Department of Neurosurgery, Ospedale 'Vito Fazzi', Piazza F. Muratore 1, 73100, Lecce, Italy; Vita-Salute San Raffaele University, Milan, Italy.
Department of Neurosurgery, Manchester Centre for Clinical Neurosciences, Salford Royal NHS Foundation Trust, Manchester Academic Health Sciences Centre, University of Manchester, Manchester, UK.
Neurochirurgie. 2021 Apr;67(2):205-209. doi: 10.1016/j.neuchi.2020.11.006. Epub 2020 Dec 1.
The Rosai Dorfman disease (RDD) is a rare and usually benign lymphoproliferative disorder of unknown aetiology, typically characterized by head and neck lymphadenopathies. However, it may occasionally present with primary extra-nodal involvement including the central nervous system. We firstly described the potential malignant behaviour and fatal evolution of primary intracranial RDD.
A 69-year-old woman sought clinical attention for recurrent episodes of headache, fever and malaise associated with bilateral proptosis and left lower limb paresis. The brain MRI revealed bilateral retro-bulbar and fronto-parieto-occipital subdural lesions. The body CT did not show extracranial lesions. A right fronto-parietal craniotomy was performed and a subtotal resection was achieved. The histopathological diagnosis was consistent with RDD. Despite the patient was commenced on high-dose corticosteroids, she developed a worsening respiratory distress syndrome and a rapid systemic disease progression with liver and kidney failure. Given the poor general status, adjuvant medical and radiation therapies were not deemed safe and feasible and the patient died of multi-organ failure a month later.
We documented an exceptional case of primary intracranial RDD with malignant behaviour characterized by rapid systemic disease progression and poor prognosis. Although RDD with intracranial location has usually a benign and self-limiting course with good response to adjuvant treatments, it hides a malignant potential that may lead patients to death.
罗萨达-多夫曼病（RDD）是一种罕见的、通常为良性的淋巴组织增生性疾病，病因不明，通常表现为头颈部淋巴结病。然而，它偶尔也可能表现为原发性结外受累，包括中枢神经系统。我们首次描述了原发性颅内 RDD 的潜在恶性行为和致命演变。
一名 69 岁女性因反复发作的头痛、发热和不适，伴有双侧眼球突出和左下肢无力而寻求临床关注。脑部 MRI 显示双侧眶后和额顶枕部硬脑膜下病变。全身 CT 未显示颅外病变。行右额顶开颅术，行大部分切除术。组织病理学诊断与 RDD 一致。尽管患者开始接受大剂量皮质类固醇治疗，但她出现了进行性加重的呼吸窘迫综合征和全身疾病快速进展，伴有肝肾功能衰竭。鉴于患者一般状况较差，辅助药物和放射治疗被认为是不安全和不可行的，一个月后患者死于多器官衰竭。
我们记录了一例罕见的原发性颅内 RDD 病例，其恶性行为表现为快速的全身疾病进展和预后不良。虽然颅内 RDD 通常具有良性和自限性的病程，对辅助治疗有良好的反应，但它隐藏着恶性潜能，可能导致患者死亡。
