Rosai-Dorfman disease affecting the maxilla
Thaís Gimenez Miniello1, Juliane Piragine Araujo1, Norberto Nobuo Sugaya1
1Stomatology Department - School of Dentistry - Universidade de São Paulo, São Paulo/SP - Brazil .
Autopsy & Case Reports
|February 18, 2017
Summary
Rosai-Dorfman disease (RDD) is a rare histiocytic disorder that can affect any organ. This case highlights extranodal RDD in the maxilla, successfully treated with conservative management.
Area of Science:
- Histiocytic Disorders
- Pathology
- Immunohistochemistry
Background:
- Rosai-Dorfman disease (RDD) is a rare, non-neoplastic proliferative histiocytic disorder.
- While commonly affecting lymph nodes, RDD can manifest in extranodal sites.
- The clinical presentation and behavior of RDD vary significantly.
Observation:
- A 39-year-old woman presented with an 11-month history of right maxillary pain and mobile teeth.
- Radiographic examination revealed extensive bone resorption and maxillary sinus infiltration.
- Histopathological analysis showed a diffuse inflammatory infiltrate with foamy histiocytes and emperipolesis.
Findings:
- Immunohistochemistry confirmed RDD with CD68 and S-100 positivity.
- Extranodal RDD of the maxilla is an uncommon presentation.
- Conservative treatment with corticotherapy led to a favorable outcome.
Implications:
- This case underscores the importance of considering extranodal RDD in the differential diagnosis of maxillary lesions.
- Early diagnosis and appropriate management are crucial for patients with Rosai-Dorfman disease.
- Long-term follow-up is essential to monitor for recurrence or progression.
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