Abstract
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Introduction: Rosai-Dorfman Disease (RDD), also known as Sinus Histiocytosis with Massive Lymphadenopathy (SHML), is a rare histiocytic disorder that classically presents in young adults as asymptomatic head and neck lymphadenopathy with spontaneous resolution in 20-40%. Extranodal involvement occurs in approximately half of cases with sites including kidneys, breasts, skin/subcutaneous tissue, bone, intra-abdominal organs, and the digestive tract. In the setting of multisystem involvement, mortality may range as high as 40%. The goal of this study is to demonstrate the role of whole body 18F-FDG PET/CT in the initial evaluation and assessment of treatment response in RDD.
Methods: We retrospectively reviewed a cohort of histopathological proven RDD patient’s charts and sequential 18F-FDG PET/CT imaging findings with attention to extranodal lesions. The relevant clinical presentation, FDG PET/CT imaging features, including lesion distribution pattern, SUV uptake, and its response to therapy were analyzed.
Results: There were 14 patients with histopathological proven RDD enrolled in our study, with an average age of 47 years old (range 17-72 years old), male:female ratio of 5:9. In this cohort of patients, external nodal FDG-avid lesions were identified in cutaneous/subcutaneous soft tissue (n=11), nasal/sinus (n=6), osseous (n=5), breast (n=2), and kidneys (n=2), pericardium (n=1) and vocal cord (n=1). Single regimen and synergic treatment approaches varied within this cohort, including, corticosteroid, chemotherapy, radiation therapy and conservative treatment. All treated 9 patients within the study have follow-up 18F-FDG PET/CT scans to monitor response to therapy.
Conclusions: Whole body 18F-FDG PET/CT is a valuable imaging modality in the diagnosis, guidance of biopsy, and assessment of treatment response of RDD. It may provide accurate evaluation of disease burden in this rare etiology.
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