Rosai-Dorfman disease in female patient and atypical age: case report and literature review

Abstract

Introduction: Rosai-Dorfman disease (RDD) is a rare benign histiocytic proliferative disorder, which usually presents with massive, painless, bilateral lymphadenopathy, mainly in the cervical and submandibular regions, and may be accompanied by fever, weight loss, night sweats, leukocytosis and increased erythrocyte sedimentation speed. The prevalence of the disease is highest in men aged up to 20 years. There is no consensus as to the etiology of the disease, although some correlations with human herpesvirus 6 (HHV6), Epstein-Barr virus (EBV) and cytomegalovirus are believed to exist. Objective: To present a rare entity in an atypical occurence and to review the actions taken by the clinical team. Case Report: MASC, female, 55 years old, reported cervical mass on the right side with compression for five months. Before a diagnosis, surgery was performed for the removal of a likely cervical lymph node. The surgical specimen was sent for anatomopathological and immunohistochemical examination, and Rosai-Dorfman disease was diagnosed. One year and five months after the operation, the case recurred. Results: a 55-year-old patient, whose case is rarer even due to the sex and age affected, aside from the unilateral presentation. Prior to the surgery, diagnosis was not possible, as it was confirmed by anatomopathological and immunohistochemical analyses. The patient relapsed and was treated with corticotherapy by the clinical team. Conclusion: To reduce situations of error or delay in diagnosis, it is important to know the main symptoms presented by patients and their possible variations, always listing RDD as a differential diagnosis in cases of cervical adenomegaly.