Department of Integrated Biomedical Sciences, University of Detroit Mercy School of Dentistry, Detroit, MI, 48208, USA.
Department of Oral and Maxillofacial Surgery, B204, 1959 NE Pacific Street, Seattle, WA, 98195, USA.
Head Neck Pathol. 2020 Jun;14(2):442-453. doi: 10.1007/s12105-019-01056-8. Epub 2019 Jul 31.
Rosai-Dorfman disease (RDD) is a benign, self-limiting histiocytosis of unknown etiology. The classic form of the condition includes a painless cervical lymphaenopathy accompanied by fever, weight loss and an elevated ESR. Extra nodal RDD (ENRDD) is most frequent in the head and neck. Thirty-eight cases of ENRDD have been described. Seven cases of ENRDD were identified in our pathology biopsy services. The demographic and clinical information was tabulated logically on the basis of age, gender, location and presence or absence of symptoms, treatment and follow-up. Radiographic and histopathological features were also examined. The findings in these cases were correlated with those available from the previously reported cases. Six cases affected women and one case was diagnosed in a male. The age ranged from 22-55 years. Three cases presented as a nasal mass. One of these lesions extended into the paranasal sinuses. One case was located in the maxilla and extended to involve the maxillary sinus. Three cases were diagnosed in the mandible. The maxillary and one mandibular lesion (Case 2) resulted in significant painful irregular bone destruction with a non-healing socket and tooth mobility respectively. One mandibular lesion was asymptomatic (Case 6). The third case affecting the mandible presented as a rapidly expansile mass following a tooth extraction (Case 7). Nasal masses presented with symptoms of obstruction. Nasal masses were excised with no recurrence from up to 2-3 years of follow-up. The mandibular lesions were curetted aggressively. The oral mass in Case 7 was excised synchronously. No recurrence up to 2 years was recorded in Case 2. Follow-up information is not available for Cases 6 and 7. The maxillary lesion was not intervened surgically. The patient has persistent but stable disease for a follow-up period of 2 years. ENRDD is rarely considered in the differential diagnosis in the absence of lymph node involvement. Lesions of ENRDD resemble many other histiocytic and histiocyte-rich lesions of the head and neck. This makes the diagnosis of ENRDD challenging with the potential for under diagnosis or misdiagnosis and delay in treatment.
罗道尔夫-多夫曼病（RDD）是一种病因不明的良性、自限性组织细胞增生症。该疾病的典型形式包括无痛性颈部淋巴结病，伴有发热、体重减轻和血沉升高。结外罗道尔夫-多夫曼病（ENRDD）最常见于头颈部。已有 38 例 ENRDD 被描述。我们的病理活检服务中发现了 7 例 ENRDD。根据年龄、性别、位置以及是否有症状、治疗和随访情况，对人口统计学和临床信息进行了逻辑表格化。还检查了影像学和组织病理学特征。将这些病例的发现与之前报道的病例进行了比较。6 例女性和 1 例男性。年龄从 22 岁到 55 岁不等。3 例表现为鼻部肿块。其中一个病变延伸到鼻旁窦。1 例位于上颌骨，延伸累及上颌窦。3 例诊断为下颌骨。上颌骨和下颌骨的一个病变（病例 2）分别导致严重的疼痛性不规则骨破坏，无愈合窦腔和牙齿移动。下颌骨的一个病变（病例 6）无症状。第三个累及下颌骨的病例在拔牙后表现为迅速扩张的肿块（病例 7）。鼻部肿块有阻塞症状。鼻部肿块切除后，随访 2-3 年无复发。下颌骨病变经积极刮除。病例 7 的口腔肿块同步切除。病例 2 随访 2 年无复发。病例 6 和 7 无随访信息。上颌骨病变未手术干预。患者在随访期间疾病持续但稳定，随访时间为 2 年。在没有淋巴结受累的情况下，ENRDD 很少被考虑作为鉴别诊断。ENRDD 的病变类似于头颈部的许多其他组织细胞和富含组织细胞的病变。这使得 ENRDD 的诊断具有挑战性，存在诊断不足或误诊以及治疗延迟的潜在风险。
