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Rosai-Dorfmann Disease: A Rare Disease Presenting as a Unilateral Neck Swelling

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Indian Journal of Otolaryngology and Head & Neck Surgery Aims and scope Submit manuscript

Abstract

Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder of unknown etiology. Usually it presents with massive painless cervical lymph node enlargement. Histologically, it shows proliferation of distinctive histiocytic cells that demonstrate emperipolesis in the background of a mixed inflammatory infiltrates. Immunohistochemically, the cells are positive for markers such as CD68 and S100. A 14-year-old boy presented with painless right sided cervical lymphadenopathy without any systemic and other ear, nose, and throat manifestations. The biopsy report of the lymph node showed infiltration of sheets of histiocytes showing emperipolesis with areas of fibrosis and hyalinisation. The sinus histiocytes were strongly positive for S-100 protein. RDD must be considered in the differential diagnosis of massive or multiple lymphadenopathies.

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References

  1. Weinstock MS, Patel NA, Smith LP (2018) Pediatric cervical lymphadenopathy. Pediatr Rev 39(9):433–443

    Article  PubMed  Google Scholar 

  2. The neck. In, Das S( ed). A concise textbook of surgery, 3rd edition. Calcutta 2001; 617–41.

  3. Feriante J, Lee RT (2018) Rosai-Dorfman disease: self-resolving unilateral lymphadenopathy and a brief review of literature. Case Rep Oncol Med 16:2018

    Google Scholar 

  4. Destombes P (1965) Adenitis with lipid excess, in children or young adults, seen in the Antilles and in Mali. (4 cases). Bull Soc Pathol Exot Filiales 58(6):1169–1175

    CAS  PubMed  Google Scholar 

  5. Pulsoni A, Anghel G, Falcucci P et al (2002) Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of a case and literature review. Am J Hematol 69:67–71

    Article  PubMed  Google Scholar 

  6. Chen HH, Zhou SH, Wang SQ, Teng XD, Fan J (2012) Factors associated with recurrence and therapeutic strategies for sinonasal Rosai-Dorfman disease. Head Neck 34:1504–1513

    Article  PubMed  Google Scholar 

  7. Dalia S, Sagatys E, Sokol L, Kubal T (2014) Rosai-Dorfman disease: tumor biology, clinical features, pathology, and treatment. Cancer Control 21:322–327

    Article  PubMed  Google Scholar 

  8. Lima FB, Barcelos PS, Constâncio AP, Nogueira CD, Melo-Filho AA (2011) Rosai-Dorfman disease with spontaneous resolution: case report of a child. Rev Bras Hematol Hemoter 33:312–314

    Article  PubMed  PubMed Central  Google Scholar 

  9. Ma YL, Liang ZP, Xu SE, Yang ZH, Peng Y, Sun XQ, Qin G (2015) Rosai-Dorfman disease (RDD) in the paraglottic space: report of a case and review of literature. Int J Clin Exp Pathol 8(10):13532

    PubMed  PubMed Central  Google Scholar 

  10. Delacrétaz F, Meugé-Moraw C, Anwar D, Borisch B, Chave JP (1991) Sinus histiocytosis with massive lymphadenopathy (Rosai Dorfman disease) in an HIV-positive patient. Virchows Arch A Pathol Anat Histopathol 419:251–254. https://doi.org/10.1007/BF01626356

    Article  PubMed  Google Scholar 

  11. Haroche J, Charlotte F, Arnaud L et al (2012) High prevalence of BRAF V600E mutations in Erdheim-Chester disease but not in other non-Langerhans cell histiocytoses. Blood 120:2700–2703. https://doi.org/10.1182/blood-2012-05-430140

    Article  CAS  PubMed  Google Scholar 

  12. Azari-Yaam A, Abdolsalehi MR, Vasei M, Safavi M, Mehdizadeh M (2021) Rosai-dorfman disease: a rare clinicopathological presentation and review of the literature. Head Neck Pathol 15:352–360

    Article  PubMed  Google Scholar 

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  1. Department of Otorhinolaryngology, Dr. B R Ambedkar Medical College and Hospital, Kadugondanahalli, Bangalore, Karnataka, 560045, India

    Stanley John & Tanubha Goel

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  1. Stanley John
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  2. Tanubha Goel
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Correspondence to Tanubha Goel.

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John, S., Goel, T. Rosai-Dorfmann Disease: A Rare Disease Presenting as a Unilateral Neck Swelling. Indian J Otolaryngol Head Neck Surg 76, 1260–1263 (2024). https://doi.org/10.1007/s12070-023-04254-7

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  • DOI: https://doi.org/10.1007/s12070-023-04254-7

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