Polymorphous low-grade adenocarcinoma of the nasal fossa.
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An unusual case of a T4N2CMx polymorphous low grade adenocarcinoma located in the nasal fossae and extending to the pterygoid area is presented. The primary tumor was excised through a Lefort I maxillotomy and the neck was managed with a supraomohyoid neck dissection. Adjuntive postoperative radiotherapy was also administered to the patient. (+info)
The role of DNA ploidy and Ki-67 in the grading of mucoepidermoid carcinomas.
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BACKGROUND: The grading of mucoepidermoid carcinomas (MEC) is based on subjective microscopic evaluation of the prevalence of cell types as well as features of atypia and aggressiveness. Our study was aimed at evaluating the role of high-resolution DNA flow cytometry and Ki-67 expression in the grading of MEC. MATERIALS AND METHODS: Fifty-five cases of intraoral and major salivary gland tumours, diagnosed as MEC, were retrieved and the grading system proposed by Brandwein et al. applied. RESULTS: Forty-nine per cent of our sample was graded as high, 35% as intermediate and 16% as low. Eighty-nine per cent of the high-grade MEC showed aneuploid DNA cell populations, while 88% of the diploid tumours were graded as intermediate or low. The mean Ki-67 positivity was significantly different between the high and intermediate grade tumours and between the aneuploid and diploid tumours. CONCLUSION: This study showed that high-resolution DNA flow cytometry of archival paraffin-embedded tissue is accurate in the grading of MEC and can be used with Ki-67 expression as an additional diagnostic tool. (+info)
Rituximab treatment in patients with primary Sjogren's syndrome: an open-label phase II study.
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OBJECTIVE: To investigate the safety and efficacy of B cell depletion treatment of patients with active primary Sjogren's syndrome of short duration (early primary SS) and patients with primary SS and mucosa-associated lymphoid tissue (MALT)-type lymphoma (MALT/primary SS). METHODS: Fifteen patients with primary SS were included in this phase II trial. Inclusion criteria for the early primary SS group were B cell hyperactivity (IgG >15 gm/liter), presence of autoantibodies (IgM rheumatoid factor, anti-SSA/SSB), and short disease duration (<4 years). Inclusion criteria for the MALT/primary SS group were primary SS and an associated MALT-type lymphoma (Ann Arbor stage IE) localized in the parotid gland. Patients were treated with 4 infusions of rituximab (375 mg/m2) given weekly after pretreatment with prednisone (25 mg) and clemastine. Patients were evaluated, using immunologic, salivary/lacrimal function, and subjective parameters, at baseline and at 5 and 12 weeks after the first infusion. RESULTS: Significant improvement of subjective symptoms and an increase in salivary gland function was observed in patients with residual salivary gland function. Immunologic analysis showed a rapid decrease of peripheral B cells and stable levels of IgG. Human anti-chimeric antibodies (HACAs) developed in 4 of 15 patients (27%), all with early primary SS. Three of these patients developed a serum sickness-like disorder. Of the 7 patients with MALT/primary SS, complete remission was achieved in 3, and disease was stable in 3 and progressive in 1. CONCLUSION: Findings of this phase II study suggest that rituximab is effective in the treatment of primary SS. The high incidence of HACAs and associated side effects observed in this study needs further evaluation. (+info)
Increased acinar damage of salivary glands of patients with Sjogren's syndrome is paralleled by simultaneous imbalance of matrix metalloproteinase 3/tissue inhibitor of metalloproteinases 1 and matrix metalloproteinase 9/tissue inhibitor of metalloproteinases 1 ratios.
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OBJECTIVE: Previous findings in labial salivary glands (LSGs) from patients with Sjogren's syndrome (SS) suggest that increased activity and expression of matrix metalloproteinase 9 (MMP-9) and MMP-3 trigger the destruction of acinar structures in these glands. Tissue inhibitors of matrix metalloproteinases (TIMPs) tightly control MMP activity, and TIMP expression is an important modulator of effects attributed to MMPs. This study was undertaken to investigate the correlation between the balance of MMPs/TIMPs in the LSGs of SS patients and the degree of inflammatory infiltration and acinar structure integrity. METHODS: Three groups of SS patients classified according to focus score and residual tissue were studied. The expression of MMP-2, MMP-3, MMP-9, TIMP-1, and TIMP-2 was examined at the messenger RNA and protein levels. The ratio of MMP/TIMP expression (R value) was calculated. Focus score and acinar structure were evaluated by histologic analysis. RESULTS: In SS patients the MMP-3/TIMP-1 ratio was higher than 1 and the MMP-9/TIMP-1 ratio was much higher than 1 whereas the MMP-2/TIMP-2 ratio nearly equaled 1, suggesting elevated proteolytic activity due mainly to MMP-9. R values were independent of the focus score of inflammatory cells, but correlated well with the dramatic changes observed in morphologic integrity of acini, as revealed mainly by the lack of nuclear polarity. Acinar changes were more evident when R values for both MMP-9/TIMP-1 and MMP-3/TIMP-1 were higher. CONCLUSION: This study provides evidence that an altered balance between MMPs and their inhibitors is associated with acinar damage. Since salivary gland acinar cells express both MMPs and TIMPs, these cells may play an important role in extracellular matrix destruction and in the LSG pathophysiology in SS. (+info)
Immunohistochemical evaluation of p27 (kip1) in pleomorphic adenomas and adenoid cystic carcinomas of the minor salivary glands.
