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首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Primary intracranial malignant ectomesenchymoma in an adult: Report of a rare case and review of the literature
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Primary intracranial malignant ectomesenchymoma in an adult: Report of a rare case and review of the literature

机译：成人的原发性颅内恶性异位表展性能：对罕见案例的报告和文献审查

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摘要

Malignant ectomesenchymoma (MEM) is an exceedingly rare rapidly progressing tumor of soft tissues of the central nervous system, believed to be derived from neural crest cells. The majority of cases have been observed in young children or adolescents. So far only 11 patients with intracranial manifestations (with confirmed clinicopathological data) have been documented. We report the first case of adult intracranial MEM in a 54‐year‐old man who presented with a 4 months history of headache and weakness of right side of the body. Magnetic resonance imaging showed a homogenously enhanced dural‐based lesion in the left fronto‐temporo‐parietal lobe with significant perilesional edema and mass effect. No metastatic disease was identified and the lesion was grossly resected. Histopathological and immunohistochemical examination revealed mature and immature neurons and bizarre astrocytes admixed with a mesenchymal spindle cell (rhabdomyoblastic) component. Specific risk factors that contribute toward the development of MEM are unknown. Due to the scarcity of reported cases the role of adjuvant therapy is unclear.
机译：恶性异位表展瘤瘤（MEM）是中枢神经系统的软组织的非常罕见的快速进展肿瘤，被认为是源自神经顶部的细胞。在幼儿或青少年中观察到大多数病例。到目前为止，仅记录了11例颅内表现（具有确诊的临床病理数据）的患者。我们在一个54岁的男子中举报了成人颅内Mem的第一个案例，他们呈现了4个月的头痛和身体右侧弱点的历史。磁共振成像显示左前颞下叶中均匀增强的基于多久的病变，具有显着的细胞水肿和质量效应。没有鉴定转移性疾病，并且病变被严重切除。组织病理学和免疫组化检查显示了与间充质主轴细胞（横纹囊泡）组分混合的成熟和未成熟的神经元和奇异的星形胶质细胞。有助于MEM发展的具体风险因素是未知的。由于报告案例的稀缺性，佐剂治疗的作用尚不清楚。

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