Differentiating idiopathic sclerosing orbital inflammation from orbital inflammation secondary to neoplasia may be challenging, as both processes can present similarly. Neoplasms in the orbit may induce inflammation with accompanying fibrosis. Limited sections of histopathological specimens may demonstrate nonspecific inflammation and lead to an inaccurate diagnosis.
The patient discussed is an 86-year-old male who presented with complaints of left eye irritation. His past ocular history was significant for a left central retinal vein occlusion, managed over several years with panretinal photocoagulation and multiple anti-VEGF injections. He also had advanced glaucoma with a superotemporal Ahmed valve tube shunt in the left eye, and his left eye was pseudophakic.
Slit lamp examination revealed an elevated inferotemporal subconjunctival mass. An MRI orbit showed soft tissue enhancement in the temporal aspect of the left orbit between the sclera and the lacrimal gland without evidence of bony involvement. A biopsy of the orbital lesion demonstrated chronic inflammation and subepithelial fibrosis but no sign of malignancy. The patient was started on 40mg oral prednisone with a gradual taper.
Four months later he developed a new subconjunctival lesion adjacent to the initial area of concern, but this time it did not resolve with corticosteroids. Another biopsy of the lesion was performed, and was consistent with an admixture of active and chronic inflammation and fibrosis but negative for malignancy. The patient was started on steroid-sparing anti-inflammatory therapy with mycophenolate motefil.
Despite treatment of his inflammation, his pain continued to worsen and vision declined gradually to no light perception, with complete restriction of the left globe but intact facial cutaneous sensation. This was treated by removal of the Ahmed valve and placement of an amniotic membrane graft. On a postoperative fundus exam, the patient was noted to have inferotemporal elevation of the retina and choroid concerning for choroidal effusion from the hypotony which had not been noted on prior fundus exams, but B-scan ultrasound demonstrated indentation of the inferotemporal aspect of the globe secondary to orbital mass effect.
A subtotal exenteration was ultimately performed given the extensive tumor burden noted intraoperatively after the surgical specimens were sent for histopathologic evaluation.
The final pathologic diagnosis was infiltrating adenocarcinoma,with some features suggestive of mucoepidermoid carcinoma. The tumor t with infiltrative growth pattern lining the internal globe suggestive of a high-grade tumor, T4N0M0.. The patient has finished adjuvant radiation therapy, and neuroimaging at six months post exenteration showed no signs of recurrence
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