Thickened Pituitary Stalk Associated with a Mass in the Sphenoidal Sinus: An Alarm to Suspect Hypophysitis by Immunoglobulin G4?

Abstract

Introduction Hypophysitis is a chronic inflammation of the pituitary gland of complex and still incompletely defined pathogenesis. It belongs to the group of non-hormone-secreting sellar masses, sharing with them comparable clinical presentation and radiographic appearance.

Objectives Describe the case of immunoglobulin G4 (IgG4)-related hypophysitis presenting as a mass in the sphenoid sinus.

Resumed Report A 40-year-old Brazilian man had a diagnosis of central diabetes insipidus since 2001 associated with pituitary insufficiency. Pituitary magnetic resonance imaging revealed a centered pituitary stalk with focal nodular thickening and the presence of heterogeneous materials inside the sphenoid sinus. The patient was treated with testosterone replacement therapy. Laboratory results revealed increased IgG4 serum.

Conclusion IgG4-related hypophysitis should be considered in patients with pituitary insufficiency associated with sellar mass and/or thickened pituitary stalk. IgG4 serum measurement for early diagnosis of IgG4-related hypophysitis should be performed.

Keywords

pituitary diseases - hypopituitarism - sphenoid sinus

• References

• 1 Howlett TA, Levy MJ, Robertson IJ. How reliably can autoimmune hypophysitis be diagnosed without pituitary biopsy. Clin Endocrinol (Oxf) 2010; 73 (1) 18-21
• 2 Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med 2012; 366 (6) 539-551
• 3 Okazaki K, Umehara H. Are classification criteria for IgG4-RD now possible? The concept of IgG4-related disease and proposal of comprehensive diagnostic criteria in Japan. Int J Rheumatol 2012; 2012: 357071
• 4 van der Vliet HJ, Perenboom RM. Multiple pseudotumors in IgG4-associated multifocal systemic fibrosis. Ann Intern Med 2004; 141 (11) 896-897
• 5 Wong S, Lam WY, Wong WK, Lee KC. Hypophysitis presented as inflammatory pseudotumor in immunoglobulin G4-related systemic disease. Hum Pathol 2007; 38 (11) 1720-1723
• 6 Leporati P, Landek-Salgado MA, Lupi I, Chiovato L, Caturegli P. IgG4-related hypophysitis: a new addition to the hypophysitis spectrum. J Clin Endocrinol Metab 2011; 96 (7) 1971-1980
• 7 Yamamoto M, Takahashi H, Ohara M , et al. A case of Mikulicz's disease (IgG4-related plasmacytic disease) complicated by autoimmune hypophysitis. Scand J Rheumatol 2006; 35 (5) 410-411
• 8 Tanabe T, Tsushima K, Yasuo M , et al. IgG4-associated multifocal systemic fibrosis complicating sclerosing sialadenitis, hypophysitis, and retroperitoneal fibrosis, but lacking pancreatic involvement. Intern Med 2006; 45 (21) 1243-1247
• 9 Isaka Y, Yoshioka K, Nishio M , et al. A case of IgG4-related multifocal fibrosclerosis complicated by central diabetes insipidus. Endocr J 2008; 55 (4) 723-728
• 10 Shimatsu A, Oki Y, Fujisawa I, Sano T. Pituitary and stalk lesions (infundibulo-hypophysitis) associated with immunoglobulin G4-related systemic disease: an emerging clinical entity. Endocr J 2009; 56 (9) 1033-1041
• 11 Kotera N, Isogawa A, Uchida L , et al. [Case report: IgG4-related hypophysitis presenting with secondary adrenal insufficiency and central diabetes insipidus in a type 1 diabetes patient]. Nippon Naika Gakkai Zasshi 2011; 100 (4) 1044-1047
• 12 Hattori Y, Tahara S, Ishii Y , et al. A case of IgG4-related hypophysitis without pituitary insufficiency. J Clin Endocrinol Metab 2013; 98 (5) 1808-1811
• 13 Kamisawa T, Funata N, Hayashi Y , et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol 2003; 38 (10) 982-984
• 14 Kotani T, Kato E, Hirai K, Kuma K, Ohtaki S. Immunoglobulin G subclasses of anti-thyroid peroxidase autoantibodies in human autoimmune thyroid diseases. Endocrinol Jpn 1986; 33 (4) 505-510
• 15 Hamano H, Kawa S, Horiuchi A , et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med 2001; 344 (10) 732-738
• 16 Umehara H, Okazaki K, Masaki Y , et al; Research Program for Intractable Disease by Ministry of Health, Labor and Welfare (MHLW) Japan G4 team. A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details. Mod Rheumatol 2012; 22 (1) 1-14
• 17 Landek-Salgado MA, Leporati P, Lupi I, Geis A, Caturegli P. Growth hormone and proopiomelanocortin are targeted by autoantibodies in a patient with biopsy-proven IgG4-related hypophysitis. Pituitary 2012; 15 (3) 412-419