Xanthomatous hypophysitis.

We present a 69-year-old woman who presented with chronic headaches and was found to have a pituitary mass on MRI, which was biopsied and said to be lymphocytic hypophysitis. The woman was placed on prednisone and followed with routine eye examinations. Two years later, the lesion gradually increased in size and the woman developed a decrease in peripheral vision in the right eye. An MRI showed abutment of the right optic nerve by the mass. A repeat endoscopic transsphenoidal biopsy/resection of the pituitary lesion was performed. Histopathological analysis of the specimen was consistent with diagnosis of xanthomatous hypophysitis (XH). XH is an inflammatory disorder of the pituitary gland characterized by an infiltration of lipid-laden histiocytes, also known as xanthoma cells. The mass was biopsied and a diagnosis of lymphocytic hypophysitis was made. The woman reported improved visual acuity and peripheral vision postoperatively. One year after the second resection, her visual symptoms worsened. Repeat MRI revealed expansion of the residual pituitary tissue. She was referred to the radiation oncology department for external beam radiation therapy and was placed on a maintenance dose of steroids. Since undergoing radiation therapy, her vision has improved slightly and her 3month MRI revealed stable lesion size. This woman illustrates a rare pituitary pathology presented with a literature review of published patients describing xanthomatous hypophysitis. A discussion of the clinical presentation, epidemiology, etiology, diagnosis, histology and treatment is provided.