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

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

› Author Affiliations

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

References
1 Caturegli P, Newschaffer C, Olivi A, Pomper MG, Burger PC, Rose NR. Autoimmune hypophysitis. Endocr Rev 2005; 26 (05) 599-614
2 Laws ER, Vance Jr ML, Jane Jr JA. Hypophysitis. Pituitary 2006; 9 (04) 331-333
3 Caturegli P, Lupi I, Landek-Salgado M, Kimura H, Rose NR. Pituitary autoimmunity: 30 years later. Autoimmun Rev 2008; 7 (08) 631-637
4 Carmichael JD. Update on the diagnosis and management of hypophysitis. Curr Opin Endocrinol Diabetes Obes 2012; 19 (04) 314-321
5 Glezer A, Bronstein MD. Pituitary autoimmune disease: nuances in clinical presentation. Endocrine 2012; 42 (01) 74-79
6 Falorni A, Minarelli V, Bartoloni E, Alunno A, Gerli R. Diagnosis and classification of autoimmune hypophysitis. Autoimmun Rev 2014; 13 (4-5): 412-416
7 Takahashi Y. Autoimmune hypophysitis: new developments. Handb Clin Neurol 2014; 124: 417-422
8 Furuoka H. Hypophysitis. Endocrinol Metab Clin North Am 2015; 44 (01) 143-149
9 Caturegli P, Iwama S. From Japan with love: another tessera in the hypophysitis mosaic. J Clin Endocrinol Metab 2013; 98 (05) 1865-1868
10 Cheung CC, Ezzat S, Smyth HS, Asa SL. The spectrum and significance of primary hypophysitis. J Clin Endocrinol Metab 2001; 86 (03) 1048-1053
11 Faje A. Immunotherapy and hypophysitis: clinical presentation, treatment, and biologic insights. Pituitary 2016; 19 (01) 82-92
12 Shi J, Zhang JM, Wu Q, Chen G, Zhang H, Bo WL. Granulomatous hypophysitis: two case reports and literature review. J Zhejiang Univ Sci B 2009; 10 (07) 552-558
13 Caputo C, Bazargan A, McKelvie PA, Sutherland T, Su CS, Inder WJ. Hypophysitis due to IgG4-related disease responding to treatment with azathioprine: an alternative to corticosteroid therapy. Pituitary 2014; 17 (03) 251-256
14 Gutenberg A, Hans V, Puchner MJ. , et al. Primary hypophysitis: clinical-pathological correlations. Eur J Endocrinol 2006; 155 (01) 101-107
15 Karaca Z, Kelestimur F. Pregnancy and other pituitary disorders (including GH deficiency). Best Pract Res Clin Endocrinol Metab 2011; 25 (06) 897-910
16 Sautner D, Saeger W, Lüdecke DK, Jansen V, Puchner MJ. Hypophysitis in surgical and autoptical specimens. Acta Neuropathol 1995; 90 (06) 637-644
17 Goudie RB, Pinkerton PH. Anterior hypophysitis and Hashimoto's disease in a young woman. J Pathol Bacteriol 1962; 83: 584-585
18 Pestell RG, Best JD, Alford FP. Lymphocytic hypophysitis. The clinical spectrum of the disorder and evidence for an autoimmune pathogenesis. Clin Endocrinol (Oxf) 1990; 33 (04) 457-466
19 Nishiki M, Murakami Y, Ozawa Y, Kato Y. Serum antibodies to human pituitary membrane antigens in patients with autoimmune lymphocytic hypophysitis and infundibuloneurohypophysitis. Clin Endocrinol (Oxf) 2001; 54 (03) 327-333
20 De Bellis A, Pane E, Bellastella G. , et al; Italian Autoimmune Hypophysitis Network Study. Detection of antipituitary and antihypothalamus antibodies to investigate the role of pituitary or hypothalamic autoimmunity in patients with selective idiopathic hypopituitarism. Clin Endocrinol (Oxf) 2011; 75 (03) 361-366
21 Lupi I, Manetti L, Raffaelli V. , et al. Pituitary autoimmunity is associated with hypopituitarism in patients with primary empty sella. J Endocrinol Invest 2011; 34 (08) e240-e244
