Martinez Derek L, Gujrati Meena, Geoffroy Francois, Tsung Andrew J
Department of Neurosurgery/Neurosurgical Oncology, Illinois Neurological Institute/University of Illinois College of Medicine, 530 NE Glen Oak, Peoria, IL 61637, USA.
CNS Oncol. 2014 Nov;3(6):383-7. doi: 10.2217/cns.14.45.
CNS involvement in the setting of lymphoid neoplasia is a clinical situation that requires specific diagnosis due to the disparate treatment regimens recommended for neoplasms of specific lymphoid cell types. Cerebrospinal fluid (CSF) sampling may provide sufficient information to determine the presence of abnormal lymphoid cells but may not be able to further specify the malignant cellular population. In cases where abnormal clinical or radiographic features are present, accurate tissue diagnosis is essential. In this report, we define a rare case of primary CNS intramedullary Hodgkin's lymphoma without leptomeningeal dissemination diagnosed via resectional biopsy of a conus medullaris lesion. The patient received post-resection radiation therapy and subsequently demonstrated radiographic and clinical improvement. Lymphoid neoplasia within the CNS comprises a diverse group with varying response and survival rates. Treatment hinges upon accurate diagnosis as chemotherapy varies widely among Hodgkin's and non-Hodgkin's lymphoma. While CSF sampling may yield a positive result with sufficiency to diagnose an abnormal lymphoid cell population, tissue is necessary for further defining cellular pathology. In this report, we define a rare case of primary CNS intramedullary Hodgkin's lymphoma without leptomeningeal dissemination via resectional biopsy of a conus medullaris lesion. In cases where abnormal enhancement is found in eloquent CNS regions and lymphoid neoplasia is suspected, management often entails either stereotactic biopsy or CSF sampling. While CSF analysis may differentiate malignancy at a low rate, tissue diagnosis via paraffin block immunohistochemistry is necessary to further classify malignancy as primary or peripheral, Hodgkin's or non-Hodgkin's lymphoma, or other such as metastatic leptomeningeal dissemination and glioma. Within the subtypes of lymphoid neoplasms, treatment regimens vastly differ and thus accurate tissue diagnosis is paramount. We therefore present a rare case of primary CNS intramedullary Hodgkin's lymphoma without leptomeningeal disease in the setting of immunocompromise diagnosed via open resectional biopsy of the conus medullaris.
中枢神经系统（CNS）受累于淋巴样肿瘤的情况是一种临床状况，由于针对特定淋巴样细胞类型肿瘤推荐的治疗方案不同，因此需要进行特定诊断。脑脊液（CSF）采样可能提供足够信息以确定异常淋巴样细胞的存在，但可能无法进一步明确恶性细胞群体。在存在异常临床或影像学特征的情况下，准确的组织诊断至关重要。在本报告中，我们定义了一例罕见的原发性中枢神经系统髓内霍奇金淋巴瘤，无软脑膜播散，通过圆锥髓质病变的切除活检确诊。患者接受切除术后放疗，随后影像学和临床症状均有改善。中枢神经系统内的淋巴样肿瘤包括一组异质性疾病，其反应和生存率各不相同。治疗取决于准确诊断，因为霍奇金淋巴瘤和非霍奇金淋巴瘤的化疗差异很大。虽然脑脊液采样可能产生足以诊断异常淋巴样细胞群体的阳性结果，但需要组织来进一步明确细胞病理学。在本报告中，我们通过圆锥髓质病变的切除活检定义了一例罕见的原发性中枢神经系统髓内霍奇金淋巴瘤，无软脑膜播散。在明确的中枢神经系统区域发现异常强化并怀疑有淋巴样肿瘤的情况下，管理通常需要立体定向活检或脑脊液采样。虽然脑脊液分析可能以较低的概率区分恶性肿瘤，但通过石蜡块免疫组织化学进行组织诊断对于进一步将恶性肿瘤分类为原发性或继发性、霍奇金淋巴瘤或非霍奇金淋巴瘤，或其他如转移性软脑膜播散和胶质瘤是必要的。在淋巴样肿瘤的亚型中治疗方案差异很大，因此准确的组织诊断至关重要。因此，我们报告一例罕见的原发性中枢神经系统髓内霍奇金淋巴瘤，在免疫妥协的情况下无软脑膜疾病，通过圆锥髓质的开放切除活检确诊。
