腫瘍性および肉芽腫性疾患の治療の進歩

抄録

The aim of this paragraph is to introduce recent advances in the studies of, intravascular lymphoma, lymphomatoid granulomatosis and neurosarcoidosis to neurological clinicians based on reports published in 2016. Any three are very rare, and their neurological manifestations are non–specific, hence they are difficult to diagnose, in early phases. Gliomatosis cerebri has been deleted from the 2016 central nervous system (CNS) World Health Organization (WHO) classification as a distinct entity, rather being considered a growth pattern found in many gliomas.

Regarding Intravascular lymphoma, a comprehensive meta–analysis of 654 cases of intravascular lymphoma patients published between 1957 and 2012 was reported in 2016. In their results, there were no significant differences between Asian and non–Asian populations in clinical features. Lymphomatoid granulomatosis (LYG) sometimes suffer from lesions of the CNS, although the pathogenesis of LYG is still unknown. A review describing pathological feature of CNS–LYG was reported in 2016. There are also case reports of successfully treatment of CNS–LYG with rituximab plus cyclophosphamide, vincristine, and prednisolone therapy. A systematic review and meta–analysis of studies on neurosarcoidosis identifying 29 articles describing 1088 patients diagnosed between 1965 and 2015 and a cross–sectional cohort study including 52 patients were reported in 2016. The recommended treatment of neurosarcoidosis consisits of corticosteroids (first line), methotrexate or azathioprine (second line), or monoclonal antibodies (third line).