Department of Pathology and Neuropathology, Timone Hospital, 264 rue Saint Pierre, 18285 CEDEX 5, Marseille, France.
Ann Hematol. 2014 Jun;93(6):937-40. doi: 10.1007/s00277-013-1994-8. Epub 2013 Dec 24.
Rosai-Dorfman disease of the central nervous system is extremely rare and difficult to diagnose also for pathologists. We describe three unusual cases of meningeal Rosai-Dorfman disease and illustrate the difficulties of preoperative and pathological diagnosis. We retrospectively analyzed three patients who underwent surgery for a suspected meningioma for whom the final diagnosis was Rosai-Dorfman disease of the central nervous system. Pathological initial diagnosis was schwannoma, lymphoplasmacyte-rich meningioma, or inflammatory tumor, but final diagnosis in all cases was Rosai-Dorfman disease. These cases underline the preoperative and pathological difficulties of such diagnosis. Pathologists and physicians should be aware of the occurrence of such rare localization of this disease and should think about this differential diagnosis in lymphocyte-rich meningeal tumors mimicking, clinically and radiologically, a meningioma. Communication of significant previous medical history to pathologists and careful examination of slides with appropriate medical history and the use of S100 antibody in the diagnosis of meningeal tumors mimicking Rosai-Dorfman disease could lower the rate of misdiagnosis.
中枢神经系统 Rosai-Dorfman 病极为罕见，病理学家也难以诊断。我们描述了三例脑膜 Rosai-Dorfman 病的不典型病例，并说明了术前和病理诊断的困难。我们回顾性分析了 3 例因疑似脑膜瘤而行手术的患者，最终诊断为中枢神经系统 Rosai-Dorfman 病。病理最初诊断为神经鞘瘤、富含淋巴浆细胞的脑膜瘤或炎性肿瘤，但所有病例的最终诊断均为 Rosai-Dorfman 病。这些病例突出了这种诊断的术前和病理困难。病理学家和医生应意识到这种疾病罕见部位的发生，并应考虑到这种具有淋巴细胞丰富的脑膜肿瘤在临床上和影像学上类似于脑膜瘤的鉴别诊断。将重要的既往病史告知病理学家，并仔细检查带有适当病史的切片，并在诊断类似于 Rosai-Dorfman 病的脑膜肿瘤时使用 S100 抗体，可降低误诊率。
