Division of Neurosurgery, University of Nebraska Medical Center, Omaha, Nebraska, USA.
World Neurosurg. 2013 Mar-Apr;79(3-4):593.e9-13. doi: 10.1016/j.wneu.2012.07.026. Epub 2012 Aug 7.
Primary central nervous system non-Hodgkin lymphoma (PCNSL) is a malignant lymphoma limited to the cranial-spinal axis in the absence of systemic lymphoma. Historically, PCNSL accounts for fewer than 5% of all cases of primary intracranial neoplasms. PCNSL is rare in immunocompetent young adults. Although the prognosis for PCNSL is poor, approximately 20%-30% percent of cases achieve a cure.
We report two cases of PCNSL originating in the ventricle in otherwise healthy immunocompetent young adults.
A 27-year-old man presented with 10 days of nausea, vomiting, and headache and was found to have a large intraventricular mass emanating from the choroid plexus with resultant hydrocephalus. He underwent placement of external ventricular drain and systemic and intrathecal chemotherapy for cytologically proven PCNSL. A 31-year-old pregnant woman presented with headaches, vision difficulties, and ataxia and was found to have a septum pellucidum mass. She underwent craniotomy and subtotal resection of the mass with subsequent systemic therapy and whole brain radiation for treatment of PCNSL.
To our knowledge, this is the first report of primary CNS lymphoma of the choroid plexus and septum pellucidum in otherwise healthy, immunocompetent young adults.
原发性中枢神经系统非霍奇金淋巴瘤（PCNSL）是一种恶性淋巴瘤，局限于颅脊柱轴，而无全身淋巴瘤。历史上，PCNSL占所有原发性颅内肿瘤的比例不到 5%。PCNSL 在免疫功能正常的年轻成年人中较为罕见。尽管 PCNSL 的预后较差，但约有 20%-30%的病例可以治愈。
我们报告了两例起源于脑室的 PCNSL，患者均为免疫功能正常的健康年轻成年人。
一名 27 岁男性因 10 天恶心、呕吐和头痛就诊，发现脑室中有一个源于脉络丛的大型肿块，导致脑积水。他接受了外部脑室引流以及全身和鞘内化疗，以治疗经细胞学证实的 PCNSL。一名 31 岁孕妇因头痛、视力困难和共济失调就诊，发现有透明隔肿块。她接受了开颅手术和肿块的大部分切除术，随后接受了全身治疗和全脑放疗，以治疗 PCNSL。
据我们所知，这是首例报告发生于健康、免疫功能正常的年轻成年人的脉络丛和透明隔的原发性中枢神经系统淋巴瘤。
