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INTRODUCTION: The pleomorphic variant is the least common and consequently, the least known subtype of liposarcomas. It represents <5 % of all liposarcomas and develops during late adulthood. PRESENTATION OF THE CASE: We report the case of a 77-year-old male who presented with pain in the right iliac fossa and right inguinal region. A CT scan revealed a stony lesion at the level of the cecum. A right hemicolectomy was performed, and the pathology report was positive for pleomorphic liposarcoma. Consequently, he was referred for adjuvant radiotherapy. CLINICAL DISCUSSION: Molecular analysis has validated the distinction of liposarcoma subtypes, although histological subtype remains the reliable prognostic parameter in daily practice, recent evidence suggests that genetic profiles may eventually influence risk stratification of individual patients. CONCLUSION: The pleomorphic subtype of liposarcomas is infrequent, and they are very rare in the cecum, so it is currently recommended that patients be evaluated by a multidisciplinary team to make a therapeutic decision as it is a subtype of very aggressive behavior, before initiating any type of oncologic treatment.
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OBJECTIVE: To evaluate and compare the expression of E-cadherin, Snail1 and Twist1 in pleomorphic adenomas (PAs), adenoid cystic carcinomas (AdCCa) and carcinoma ex-pleomorphic adenomas (CaexPA) of salivary glands, as well as investigate possible associations with clinicopathological parameters. STUDY DESIGN: E-cadherin, Snail1 and Twist1 antibody immunostaining were analyzed semiquantitatively in 20 PAs, 20 AdCCas and 10 CaexPAs. Cases were classified as low and high expression for analysis of the association with clinicopathological parameters. RESULTS: Compared to PAs, AdCCas and CaexPAs exhibited higher nuclear expression of Snail1 (p = 0.021 and p = 0.028, respectively) and Twist1 (p = 0.009 and p = 0.001). Membranous and cytoplasmic expression of E-cadherin were positively correlated in PAs, AdCCas and CaexPAs (r = 0.645, p = 0.002; r = 0.824, p < 0.001; r = 0.677, p = 0.031). In PAs, positive correlation was found between nuclear expression of Snail1 and membrane expression of E-cadherin (r = 0.634; p = 0.003), as well as between nuclear expression of Snail1 and Twist1 (r = 0.580; p = 0.007). Negative correlations were detected between membrane expression of E-cadherin and cytoplasmic expression of Snail1 in AdCCas (r = - 0.489; p = 0.029). CONCLUSIONS: E-cadherin, Twist1, and Snail1 may participate in modulating events related to cell differentiation and adhesion in PAs and to biological behavior in AdCCas and CaexPAs, which indicates the involvement of EMT in these processes. Furthermore, the expression of these proteins in these carcinomas may reflect the plasticity feature of EMT.
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Adenoma Pleomorfo , Caderinas , Carcinoma Adenoide Cístico , Transição Epitelial-Mesenquimal , Proteínas Nucleares , Neoplasias das Glândulas Salivares , Fatores de Transcrição da Família Snail , Proteína 1 Relacionada a Twist , Humanos , Neoplasias das Glândulas Salivares/patologia , Neoplasias das Glândulas Salivares/metabolismo , Fatores de Transcrição da Família Snail/metabolismo , Caderinas/metabolismo , Feminino , Masculino , Proteína 1 Relacionada a Twist/metabolismo , Pessoa de Meia-Idade , Carcinoma Adenoide Cístico/patologia , Carcinoma Adenoide Cístico/metabolismo , Proteínas Nucleares/metabolismo , Adulto , Adenoma Pleomorfo/metabolismo , Adenoma Pleomorfo/patologia , Idoso , Fatores de Transcrição Twist/metabolismo , Imuno-Histoquímica , Fatores de Transcrição/metabolismo , Biomarcadores Tumorais/metabolismoRESUMO
Primary hepatic liposarcoma is an extremely rare malignant tumour derived from adipocytes and is part of the group of mesenchymal tumours. We present the case of a 43-year-old Hispanic male patient with a pleomorphic hepatic liposarcoma and absence of MDM2 gene amplification. Two years and six months after surgery, the patient is asymptomatic. The present case is the first report of this entity with positive immunohistochemical testing for p16, p53, S100, vimentin and absence of MDM2 gene amplification.
