Alper G, Crumrine P K, Hamilton R L, Albright A L, Wald E R
Division of Pediatric Neurology, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh, Pennsylvania.
Pediatr Neurol. 1996 Jul;15(1):60-2. doi: 10.1016/0887-8994(96)00090-2.
Castleman syndrome (giant lymph node hyperplasia) is a rare, heterogeneous lymphoproliferative disorder of unknown etiology and pathogenesis. Most cases occur as mediastinal masses, although extrathoracic involvement including nodal and extranodal locations have been reported. The localized variants (solitary lesions) respond well to surgical excision. We report a 10-year-old boy who presented with headache, intermittent fever, and progressive weakness of his legs. MRI imaging showed an enhancing epidural mass with impingement on the spinal cord at the C6-T2 level. Other laboratory abnormalities included anemia, hypergammaglobulinemia, increased erythrocyte sedimentation rate, and cerebrospinal fluid pleocytosis with slightly increased cerebrospinal protein. The mass was partially resected and the histopathology showed lymphoplasmocytic infiltration compatible with Castleman syndrome. There was no evidence of malignancy. Castleman syndrome is the most likely diagnosis in the presence of the associated systemic findings, although the epidural site for lymphoplasmocytic inflammation is atypical.
卡斯特曼病（巨大淋巴结增生症）是一种病因和发病机制不明的罕见异质性淋巴增殖性疾病。大多数病例表现为纵隔肿块，不过也有报道称其可累及胸外部位，包括淋巴结及结外部位。局限性变体（孤立性病变）对手术切除反应良好。我们报告了一名10岁男孩，他出现头痛、间歇性发热及双下肢进行性无力。MRI成像显示C6 - T2水平有一个强化的硬膜外肿块，压迫脊髓。其他实验室异常包括贫血、高球蛋白血症、红细胞沉降率升高，以及脑脊液细胞增多伴脑脊液蛋白轻度升高。肿块部分切除，组织病理学显示为与卡斯特曼病相符的淋巴浆细胞浸润。无恶性肿瘤证据。尽管淋巴浆细胞炎症的硬膜外部位不典型，但结合相关全身表现，卡斯特曼病是最可能的诊断。
