[Diagnosis and treatment of extranodal Rosai-Dorfman disease in nasal skull base: a report of 5 cases].
This retrospective five-patient series, including one adolescent, reports prolonged recurrence-free follow-up after gross total endoscopic resection of localized nasal skull-base Rosai-Dorfman disease, while a partially resected lesion required repeat surgery after limited symptomatic response to postoperative methylprednisolone.
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This retrospective five-patient series, including one adolescent, reports prolonged recurrence-free follow-up after gross total endoscopic resection of localized nasal skull-base Rosai-Dorfman disease, while a partially resected lesion required repeat surgery after limited symptomatic response to postoperative methylprednisolone.
Research significance
The reported observations support maximal safe endoscopic resection as a potentially effective local treatment for anatomically resectable nasal skull-base RDD; however, comparative benefit, pediatric applicability, safety, and the added value of postoperative corticosteroids remain unproven.
Source abstract
Objective:To investigate the clinical features and diagnosis and treatment of extranodal Rosai-Dorfman disease（RDD） in nasal skull base. Methods:A retrospective analysis was conducted on 5 cases（2 males and 3 females; aged 13 to 61 years） of extranodal RDD in nasal skull base, diagnosed at Xiangya Hospital, Central South University, from February 2015 to May 2025. The lesions were found in various parts, including the nasal cavity, paranasal sinuses, pterygopalatine fossa, and nasopharynx, manifesting as nasal congestion, headache, visual impairment, and hearing loss, etc. Two patients were admitted to Xiangya Hospital due to postoperative residual lesions from other hospitals and unclear pathology. All patients had nasal endoscopic tumor resection surgery, where one patient underwent partial resection of the lesion due to the large lesion range. Five patients received oral methylprednisolone after surgery. Results:During a follow-up period of 49 to 126 months, none of 4 cases（4/5） who underwent gross total resection experienced a recurrence. The remaining patient （1/5） who underwent partial resection showed no significant symptomatic improvement after postoperative methylprednisolone treatment and requested a second surgery. Following further endoscopic tumor resection, this patient achieved a good postoperative recovery. Conclusion:Extranodal RDD in the nasal skull base is clinically rare and highly susceptible to misdiagnosis. Endoscopic surgical resection of localized nasal skull base RDD has shown favorable therapeutic outcomes, suggesting the importance of achieving maximal safe resection. The specific regimen of postoperative adjuvant steroid therapy still requires further investigation.