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[一例起源于视交叉的原发性中枢神经系统淋巴瘤及诊断困难病例]

[A Case of Primary Central Nervous System Lymphoma Originated from Optic Chiasma and Presenting Difficulty in Diagnosis].

作者信息

Gobayashi Yuko, Uryu Yasuhiro, Noda Naoyuki, Sekiguchi Noriaki, Suzuki Koji, Tanaka Yusuke, Hataoka Syunsuke, Tanino Shin, Okada Tomu, Miyahara Kosuke

机构信息

Department of Neurosurgery, National Hospital Organization Yokohama Medical Center.

出版信息

No Shinkei Geka. 2020 Dec;48(12):1165-1170. doi: 10.11477/mf.1436204340.

DOI:10.11477/mf.1436204340
PMID:33353879
Abstract

We report a case of primary central nervous system lymphoma(PCNSL)originating from an optic chiasma, which was difficult to diagnose but was finally diagnosed by biopsy. A 62-year-old immunocompetent man presented with bilateral visual field disturbance, hypopituitarism, and diabetes insipidus;an optic chiasm lesion was detected on MRI. After starting steroid supplementation for adrenal insufficiency, visual field disturbance immediately improved. Since the lesion completely disappeared three months after its onset, it became the follow-up without histological confirmation. Six months after the onset, visual field disturbance progressed, and the lesion recurred. We performed a left optic nerve biopsy to maintain the right visual field, which remained partially. The pathology was PCNSL. We performed postoperative chemoradiotherapy, and the patient showed remission and improvement of the visual field. Isolated PCNSLs arising from optic chiasma are very rare. The diagnosis of optic chiasm lesions is difficult due to their similarity with a variety of inflammatory/autoimmune disease and neoplastic lesions. When a lymphoma is considered to be differentiated, early biopsy should be performed before administering a steroid. The approach and sampling site to prevent the function are also important for biopsy.

摘要

我们报告一例起源于视交叉的原发性中枢神经系统淋巴瘤(PCNSL),该病例诊断困难,但最终通过活检得以确诊。一名62岁免疫功能正常的男性出现双侧视野障碍、垂体功能减退和尿崩症;MRI检查发现视交叉病变。在开始补充类固醇治疗肾上腺功能不全后,视野障碍立即改善。由于病变在发病三个月后完全消失,因此在未进行组织学确认的情况下进行随访。发病六个月后,视野障碍进展,病变复发。为了保留部分右侧视野,我们对左侧视神经进行了活检。病理结果为PCNSL。我们进行了术后放化疗,患者病情缓解,视野得到改善。起源于视交叉的孤立性PCNSL非常罕见。由于视交叉病变与多种炎症/自身免疫性疾病及肿瘤性病变相似,其诊断较为困难。当考虑为淋巴瘤时,应在使用类固醇之前尽早进行活检。活检时防止功能受损的方法和取材部位也很重要。

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