Heckmann J G, Druschky A, Kern P M, Romstöck J, Huk W, Neundörfer B
Neurologische Universitätsklinik, Erlangen-Nürnberg.
Nervenarzt. 2000 Apr;71(4):305-10. doi: 10.1007/s001150050562.
In recent years, the frequency of primary cerebral lymphoma (PCNSL) has increased, even among immunocompetent patients. In order to treat the disease optimally, early diagnosis is important. We present three patients with atypical courses of this disease and stress the importance of PCNSL in the differential diagnosis for optimal treatment. In a 75-year-old man, a space-occupying, radiopaque, enhancing CNS lesion disappeared completely after biopsy and short steroid therapy. One year later, the tumor recurred on the other side and again regressed after steroid therapy. The first biopsy showed signs of a papillary tumor, so a choroid plexus papilloma was suspected initially. A 57-year-old woman developed progressive bilateral hearing dysfunction. Lymphocytic pleocytosis led to a primary diagnosis of chronic lymphocytic meningitis. During the further course of disease, the patient developed multiple space-occupying cerebral lesions. Stereotactic biopsy revealed PCNSL. Despite combined chemo- and radiotherapy, a relapse occurred. A 49-year-old woman rapidly developed memory and concentration disturbances. Computed tomography revealed diffuse edema in both hemispheres and MRI detected severe, diffuse, white matter lesions. The CSF revealed lymphocytic pleocytosis including plasma cells. Herpes encephalitis and, after lack of clinical improvement and progression of the MRI changes, acute disseminated encephalomyelitis (ADEM) were suspected. Corticosteroid treatment was initiated. After enormous clinical improvement, a clinical relapse occurred and MRI detected bitemporal and singular space-occupying lesions in the corpus callosum and hypothalamus. Finally, open biopsy showed PCNSL. However, on examination of the treatment history of patient 1, the initial diagnosis must be revised; a PCNSL seems most probable. The phenomenon of tumor remission under steroid administration is rare. In patients 2 and 3, atypical clinical signs and symptoms delayed diagnosis of PCNSL. This tumor can mimic diverse neurological diseases and remit following corticosteroid treatment alone. In unclear cerebral disease, biopsy should be performed early for exact diagnosis and optimal treatment.
近年来,原发性中枢神经系统淋巴瘤(PCNSL)的发病率有所上升,即使在免疫功能正常的患者中也是如此。为了实现对该疾病的最佳治疗,早期诊断至关重要。我们介绍了三例患有该疾病非典型病程的患者,并强调了PCNSL在鉴别诊断以实现最佳治疗中的重要性。在一名75岁男性中,一个占位性、不透射线、增强的中枢神经系统病变在活检和短期类固醇治疗后完全消失。一年后,肿瘤在另一侧复发,类固醇治疗后再次消退。首次活检显示有乳头状肿瘤的迹象,因此最初怀疑为脉络丛乳头状瘤。一名57岁女性出现进行性双侧听力功能障碍。淋巴细胞增多导致初步诊断为慢性淋巴细胞性脑膜炎。在疾病的进一步发展过程中,患者出现了多个占位性脑病变。立体定向活检显示为PCNSL。尽管进行了联合化疗和放疗,但仍出现了复发。一名49岁女性迅速出现记忆和注意力障碍。计算机断层扫描显示双侧半球弥漫性水肿,磁共振成像检测到严重的、弥漫性的白质病变。脑脊液显示淋巴细胞增多,包括浆细胞。怀疑为疱疹性脑炎,在临床无改善且磁共振成像改变进展后,怀疑为急性播散性脑脊髓炎(ADEM)。开始使用皮质类固醇治疗。在临床有巨大改善后,出现了临床复发,磁共振成像检测到胼胝体和下丘脑有双侧颞叶和单个占位性病变。最后,开放活检显示为PCNSL。然而,在检查患者1的治疗史时,必须修正初始诊断;PCNSL似乎最有可能。类固醇给药后肿瘤缓解的现象很少见。在患者2和3中,非典型的临床体征和症状延误了PCNSL的诊断。这种肿瘤可以模仿多种神经系统疾病,仅皮质类固醇治疗后即可缓解。在不明原因的脑部疾病中,应尽早进行活检以获得准确诊断和最佳治疗。