Rosai Dorfman disease: a rare entity
DOI:
https://doi.org/10.18203/2349-3291.ijcp20251880Keywords:
Lymph nodes, Histopathology, Non-Langerhan cells, SteroidsAbstract
Rosai Dorfman disease (RDD), a rare benign proliferative disorder of unknown etiology, stems from abnormal histiocyte accumulation within lymph node sinusoids and extra nodal tissues. While uncommon in paediatric population, RDD clinically simulates lymphoproliferative disorders. This report details a 4-year male patient presenting at a tertiary care centre with progressive bilateral cervical and submandibular lymphadenopathy lacking febrile symptoms. Diagnostic confirmation was achieved through excisional biopsy. RDD is an exclusion-based diagnosis which requires multidisciplinary collaboration and heightened clinical suspicion remains essential for paediatricians and oncologists to facilitate timely intervention and management of this rare entity.
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References
Goyal G, Ravindran A, Young JR, Shah MV, Bennani NN, Patnaik MM, et al. Clinicopathological features, treatment approaches, and outcomes in Rosai-Dorfman disease. Haematologica. 2019;105(2):348. DOI: https://doi.org/10.3324/haematol.2019.219626
Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagnost Pathol. 1990;7(1):19-73.
Singh A, Kanaujiya SK. The Rosai–Dorfman Disease: A Differential Diagnosis in Cervical Swelling. Indian J Otolaryngol Head Neck Surg. 2019;71(1):107-12. DOI: https://doi.org/10.1007/s12070-017-1133-2
Destombes P. Adenitis with lipid excess, in children or young adults, seen in the Antilles and in Mali.(4 cases). Bulletin de la Societe de pathologie exotique et de ses filiales. 1965;58(6):1169-75.
Rosai J. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol. 1969;87:63-70.
Bruce-Brand C, Schneider JW, Schubert P. Rosai-Dorfman disease: an overview. J Clin Pathol. 2020;73(11):697-705. DOI: https://doi.org/10.1136/jclinpath-2020-206733
Abraham ZS, Francis Z, Ngaiza AI, Kahinga AA, Bukanu F. Histocytopathological diagnosis of Rosai–Dorfman disease: Case report. Clin Case Rep. 2022;10(2):e05412. DOI: https://doi.org/10.1002/ccr3.5412
Pulsoni A, Anghel G, Falcucci P, Matera R, Pescarmona E, Ribersani M, et al. Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai‐Dorfman disease): report of a case and literature review. Am J Hematol. 2002;69(1):67-71. DOI: https://doi.org/10.1002/ajh.10008