Rosai-Dorfman Disease: The MD Anderson Cancer Center Experience

Sathyanarayanan, Vishwanath; Issa, Amir K; Pinto, Raisa; Fayad, Luis; Loghavi, Sanam; Hagemeister, Fredrick; Westin, Jason R.

doi:10.1016/j.clml.2019.06.013

Cited by 15 publications

(13 citation statements)

References 35 publications

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“…5,6,[13][14][15][16] Consistent with historical literature, we found a preponderance in males and African Americans; 6,17 however, some suggest equal or female preponderance and a higher prevalence in Asian descendants among children. 5,12,14,15 Variability in pathogenesis offers a potential rationale for wide-ranging clinical manifestations seen in RDD. 5 Studies associate numerous viruses including HIV, EBV, parvovirus, CMV, and HSV, 6,[18][19][20][21] neoplasms, and autoimmunity 16,19 with RDD.…”

Section: Discussionsupporting

confidence: 85%

“…In contrast to the 87.3% rate of cervical lymphadenopathy reported by Foucar et al, 4 many studies observed significantly less, around 50%. [14][15][16] Deviation from the traditional description of cervical lymphadenopathy is also found in older patients, who present with smaller foci that range between 1 to 2 cm. 5 This highlights the differences in presenting characteristics between children and adults, who may have different mechanisms of pathogenesis or phenotypical drift with older patients having a higher incidence of extranodal disease.…”

Section: Discussionmentioning

confidence: 99%

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Rosai-Dorfman-Destombes Disease in the Pediatric Head and Neck

Lee

Lovell

Herrmann

2022

Ann Otol Rhinol Laryngol

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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.

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Section: Discussionsupporting

confidence: 85%

Section: Discussionmentioning

confidence: 99%

Rosai-Dorfman-Destombes Disease in the Pediatric Head and Neck

Lee

Lovell

Herrmann

2022

Ann Otol Rhinol Laryngol

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“…After screening, five retrospective studies were included [ 13 – 17 ] that reported 18 F-FDG PET/CT results of RDD (Table 4 ). There were 61 cases in which specific RDD lesions were observed on PET/CT.…”

Section: Resultsmentioning

confidence: 99%

The value of 18F-FDG PET/CT in the systemic evaluation of patients with Rosai–Dorfman disease: a retrospective study and literature review

Wang

Zhu

2023

Orphanet J Rare Dis

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Background Rosai–Dorfman disease (RDD) is a rare form of non-Langerhans cell histiocytic disease. The aim of this study was to review the characteristics of RDD using 18F-FDG PET/CT and determine its efficacy in the disease management. Results A total of 28 RDD patients underwent 33 18F-FDG PET/CT scans for systematic assessment and follow-up. The common involved sites included the lymph nodes (17, 60.7%), upper respiratory tract (11, 39.3%), and skin (9, 32.1%). Five patients had more lesions detected in PET/CT images than in CT and/or MRI, including inapparent nodules (n = 5) and bone destruction (n = 3). After thorough treatment evaluation using PET/CT, the treatment strategies of 14 patients (14/16, 87.5%) were changed. Five patients underwent PET/CT twice during follow-up and the SUVs were significantly decreased (15.3 ± 3.4 vs. 4.4 ± 1.0, p = 0.02), which demonstrated disease improvement. Conclusions 18F-FDG PET/CT contributed to displaying the holistic characteristics of RDD, in particular during initial assessment, treatment strategy adjustment, or efficacy evaluation, and could compensate for some disadvantages of CT and MRI images.

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“…In contrast, a report of 64 patients published in 2020 showed that the median age at diagnosis was 50 years (interquartile range [IQR] 2–79 years), with the median period from onset to diagnosis being 7 months (IQR 0–128 months) ( 6 ). Moreover, a report of 10 patients at a single facility published in 2019 showed that the mean age was 56 years (IQR 20–81 years) ( 13 ). The present patient was diagnosed with RDD at age 74 years.…”

Section: Discussionmentioning

confidence: 99%

Case report: Rosai-Dorfman disease with rare extranodal lesions in the pelvis, heart, liver and skin

Yoshida

Zoshima²,

Hara³

et al. 2023

Front. Oncol.

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Rosai-Dorfman disease (RDD), a rare form of non-Langerhans cell histiocytosis, can involve systemic extranodal lesions. Skin lesions are the most common, whereas intrapelvic, cardiac, and hepatic lesions are infrequent. The present study describes a 74-year-old woman with multiple extranodal lesions in the pelvis, heart, liver, and skin that were successfully treated with glucocorticoid therapy. She had experienced fever and persistent inflammation without cervical lymphadenopathy for several months and 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) showed abnormal FDG uptake in the left cheek; cervical, axillary, inguinal lymph nodes; periatrium; and pelvis. She was diagnosed with RDD based on skin and pelvic biopsies. Although this was an atypical case without bilateral cervical lymphadenopathy, the FDG-PET detection of inflammatory lesions led to selection of suitable biopsy sites, and pathological examination led to a correct diagnosis. Findings in this patient indicate that RDD can present with an atypical distribution of infrequent extranodal lesions, with attention required to prevent a delayed diagnosis.

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Rosai-Dorfman Disease: The MD Anderson Cancer Center Experience

Cited by 15 publications

References 35 publications

Rosai-Dorfman-Destombes Disease in the Pediatric Head and Neck

Rosai-Dorfman-Destombes Disease in the Pediatric Head and Neck

The value of 18F-FDG PET/CT in the systemic evaluation of patients with Rosai–Dorfman disease: a retrospective study and literature review

Case report: Rosai-Dorfman disease with rare extranodal lesions in the pelvis, heart, liver and skin

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