Lymphocytic Hypophysitis Successfully Treated with Stereotactic Radiosurgery: Case Report and Review of the Literature

Sandra Pekic; Vojislav Bogosavljevic; Selcuk Peker; Mirjana Doknic; Dragana Miljic; Marko Stojanovic; Milica Skender-Gazibara; Emilija Manojlovic Gacic; Vera Popovic; Milan Petakov

doi:10.1055/s-0037-1604079

Journal of Neurological Surgery Part A: Central European Neurosurgery, Table of Contents

J Neurol Surg A Cent Eur Neurosurg 2018; 79(01): 077-085
DOI: 10.1055/s-0037-1604079

Case Report

Georg Thieme Verlag KG Stuttgart · New York

Lymphocytic Hypophysitis Successfully Treated with Stereotactic Radiosurgery: Case Report and Review of the Literature

Authors

Sandra Pekic

¹Department of Neuroendocrinology, Clinic for Endocrinology, Diabetes and Metabolic Diseases, University Clinical Center Belgrade, Belgrade, Serbia

²University of Belgrade, School of Medicine, Belgrade, Serbia
Vojislav Bogosavljevic

²University of Belgrade, School of Medicine, Belgrade, Serbia

³Clinic of Neurosurgery, University Clinical Center, Belgrade, Serbia
Selcuk Peker

⁴Department of Neurosurgery, Acibadem University School of Medicine, Istanbul, Turkey
Mirjana Doknic

¹Department of Neuroendocrinology, Clinic for Endocrinology, Diabetes and Metabolic Diseases, University Clinical Center Belgrade, Belgrade, Serbia

²University of Belgrade, School of Medicine, Belgrade, Serbia
Dragana Miljic

¹Department of Neuroendocrinology, Clinic for Endocrinology, Diabetes and Metabolic Diseases, University Clinical Center Belgrade, Belgrade, Serbia

²University of Belgrade, School of Medicine, Belgrade, Serbia
Marko Stojanovic

¹Department of Neuroendocrinology, Clinic for Endocrinology, Diabetes and Metabolic Diseases, University Clinical Center Belgrade, Belgrade, Serbia

²University of Belgrade, School of Medicine, Belgrade, Serbia
Milica Skender-Gazibara

⁵Institute of Pathology, University of Belgrade, School of Medicine, Belgrade, Serbia
Emilija Manojlovic Gacic

⁵Institute of Pathology, University of Belgrade, School of Medicine, Belgrade, Serbia
Vera Popovic

²University of Belgrade, School of Medicine, Belgrade, Serbia
Milan Petakov

¹Department of Neuroendocrinology, Clinic for Endocrinology, Diabetes and Metabolic Diseases, University Clinical Center Belgrade, Belgrade, Serbia

²University of Belgrade, School of Medicine, Belgrade, Serbia

Abstract

Lymphocytic hypophysitis (LH) is an autoimmune inflammatory infiltration of the pituitary gland, usually with a benign evolution. In rare circumstances the inflammatory process may extend beyond the pituitary and infiltrate the surrounding structures. We present a 42-year-old woman affected by an aggressive form of LH with extension to the cavernous sinus causing internal carotid artery occlusion and right sixth cranial nerve palsy. Prednisone therapy caused severe iatrogenic Cushing's syndrome, and the patient underwent transsphenoidal decompression. The histopathology report was consistent with LH. The patient was symptom free for a short period with reappearance of severe headache, diplopia, and hearing loss (middle ear inflammation) 3 months after surgery. Corticosteroids were reintroduced with the addition of azathioprine, but there was no regression of the pituitary mass. The patient was referred for stereotactic radiosurgery (SRS) using Gamma Knife (15 Gy to the margin). After 26 months, azathioprine was stopped, and the dose of prednisone was gradually tapered to 7.5 mg/day. Sellar magnetic resonance imaging showed regression of the pituitary mass. After follow-up for > 3 years after SRS, there was no clinical or radiologic evidence of the disease, but carotid arteries remained occluded. The patient developed secondary hypothyroidism and hypogonadism as consequences of SRS. An aggressive form of LH extending beyond the pituitary gland infiltrating surrounding structures is described. It was successfully treated with SRS after failure of transsphenoidal surgery and combined immunosuppressive therapy (prednisone, azathioprine). The review of the literature presents timely information concerning treatment with azathioprine and SRS of patients with an aggressive form of LH.

Keywords

lymphocytic hypophysitis - prednisone - azathioprine - stereotactic radiosurgery

Full Text

References

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