Wong Shun, Lam Wai Y, Wong Wai K, Lee Kam C
Department of Pathology, Princess Margaret Hospital, Kowloon, Hong Kong.
Hum Pathol. 2007 Nov;38(11):1720-3. doi: 10.1016/j.humpath.2007.06.011.
Immunoglobulin (Ig) G4-related systemic disease is a recently characterized entity. The best-known manifestation is pancreatitis. Other systemic involvements are also described. Three cases of this disease with hypophyseal involvement have been reported in the literature, all diagnosed clinically. We herein present the first case of IgG4-related hypophysitis diagnosed histopathologically. The patient is a 77-year-old Chinese man with a pituitary tumor. Histologic examination of the resected tumor showed hypophysitis with features of inflammatory pseudotumor. Clinical review showed history of pancreatitis and cholecystitis 4 years ago. The pancreatic biopsy and gall bladder specimens obtained previously had the same pathologic features of inflammatory pseudotumor. Immunohistochemistry highlighted abundant IgG4-positive plasma cells in all 3 specimens. Serum IgG4 level was also elevated. A diagnosis of IgG4-related systemic disease was confirmed. This is the first case of intracranial inflammatory pseudotumor shown to be associated with IgG4-related systemic disease.
免疫球蛋白（Ig）G4相关系统性疾病是一种最近才被明确的疾病实体。最常见的表现是胰腺炎，也有其他系统性受累的描述。文献中报道了3例该疾病累及垂体的病例，均为临床诊断。我们在此报告首例经组织病理学诊断的IgG4相关性垂体炎病例。患者为一名77岁的中国男性，患有垂体肿瘤。对切除肿瘤的组织学检查显示为具有炎性假瘤特征的垂体炎。临床回顾显示患者4年前有胰腺炎和胆囊炎病史。之前获取的胰腺活检和胆囊标本具有相同的炎性假瘤病理特征。免疫组化显示所有3个标本中均有大量IgG4阳性浆细胞。血清IgG4水平也升高。确诊为IgG4相关系统性疾病。这是首例显示与IgG4相关系统性疾病相关的颅内炎性假瘤病例。
