Stein Moshe, Farrar Nathan, Jones Glenn W, Wilson Lynn D, Fox Lisa, Wong Raimond K, Kuten Abrahem
The Northern Israel Oncology Center, Rambam Medical Center and the Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel.
Cancer J. 2006 Jan-Feb;12(1):55-62. doi: 10.1097/00130404-200601000-00010.
Neurologic involvement in mycosis fungoides is rare. Isolated case reports in the literature suggest the pattern and the natural history for such occurrences, while a literature summary can provide direction on diagnosis and management. Although case series may confirm such information, cohort data are required to establish an overall risk of occurrence and to evaluate possible predictive factors.
We presented a case of central nervous system involvement in mycosis fungoides from Haifa, Israel and tabulated a series of nine cases from Canada. To estimate the risk of neurologic involvement, a cohort of 680 consecutive patients with newly diagnosed mycosis fungoides, of which the nine cases of neurologic involvement emerged during follow up, was analyzed using the Kaplan-Meier method. The actuarial risk of developing neurologic involvement was related to the baseline tumor-node-metastasis-blood classification factors.
The pattern of disease in these 10 additional cases confirms the overall pattern in the approximately 40 patients described in the literature. The main symptoms are fluctuating higher cognitive functions and cranial nerve dysfunction, with fairly rapid clinical onset of symptoms. Most cases of central neurologic involvement with mycosis fungoides emerge within a setting of advanced disease. In patients with newly diagnosed mycosis fungoides, the greatest risk of developing neurologic involvement is within the first several years after diagnosis and is associated with the initial stage of disease. Patients with two or more of the T3-4, N3, M1, and B1 classification factors have a one in six chance of developing central neurologic involvement, while there is about a one in a hundred chance for the corresponding control group.
Neurologic involvement with mycosis fungoides is indeed rare, but it is associated with a more advanced stage at diagnosis and with other visceral disease that can precede it. Although the role of low-dose prophylactic cranial radiation is uncertain, overt neurologic involvement requires urgent palliative treatment.
蕈样肉芽肿累及神经系统较为罕见。文献中的个别病例报告提示了此类情况的发病模式和自然病程，而文献综述可为诊断和治疗提供指导。尽管病例系列研究可证实此类信息，但需要队列数据来确定总体发病风险并评估可能的预测因素。
我们报告了1例来自以色列海法的蕈样肉芽肿累及中枢神经系统的病例，并整理了加拿大的9例病例系列。为评估神经系统受累风险，我们使用Kaplan-Meier方法对680例新诊断的蕈样肉芽肿患者队列进行了分析，其中9例在随访期间出现神经系统受累。发生神经系统受累的精算风险与基线肿瘤-淋巴结-转移-血液分类因素相关。
这10例额外病例的疾病模式证实了文献中描述的约40例患者的总体模式。主要症状为认知功能波动及颅神经功能障碍，症状临床起病较快。大多数蕈样肉芽肿中枢神经系统受累病例出现在疾病晚期。在新诊断的蕈样肉芽肿患者中，发生神经系统受累的最大风险在诊断后的头几年内，且与疾病初期相关。具有T3-4、N3、M1和B1分类因素中两项或更多项的患者发生中枢神经系统受累的几率为六分之一，而相应对照组的几率约为百分之一。
蕈样肉芽肿累及神经系统确实罕见，但与诊断时更晚期的疾病阶段以及可能先于其出现的其他内脏疾病相关。尽管低剂量预防性颅脑放疗的作用尚不确定，但明显的神经系统受累需要紧急姑息治疗。
