Rosai Dorfman disease: appearances can be deceptive

Area of Science:

• Oncology
• Pathology
• Pediatrics

Background:

• Lymphadenopathy is a common clinical finding in children and young adults.
• Malignancies like lymphoma are often suspected in cases of massive cervical lymphadenopathy.
• Distinguishing benign conditions from malignancies is critical for appropriate patient management.

Observation:

• A 14-year-old boy presented with progressive, painless, symmetrical cervical lymphadenopathy.
• Initial biopsies were inconclusive, leading to further investigation.
• Histopathological examination confirmed Rosai Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy.

Findings:

• Rosai Dorfman disease is a rare, benign condition characterized by sinus histiocytosis and massive lymphadenopathy.
• Histological hallmarks include a fibrous capsule, distended sinuses, plasma cell accumulation, and emperipolesis by large histiocytes.
• Extranodal involvement can occur in sites such as skin, airways, GI tract, and CNS.

Implications:

• RDD is typically benign and self-limiting, requiring accurate diagnosis to avoid unnecessary aggressive treatment.
• The case highlights the importance of repeat lymph node biopsies in persistent lymphadenopathy, especially when initial findings suggest reactive hyperplasia.
• Increased awareness and prompt diagnosis of RDD can prevent misdiagnosis and ensure appropriate clinical surveillance.