2023 Volume 116 Issue 3 Pages 227-234
The superior orbital fissure syndrome (SOFS) is caused by impaired functions of the cranial nerves (III, IV, V1, VI) that enter the orbit through the superior orbital fissure. SOFS associated with malignant lymphoma is rare.
We present the case of an 88-year-old female patient who presented with left-sided ptosis and total ophthalmoplegia, with pain in the left eye. Examination revealed small tumors in the left lower eyelid and a nodule in the left leg. CT of the orbits and the nasal sinus area showed mass lesions in the ethmoid sinuses of both sides and the left sphenoid sinus. We suspected infection or autoimmune disorder and performed endoscopic sinus surgery for the purpose of histopathologic diagnosis and treatment.
Both frozen-section and permanent section diagnosis of the resected specimen revealed features consistent with malignant lymphoma (diffuse large B-cell lymphoma (DLBCL)). Biopsy of the skin nodule in the left leg performed at the same time also revealed features consistent with the diagnosis of DLBCL.
Although the standard treatment for DLBCL is combination chemotherapy (R-CHOP), this patient and her family decided against aggressive chemotherapy in favor of best supportive care, in view of her age and performance status.
A large variety of disease processes are known to underlie the etiopathogenesis of SOFS, such as infections and autoimmune diseases. When the onset of symptoms is slow, a neoplastic cause should be considered.
The greater the degree of disease progression, the higher the risk of vision loss from optic neuropathy. Therefore, the condition should be diagnosed early and treated promptly to avoid blindness.
