전체눈근육 마비로 처음 진단된 원발성 뇌하수체 림프종 1예

Primary Pituitary Lymphoma with Complete Ophthalmoplegia as the 1st Manifestation

Purpose: To report a patient with total ophthalmoplegia as the 1st manifestation due to a primary pituitary lymphoma (PPL). Case summary: A 62-year-old woman visited the ophthalmology clinic with 2-week history of right upper eyelid ptosis and binocular diplopia. Ophthalmologic evaluation showed a 7 mm dilated pupil in the right eye, relative afferent pupillary defect (RAPD), exotropia of 70 prism diopters in primary position and eye movement limitation in adduction (-4), elevation (-4), depression (-4), and abduction (-2) of the right eye. The patient suffered from right facial pain and headache, but no definite other systemic symptoms were found. Brain magnetic resonance imaging showed the pituitary macroadenoma. Trans-sphenoidal surgery was performed and the pathology confirmed an infiltration of the pituitary gland by a diffuse large B-cell lymphoma. A bone marrow biopsy, abdomen, pelvis, chest computed tomography, and total body positron emission tomography–computed tomography confirmed the absence of systemic involvement and thus the diagnosis of PPL was posed. The postoperative course was without complications. The patient underwent treatment with systemic chemotherapy. By 2 months postoperatively, the patient had complete resolution of the ptosis, relative afferent pupillary defect, pupil dilatation, diplopia, exotropia and eye movement limitation, and the best corrected visual acuity was approved. Conclusions: Although primary pituitary lymphoma hardly occurs, ophthalmic symptoms like ophthalmoplegia, diplopia, and ptosis can be the first sign of primary pituitary lymphoma. The patient can show recovery from ophthalmic symptoms and nervous symptoms with appropriate diagnosis and treatment.