Garzón-Recalde Diego Alejandro, Rentería-Castillo Elizabeth, Esparza-Ibarra Carlos César
Instituto Mexicano del Seguro Social. Centro Médico Nacional Siglo XXI, Hospital de Especialidades "Dr. Bernardo Sepúlveda Gutiérrez", Servicio de Medicina Interna. Ciudad de México, México.
Instituto Mexicano del Seguro Social. Hospital General de Zona 2-A "Francisco del Paso y Troncoso", Servicio de Hematología. Ciudad de México, México.
Rev Med Inst Mex Seguro Soc. 2024 Jul 1;62(4):1-6. doi: 10.5281/zenodo.11397215.
Primary central nervous system lymphoma (PCNSL) is an extranodal lymphoid neoplasm that affects the brain, spinal cord, leptomeninges or the vitreous-retina space, without systemic involvement. It represents 3% of CNS tumors predominantly in the context of immunosuppression (HIV infection, post-transplant). The clinical presentation and radiological findings are highly variable, representing a diagnostic challenge especially in immunocompetent patients. Its prognosis is unfavorable despite the high response to initial treatment.
69-year-old man, hospitalized for upper motor neuron syndrome and intracranial hypertension. No abnormal laboratory studies were found and a contrast-enhanced tomography of the head revealed multiple contrast-enhancing lesions with vasogenic edema. Extension studies in search of a primary tumor were negative. MRI demonstrated lesions with characteristics consistent with lymphoma. A PET/CT with 18-fluorodeoxyglucose was performed, which ruled out systemic involvement, and a brain biopsy confirmed PCNSL with histopathologic features of a diffuse large B cell lymphoma. The patient was sent to the hemato-oncology department at a third level hospital for chemotherapy, he completed two cycles of high-dose methotrexate plus rituximab without response and fatal outcome.
PCNSL is an aggressive, low-incidence, extranodal lymphoma, which should be considered in immunocompetent patients over 60 years old with multiple brain lesions, in order to initiate treatment in a timely manner.
原发性中枢神经系统淋巴瘤(PCNSL)是一种结外淋巴样肿瘤,累及脑、脊髓、软脑膜或玻璃体 - 视网膜间隙,无全身受累。它占中枢神经系统肿瘤的3%,主要发生在免疫抑制(HIV感染、移植后)的情况下。其临床表现和影像学表现高度多变,对诊断构成挑战,尤其是在免疫功能正常的患者中。尽管对初始治疗反应良好,但其预后不佳。
一名69岁男性,因上运动神经元综合征和颅内高压入院。实验室检查未发现异常,头部增强CT显示多个强化病灶伴血管源性水肿。寻找原发肿瘤的扩展检查结果为阴性。MRI显示病灶特征与淋巴瘤一致。进行了18氟脱氧葡萄糖PET/CT检查,排除了全身受累,脑活检证实为PCNSL,具有弥漫性大B细胞淋巴瘤的组织病理学特征。患者被送往三级医院血液肿瘤科进行化疗,他完成了两个周期的大剂量甲氨蝶呤加利妥昔单抗治疗,但无反应,最终死亡。
PCNSL是一种侵袭性、低发病率的结外淋巴瘤,对于60岁以上有多个脑病灶的免疫功能正常患者应考虑此病,以便及时开始治疗。