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Lipossarcoma , Proteínas Proto-Oncogênicas c-mdm2 , Humanos , Masculino , Adulto , Proteínas Proto-Oncogênicas c-mdm2/genética , Lipossarcoma/patologia , Adipócitos/patologiaRESUMO
OBJECTIVE: This study used array comparative genomic hybridization to assess copy number alterations (CNAs) involving miRNA genes in pleomorphic adenoma (PA), recurrent pleomorphic adenoma (RPA), residual PA, and carcinoma ex pleomorphic adenoma (CXPA). MATERIALS AND METHODS: We analyzed 13 PA, 4 RPA, 29 CXPA, and 14 residual PA using Nexus Copy Number Discovery software. The miRNAs genes affected by CNAs were evaluated based on their expression patterns and subjected to pathway enrichment analysis. RESULTS: Across the groups, we found 216 CNAs affecting 2261 miRNA genes, with 117 in PA, 59 in RPA, 846 in residual PA, and 2555 in CXPA. The chromosome 8 showed higher involvement in altered miRNAs in PAs and CXPA patients. Six miRNA genes were shared among all groups. Additionally, miR-21, miR-455-3p, miR-140, miR-320a, miR-383, miR-598, and miR-486 were prominent CNAs found and is implicated in carcinogenesis of several malignant tumors. These miRNAs regulate critical signaling pathways such as aerobic glycolysis, fatty acid biosynthesis, and cancer-related pathways. CONCLUSION: This study was the first to explore CNAs in miRNA-encoding genes in the PA-CXPA sequence. The findings suggest the involvement of numerous miRNA genes in CXPA development and progression by regulating oncogenic signaling pathways.
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Adenocarcinoma , Adenoma Pleomorfo , MicroRNAs , Neoplasias das Glândulas Salivares , Humanos , Adenoma Pleomorfo/genética , Adenoma Pleomorfo/patologia , Variações do Número de Cópias de DNA , Neoplasias das Glândulas Salivares/patologia , MicroRNAs/genética , Hibridização Genômica Comparativa , Transformação Celular Neoplásica/patologia , Adenocarcinoma/patologiaRESUMO
OBJECTIVE: Pleomorphic adenoma (PA), mucoepidermoid carcinoma (MEC), and adenoid cystic carcinoma (ACC) are the most prevalent salivary gland tumors. Their pathogenesis has been recently associated with complex molecular cascades, including the TGFß signaling pathway. The aim of this study was to evaluate the expression of genes associated with the TGFß signaling pathway (TGFB1, ITGB6, SMAD2, SMAD4, FBN1, LTBP1, and c-MYC) to map possible downstream alterations in the TGFß cascade. DESIGN: Thirteen PA, 17 MEC, 13 ACC, and 10 non-neoplastic salivary gland samples were analyzed by real-time RT-PCR. RESULTS: Cases of PA presented increased TGFB1, LTPB1, c-MYC, and FBN1 expressions, whereas SMAD2 expression was decreased when compared to non-neoplastic tissue. MEC patients displayed increased expressions of TGFB1, ITGB6, FBN1, and c-MYC and decreased expressions of SMAD2 and SMAD4. ACC cases exhibited elevated expressions of the investigated genes except TGFB1. The present results suggest that decreased expression of SMAD2 and SMAD4 does not impede the transcriptional regulation of c-MYC, especially in PA and MEC. Increased expressions of ITGB6, TGFB1, LTBP1, and FBN1 appear to be related to the regulation of the TGFß signaling pathway in these tumors. Additionally, we observed a higher expression of SMAD4 in ACC and a raised expression of ITGB6 and lowered expression of SMAD2 in MEC. CONCLUSIONS: Our study demonstrated the differential expression of TGFß cascade members in salivary gland tumors such as SMAD2/SMAD4 and c-MYC as well as the participation of ITGB6, TGFB1, LTBP1, and FBN1, contributing to the understanding of the mechanisms involved in tumor progression.
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Adenoma Pleomorfo , Carcinoma Adenoide Cístico , Carcinoma Mucoepidermoide , Neoplasias das Glândulas Salivares , Fator de Crescimento Transformador beta , Humanos , Adenoma Pleomorfo/genética , Adenoma Pleomorfo/metabolismo , Adenoma Pleomorfo/patologia , Biomarcadores Tumorais/metabolismo , Carcinoma Adenoide Cístico/genética , Carcinoma Adenoide Cístico/metabolismo , Carcinoma Mucoepidermoide/metabolismo , Neoplasias das Glândulas Salivares/genética , Neoplasias das Glândulas Salivares/metabolismo , Transdução de Sinais , Fator de Crescimento Transformador beta/metabolismoRESUMO
Pleomorphic hyalinizing angiectatic tumour (PHAT) is a very rare low-grade indeterminate neoplasm of connective and other soft tissue, which is not known to metastasize though local recurrence has been documented. It most commonly presents in the lower extremities, but other anatomical locations have been described. This is the second known case of PHAT from the Caribbean region and adds to the limited reported cases of the condition in the literature.
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Abstract The aim of this study was to evaluate the expression of the EZH2 protein and describe the clinical and microscopic characteristics of adenoid cystic carcinoma (ACC) and pleomorphic adenoma (PA). The study included 16 ACC cases and 12 PA. All ACC and PA cases were positive for EZH2 and the ACC samples showed significantly higher EZH2 expression. The clinical and microscopic covariates were described in relation to EZH2 staining in ACC samples. The highest mean values of EZH2 were observed in cases with local metastasis, recurrence, perineural invasion, and predominantly cribriform growth pattern without solid areas. EZH2 is a potential marker of malignancy.
