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颅骨多中心性婴儿肌纤维瘤病:病例报告

Multicentric infantile myofibromatosis in the cranium: case report.

作者信息

Hasegawa M, Kida S, Yamashima T, Yamashita J, Takakuwa S

机构信息

Department of Neurosurgery, Kanazawa University School of Medicine, Japan.

出版信息

Neurosurgery. 1995 Jun;36(6):1200-3. doi: 10.1227/00006123-199506000-00023.

Abstract

Infantile myofibromatosis is a rare clinical entity characterized by multiple mesenchymal tumors in the neonatal period. We describe a 15-month-old girl with multicentric cranial lesions involving the parietal and occipital bones associated with a single small subcutaneous lesion in the back. Magnetic resonance imaging clearly demonstrated the isointense lesions on T1-, T2-, and proton density-weighted images, which showed marked gadolinium enhancement of the tumors and adjacent dura mater. A histological examination of the resected temporal lesion revealed the myofibroblastic nature of the tumor cells. This is the first description of magnetic resonance features of multicentric infantile myofibromatosis in the cranium, and gadolinium-enhanced magnetic resonance images were useful in showing dural involvement. The importance of recognizing this disorder is emphasized because of its special clinical behavior.

摘要

婴儿肌纤维瘤病是一种罕见的临床病症,其特征为新生儿期出现多个间充质肿瘤。我们描述了一名15个月大的女孩,患有累及顶骨和枕骨的多中心颅骨病变,并伴有背部单个小的皮下病变。磁共振成像在T1加权、T2加权和质子密度加权图像上清晰显示等信号病变,肿瘤及相邻硬脑膜有明显钆增强。对切除的颞部病变进行组织学检查显示肿瘤细胞具有肌成纤维细胞性质。这是关于颅骨多中心婴儿肌纤维瘤病磁共振特征的首次描述,钆增强磁共振图像有助于显示硬脑膜受累情况。鉴于其特殊的临床行为,强调了认识这种疾病的重要性。

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