Eleven year-old boy presenting a painless right sub-mandibular mass for 1 week. He was otherwise asymptomatic. Diagnosis of adenitis. As there were no modifications after antibiotic treatment, biopsy was performed. F18-FDG PET/CT requested for staging.
Hypermetabolic right cervical mass measuring 3 x 2 cm, SUV max 5.9. Additional small hypermetabolic lymph node, 1.5 x 0.8 cm, SUV max 4.5, station I-B. No other lesion.
Rosai Dorfman disease (RDD) or Sinus Histiocytosis with Massive Lymphadenopathy ( SHML) is a rare disease found mostly in children and young adults. It is included in the non Langerhans cell histiocytosis group. It is characterized by massive, painless bilateral cervical adenopathies. In some patients, such as this patient, disease can be localized to one lymphatic region. Biopsy revealed sinus infiltration by large histiocytes staining CD68 and S-100. As most lesions resolve spontaneously, the patient is follow-up clinically and by ultrasound, without further treatment.
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