Journal of Prevention and Treatment for Stomatological Diseases ›› 2020, Vol. 28 ›› Issue (2): 97-101.DOI: 10.12016/j.issn.2096-1456.2020.02.007

• Prevention and Treatment Practice • Previous Articles     Next Articles

Maxillofacial Rosai-Dorfman′s disease: a case report and literature review

CHEN Yi1,XIE Hongliang1(),TANG Jianming1,WENG Rulian1,FENG Li1,ZUO Min2   

  1. 1. Department of Oral and Maxillofacial Surgery, Shenzhen People′s Hospital,Shenzhen 518000,China
    2. Department of Pathology, Shenzhen People′s Hospital,Shenzhen 518000,China
  • Received:2019-06-02 Revised:2019-08-30 Online:2020-02-20 Published:2020-02-25
  • Contact: Hongliang XIE

颌面部Rosai-Dorfman病1例报道及文献复习

陈毅1,谢宏亮1(),汤剑明1,翁汝涟1,冯力1,左敏2   

  1. 1. 深圳市人民医院口腔医学中心口腔颌面外科,广东 深圳(518000)
    2. 深圳市人民医院病理科,广东 深圳(518000)
  • 通讯作者: 谢宏亮
  • 作者简介:陈毅,住院医师,学士,Email: 1534010252@qq.com

Abstract:

Objective To explore the clinical manifestations, histopathological features, diagnosis, treatment and prognosis of Rosai-Dorfman′s disease (RDD) in the maxillofacial region and to review the relevant literature in order to improve the understanding, diagnosis and treatment of oral and maxillofacial RDD. Methods The clinical manifestations, histopathological features, diagnosis, treatment, and prognosis of a patient with RDD in the maxillofacial region admitted to Shenzhen People′s Hospital were analyzed, and the literature was reviewed for analysis. Results The clinical manifestations were palpable masses of 3.5 cm × 2.0 cm × 1.0 cm in the right cheek and 3.0 cm × 2.0 cm × 1.0 cm in the right submaxillary area, with clear boundaries, good mobility, medium and hard textures, respectively, no tenderness, smooth surfaces, and no obvious nodules. On contrast-enhanced and plain CT scans of the maxilla and neck, a diffuse soft tissue shadow was seen in the right maxillofacial region with an unclear boundary and uniform density, and the contrast-enhanced scan also showed moderate and uniform enhancement. The primary diagnosis was right maxillofacial lesions. The tumor was resected surgically. The pathological report was right buccal and right submaxillary extranodal RDD. Under light microscopy, nodular lesions in the fibrous fat tissue were found, which were composed of light and deep staining areas. The light staining areas consisted of patchy, polygonal cells with large volumes and rich cytoplasm, in which lymphocytes and neutrophils could be seen stretching into the movement; the deep staining areas were composed of lymphocytes and plasma cells. IHC: S-100 (+), CD68 (+), CD163 (+), CD1a (-), CD21FDC (+), langerin (-), IgG (+), IgG4 (+). No recurrence was found 11 months after the operation. RDD is a rare, benign and self-limited tissue and cell disease and consists of multiple lesions in the maxillofacial region. Its imaging features are similar to those of lymphoma. Its pathological features are large volumes, rich cytoplasm and phagocytosis of lymphocytes and plasma cells. Generally, RDD only needs to be observed, and individuals with symptoms or the involvement of important organs need to be treated; the first choice for the extranodal type is drug treatment, with radiotherapy administered if the central nervous system is involved. Surgery is recommended if involvement of important organs and compression of the trachea are observed; chemotherapy should be used for diffuse RDD. Most patients with RDD experienced relapse or remission of the disease; a few patients died because of the involvement of important organs or complications. Conclusion The clinical manifestations of maxillofacial RDD vary and lack specific imaging features, and pathological immunohistochemistry is the gold standard for diagnosis. The etiology is not completely clear, the treatment methods are varied, and the prognosis is related to the involved range of the disease.

Key words: Rosai-Dorfman disease, sinus histiocytosis with giant lymphadenopathy, maxillofacial, pthology, emperipolesis, immunohistochemistry

摘要:

目的 探讨颌面部Rosai-Dorfman病(Rosai-Dorfman disease, RDD)的临床表现、组织病理学特点、诊断治疗及预后,并进行相关文献复习,提高对口腔颌面部Rosai-Dorfman病的认识与诊治水平。 方法 对深圳市人民医院收治的1例累及颌面部的RDD的临床表现、组织病理学特点、诊断治疗及预后进行观察,结合文献复习进行分析。结果 该患者临床表现为右颊部可扪及3.5 cm × 2.0 cm × 1.0 cm大小肿物,右颌下区可扪及3.0 cm × 2.0 cm × 1.0 cm大小肿物,边界清晰,活动度好,质地中等偏硬,无压痛,表面较光滑、未扪及明显结节。上颌骨、颈部增强+平扫CT示:右侧颌面部见弥漫性分布的软组织影,边界欠清,密度均匀,增强扫描见中等均匀强化,初步诊断为右侧颌面部病变。对肿物进行手术切除,病理报告为右颊部、右颌下结外型RDD,光镜下见纤维脂肪组织中结节性病灶,由淡染区和深染区组成。淡染区为片状分布的多边形细胞,体积大,胞质丰富,其内可见淋巴细胞及中性粒细胞(伸入运动);深染区由淋巴细胞及浆细胞构成。免疫组化IHC:S-100(+)、CD68(+)、CD163(+)、CD1a(-)、CD21FDC(+)、Langerin(-)、IgG(+)、IgG4(+)。患者术后11个月复查,未见复发。经相关文献复习,RDD是一种良性、具有自限性的组织细胞疾病。颌面部RDD是一种罕见的多发病变,影像学特征与淋巴瘤相似,病理特征为体积大、细胞质丰富的组织细胞吞噬淋巴细胞和浆细胞。RDD一般只需观察,有症状或累及重要器官者需治疗,结外型首选药物治疗;中枢神经系统受累下选择放疗;重要器官受累及压迫气管选择手术方式治疗;对于弥散的RDD选择化疗。RDD绝大多数可自行消退或病情持续迁延,极少数患者累及重要器官或因并发症而死亡。结论 颌面部RDD临床表现多样,无特定的影像学特征,病理免疫组化是诊断的金标准,病因尚未完全明确,治疗方法多样,预后与累及的范围有关。

关键词: Rosai-Dorfman病, 窦组织细胞增生伴巨大淋巴结病, 颌面部, 病理学, 吞噬运动, 免疫组化

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