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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

Sandra Pekic
1   Department of Neuroendocrinology, Clinic for Endocrinology, Diabetes and Metabolic Diseases, University Clinical Center Belgrade, Belgrade, Serbia
2   University of Belgrade, School of Medicine, Belgrade, Serbia
,
Vojislav Bogosavljevic
2   University of Belgrade, School of Medicine, Belgrade, Serbia
3   Clinic of Neurosurgery, University Clinical Center, Belgrade, Serbia
,
Selcuk Peker
4   Department of Neurosurgery, Acibadem University School of Medicine, Istanbul, Turkey
,
Mirjana Doknic
1   Department of Neuroendocrinology, Clinic for Endocrinology, Diabetes and Metabolic Diseases, University Clinical Center Belgrade, Belgrade, Serbia
2   University of Belgrade, School of Medicine, Belgrade, Serbia
,
Dragana Miljic
1   Department of Neuroendocrinology, Clinic for Endocrinology, Diabetes and Metabolic Diseases, University Clinical Center Belgrade, Belgrade, Serbia
2   University of Belgrade, School of Medicine, Belgrade, Serbia
,
Marko Stojanovic
1   Department of Neuroendocrinology, Clinic for Endocrinology, Diabetes and Metabolic Diseases, University Clinical Center Belgrade, Belgrade, Serbia
2   University of Belgrade, School of Medicine, Belgrade, Serbia
,
Milica Skender-Gazibara
5   Institute of Pathology, University of Belgrade, School of Medicine, Belgrade, Serbia
,
Emilija Manojlovic Gacic
5   Institute of Pathology, University of Belgrade, School of Medicine, Belgrade, Serbia
,
Vera Popovic
2   University of Belgrade, School of Medicine, Belgrade, Serbia
,
Milan Petakov
1   Department of Neuroendocrinology, Clinic for Endocrinology, Diabetes and Metabolic Diseases, University Clinical Center Belgrade, Belgrade, Serbia
2   University of Belgrade, School of Medicine, Belgrade, Serbia
› Author Affiliations
Further Information

Publication History

15 August 2016

28 December 2016

Publication Date:
25 July 2017 (online)

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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
 

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