Pathologic Progression, Possible Origin, and Management of Multiple Primary Intracranial Neuroendocrine Carcinomas
Pathologic Progression, Possible Origin, and Management of Multiple Primary Intracranial Neuroendocrine Carcinomas
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多发性原发性颅内神经内分泌癌的病理进展、可能的起源和治疗
DOI：
10.1016/j.wneu.2017.07.053
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发表时间：
2017-10-01
影响因子：
2
通讯作者：
Liu, Yuguang
中科院分区：
文献类型：
--
作者：
Cao, Jingwei;Xu, Wenzhe;Liu, Yuguang
背景：原发性颅内神经内分泌癌（NECs）是一种极为罕见的恶性肿瘤，文献中未见多发病例的报道。病例描述：本文报告一名56岁女性患者的临床表现、术前和复查磁共振成像结果以及组织病理学研究，该患者患有多发性颅内NECs，模仿多发性颅内脑膜瘤，分别进行了左顶骨开颅、右枕顶骨开颅和左额骨开颅3次手术，并按时间顺序进行了报道。值得注意的是，第1例和第2例肿瘤被证实为NEC，其组织学特征为典型的间变性脑膜瘤，具有螺旋状形成的特征，而第3例肿瘤为典型的NEC，具有类器官癌巢的特征。换句话说，前两个病变被诊断为脑膜瘤而不是NEC。仅在第三次手术后，对前2例的病理进行了回顾并修改了诊断。第三次手术切除后，患者进一步接受全脑放疗和全身化疗（替莫唑胺联合YH-16）。在10个月的随访中，患者获得了良好的预后。结论：多发原发性颅内NECs极为罕见。肿瘤可能起源于蛛网膜或脑膜，其组织学模式可能导致转化和/或进展。最大的手术切除是必要的症状肿块效应。术后辅助治疗包括放疗和化疗应是推荐的治疗方式。
BACKGROUND: Primary intracranial neuroendocrine carcinomas (NECs) are extremely rare malignant tumors with no previous reports of multiple ones in the literatures.CASE DESCRIPTION: The 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.CONCLUSIONS: Multiple 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.