1 Department of Pathology, the Second Affiliated Hospital of Fujian Medical University, Quanzhou 362000, China ; 2 Department of Pathology, 3 Department of Neurosurgery, 4 Department of Radiology, the First Affiliated Hospital of Fujian Medical University, Fuzhou 350005, China.
Chin J Cancer Res. 2014 Apr;26(2):211-4. doi: 10.3978/j.issn.1000-9604.2014.02.12.
The brain parenchymal Langerhans cell histiocytosis (LCH) without systemic disease or lytic skull lesions is extremely rare. We report a 23-year-old male presenting with new onset 1 hour seizure with loss of consciousness 20 days prior to admission, and recurrent seizure 2 weeks later. Brain magnetic resonance imaging (MRI) showed an irregularly mass with enhancement involving the right frontal lobe. Microscopically, the lesion was characterized by sheets of Langerhans cells in addition to reactive inflammatory elements. Immunohistochemically, Langerhans cells were positive for Langerin, CD1a and S-100 protein. The patient received no chemotherapy or radiotherapy after surgery. After 24 months of follow-up, no recurrence or other systemic lesions were observed. Although there is no standard treatment for solitary cerebral LCH, the prognosis generally appears to be good.
脑实质朗格汉斯细胞组织细胞增生症（LCH）无全身疾病或溶骨性颅骨病变极为罕见。我们报告了一名 23 岁男性，入院前 20 天出现新发 1 小时癫痫发作伴意识丧失，2 周后再次出现癫痫发作。脑部磁共振成像（MRI）显示右额叶不规则肿块伴增强。显微镜下，病变的特征是朗格汉斯细胞片，伴有反应性炎症成分。免疫组化染色显示，朗格汉斯细胞对朗格汉斯蛋白、CD1a 和 S-100 蛋白呈阳性。患者手术后未接受化疗或放疗。24 个月随访后，未观察到复发或其他全身病变。虽然孤立性脑朗格汉斯细胞组织细胞增生症没有标准的治疗方法，但预后通常较好。
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