Enfermedad Esclerosante Asociada a Ig4

Pathology International 2009; 59: 670–675

doi:10.1111/j.1440-1827.2009.02425.x

Case Report

Multiple IgG4-related sclerosing lesions in the maxillary sinus, parotid gland and nasal septum
pin_2425 670..675

Mitsuaki Ishida,1 Machiko Hotta,1 Ryoji Kushima,2 Masayuki Shibayama,3 Takeshi Shimizu3 and Hidetoshi Okabe1,2 Department of Clinical Laboratory Medicine, 2Division of DiagnosticPathology and 3Department of Otolaryngology, Head and Neck Surgery, Shiga University of Medical Science, Shiga, Japan
1

IgG4-related sclerosing disease is recognized as a distinct clinicopathological entity. It is well known that this disease can occur in the salivary, lacrimal and pituitary glands, in the head and neck region. The nasal cavity is an extremely rare site of involvement ofIgG4-related sclerosing disease. Herein is reported a case of multiple IgG4-related sclerosing lesions in the maxillary sinus, parotid gland and nasal septum. A 73-year-old Japanese man presented with nasal obstruction and tumors of the right maxillary sinus and parotid gland were detected, after which resections of these tumors were performed. One year after the last surgery, he noted swelling of thenasal septum, and the tumor was resected. These three tumors had similar histopathology, such as conspicuous fibrosclerotic changes with dense lymphoplasmacytic infiltration and occasional obliterative phlebitis. Immunohistochemistry indicated abundant IgG4positive plasma cell infiltration and high ratios of IgG4positive/IgG-positive plasma cells (>70%) in all three lesions. The diagnosis of multipleIgG4-related sclerosing lesions was made. The present case suggests that IgG4related sclerosing lesion can occur in the maxillary sinus and nasal septum, and represents an extension of the spectrum of IgG4-related sclerosing disease. Key words: IgG4, maxillary sinus, nasal septum, parotid gland, tumefactive fibroinflammatory lesion

pancreatitis.2 IgG4-related sclerosing disease occurs in the bileduct (sclerosing cholangitis),3 liver (inflammatory pseudotumor),3 retroperitoneum (retroperitoneal fibrosis)4 and lung (inflammatory pseudotumor and interstitial pneumonia);5,6 and, most recently, some inflammatory aortic aneurysms have also been characterized as IgG4-related sclerosing disease.7,8 In the head and neck region it is well known that IgG4-related sclerosing disease can occur in thesubmandibular gland (Küttner’s tumor),9 lacrimal and parotid glands (Mikulicz’s disease),10 and pituitary gland (lymphoplasmacytic hypophysitis).11 The nasal cavity is an extremely rare site of involvement of IgG4-related sclerosing disease.12 Here we report a case of multiple IgG4-related sclerosing lesions involving the maxillary sinus, parotid gland and nasal septum, and discuss the relationship ofIgG4-related sclerosing disease and inflammatory fibrosclerotic lesions of the head and neck, including tumefactive fibroinflammatory lesion.

CLINICAL SUMMARY A 73-year-old Japanese man presented to the outpatient clinic with a 5 year history of right nasal obstruction. Tumors of the right maxillary sinus and parotid gland were detected, and he was referred to Shiga University of Medical ScienceHospital. There was no significant past history except for asthma and otitis media during childhood. Physical examination indicated a firm tumor measuring 2.5 ¥ 2.0 cm in the right parotid gland. CT showed a tumor with destruction of the surrounding bone tissues in the right maxillary sinus (Fig. 1) and a relatively well-circumscribed tumor in the right parotid gland. No abnormality was detected inthe other organs including the pancreas, submandibular and pituitary glands, and nasal septum on systemic CT. Laboratory tests were normal, and CRP and serum IgG concentration were within

Recently, IgG4-related sclerosing disease has been established as a distinct clinicopathological entity.1 The spectrum of IgG4-related sclerosing disease has expanded1 since this disease entity was first...