Okubo T, Okabe H, Kato G
Department of Laboratory Medicine, Shiga University of Medical Science, Japan.
Acta Neuropathol. 1995;90(1):87-92. doi: 10.1007/BF00294464.
Juvenile xanthogranuloma is usually a self-limiting disease of the skin. Intracranial manifestations are extremely rare. We report the clinico-pathological features of an 8-month-old boy suffering from a gradually enlarging nodule of the chest wall and subsequent epileptic seizures. The subcutaneous tumor and a cerebral subcortical tumor of the left temporal lobe were resected. The histological appearance of both tumors corresponded to juvenile xanthogranuloma and included histiocytes, foamy cells, giant cells, inflammatory cells, and collagen-producing fibroblasts showing a storiform pattern. Immunohistochemical studies demonstrated positivity of the tumor cells for lysozyme, CD68 and myeloid-histiocytic antigen, but not S-100 protein, supporting mono-histiocytic differentiation. This case indicates that juvenile xanthogranuloma should be considered in the differential diagnosis of intracranial "xanthomatous" and histiocytic lesions.
幼年性黄色肉芽肿通常是一种皮肤自限性疾病。颅内表现极为罕见。我们报告了一名8个月大男孩的临床病理特征，该男孩患有逐渐增大的胸壁结节并随后出现癫痫发作。切除了皮下肿瘤和左侧颞叶的脑皮质下肿瘤。两个肿瘤的组织学表现均符合幼年性黄色肉芽肿，包括组织细胞、泡沫细胞、巨细胞、炎性细胞以及呈束状排列的产生胶原的成纤维细胞。免疫组织化学研究显示肿瘤细胞对溶菌酶、CD68和髓样组织细胞抗原呈阳性，但对S-100蛋白呈阴性，支持单核组织细胞分化。该病例表明，在颅内“黄色瘤样”和组织细胞性病变的鉴别诊断中应考虑幼年性黄色肉芽肿。
