Kim Min-Kyung, Cho Chang-Ho, Sung Woo-Jung, Ryoo Hun-Mo, Lee Ho-Jun, Youn Sung-Won, Park Kwan-Kyu
Department of Pathology, School of Medicine, Catholic University of Daegu, Daegu, Republic of Korea.
Int J Clin Exp Pathol. 2013 Jul 15;6(8):1643-51. Print 2013.
Anaplastic large cell lymphoma (ALCL) is a rare T-cell lymphoma composed of CD30-positive lymphoid cells. ALCL arising in the dura matter of the brain is even more infrequent, in which only one case has been reported worldwide so far. We report a case of a 30-year-old immunocompetent male with a dura-based mass, radiographically consistent with meningioma. However, the excised mass via a left parieto-occipital craniotomy was composed of large, pleomorphic lymphoid cells to be immunopositive for CD3, CD30, anaplastic lymphoma kinase protein-1 (ALK-1) and epithelial membrane antigen (EMA), and immunonegative for CD20, CD15 and CD68. Multiple ALK gene fusion signals in the ALK locus were detected by fluorescence in situ hybridization (FISH) analysis. The patient was treated with CHOP chemotherapy and intrathecal methotrexate along with brain radiation therapy, which resulted in a complete remission. In an analysis of 25 previously reported primary CNS ALCLs, ALK-1 positivity was shown to be prevalent in younger age, as ALCL occurs outside the brain. Patient less than 23 years, ALK-1 positivity and unifocal tumor may be associated with a better prognosis. However, sex, dural or leptomeningeal involvement, immune status, and tumor necrosis do not appear to have any influence on survival.
间变性大细胞淋巴瘤（ALCL）是一种罕见的由CD30阳性淋巴细胞组成的T细胞淋巴瘤。起源于脑硬膜的ALCL更为罕见，迄今为止全球仅报道过1例。我们报告1例30岁免疫功能正常的男性，其硬膜有占位，影像学表现与脑膜瘤一致。然而，经左顶枕开颅手术切除的肿物由大的多形性淋巴细胞组成，免疫组化显示CD3、CD30、间变性淋巴瘤激酶蛋白-1（ALK-1）和上皮膜抗原（EMA）呈阳性，而CD20、CD15和CD68呈阴性。通过荧光原位杂交（FISH）分析在ALK基因座检测到多个ALK基因融合信号。该患者接受了CHOP化疗、鞘内注射甲氨蝶呤以及脑部放射治疗，最终完全缓解。在对先前报道的25例原发性中枢神经系统ALCL的分析中，发现ALK-1阳性在较年轻患者中更为普遍，如同ALCL发生在脑外一样。年龄小于23岁、ALK-1阳性及单灶性肿瘤可能预后较好。然而，性别、硬脑膜或软脑膜受累情况、免疫状态及肿瘤坏死似乎对生存无任何影响。
