Rosai–Dorfman disease

Case contributed by Hani Makky Al Salam
Diagnosis certain

Presentation

Uveitis and sinusitis.

Patient Data

Age: 10 years
Gender: Female

Note: This case has been tagged as "legacy" as it no longer meets image preparation and/or other case publication guidelines.

x-ray

Chest x-ray revealed enlarged heart and prominent pulmonary interstitium. Broadening of upper mediastinum.

Multiple lucent areas of tibial shafts.

ct

Ethmoid and maxillary sinuses are opacified with soft tissue density with postcontrast enhancement. Rarefaction of sinus walls and bone septae.

Enlarged bilateral axillary and cervical lymph nodes.

Diffuse interstitial thickening of both lungs. Enlarged mediastinal and bilateral hilar lymph nodes.

Moderately enlarged heart.

Markedly enlarged liver with interstitial edema. Mildly dilated hepatic veins. Mild gallbladder wall edema. Mild splenomegaly.

Multiple enlarged para-aortic lymph nodes.

Mild free ascites at the pelvis.

mri

Opacification of ethmoid and maxillary sinuses.

Gallium scan

Nuclear medicine

Increased uptake of Gallium within the mediastinum. 

Case Discussion

Path proven Rosai-Dorfman disease.

Pathology report:

Lymph node biopsy from right axilla: Sinus histiocytosis with morphological and immunohistochemical features suggestive of Rosai-Dorfman disease.

Immunohistochemistry:

- CD68 (KP1) : Positive

- S100 (Polyclonal) : Positive

- CD30 (Ber-H2) : Negative

- CD1a : Negative

- CD21 : Negative

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