Bobby R. Alford Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, Houston, TX, USA.
Department of Otolaryngology, Texas Children's Hospital, Houston, USA.
Ear Nose Throat J. 2023 Jul;102(7):NP303-NP307. doi: 10.1177/01455613211016704. Epub 2021 May 11.
Rosai-Dorfman disease (RDD) is a rare benign systemic histiocytic proliferation characterized by massive lymph node enlargement and sometimes associated with extranodal involvement. Even though it is considered to be benign, death can occur depending on the extent and location. Our case highlights a primary extranodal site of the right pinna with extension through the Eustachian tube to the subglottis. A previously healthy 15-year-old female presented with 1-year right pinna swelling, slowly enlarging and becoming more bothersome. An incisional biopsy was performed on the ear along with S100 staining yielding a diagnosis. After multidisciplinary case discussion, clofarabine monotherapy and systemic therapy for Langerhans cell histiocytosis has started. Rosai-Dorfman disease can be a general disorder, often affecting the lymph nodes. Unlike a nodal disease, extranodal disease could involve any site on the patient's anatomy. Head and neck lesions are the most common extranodal lesions. Rosai-Dorfman disease is self-limited in more than 20% of the cases with spontaneous regression without intervention; 70% of the patients have noticeable symptoms and vital organ involvement requiring treatments such as surgery, steroids, radiation, and chemotherapy. In our case, the patient had wide involvement and presented without any serious breathing difficulties; we decided to start with monotherapy with chemotherapy and systematic glucocorticoid treatment.
罗萨达-多夫曼病（RDD）是一种罕见的良性全身性组织细胞增生症，其特征为大量淋巴结肿大，有时伴有结外累及。尽管它被认为是良性的，但根据病变的范围和部位，也可能导致死亡。我们的病例突出了一个右耳廓原发性的结外部位，通过咽鼓管延伸至声门下区。一位之前健康的 15 岁女性因右耳廓肿胀 1 年就诊，肿胀逐渐增大且变得更加恼人。对耳部进行了切开活检，并进行了 S100 染色，得出了诊断。经过多学科病例讨论，开始使用克拉屈滨单药治疗和朗格汉斯细胞组织细胞增生症的全身治疗。罗萨达-多夫曼病可以是一种全身性疾病，常累及淋巴结。与结内疾病不同，结外疾病可累及患者解剖结构的任何部位。头颈部病变是最常见的结外病变。罗萨达-多夫曼病有 20%以上的病例可自行缓解，无需干预即可自行消退；70%的患者有明显的症状和重要器官受累，需要治疗，如手术、类固醇、放疗和化疗。在我们的病例中，患者广泛受累，且无明显呼吸困难；我们决定开始采用单药化疗和系统性糖皮质激素治疗。
