原发性中枢神经系统恶性淋巴瘤22例临床分析

Clinical analysis of 22 cases with primary central nervous system malignant lymphoma

目的探讨原发性中枢神经系统恶性淋巴瘤(PCNSML)的临床和影像学特点。方法对22例PCNSML患者的临床资料进行回顾性分析。结果本组患者头痛16例、呕吐11例,肢体无力5例、麻木1例。病变位于幕上19例、幕下3例,其中多发病灶5例。CT表现为等或高密度病灶,瘤周轻度水肿。MRI显示T1加权像略低信号9例、等信号5例;T2加权像略高信号11例,等信号3例。CT和MRI强化扫描示均匀明显强化。手术前误诊17例。病理检查均为B细胞型恶性淋巴瘤。随访18例,5例多发病灶仅做活检的患者,均在6个月内死亡;肿瘤全切除加放疗6例,平均生存期27个月;部分切除加γ-射线聚焦治疗4例,平均生存期21个月;部分切除加放疗3例,平均生存期10个月;单纯全切除肿瘤1例,11个月后死于肿瘤复发;2例单纯肿瘤大部切除者在术后5个月和8个月死亡。结论PCNSML临床表现无特异性,术前误诊率高,CT和MRI表现有一定特点,确诊依靠病理检查。综合治疗可延长生存期。

Objective To explore the clinical characteristics of primary central nervous system malignant lymphoma （PCNSML）. Methods The clinical data of 22 cases of PCNSML were analyzed retrospectively. Results The patients in our study mainly presented with headache （16 cases） , vomiting （11 cases） , weakness of extremities （5 cases） and numbness （ 1 case）. The tumors were supratentorial in 19cases, infratentorial in 3 cases and multiple in 5 cases. CT scan showed same density or high-density lesions with surrounding mild edema. Brain MRI demonstrated slight-hypointense lesion of 9 cases and isointense lesion of 5 cases on T1 -weighted imaging. On the T2- weighted imaging, the lesions were slight-hyperintense in 11 cases and isointense in 3 cases. Enhanced scanning showed that the lesions were contrasted evenly and obviously on CT and MRI. Misdiagnosis was given preoperatively in 17 patients. The pathological examination revealed B cell malignant lymphoma in all cases. In 18 cases of followup, 5 patients with multiple lesions who only received stereotactic biopsy all died within 6 months, the mean survival time of 6 patients who were treated with total resection of the tumors and radiotherapy was 27 months, 4 patients treated with partial resection and γ-radiotherapy was 21 months, 4 patients treated with partial resection and radiotherapy was 10 months. The patient who was treated with only total resection of the tumor died in 11 months because of recurrent. Two patients with only partial resection died in 5 and 8 months, respectively. Conclusions There are no specific clinical manifestations of PCNSML and the disease is often misdiagnosed preoperatively. Although certain characteristic performance may be found on CT and MRI scans, the final diagnosis of this disease depends on pathological examination. Comprehensive treatments may prolong the survival time of the patients.