Rosai-Dorfman-Destombes (RDD) disease also known as sinus histiocytosis with massive lymphadenopathy is a rare disease characterized by disorder of histiocytes within lymph nodes or other affected tissues. Extranodal forms of disease are even more uncommon. Only small percent of patients present with intracranial extranodal form of disease. Lesion biopsy and characteristic histopathological findings are crucial for definitive diagnosis. Histological findings typically show emperipolesis of affected histiocytes or hyperplastic lymph node sinuses containing enlarged histiocytes with phagocyted intact lymphocytes with specific immunoassay findings of the characteristic histiocytic non-Langerhans cells. Therapy includes various modalities such as surgery, steroids, radiotherapy, and/or chemotherapy. In case of extranodal intracranial form of disease, resection (single or combined with other treatment) was reported as effective. There are only few cases of extranodal intracranial disease in pediatric population described in the world so far. We aimed to provide brief comprehensive review of published cases referring on pediatric extranodal intracranial RDD.

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儿童结外颅内Rosai-Dorfman-Destombes病：文献综述

Rosai-Dorfman-Destombes（RDD）疾病也称为窦性组织细胞增生伴大量淋巴结肿大，是一种罕见的疾病，其特征在于淋巴结或其他受影响的组织内的组织细胞紊乱。结外型疾病更加罕见。仅一小部分患者出现颅内结外形式的疾病。病变活检和特征性组织病理学发现对于明确诊断至关重要。组织学发现通常表现为受影响的组织细胞或增生性淋巴结窦的经验性增强，其中包含组织细胞增生和吞噬细胞完整淋巴细胞，并具有特征性组织细胞非朗格汉斯细胞的特异性免疫测定结果。治疗包括各种方式，例如手术，类固醇，放射疗法和/或化学疗法。如果是结外颅内疾病，切除（单次或联合其他治疗）报告有效。迄今为止，在世界范围内，在儿科人群中仅有很少的结外颅内疾病病例。我们旨在对涉及小儿结外颅内RDD的已发表病例进行简要的综合回顾。