Masuoka Jun, Sakata Shuji, Maeda Kenji, Sugita Yasuo
Department of Neurosurgery, Saga University faculty of Medicine, Saga 840-8501, Japan.
Surg Neurol. 2008 May;69(5):530-3; discussion 533-4. doi: 10.1016/j.surneu.2007.02.057. Epub 2008 Feb 8.
Except for cases with radiotherapy or phacomatosis, multiple primary brain tumors of different histologic types are rare. The authors report the association of an epidermoid cyst and a primary CNS lymphoma.
A 66-year-old man with a 20-year history of gait disturbance was admitted because of recently worsening symptoms. Magnetic resonance imaging showed an abnormal mass in the posterior fossa. The mass lesion had the appearance of a cystic tumor with a large mural nodule. Gross total removal of the tumor was performed. Histologic examination disclosed the cystic portion to be an epidermoid, whereas the nodular portion exhibited the histologic and immunohistochemical features of a malignant lymphoma. The lymphoma cells were shown to harbor EBV by in situ hybridization.
To our knowledge, this is the first report of the coexistence of an epidermoid cyst and primary CNS lymphoma. When the lesions are adjacent, a definite preoperative diagnosis can be difficult. Epstein-Barr virus-associated CNS lymphoma is rare among the immunologically normal population. The possible pathogenesis for the coexistence of these 2 tumors is discussed.
除放疗或神经皮肤综合征病例外,不同组织学类型的多发性原发性脑肿瘤较为罕见。作者报告了1例表皮样囊肿与原发性中枢神经系统淋巴瘤并存的病例。
一名有20年步态障碍病史的66岁男性因近期症状加重入院。磁共振成像显示后颅窝有一异常肿块。该肿块病变表现为带有大的壁结节的囊性肿瘤。对肿瘤进行了全切除。组织学检查显示囊肿部分为表皮样囊肿,而结节部分表现出恶性淋巴瘤的组织学和免疫组化特征。原位杂交显示淋巴瘤细胞携带EB病毒。
据我们所知,这是表皮样囊肿与原发性中枢神经系统淋巴瘤并存的首例报告。当病变相邻时,术前明确诊断可能困难。在免疫功能正常人群中,爱泼斯坦-巴尔病毒相关的中枢神经系统淋巴瘤罕见。讨论了这两种肿瘤并存的可能发病机制。