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Extra Nodal Rosai-Dorfman Disease Originating in the Nasal and Paranasal Complex and Gnathic Bones: A Systematic Analysis of Seven Cases and Review of Literature

Head and Neck Pathology volume 14, pages 442–453 (2020)Cite this article

Abstract

Rosai-Dorfman disease (RDD) is a benign, self-limiting histiocytosis of unknown etiology. The classic form of the condition includes a painless cervical lymphaenopathy accompanied by fever, weight loss and an elevated ESR. Extra nodal RDD (ENRDD) is most frequent in the head and neck. Thirty-eight cases of ENRDD have been described. Seven cases of ENRDD were identified in our pathology biopsy services. The demographic and clinical information was tabulated logically on the basis of age, gender, location and presence or absence of symptoms, treatment and follow-up. Radiographic and histopathological features were also examined. The findings in these cases were correlated with those available from the previously reported cases. Six cases affected women and one case was diagnosed in a male. The age ranged from 22–55 years. Three cases presented as a nasal mass. One of these lesions extended into the paranasal sinuses. One case was located in the maxilla and extended to involve the maxillary sinus. Three cases were diagnosed in the mandible. The maxillary and one mandibular lesion (Case 2) resulted in significant painful irregular bone destruction with a non-healing socket and tooth mobility respectively. One mandibular lesion was asymptomatic (Case 6). The third case affecting the mandible presented as a rapidly expansile mass following a tooth extraction (Case 7). Nasal masses presented with symptoms of obstruction. Nasal masses were excised with no recurrence from up to 2–3 years of follow-up. The mandibular lesions were curetted aggressively. The oral mass in Case 7 was excised synchronously. No recurrence up to 2 years was recorded in Case 2. Follow-up information is not available for Cases 6 and 7. The maxillary lesion was not intervened surgically. The patient has persistent but stable disease for a follow-up period of 2 years. ENRDD is rarely considered in the differential diagnosis in the absence of lymph node involvement. Lesions of ENRDD resemble many other histiocytic and histiocyte-rich lesions of the head and neck. This makes the diagnosis of ENRDD challenging with the potential for under diagnosis or misdiagnosis and delay in treatment.

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Author information

Authors and Affiliations

  1. Department of Integrated Biomedical Sciences, University of Detroit Mercy School of Dentistry, Detroit, MI, 48208, USA

    Junu Ojha

  2. Department of Oral and Maxillofacial Surgery, B204, 1959 NE Pacific Street, Seattle, WA, 98195, USA

    Yeshwant B. Rawal

  3. Department of Pathology, Harvard Medical School, Brigham & Women’s Hospital, 75 Francis Street, Boston, MA, 02115, USA

    Jason L. Hornick

  4. Department of Pathology & Laboratory Medicine, Emory University School of Medicine, 1364 Clifton Rd NE, Atlanta, GA, 30322, USA

    Kelly Magliocca

  5. Oral & Maxillofacial Surgeon, Private Practice, Detroit, MI, 48208, USA

    David R. Montgomery

  6. Head and Neck Pathology, Joint Pathology Center, 606, Stephen Sitter Ave, Silver Spring, MD, 20910, USA

    Robert D. Foss

  7. Department of Pathology, Naval Medical Center Portsmouth, Portsmouth, VA, 23708, USA

    Kevin R. Torske

  8. Oral Pathology Consultants, St. Joseph Mercy-Oakland Hospital, Pontiac, MI, 48341, USA

    Brent Accurso

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  1. Junu Ojha
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Ojha, J., Rawal, Y.B., Hornick, J.L. et al. Extra Nodal Rosai-Dorfman Disease Originating in the Nasal and Paranasal Complex and Gnathic Bones: A Systematic Analysis of Seven Cases and Review of Literature. Head and Neck Pathol 14, 442–453 (2020). https://doi.org/10.1007/s12105-019-01056-8

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