DEMYELINATING DISEASE VERSUS TUMOR IN SURGICAL NEUROPATHOLOGY - CLUES TO A CORRECT PATHOLOGICAL DIAGNOSIS
DEMYELINATING DISEASE VERSUS TUMOR IN SURGICAL NEUROPATHOLOGY - CLUES TO A CORRECT PATHOLOGICAL DIAGNOSIS
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DOI：
10.1097/00000478-199306000-00001
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发表时间：
1993-06-01
影响因子：
5.6
通讯作者：
BUDZILOVICH, GN
中科院分区：
文献类型：
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作者：
ZAGZAG, D;MILLER, DC;BUDZILOVICH, GN
多发性硬化症（MS）或其他中枢神经系统脱髓鞘疾病患者的临床表现以及放射学和组织病理学活检结果有时会产生误导，导致脑或脊髓肿瘤的误诊。我们报告了17例患者，他们表现出类似脑肿瘤（14例）或脊髓肿瘤（3例）的症状。在每种情况下，计算机断层扫描或磁共振成像研究或两者都被解释为与肿瘤一致。所有患者均接受手术，所有17个病理标本最终诊断为脱髓鞘疾病，通常与MS一致。在每种情况下，我们检查了各种组织学特征和免疫组化研究，并考虑MS的诊断解决其相对重要性。所有病例均显示血管周围淋巴细胞炎症与不同量的巨噬细胞浸润，坏死和水肿。病变的细胞过多和存在具有核分裂像的非典型反应性星形胶质细胞是可能导致星形胶质细胞肿瘤误诊的令人不安的特征。星形胶质细胞（胶质细胞酸性蛋白）和巨噬细胞（HAM-56）标记物的免疫组化有助于评估活检。我们的研究结果强调需要进行特殊染色（即，对于髓鞘和轴突），其显示髓鞘损失和轴突的相对保留，并允许正确的诊断。
Clinical presentations as well as radiological and histopathological findings in biopsies from patients with multiple sclerosis (MS) or other demyelinating disorders of the central nervous system are sometimes misleading, resulting in an erroneous diagnosis of brain or spinal cord tumor. We report 17 patients who presented with symptoms mimicking those of brain (14 cases) or spinal cord (three cases) tumors. Computerized tomography or magnetic resonance imaging studies or both were interpreted as consistent with a tumor in each case. All patients underwent surgery, and all 17 pathological specimens were eventually diagnosed as showing demyelinating disease, usually consistent with MS. In each case we examined a variety of histological features and immunohistochemical studies and addressed their relative importance in considering the diagnosis of MS. All cases showed perivascular lymphocytic inflammation with variable amounts of macrophage infiltration, necrosis, and edema. The hypercellularity of the lesions and the presence of atypical reactive astrocytes with mitotic figures were the disturbing features that might have led to the erroneous diagnosis of an astrocytic neoplasm. Immunohistochemistry for astrocytic (glial fibrillary acidic protein) and macrophage (HAM-56) markers are helpful in evaluating biopsies. Our results emphasize the need to perform special stains (i.e., for myelin and axons) that demonstrate myelin loss and relative preservation of axons and allow a correct diagnosis.