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Purpose: This systematic review analyzed the clinical behavior and odds of malignancy of the palatal recurrent pleomorphic adenomas. Methods: Systematic review of patients with recurrent pleomorphic adenoma arising in the palate. Database search: MEDLINE, Scopus, Web of Science, Cochrane, EMBASE, Virtual Health Library, Google Scholar, and OpenGrey. A binomial logistic regression was performed to assess the odds of detecting recurrence five, 10 and 20 years after the treatment of primary tumor. Results: Thirteen studies (n = 18 patients) out of 336 were included. The recurrent pleomorphic adenoma in palate was more common in females (61.6%), average age was 49 years old (range 9-73 years old). Four patients progressed to malignant transformation. The odds ratio (OR) of detecting a recurrence at 10 (OR = 5.57; 95% confidence interval - 95%CI 1.13-27.52), and 20 years (OR = 18.78; 95%CI 3.18-110.84) after treatment of primary pleomorphic adenoma was significantly higher than at one-year follow-up. Conclusions: The recurrence of pleomorphic adenoma in palate remains a rare event of late occurrence. It mainly affects middle-aged female and carries a risk of malignant transformation. Although uncommon, patients with palatal pleomorphic adenoma should be warned about the possibility of recurrence or malignant transformation of tumor at advanced ages.
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Palato , Glândulas Salivares , Adenoma Pleomorfo , NeoplasiasRESUMO
ABSTRACT We present a literature review of 57 publications describing this pathology, published from the year 2012. In all these studies patients were reported to depict a slow-growing, motionless mass, which is painless at most times. All cases were managed by total excision, except for one report where adjuvant radiotherapy was applied. Among the several therapeutic strategies, the total tumor resection, preserving the tumor pseudocapsule intact, appears to be a consensus in treating the disease efficiently. Furthermore, fine-needle aspiration biopsy, including the assessment of genetic alterations, has proved to be a valuable tool in the diagnosis of challenging cases. Our literature survey also suggests that an incisional biopsy before the surgery may lead to the pseudocapsule disruption, thus considerably increasing the chances of adenoma recurrence, enabling its malignization. At present, genetics studies indicate that the molecular aberrations involved in the adenoma are similar to those represented in the salivary gland tumor pathogenesis. Further, in the recurrent cases, the pathology becomes difficult to treat and multiple surgeries may be required, occasionally, leading to radical surgery treatment.
RESUMO Uma revisão narrativa da literatura de 57 publicações que descrevem esta patologia, publicada a partir de 2012. Os pacientes têm uma massa de crescimento lento e imóvel, que na maioria das vezes é indolor. Todos os casos foram tratados por excisão total, com exceção de um relatório de radioterapia adjuvante. Entre as estratégias terapêuticas encontradas, a ressecção total do tumor, preservando a pseudocápsula tumoral intacta, parece ser um consenso. Alternativamente, a biópsia por aspiração de agulha fina incluindo a avaliação de alterações genéticas pode representar uma ferramenta valiosa nos casos diagnósticos desafiadores. Uma biópsia incisional antes da cirurgia não é recomendada, pois a ruptura da pseudocápsula aumenta consideravelmente a recorrência do adenoma, permitindo até mesmo sua malignização. Com relação à genética, estudos atuais indicam que as aberrações moleculares envolvidas no adenoma são semelhantes às da patogênese do tumor da glândula salivar. Para casos de recorrência, a patologia torna-se difícil de tratar e múltiplas cirurgias podem ser necessárias, às vezes levando a um tratamento cirúrgico radical.
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The objective of this study was to evaluate the role of p16 in histologic characteristics and transition of Pleomorphic Adenoma (PA) to Carcinoma ex-PA (CxPA). So, 60 PA and 4 CxPA were histologic reviewed based on microscopic characteristics proposed by Hellquist, Triantafyllou and Dulguerov (PA) and Morais, Antony and Toluie (CxPA). Immunostaining for p16 was associated in different parenchyma and stroma of both tumors and Fisher's/chi-square tests and Mann-Whitney test were performed (SPSS v20.0, p<0.05). In PA the periductal cells were predominantly p16- and that ductal and myoepithelial cells showed a significant increase in p16+ cells (p<0.001). In CxPA, none of the cases showed p16+ in periductal cells, most parotid cases showed p16+ in ductal cells, and one case of parotid and the submandibular case showed mild immunostaining for myoepithelial cells. There was a small reduction in p16+ in CxPA compared to PA (p=0.537), but in both tumors there was less p16+ cells in solid stroma than other (p<0.001). The p16+ cases of PA had a higher capsular thickness (p=0.047). So, the loss of p16 immunostaining does not seem to be associated with the transition from PA to CxPA, but in both tumors the loss of p16+ cells are related to microscopic aggressiveness.
