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- Title
Rosai-Dorfman-Destombes Disease in the Pediatric Head and Neck.
- Authors
Lee, Nam K.; Lovell, Mark A.; Herrmann, Brian W.
- Abstract
Background: Rosai-Dorfman-Destombes disease (RDD), or sinus histiocytosis with massive lymphadenopathy, is a rare form of non-Langerhans cells histiocytosis. It has a wide-ranging variability in presentation since first described in 1969 but much of its characteristics in children remain unknown. Methods: A retrospective chart review of children diagnosed with RDD at a tertiary care children's hospital was conducted from 2000 to 2021. Results: Twelve RDD patients were identified, with an average age of 7 years (SD 4.3). Males comprised 58% of the cohort, and African American ethnicity was most common (42%). Nodal RDD was found in 7 patients (58%). Nine patients (75%) presented RDD within the head and neck, 6 of whom had nodal RDD. The most common presentation was cervical lymphadenopathy, which most often involved levels V (67%), II (56%), III (44%), and I (11%), in order of frequency. Recurrence and persistence of disease after initial treatment was common, with 5 (42%) being disease free at the time of the last follow up. Fifty-eight percent (7/12) developed recurrence or had persistent disease and 4 required adjuvant systemic treatment with corticosteroids and/or chemotherapy. One patient succumbed after developing treatment related acute myelodysplastic leukemia (t-AML) from chemotherapy used to treat recurrent RDD. Conclusion: Pediatric RDD presents at a young age and most commonly involving cervical lymphadenopathy. Ongoing surveillance in the setting of persistence or recurrence without clearly defined prognostic risk factors is important.
- Subjects
ADRENOCORTICAL hormones; CHRONIC diseases; LYMPHOBLASTIC leukemia; CANCER chemotherapy; PEDIATRICS; RETROSPECTIVE studies; ACQUISITION of data; TERTIARY care; NON-langerhans-cell histiocytosis; HEAD; LYMPHATIC diseases; RISK assessment; DISEASE relapse; MEDICAL records; DESCRIPTIVE statistics; NECK; AFRICAN Americans; DISEASE risk factors; DISEASE complications
- Publication
Annals of Otology, Rhinology & Laryngology, 2023, Vol 132, Issue 9, p1121
- ISSN
0003-4894
- Publication type
Article
- DOI
10.1177/00034894221130822