intracranial NECs mimicking multiple intracranial meningiomas, who underwent 3 operations with left parietal craniotomy, right occipital parietal craniotomy, and left frontal craniotomy, separately and chronologically, are presented in this article. Noteworthy, the first and second tumors were confirmed as NECs exhibiting histologic characteristics of typical anaplastic meningiomas with features of whorl formation, while the third tumor was a typical NEC with features of organoid cancer nests. In other words, the first 2 lesions were diagnosed as meningioma as opposed to NEC. It was only after the third surgery that the pathology for the first 2 cases was reviewed and had a revised diagnosis. After the third surgical resection, the patient further received whole brain radiotherapy and systemic chemotherapy (temozolomide combined with YH-16). At her 10-month follow-up, the patient achieved a good outcome.ConclusionsMultiple primary intracranial NECs are extremely rare. The tumor might be of arachnoidal or leptomeningeal origin, with histologic patterns that might lead to transformation and/or progression. Maximal surgical resection is warranted for symptomatic mass effect. Postoperative adjuvant treatments including radiotherapy and chemotherapy should be a recommended therapeutic modality.Copyright © 2017 Elsevier Inc. All rights reserved.

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