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[Rosai-Dorfman disease: Case report]
Silvia A Maffia1, María V Peruffo1, Roque Malvaso1
1Hospital de Niños Sor María Ludovica.
Archivos Argentinos De Pediatria
|November 24, 2015
Summary
Rosai-Dorfman disease, a rare condition causing enlarged lymph nodes, typically does not require treatment. This report details a rare case of a 10-month-old infant presenting with a cervical tumor.
Area of Science:
- Pediatric Pathology
- Rare Diseases
- Histiocytic Disorders
Background:
- Rosai-Dorfman disease (RDD), or sinus histiocytosis with massive lymphadenopathy, is a rare, benign histiocytic proliferative disorder.
- Its etiology remains largely unknown, but it is characterized by the proliferation of histiocytes in various tissues, most commonly lymph nodes.
Observation:
- This report focuses on a rare pediatric case of RDD presenting as a distinct clinical challenge.
- A 10-month-old infant was admitted with a rapidly growing left cervical tumor, exhibiting symptoms not typical for the usual presentation of RDD.
Findings:
- The cervical tumor in the infant represented an unusual manifestation of Rosai-Dorfman disease.
- While most RDD cases are self-limiting and require no intervention, this infant's presentation necessitated further investigation and management considerations.
Implications:
- This case highlights the importance of considering RDD in the differential diagnosis of pediatric cervical masses, even with atypical features.
- Understanding rare presentations of Rosai-Dorfman disease is crucial for accurate diagnosis and appropriate patient management in pediatric oncology and pathology.
- Further research into the diverse etiologies and clinical behaviors of RDD may improve diagnostic and therapeutic strategies.
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