A rare case of frequently recurring lympocytic hypophysitis: How remission was finally achieved

Autoimmune (lymphocytic) hypophysitis is a rare disease with a tendency to recover spontaneously with or without persisting pituitary hormone deficits. Medical (steroids) or neurourgical (decompressive) therapy is usually performed in symptomatic patients, although controlled studies which determine treatment benefit are lacking. Patients whose disease cannot be controlled by these forms of treatment are rarely discussed in the literature, merely two patients have been reported to have profited from additional stereotactic radiotherapy.

We report the clinical course of a 49 years old woman who presented 7 years ago with bitemporal hemianopia and underwent transsphenoidal surgery due to a pituitary mass lesion. Histological examination then revealed hemorrhagic pituitary tissue. Because of recurring pituitary masses with renewed visual field compromise, the patient was operated upon again two years later with histological evidence of lympocytic hypophysitis. Due to recurring intrasellar and suprasellar mass lesion, the patient underwent two courses of fluocortolon treatment which led to a reduction of sellar masses with invariable recurrence upon termination of corticosteroid therapy. Because the patient had developed deficiency of all pituitary hormone axes, surgical hypophysectomy was then performed. However, even the complete resection of all sellar and suprasellar masses with postoperative evidence of empty sella on MRI scans was unable to prevent the renewed occurrence of intrasellar tissue with extension to the optic chiasm. At that point of time the patient underwent 5 courses of percutaneous radiotherapy at the linear accelerator with single doses of 1.8 Gy. There were no adverse sequelae of radiation treatment and on imaging studies the pituitary mass has shown stability with a tendency to regression for the last three years. We conclude that percutaneous radiotherapy may represent a non-invasive treatment option for patients with lymphocytic hypophysitis, particularly if the disease is recurrent after surgery or resistant to corticosteroid medication.