探讨头颈部混合型Rosai-Dorfman病（RDD）的临床表现、组织病理学特征、诊断、治疗及预后。

对1例混合型RDD行苏木精-伊红染色和免疫组织化学染色观察，对其进行随访并结合文献回顾分析。

光镜下见大量淋巴细胞、浆细胞以及胞质苍白或呈嗜酸性淡染的特征性组织细胞，可见明显的淋巴吞噬现象（emperipolesis），免疫组化S-100和CD68染色阳性，CD1a阴性。本病的临床表现不典型，伴有颌面骨破坏吸收，容易误诊。予重组人粒细胞巨噬细胞刺激因子等治疗后病情控制。

头颈部混合型RDD具有一定的组织病理学特征，但临床表现多样，诊断较困难。该病的治疗方法较多，效果不确定，应以保守治疗为主，密切随访。

To evaluate the pathological features, clinical manifestations, diagnosis, treatments and prognosis of Rosai-Dorfman disease (RDD) in head and neck.

HE staining and immunohistochemical analysis were performed in a case with both nodal and extranodal RDD in head and neck, follow-up data was collected and pertinent literatures were reviewed.

Characteristic histiocytes with abundant pale or eosinophilic cytoplasm, and many lymphocytes and plasma cells were observed under light microscopy. A condition termed emperipolesis was found. Immunohistochemical staining was positive for both S-100 and CD68 but negative for CD1a. The clinical manifestation, along with destruction and resorption in maxillofacial bone, was atypical in this case and therefore apt to make a misdiagnosis. The patient shows partial remission on regular clinical and radiological follow-up followed immunotherapy.

Although RDD in head and neck has its specific pathological features, it presents differential clinical presentations and is hard to make an accurate diagnosis. Various treatments were used but none of them proved to be widely effective. Conservative treatment may be suitable for RDD in head and neck, and moreover, a long-term follow-up is indispensable.