Rosai Dorfman Disease: A Rare Cause of Bilateral Orbital Mass Lesions

Rosai Dorfman Disease: A Rare Cause of Bilateral Orbital Mass Lesions:

A 36-year-old male presented with a 12-month history of progressive, painless, bilateral lower eyelid swelling. On examination, visual acuity was 6/9 in both eyes with normal pupil reflexes, unrestrained ocular motility, and no proptosis. Large, nontender, firm, fixed lower eyelid masses with rubbery consistency were identified (Fig. A), while anterior and posterior segment examination was otherwise normal. No palpable lymphadenopathy or hepatosplenomegaly were detected on physical examination. Laboratory investigations were unremarkable except for mild hypergammaglobulinemia (IgG 17.40 g/l). CT and gadolinium-enhanced MRI orbital scans demonstrated bilateral anterior orbital, extraconal, enhancing soft tissue masses with nasal cavity and maxillary sinus involvement, measuring 20 × 20 × 11 mm (Fig. B1–B3, long arrows) without bone destruction. Differential diagnosis included lymphoma, metastatic deposits, and inflammatory disease (sarcoidosis, idiopathic orbital inflammation, and histiocytosis). A full-body CT scan did not demonstrate systemic lymphadenopathy and incisional biopsy of the lesions followed. Histology (Fig. B4) revealed confluent histiocytes with pale, foamy vacuolated cytoplasm, prominent fibrosis and emperipolesis (intact lymphocytes within large histiocytes, Fig. B4, long arrows), consistent with Rosai–Dorfman disease of the orbits and paranasal sinuses (extranodal Rosai–Dorfman disease). Due to lack of symptomatology, a conservative approach was decided with close monitoring. However, further growth of the lesions ensued with ocular discomfort and proptosis, necessitating orbital debulking via transconjunctival approach, along with oral corticosteroids (prednisolone 1 mg/kg/day tapered over 8 weeks). This successfully controlled symptoms and noticeably improved the cosmetic appearance. Orbital Rosai–Dorfman disease is a rare entity, hence no treatment consensus exists. Surgical excision or debulking is usually employed for symptomatic or progressive orbital disease.

A: 

, Clinical photograph demonstrating bilateral lower eyelid masses, B1-B2, MRI STIR orbital scan, coronal section, and B3, T1-weigted sagital MRI showing bilateral anterior extraconal orbital and maxilary sinus masses, B4, Histopathology illustrating emperipolesis and prominent fibrosis

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