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Background <p>Rosai-Dorfman Disease (RDD), or sinus histiocytosis with massive lymphadenopathy, is a rare, non-Langerhans histiocytic disorder. Cervical lymphadenopathy is the most common presentation, especially in adolescents and young adults, often mimicking lymphoma.</p> Objective <p>To present a cytological case series of three young patients with RDD involving cervical lymph nodes, with a focus on cytomorphological diagnosis, diagnostic pitfalls, and literature review.</p> Case Summary <p>Three patients aged 7–10 years presented with unilateral cervical lymphadenopathy. Fine-needle aspiration cytology (FNAC) showed large histiocytes with emperipolesis, a polymorphous inflammatory background, and absence of necrosis—features diagnostic of RDD. One case was histopathologically confirmed. All were managed conservatively with resolution or stability.</p> Conclusion <p>RDD should be considered in the differential diagnosis of lymphadenopathy in young individuals. FNAC, when carefully interpreted, can obviate the need for biopsy and avoid misdiagnosis as lymphoma.</p>

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Rosai-Dorfman Disease Presenting as Massive Cervical Lymphadenopathy in Pediatric Population: A Rare Cytological Case Series with Review of Literature

  • 期刊论文

  • 发表日期：

    2026年3月12日

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  • Beant Kaur,
  • Arti Agarwal,
  • Shikhar Chohan,
  • Tejaswi Gupta,
  • Shruti V. Dixit

摘要

Background

Rosai-Dorfman Disease (RDD), or sinus histiocytosis with massive lymphadenopathy, is a rare, non-Langerhans histiocytic disorder. Cervical lymphadenopathy is the most common presentation, especially in adolescents and young adults, often mimicking lymphoma.

Objective

To present a cytological case series of three young patients with RDD involving cervical lymph nodes, with a focus on cytomorphological diagnosis, diagnostic pitfalls, and literature review.

Case Summary

Three patients aged 7–10 years presented with unilateral cervical lymphadenopathy. Fine-needle aspiration cytology (FNAC) showed large histiocytes with emperipolesis, a polymorphous inflammatory background, and absence of necrosis—features diagnostic of RDD. One case was histopathologically confirmed. All were managed conservatively with resolution or stability.

Conclusion

RDD should be considered in the differential diagnosis of lymphadenopathy in young individuals. FNAC, when carefully interpreted, can obviate the need for biopsy and avoid misdiagnosis as lymphoma.