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BACKGROUND: p27(kip1), a universal cyclin-dependent kinase inhibitor, is a useful marker for predicting clinical aggressiveness with various human tumors. In this study, p27 expression was investigated in pleomorphic adenomas (PAs) and adenoid cystic carcinomas (ACCs) of minor salivary glands to evaluate its utility for differentiation purposes. At the same time, the correlation between p27 and ACC grading was evaluated. MATERIALS AND METHODS: Clinicopathological features of 22 patients (11 ACCs, 11 PAs), including age, sex and size of tumor were obtained from medical records. Immunohistochemical staining with p27(kip1) was performed for each specimen and p27 labelling indices were determined with a computer-assisted image-analyzing system (CAS 200). Pearson's correlation coefficient, Spearman's correlation coefficient, Students t-test, Kruskal-Wallis test and ANOVA were applied for statistical analyses using SPSS 11.5. RESULTS: p27 LIs for all PAs were above 25% whereas for ACCs they were under 25% (except one case). p27 expression (LI and intensity) was significantly lower in ACCs than PAs. The correlation between p27 expression and ACC grading was not significant. CONCLUSION: Overall, these findings suggest that reduced expression of p27 might be correlated with the development of ACC and could be an indicator of malignant behavior. (+info)
Sjogren's syndrome: an autoimmune disorder with otolaryngological involvement.
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Sjorgen's syndrome (SS) is an autoimmune exocrinopathy characterized by lymphocyte infiltration of salivary and lacrimal glands that leads to progressive xerostomia and xerophtalmia. One-third of patients suffer of systemic manifestations including arthritis, fever, fatigue and mucosal dryness whereas those with major salivary involvement show an increased risk to develop low-grade non-Hpdgkin lymphomas. In addition, a minority of patients show symptoms related to progressive hearing loss whose pathogenesis remains undefined. Both deposition of autoantibodies to antigens of the inner-ear structures and infiltration by autoreactive T-cells have been implicated in its pathogenesis. In this context, high levels of autoantibodies to both cardiolipin and M3 muscarinic receprtors as well as to ciliar epitopes of the cochlear cells have been recently described. Here we review recent advances on the pathodgenesis of SS with a particular focus to otolaryngological manifestations. (+info)
Activation of IFN pathways and plasmacytoid dendritic cell recruitment in target organs of primary Sjogren's syndrome.
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Gene expression analysis of target organs might help provide new insights into the pathogenesis of autoimmune diseases. We used global gene expression profiling of minor salivary glands to identify patterns of gene expression in patients with primary Sjogren's syndrome (pSS), a common and prototypic systemic autoimmune disease. Gene expression analysis allowed for differentiating most patients with pSS from controls. The expression of 23 genes in the IFN pathways, including two Toll-like receptors (TLR8 and TLR9), was significantly different between patients and controls. Furthermore, the increased expression of IFN-inducible genes, BAFF and IFN-induced transmembrane protein 1, was also demonstrated in ocular epithelial cells by quantitative RT-PCR. In vitro activation showed that these genes were effectively modulated by IFNs in salivary gland epithelial cells, the target cells of autoimmunity in pSS. The activation of IFN pathways led us to investigate whether plasmacytoid dendritic cells were recruited in salivary glands. These IFN-producing cells were detected by immunohistochemistry in all patients with pSS, whereas none was observed in controls. In conclusion, our results support the pathogenic interaction between the innate and adaptive immune system in pSS. The persistence of the IFN signature might be related to a vicious circle, in which the environment interacts with genetic factors to drive the stimulation of salivary TLRs. (+info)
Changing spectrum of the diffuse infiltrative lymphocytosis syndrome.
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OBJECTIVE: To describe the changing clinical spectrum of patients with diffuse infiltrative lymphocytosis syndrome (DILS) after the introduction of highly active antiretroviral treatment (HAART), and to carry out HLA class II oligotyping in these patients. METHODS: A retrospective chart review of patients with DILS who were referred to an outpatient facility for human immunodeficiency virus (HIV)-positive individuals between 1994 and 2003 was performed. DILS was diagnosed as suggested by previous criteria. Demographic features and relevant clinical, laboratory, and radiologic data were recorded and results analyzed. RESULTS: A total of 129 patients with DILS were identified. Of them, 56 (43%) were African American, 41 (32%) were white, and 32 (25%) were Hispanic. Parotid gland swelling appeared to be the sine qua non of DILS. Twenty-seven percent of patients had opportunistic infections. The status of 103 patients was available as of December 2003: 26 (25%) had died, of which only 6 (6%) succumbed to opportunistic infections. The prevalence of DILS had significantly decreased in the post-HAART era (1998 onwards) compared with that of the pre-HAART period (P < 0.000001). The prevalence of lymphocytic interstitial pneumonitis had also dropped significantly following introduction of HAART therapy (P = 0.015). A higher frequency of certain HLA class II alleles (DRB1) was found in African Americans with DILS compared with those with HIV without DILS (P = 0.006). CONCLUSION: The epidemiology, clinical presentation, and certain extraglandular manifestations of DILS have changed, concomitant with the introduction of HAART, further suggesting that DILS is an antigen (viral)-driven response and the primary treatment for it is anti-HIV therapy. (+info)