Salivary duct carcinoma arising in IgG4-related autoimmune disease of the parotid gland
ABSTRACT Hyper IgG4 disease or IgG4-related sclerosing/autoimmune disease is a multisystem condition characterized histologically by fibrosis, lymphoplasmacytic infiltration, and abundant IgG4 plasma cells associated with raised serum IgG4 levels. We present a case of salivary duct carcinoma of the parotid gland in a background of chronic sclerosing sialadenitis that also involved the submandibular gland with associated regional lymphadenopathy. The serology showed raised total IgG levels of 16.3 g/L (reference range, 6.0-15.0) and raised IgG4 levels of 3.41 g/L (reference range, 0.07-1.70). The salivary duct carcinoma contained areas of dense fibrosis and abundant IgG4-positive plasma cells (>100 per high-power field [hpf]). The adjacent noncarcinomatous areas, submandibular gland, and regional lymph nodes also contained plasma cells immunoreactive to IgG4 with densities higher than 100/hpf. To the best of our knowledge, this case is the first documentation of malignancy occurring in a background of IgG4-related autoimmune disease of the salivary gland.
SourceAvailable from: Soichiro Ibaragi[Show abstract] [Hide abstract]
ABSTRACT: Immunoglobulin G4-related disease (IgG4-RD) is an inflammatory condition associated with elevated serum IgG4 levels and tissue infiltration by IgG4-expressing plasma cells. We present a case of adenoid cystic carcinoma (ACC) of the submandibular gland with possible involvement of IgG4-RD. The patient was a 59-year-old man presenting with a swollen right submandibular gland. Laboratory tests revealed IgG4 levels of 176mg/dl (reference range: 4.8-105). An initial open biopsy for histological diagnosis showed chronic sialadenitis. The region was monitored on an outpatient basis, and finally the right submandibular was totally resected because malignant tumor could not be excluded. Histological examination of the submandibular gland showed an ACC with lymphocytic infiltration containing many IgG4-positive plasma cells in the tumor stroma. We have described a case that indicated a possible involvement of ACC with IgG4-RD. This allows us to speculate that longstanding IgG4-RD may progress to malignancy or infiltration of IgG4-positive plasma cells through the signals of tumor stimuli. Further investigations are required to determine the potential pathogenic mechanism underlying this unique tumor. This case underscores that caution is needed in the diagnosis of masses with high serum IgG4 levels, as the differential diagnosis includes malignancy. Copyright © 2015 Z. Published by Elsevier Ltd.. All rights reserved.01/2015; 68. DOI:10.1016/j.ijscr.2015.01.022
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ABSTRACT: Idiopathic enlargement of salivary glands used to be confusing in diagnosis until immunoglobulin G4 (IgG4)-related sclerosing sialadenitis was proposed as a possible answer. In this case series, we reported the clinical features and management outcomes in 16 patients with IgG4-related sclerosing sialadenitis. We retrospectively studied 16 patients in clinical examination, serology, pathology, and sonography features. All patients were treated by corticosteroids and followed up for at least 3 months. The results of clinical features showed that all of the patients presented persistent, symmetric bilateral swelling of the salivary glands, elevated levels of serum IgG4, and/or IgG4-positive plasmacytes infiltration and tissue fibrosis. The results of all autoantibody tests were negative. The typical sonographic manifestation revealed multiple hypoechoic foci with an irregular netlike diffuse lesion in salivary glands. Most patients showed excellent response to steroids treatment. We conclude that, for patients who present (1) symmetric swelling of bilateral salivary glands for more than 3 months, (2) elevated serum IgG4 level (>135 mg/dL), and (3) enlargement in bilateral salivary glands with multiple hypoechoic areas (irregular netlike appearance) in the sonography, the diagnosis of IgG4-related sclerosing sialadenitis should be considered. A comprehensive understanding of the medical condition and appropriate pathology examination are the key to diagnose. Steroids treatment is effective, and a treatment plan should be set up and followed in the long-term.Journal of Craniofacial Surgery 10/2014; 25(6). DOI:10.1097/SCS.0000000000001016 · 0.68 Impact Factor
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ABSTRACT: IgG4-related dacryoadenitis and sialoadenitis (IgG4-DS) is characterized by serum IgG4 elevation and the infiltration of IgG4-positive plasma cells in glandular tissues. For definitive diagnosis of IgG4-DS, biopsies of local lesions are recommended to exclude Sjögren's syndrome (SS), malignant tumours, and similar disorders. In this study, we examined the diagnostic utility of submandibular gland (SMG) and labial salivary gland (LSG) biopsies in IgG4-DS. Fourteen patients presenting with swelling of the SMG (eight females and six males) underwent both SMG and LSG biopsies. The sensitivity, specificity, and accuracy of SMG biopsies were all 100.0%. In contrast, those of LSG biopsies were 69.2%, 100.0%, and 71.4%, respectively. Thirty-three out of 61 LSG biopsies (54.1%) from all 14 patients were positive for the diagnostic criteria of IgG4-DS (IgG4-positive/IgG-positive plasma cells >0.4). None of the patients experienced complications such as facial nerve palsy, sialocele, or hyposalivation. The IgG4/IgG ratio showed no significant correlation between the LSG and SMG. The final diagnosis was IgG4-DS in 13 patients and marginal zone B-cell lymphoma (MZL) in one. These results suggest that incisional biopsy of the SMG is useful and appropriate for the definitive diagnosis of IgG4-DS, while diagnosis by LSG biopsy alone requires more caution.International Journal of Oral and Maxillofacial Surgery 10/2014; 43(10). DOI:10.1016/j.ijom.2014.06.014 · 1.36 Impact Factor