Actins ; metabolism ; Diagnosis, Differential ; Granuloma, Plasma Cell ; diagnostic imaging ; drug therapy ; metabolism ; pathology ; surgery ; Head and Neck Neoplasms ; diagnostic imaging ; drug therapy ; metabolism ; pathology ; surgery ; Humans ; Neoplasm Recurrence, Local ; Protein Kinase Inhibitors ; therapeutic use ; Pyrazoles ; therapeutic use ; Pyridines ; therapeutic use ; Tomography, X-Ray Computed ; Vimentin ; metabolism

OBJECTIVETo study the clinicopathologic characteristics of IgG4-related disease in the orbital and periorbital tissue.

METHODSThe clinical manifestations and pathologic features of 17 cases of IgG4-related disease affecting the orbital and periorbital tissue encountered during the period from 2012 to 2013 were studied.

RESULTSThere were 9 male patients and 8 female patients. The age of patients ranged from 11 to 71 years (mean = 48.5 years). The main clinical manifestation was swelling of the eyelids: bilateral in 11 patients and unilateral in 6 patients. The duration of disease ranged from 5 months to 7 years (more than 2 years in 13 cases). Six patients had history of allergic disorders. In addition to orbital/periorbital involvement, the disease also affected salivary gland, lymph node, lung and kidney. The disease relapsed in 9 patients. Amongst the 8 patients treated with steroids, 5 of them achieved complete remission and the remaining 3 patients had partial remission. The IgG4 level of the 17 cases ranged from 1.49 to 14.88 g/L. Histologic examination showed pseudolymphoma pattern in 8 cases, mixed pattern in 8 cases and sclerotic pattern in 1 case. There were various degrees of lymphoplasmacytic infiltrates (with lymphoid follicle formation) and stromal fibrosis. Classical obliterative phlebitis was absent. Tissue eosinophilia was demonstrated in the 17 cases studied. Immunohistochemical study showed the presence of more than 50 IgG4-positive plasma cells per high-power field, with IgG4/IgG plasma cells ratio more than 40%.

CONCLUSIONThere are characteristic clinical manifestations, pathologic features and laboratory findings in orbital and periorbital IgG4-related disease. Thorough understanding is important in arriving at a correct diagnosis.

Adult ; Aged ; Autoimmune Diseases ; immunology ; pathology ; Child ; Female ; Humans ; Immunoglobulin G ; Kidney Diseases ; immunology ; pathology ; Lung Diseases ; immunology ; pathology ; Lymphatic Diseases ; immunology ; pathology ; Male ; Middle Aged ; Orbital Diseases ; immunology ; pathology ; Plasma Cells ; immunology

OBJECTIVETo investigate the clinical and pathologic features of middle ear adenoma (MEA).

METHODSEight cases of MEA were collected from Beijing Tongren Hospital, Capital Medical University between 2004 and 2014, and immunohistochemical staining was performed.

RESULTSThe patients included five women and three men (mean age, 37.5 years; median 37 years; range, 21-51 years). All patients had unilateral lesions. Five MEA occurred on the left side, and three on the right. In seven patients the MEA was primary, and they presented with hearing loss (6 cases), tinnitus (5 cases), sense of ear blockage (3 cases), otalgia (1 case) and facial nerve paralysis (1 case). The remaining patient had recurrent MEA, and presented with otorrhea, aural fullness and tinnitus. Histologically, the tumor cells were arranged in a variety of patterns, including solid sheets, nests, glands, ribbons or trabeculae. Glandular structures were prominent in one case only. Immunohistochemically, the tumor cells were diffusely positive for keratin (8/8) and vimentin (8/8), and focally positive for CK 7(8/8) and CK5/6(8/8). CK7 and CK5/6 were predominantly positive in tumor cells with glandular growth pattern; CK7 was positive in the luminal cells while CK5/6 was positive in the abluminal cells. Both were also expressed focally in scattered tumor cells with non-glandular pattern. The tumor cells were also diffusely positive for synaptophysin(8/8), diffusely but weakly positive for NSE (5/8), and were diffusely or focally positive for chromogranin A (4/8). Both S-100 protein and calponin were negative in all cases. The proliferation rate was low, about 1%-2%. Six cases were followed up for one year and three months to ten years and six months, with an average follow-up period of four years and two months. Two patients developed recurrence, but there were no regional or distant metastases.

CONCLUSIONSDiagnosis of MEA requires pathologic confirmation since the clinical symptoms are non-specific. MEA can show a variety of histologic patterns, and should be distinguished from other space-occupying lesions at this site. Immunohistochemical staining has greatly contributed to the diagnosis and differential diagnosis of MEA. The prognosis of this tumor is good. Patients with MEA require long-term follow-up for recurrences.

Adenoma ; pathology ; Adult ; Beijing ; Diagnosis, Differential ; Ear Neoplasms ; pathology ; Ear, Middle ; pathology ; Female ; Humans ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Young Adult