Rosai-Dorfman disease with extranodal involvement

Area of Science:

• Oncology
• Pathology
• Hematology

Background:

• Rosai-Dorfman disease (RDD) is a rare, benign proliferative disorder of histiocytes, typically originating from marrow hematopoietic stem cells.
• While RDD can involve lymph nodes and extranodal sites, intracranial involvement is uncommon, occurring in only about 5% of extranodal cases.

Observation:

• A 53-year-old male presented with significant bilateral cervical lymphadenopathy.
• Imaging revealed intracranial tumors and bone lesions.
• Histopathological examination of cervical lymph nodes and resected intracranial tumor confirmed Rosai-Dorfman disease.

Findings:

• The patient's Rosai-Dorfman disease presented with massive cervical lymphadenopathy, mimicking a malignant neoplasm.
• Despite extensive involvement, the patient demonstrated a favorable clinical course with no significant tumor enlargement post-diagnosis.

Implications:

• This case underscores the importance of considering Rosai-Dorfman disease in the differential diagnosis of cervical lymphadenopathy and intracranial lesions, even when malignancy is suspected.
• Accurate histopathological diagnosis is crucial for appropriate management and to avoid unnecessary aggressive treatments.
• Understanding the diverse clinical manifestations of Rosai-Dorfman disease aids in timely diagnosis and patient management.