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Introduction and purpose: Carcinoma ex-pleomorphic adenoma (CXPA) is a rare tumor with different prevalence rates found among studies. There are few epidemiological studies of large series of CXPAs in developing countries. Objective: The aim of the present study is to present a Brazilian retrospective study of 38 patients with CXPA. Material and methods: Thirty-eight cases of CXPA from three Brazilian institutions, São Paulo State were evaluated. The patients were analyzed according to gender, age, location, clinical stage, histopathological subtype, invasiveness of the tumor, and follow-up information. Results: No preferential gender was found, and the mean age of diagnosis was 57.6years. The most commonly involved site was the parotid (28 cases, 73.7%), followed by the submandibular (six cases, 15.8%), and the minor salivary gland (four cases, 10.5%). At diagnosis, 9 (23.6%) were classified as clinical stage I or II, and 19 (50.1%) as stage III or IV, with 10 (26.3%) having no information. The histological types were: 16 salivary duct carcinomas (42.2%), 8 adenocarcinomas not otherwise specified (21.1%), 7 myoepithelial carcinomas (18.4%), 5 epithelial-myoepithelial carcinomas (13.1%), 1 epidermoid carcinoma (2.6%), and 1 sarcomatoid carcinoma (2.6%). Moreover, according to the invasive phase, 6 (15.8%) were classified as intra-capsular, 9 (23.7%) minimally invasive and 23 (60.5%) frankly invasive. Recurrence was observed in 7 out 24 patients with follow-up information available, which were all invasive cases. Seven out 24 patients died of causes related to the disease. Conclusion: The distribution of cases according to age, gender, tumor location and clinical stage was similar to the reported literature. Frankly invasive cases presented worse prognosis. [ABSTRACT FROM AUTHOR]

Introduction: Carcinoma ex-pleomorphic adenoma (CXPA) is a rare and aggressive malignancy of the salivary glands that arises from a previous PA. CXPA present different histological subtypes and grade of invasiveness, leading to variable clinical behavior. Objective: Determine the proliferative index of 36 cases of CXPA, considering the histological subtypes and invasiveness phase. Material and methods: It was included on this study 36 each of CXPA and PA, and 22 areas of PA in CXPA. All cases of CXPA were classified according to invasiveness (early and frankly invasive phase) and histological subtype. Proliferative index was determined by Ki-67, and the data were statistically analyzed by Wilcoxon’s, Mann–Whitney and Kruskal–Wallis’ tests. Results: CXPA included 14 cases classified as early invasive and 22 as frankly invasive phases. Fifteen cases corresponded to salivary duct carcinoma, 7 adenocarcinoma NOS, 7 myoepithelial carcinoma, 5 epithelial-myoepithelial carcinoma, one each squamous cell carcinoma and sarcomatoid carcinoma. The Ki-67 mean index of PA and residual PA were 6.7±2.5 and 6.9±8.2, respectively, significantly lower than CXPA (49.3±21.7). Early invasive carcinoma (44.3±23.0) showed smaller proliferative index than frankly invasive (52.5±20.8). Considering the subtypes of CXPA, there was not a statistic difference among them. Conclusion: Ki-67 is a useful marker in the differential diagnosis of PA and CXPA, even when in the early invasive phase. Ki67 index varies according to the histological subtype of CXPA, but are not statistically significant. [ABSTRACT FROM AUTHOR]

Purpose: Carcinoma ex-pleomorphic adenoma (CXPA) is a malignant salivary gland tumor that arises rarely in the minor salivary glands. Although the etiology of CXPA remains unclear, the role of some tumor suppressor genes and oncogenes in CXPA is well known, however other genes still need to be studied. Material and methods: An uncommon case of CXPA involving the upper lip is presented, which was analyzed by a panel of genes (the most are tumor suppressor) by multiplex ligation dependent-probe amplification (MLPA). Results: A 69-year-old male was submitted to surgical resection of a mass in the mucous of the upper lip histologically diagnosed as CXPA. The patient is free of disease after 30months postoperatively. From all of the genes investigated in this study, a loss of copy number was detected for BCL2, CASP8, CD44, CDH1, DAPK1, ESR1, RASSF1, and TP73. Immunohistochemical reactions for the validation of some of these results showed negativity for CD44, Bcl-2, RASSF1 and p73. Conclusion: A loss of copy number of the genes CD44, BCL-2, RASSF1, and TP73, may contribute with the formations of CXPAs. The genomic findings this case may be compared to malignant tumors of other sites aiming a better understanding of the carcinogenesis process. [Copyright &y& Elsevier]