Takeuchi Y, Hashizume Y, Hoshiyama M, Hirose Y, Takahashi A
Department of Neurology, Nagoya University, School of Medicine.
Rinsho Shinkeigaku. 1995 Feb;35(2):158-63.
A 50-year-old man was admitted to our hospital, because of weakness in his right leg. On admission, he had generalized hyperreflexia and positive Babinski signs bilaterally. After admission, weakness in his left leg and confusion developed. A cerebrospinal fluid examination revealed increases in cell counts and protein but was negative for malignant cells. Blood chemistry was normal except for elevated LDH. A bone marrow biopsy yielded lymphoma cells. Successive T2-weighted cranial MRI showed a progressively expanding high signal area in the right parieto-occipital lobe. His confusional state improved after chemotherapy; however, two months later another cranial CT showed multiple enhanced mass lesions. The patient died 20 months after the onset of illness. Postmortem examination revealed widespread intravascular aggregates of malignant lymphomatous cells in the cerebrum, lungs and kidney, as well as multiple infarcts without atherosclerosis in the cerebrum. These pathological findings are compatible with those of intravascular malignant lymphomatosis. In addition, extravascularly expanding tumor cells formed multiple nodular lesions in the cerebral hemisphere. An immunohistochemical study showed that the tumor cells were positive for B-cell marker L26. Intracranial lymphomatous mass lesion rarely occurs in cases of intravascular malignant lymphomatosis. In the present case, tumor cells were presumably restricted to intravascular spaces, occluded them and resulted in ischemic lesions in the cerebrum in the early phase, but they expanded extravascularly and developed mass lesions in the terminal stages. In conclusion, intravascular malignant lymphomatosis is considered to be phenotypes of malignant lymphoma. This is the first case of intravascular malignant lymphomatosis associated with intracranial lymphomatous mass lesions in Japan.
一名50岁男性因右腿无力入院。入院时，他双侧存在全身性反射亢进和巴宾斯基征阳性。入院后，左腿出现无力并伴有意识模糊。脑脊液检查显示细胞计数和蛋白质增加，但恶性细胞检查为阴性。除乳酸脱氢酶升高外，血液生化指标正常。骨髓活检发现淋巴瘤细胞。连续的头颅T2加权磁共振成像显示右顶枕叶有一个逐渐扩大的高信号区。化疗后他的意识模糊状态有所改善；然而，两个月后另一次头颅CT显示有多个强化的肿块病变。患者在发病20个月后死亡。尸检发现大脑、肺和肾脏中存在广泛的恶性淋巴瘤细胞血管内聚集，以及大脑中多处无动脉粥样硬化的梗死灶。这些病理表现与血管内恶性淋巴瘤相符。此外，血管外扩张的肿瘤细胞在大脑半球形成了多个结节性病变。免疫组织化学研究显示肿瘤细胞B细胞标志物L26呈阳性。颅内淋巴瘤性肿块病变在血管内恶性淋巴瘤病例中很少见。在本病例中，肿瘤细胞可能最初局限于血管内空间，阻塞血管并导致大脑早期出现缺血性病变，但在晚期它们扩展到血管外并形成肿块病变。总之，血管内恶性淋巴瘤被认为是恶性淋巴瘤的一种表型。这是日本首例与颅内淋巴瘤性肿块病变相关的血管内恶性淋巴瘤病例。
