A Case of Extranodal Rosai-Dorfman Disease Presenting as an Isolated Mass on the Base of the Tongue in a 57-Year-old Woman

Patient: Female, 57-year-old Final Diagnosis: Rosai-Dorfman disease Symptoms: Cough Medication:— Clinical Procedure: — Specialty: Otolaryngology OBJECTIVE: Rare disease BACKGROUND: Rosai-Dorfman disease (RDD), is a rare, benign, proliferative, histiocytic disorder characterized by persistent massive lymphadenopathy, which mimics malignant tumors. Diagnosis of extranodal RDD without lymphadenopathy is difficult due to its unusual clinical manifestation and lack of typical histopathologic features. Hence, it requires both a high degree of clinical suspicion and careful histopathologic examination. CASE REPORT: A 57-year-old woman presented with an isolated mass on the base of the tongue (BOT) without lymphadenopathy. Laryngoscopic examination revealed a mass on the midline of the BOT. The patient underwent complete surgical excision via suspension laryngoscopy with a CO(2) laser. Based on the histopathologic features, including numerous histiocytic infiltrations with emperipolesis and cytoplasmic expression of S100 and CD68 in histiocytes, the diagnosis was confirmed as extranodal RDD. No further treatment was required, and follow-up evaluation revealed no evidence of recurrence. CONCLUSIONS: Because no ideal therapeutic approach is available for RDD, treatment should be tailored to the clinical manifestations. To prevent airway obstruction and recurrence, surgery is considered an appropriate option in cases of localized RDD arising on the upper respiratory tract. We report an extremely rare case of extranodal RDD without lymphadenopathy in the BOT, and provide a detailed discussion of its clinical and histopathologic features and treatment with a brief review of the relevant literature.