Recurrent Steroid-Responsive Exophthalmos as a Paraneoplastic Manifestation of Esophageal Adenocarcinoma

Exophthalmos is most often associated with autoimmune thyroid disease, particularly Graves disease. Paraneoplastic syndromes rarely manifest as orbital inflammation, and only a few cases have been described in association with solid tumors. We present a case of a 67-year-old male diagnosed with esophageal cancer. Shortly after diagnosis, he developed rapidly progressive bilateral exophthalmos with ophthalmoplegia. Thyroid function and thyroid-stimulating immunoglobulin were normal, and MRI orbits demonstrated enlargement of extraocular muscles with apical crowding, consistent with inflammatory orbitopathy. Given the absence of thyroid disease, a paraneoplastic process was suspected. The patient was treated with prednisone 60 mg daily, tapered over 5 weeks, resulting in complete resolution of symptoms within 2 weeks. He underwent chemoradiation with interval improvement in the primary tumor. Several months later, disease progression occurred with new mediastinal and iliac lymphadenopathy, coinciding with recurrence of exophthalmos. A second course of corticosteroids again resulted in full remission of the orbital findings. This case highlights paraneoplastic exophthalmos as a rare manifestation of esophageal adenocarcinoma. The temporal association between tumor activity and orbital inflammation, coupled with steroid responsiveness, supports an immune-mediated mechanism. Recognition of this phenomenon is important to avoid misdiagnosis and to guide prompt corticosteroid therapy and oncologic management. In summary, exophthalmos is a very rare paraneoplastic finding. Workup needs to include brain imaging to exclude direct metastasis to the retro-orbital space. Immediate treatment for neoplastic disease is likely to resolve symptoms. High-dose steroids are effective in relieving symptoms.