Evaluation of the status of the parotid and submandibular salivary glands in the hypohidrotic ectodermal dysplasia by ultrasonography

Displasia ectodérmica hipoidrótica é uma doença genética rara, clinicamente caracterizada por alterações envolvendo os dentes, pele e suas estruturas anexas. Manifestações orais comuns incluem anadontia ou oligodontia, dentes conóides e xerostomia. Poucos relatos associam displasia ectodérmica e redução do fluxo salivar. O objetivo desse estudo é analisar possíveis alterações nas glândulas salivares de dez portadores de displasia ectodérmica que fazem acompanhamento no Departamento de Dermatologia Pediátrica da Universidade de São Paulo. Ultrassonografia foi realizada em dez casos em infantes portadores de displasia ectodérmica hipoidrótica nas suas glândulas salivares parótida e submandibular. Três apresentaram alterações na glândula parótida e/ou submandibular. Aplasia ou hipoplasia das glândulas salivares maiores é associada a casos de displasia ectodérmica e sugere-se acompanhamento rotineiro das glândulas salivares maiores usando ultrassonografia para prevenção de alterações decorrentes da hiposalivação na cavidade oral.Background- Hypohidrotic ectodermal dysplasia is a rare genetic disease, clinically characterized by defects involving skin and their adnexal structures, as well as oral structures such as teeth, and occasionally salivary glands. These latter manifestations include anodontia or hypodontia, conical teeth and xerostomia. Objective- To analyze possible alterations in major salivary glands of ten patients with ectodermal dysplasia, using image exam. Methods- Ultrasonography was performed in ten pediatric cases of hypohidrotic ectodermal dysplasia to investigate the status of parotid and submandibular salivary gland. Results- Three patients presented aplasia/hypoplasia of at least one gland examined. The other 7 patients did not present any alterations in parotid and submandibular glands. Limitations- Although ultrasonography exam is adequate to investigate the presence and status of major salivary glands, other important glands such as sublingual and minor mucous salivary glands are not well-visualized using this technique. Conclusions- Aplasia or hypoplasia of the major salivary gland is strongly related to ectodermal dysplasia and we suggest routine evaluation of the major salivary gland using ultrasound to monitor and manage the possible impact of xerostomia in oral health

Immunohistochemistry expression of Jab1, p27, c-jun and c-fos proteins in pleomorphic adenoma, low grade polymorphous adenocarcinoma and adenoid cystic carcinoma of the salivary glands

Os tumores de glândula salivar compreendem em torno de 2 a 6,5% dos tumores de cabeça e pescoço. Entre os tumores de glândula salivar, o adenoma pleomórfico é benigno e o mais comum. O carcinoma adenoide cístico e adenocarcinoma polimorfo de baixo grau encontram-se entre os mais frequentes malignos. Jab1 é uma de muitas proteínas que afetam diversos estágios da tumorigênese sendo importante na regulação variadas vias de sinalização e/ou proteínas como p27 e AP-1, a última composta por c-jun e c-fos, que são principalmente relacionadas com o ciclo celular e proliferação celular. O objetivo desse trabalho foi avaliar a expressão imuno-histoquímica das proteínas Jab1, p27, c-jun e c-fos no adenoma pleomórfico, adenocarcinoma polimorfo de baixo grau e carcinoma adenoide cístico das glândulas salivares. Foi realizada análise imuno-histoquímica semi-quantitativa das células marcadas nos tumores de glândula salivar e glândula salivar normal de acordo com o escore 0 (células sem expressão), 1(> 0 5 50%). Para Jab1, c-jun e c-fos foi considerado apenas marcação nuclear e para p27, nuclear e citoplasmática, separadamente. Os resultados foram analisados utilizando-se os testes de Kruskal-Wallis, de Mann-Whitney, do Qui-quadrado e o teste de correlação de Spearman, cujo nível de significância foi de p 0 5 50%). Nuclear immunostaining alone was considered for Jab1, c-jun and c-fos proteins and cytoplasmic and nuclear staining for p27. Results were analyzed in GraphPad Prisma 5.0 software using Kruskal-Wallis, Mann-Whitney and Chi-square tests and Spearman correlation test in which significancy level was p<0,05. Statistical analysis revealed that Jab1 expression was significant in pleomorphic adenoma and adenoid cystic carcinoma in relation to ducts and in polymorphous low-grade adenocarcinoma in relation to adenoid cystic carcinoma (p=0,0136, 0,0001 e 0,0344, respectively); the p27 nuclear expression was significant in pleomorphic adenoma and in polymorphous low-grade adenocarcinoma when compared to adenoid cystic carcinoma (p=0,0074 e 0,0004, respectively) and cytoplasmic immunostaining was significant in all groups when compared to acini; c-fos expression was significant in ducts if compared to pleomorphic adenoma, polymorphous low-grade adenocarcinoma and adenoid cystic carcinoma (p=0,0002, 0,0048 e 0,0352, respectively). Spearman correlation test to Jab1, p27, c-jun and c-fos in each lesion separately revealed significant correlation between Jab1 and p27 (r=0,371; p=0,020) and c-jun and c-fos (r=0,452; p=0,004) in pleomorphic adenoma. There was correlation between Jab1 and p27 (r=0,494; p=0,044) in polymorphous low-grade adenocarcinoma and between p27 and c-fos (r=0,513; p=0,035) in adenoid cystic carcinoma. In conclusion, tumorigenesis in pleomorphic adenoma, polymorphous low-grade adenocarcinoma and adenoid cystic carcinoma seems to be associated to expression of Jab1 and p27

Carcinoma ex pleomorphic adenoma of minor salivary glands with major epithelial-myoepithelial component: clinicopathologic and immunohistochemical study of 3 cases

In the present study, 3 cases of very rare intraoral carcinomas ex pleomorphic adenomas showing a striking differentiation of the malignant component towards epithelial-myoepithelial carcinoma were described. The tumors occurred in 2 men and 1 woman with median age of 56 years. Involved sites included palate and buccal mucosa. Two patients experienced local recurrences, of which one died of disease complications. In all cases, residual pleomorphic adenoma was present The malignant component in all cases shared morphological aspects with epithelial-myoepithelial carcinoma. Those areas were characterized by eosinophilic duct-forming cells surrounded by layers of clear cells. The studied immunohistochemical markers highlighted a biphasic cell population. Duct-forming cells expressed pan-cytokeratin, cytokeratin 7, and focally cytokeratin 14, whereas the clear cell component strongly stained to cytokeratin 14, vimentin, and p63 but weakly stained to pancytokeratin and focally to a-smooth muscle actin, an immunophenotype compatible with both epithelial and myoepithelial differentiation. The Ki-67 proliferation index was up to 40% in malignant areas. Carcinoma ex pleomorphic adenomas of minor salivary glands with major epithelial-myoepithelial component are rare, locally aggressive, and potentially lethal tumors. The peculiar morphological and immunohistochemical aspects described may raise problems in diagnosis and classification of such cases, particularly in incisional biopsies. (C) 2015 Elsevier Inc. All rights reserved.In the present study, 3 cases of very rare intraoral carcinomas ex pleomorphic adenomas showing a striking differentiation of the malignant component towards epithelial-myoepithelial carcinoma were described. The tumors occurred in 2 men and 1 woman with193164168sem informaçãosem informaçã