头颈部淋巴结外Rosai-Dorfman病7例报告并文献复习

目的 分析头颈部淋巴结外Rosai-Dorfman病（RDD）的影像学表现。方法 回顾性分析7例经病理证实的头颈部淋巴结外RDD的影像学资料。结果 7例患者中，发生于眼眶3例，颅内2例，颅外1例，鼻腔1例。7例中除1例眼眶RDD伴颈部淋巴结肿大外，余6例均为单纯淋巴结外病变。3例眼眶病变表现为眼眶浸润性肿块，伴或不伴邻近骨质破坏；2例颅内病变发生于硬脑膜，T2WI呈明显低信号；1例颅外病变表现为帽状腱膜下软组织肿块，侵袭颅骨向颅内生长；1例鼻腔病变患者表现为鼻腔均质肿块。结论 头颈部淋巴结外RDD在不同部位可有不同的影像学表现，不具特征性。当发现颅内硬脑膜肿块、T2WI呈明显低信号、眼眶浸润性肿块以及鼻腔内占位时应考虑到RDD的可能。

Extra-nodal Rosai-Dorfman disease in head and neck: Seven cases report and literature review

Objective To analyze the image findings of extra-nodal Rosai-Dorfman disease (RDD) in the head and neck.Methods The image data of 7 RDD patients in the head and neck confirmed by pathology were analyzed retrospectively.Results The locations included orbit (n=3), intracranial area (n=2), extracranial region (n=1) and nasal cavity (n=1) in 7 RDD patients. Of the 7 RDDs, 6 manifested as pure extranodal disease without lymphadenopathy, except 1 patient in orbit. Three orbital RDDs demonstrated infiltrating mass with or without bone erosion; 2 intracranial RDDs demonstrated dura mater masses with extremely low signal intensity on T2WI; 1 extracranial RDD displayed subgaleal component with transcranial extension; 1 RDD of nasal cavity demonstrated homogenous soft tissue.Conclusion The image findings of extra-nodal RDD in the head and neck vary upon the location and lack typical characteristics. RDD should be taken into account in the presence of extra-axial dura mater mass with markedly low signal intensity on T2WI, infiltrating mass in the orbit and soft tissue in the nasal cavity.