22 Ward L, Paquette J, Seidman E. , et al. Severe autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy in an adolescent girl with a novel AIRE mutation: response to immunosuppressive therapy. J Clin Endocrinol Metab 1999; 84 (03) 844-852
23 Tubridy N, Saunders D, Thom M. , et al. Infundibulohypophysitis in a man presenting with diabetes insipidus and cavernous sinus involvement. J Neurol Neurosurg Psychiatry 2001; 71 (06) 798-801
24 Lecube A, Francisco G, Rodríguez D. , et al. Lymphocytic hypophysitis successfully treated with azathioprine: first case report. J Neurol Neurosurg Psychiatry 2003; 74 (11) 1581-1583
25 Curtò L, Torre ML, Cotta OR. , et al. Lymphocytic hypophysitis: differential diagnosis and effects of high-dose pulse steroids, followed by azathioprine, on the pituitary mass and endocrine abnormalities—report of a case and literature review. Sci World J 2010; 10: 126-134
26 Lu Z, Yang G, Ba J, Dou J, Mu Y, Lu J. Spontaneous pregnancy in a patient with a relapse of lymphocytic hypophysitis successfully treated with azathioprine and glucocorticoids. Endocrine 2011; 39 (03) 296-297
27 Papanastasiou L, Pappa T, Tsiavos V. , et al. Azathioprine as an alternative treatment in primary hypophysitis. Pituitary 2011; 14 (01) 16-22
28 Yang GQ, Lu ZH, Gu WJ. , et al. Recurrent autoimmune hypophysitis successfully treated with glucocorticoids plus azathioprine: a report of three cases. Endocr J 2011; 58 (08) 675-683
29 Schreckinger M, Francis T, Rajah G, Jagannathan J, Guthikonda M, Mittal S. Novel strategy to treat a case of recurrent lymphocytic hypophysitis using rituximab. J Neurosurg 2012; 116 (06) 1318-1323
30 Bianchi A, Mormando M, Doglietto F. , et al. Hypothalamitis: a diagnostic and therapeutic challenge. Pituitary 2014; 17 (03) 197-202
31 Selch MT, DeSalles AA, Kelly DF. , et al. Stereotactic radiotherapy for the treatment of lymphocytic hypophysitis. Report of two cases. J Neurosurg 2003; 99 (03) 591-596
32 Leung GK, Lopes MB, Thorner MO, Vance ML, Laws Jr ER. Primary hypophysitis: a single-center experience in 16 cases. J Neurosurg 2004; 101 (02) 262-271
33 Ray DK, Yen CP, Vance ML, Laws ER, Lopes B, Sheehan JP. Gamma knife surgery for lymphocytic hypophysitis. J Neurosurg 2010; 112 (01) 118-121
34 Kartal I, Yarman S, Tanakol R, Bilgic B. Lymphocytic panhypophysitis in a young man with involvement of the cavernous sinus and clivus. Pituitary 2007; 10 (01) 75-80
35 Gellner V, Kurschel S, Scarpatetti M, Mokry M. Lymphocytic hypophysitis in the pediatric population. Childs Nerv Syst 2008; 24 (07) 785-792
36 Yamagami K, Yoshioka K, Sakai H. , et al. Treatment of lymphocytic hypophysitis by high-dose methylprednisolone pulse therapy. Intern Med 2003; 42 (02) 168-173
37 Minniti G, Osti MF, Niyazi M. Target delineation and optimal radiosurgical dose for pituitary tumors. Radiat Oncol 2016; 11 (01) 135
38 Lanciano R, Fowble B, Sergott RC. , et al. The results of radiotherapy for orbital pseudotumor. Int J Radiat Oncol Biol Phys 1990; 18 (02) 407-411
39 Sheehan JP, Niranjan A, Sheehan JM. , et al. Stereotactic radiosurgery for pituitary adenomas: an intermediate review of its safety, efficacy, and role in the neurosurgical treatment armamentarium. J Neurosurg 2005; 102 (04) 678-691