Department of Neurosurgery, NIMHANS, Bangalore, India.
Department of Neurosurgery, NIMHANS, Bangalore, India.
J Clin Neurosci. 2024 Sep;127:110758. doi: 10.1016/j.jocn.2024.110758. Epub 2024 Jul 24.
Rosai-Dorfman disease (RDD) is a rare benign proliferative disorder of histiocytes. The study discusses the intracranial RDD approach, its management, and its outcome.
It is a retrospective study performed in a tertiary center, the National Institute of Mental Health and Neuroscience, Bangalore, from January 2010 to December 2022. The biopsy-proven patients of RDD were recruited in the present cohort. Demographic and surgical details were collected from the record section, and radiology was collected from the internal storage system. Follow-up assessments were done clinically and telephonically.
A total of 25 patients matched the criteria. The mean age was 32 ± 13.4 years, with male predominance. We have included only cranial cases (N=25). Among the intracranial lesions, 5/25 (20 %) patients had multicentric lesions. All the lesions were avidly enhancing on contrast, and 16 (64 %) lesions were hypointense on T2. Perilesional edema (T2/Flair hyperintensities in the surrounding white matter) was seen in 12 (48 %) patients. Gross total resection (GTR) was carried out in six (24 %) cases. Sub-total resection was in 14 (56 %), and biopsy was in five cases (20 %). Nineteen patients received adjuvant therapy, either only steroid (40 %), only low-dose radiotherapy (16 %), only Chemotherapy (4 %), or a combination of both. At follow-up,44 % of patients had stable disease,28 % had primary disease or recurrence growth, and regression in 12 % of cases.
We demonstrate that surgical resection is an effective therapy for treating isolated intracranial RDD. Adjuvant therapy is an add-on treatment for skull base locations in multicentric locations or surgically inaccessible locations.
罗萨达-多夫曼病（RDD）是一种罕见的组织细胞良性增生性疾病。本研究探讨颅内 RDD 的治疗方法、管理及预后。
这是一项在班加罗尔的国家心理健康与神经科学研究所进行的回顾性研究，时间为 2010 年 1 月至 2022 年 12 月。本研究纳入了经活检证实的 RDD 患者。从病历部分收集人口统计学和手术细节，从内部存储系统收集影像学资料。通过临床和电话进行随访评估。
共有 25 例患者符合标准。平均年龄为 32 ± 13.4 岁，男性居多。我们仅纳入了颅内病例（N=25）。颅内病变中，5/25（20%）患者有多发性病变。所有病变在对比增强上均呈明显强化，16 个（64%）病变在 T2 上呈低信号。12 例（48%）患者周围白质有瘤周水肿（T2/Flair 周围高信号）。6 例（24%）患者行全切除（GTR），14 例（56%）患者行次全切除，5 例（20%）患者行活检。19 例患者接受辅助治疗，其中 40%仅接受激素治疗，16%仅接受低剂量放疗，4%仅接受化疗，或两者联合治疗。随访时，44%的患者疾病稳定，28%的患者出现原发性疾病或复发生长，12%的患者病情消退。
我们证明手术切除是治疗孤立性颅内 RDD 的有效方法。对于颅底位置或手术无法到达的多灶性位置，辅助治疗是一种附加治疗。