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Adenocarcinoma , Adenoma Pleomorfo , Humanos , Células Epiteliais , Inibidor p16 de Quinase Dependente de CiclinaRESUMO
ABSTRACT Purpose: To compare the radiologic and clinical features of primary lacrimal gland pleomorphic adenoma and adenoid cystic carcinoma. Methods: This study retrospectively reviewed imaging findings and medical records of lacrimal gland pleomorphic adenoma and adenoid cystic carcinoma. Results: Eleven patients with pleomorphic adenoma and 16 patients with adenoid cystic carcinoma were evaluated. There were no statistically significant differences between groups regarding age or sex. Proptosis was the most common presenting symptom in both groups. Adenoid cystic carcinomas were more likely to present with a palpable mass, diplopia, pain, and sensory loss than pleomorphic adenomas, although the differences were not statistically significant between groups. Furthermore, there were no significant differences in terms of homogeneity and globe indentation between lacrimal gland pleomorphic adenoma and adenoid cystic carcinoma on computed tomography (CT)(all p>0.05). The rates of bone invasion, tumor calcification, and wedge sign were significantly higher in adenoid cystic carcinomas, and bone remodeling was statistically significantly higher in pleomorphic adenomas, on CT(all p<0.05). Pleomorphic adenomas were significantly more likely to show well-defined margins, lobulated contours, heterogeneous contrast enhancement, and hyperintensity on T2-weighted magnetic resonance images (all p<0.05). Conclusion: When differentiating between lacrimal gland pleomorphic adenoma and adenoid cystic carcinoma, evaluation of radiologic features along with clinical features is of great importance. Lobulated contours may be a significant distinguishing radiologic feature suggesting pleomorphic adenoma.
RESUMO Objetivo: Comparar as características radiológicas e clínicas do adenoma pleomórfico primário e do carcinoma adenoide cístico da glândula lacrimal. Métodos: Este estudo revisou retrospectivamente os achados de imagem e os prontuários médicos de casos de adenoma pleomórfico e carcinoma adenoide cístico da glândula lacrimal. Resultados: Foram avaliados 11 pacientes com adenoma pleomórfico e 16 pacientes com carcinoma adenoide cístico. Não houve diferenças estatisticamente significativas em relação à idade e sexo. Proptose foi o sintoma de apresentação mais comum em ambos os grupos. Os carcinomas adenoides císticos foram mais propensos que os adenomas pleomórficos a apresentarem massas palpáveis, diplopia, dor e perda sensorial, mas essa diferença entre os grupos não foi estatisticamente significativa. Não houve diferenças estatísticas em termos de homogeneidade e indentação do globo ocular entre os dois tipos de tumores em imagens de tomografia computadorizada (p>0,05). Também à tomografia computadorizada, a invasão óssea, a calcificação do tumor e o sinal em cunha foram mais frequentes nos carcinomas adenoides císticos, enquanto a remodelação óssea foi mais frequente nos adenomas pleomórficos, com significância estatística para todas essas manifestações (p<0,05). À ressonância magnética, os adenomas pleomórficos foram significativamente mais propensos a terem margens bem definidas, contornos lobulados, realce heterogêneo pelo contraste e hiperintensidade na ressonância magnética ponderada em T2 (p<0,05). Conclusão: Ao se diferenciar o adenoma pleomórfico e o carcinoma adenoide cístico da glândula lacrimal, é muito importante avaliar as características radiológicas juntamente com as características clínicas. Os contornos lobulados podem ser uma característica radiológica significativamente distinta em favor do adenoma pleomórfico.
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Pleomorphic adenoma (PA) is the most common salivary gland tumor, accounting for 50%-60% of these neoplasms. If untreated, 6.2% of PA may undergo malignant transformation to carcinoma ex-pleomorphic adenoma (CXPA). CXPA is a rare and aggressive malignant tumor, whose prevalence represents approximately 3%-6% of all salivary gland tumors. Although the pathogenesis of the PA-CXPA transition remains unclear, CXPA development requires the participation of cellular components and the tumor microenvironment for its progression. The extracellular matrix (ECM) comprises a heterogeneous and versatile network of macromolecules synthesized and secreted by embryonic cells. In the PA-CXPA sequence, ECM is formed by a variety of components including collagen, elastin, fibronectin, laminins, glycosaminoglycans, proteoglycans, and other glycoproteins, mainly secreted by epithelial cells, myoepithelial cells, cancer-associated fibroblasts, immune cells, and endothelial cells. Like in other tumors including breast cancer, ECM changes play an important role in the PA-CXPA sequence. This review summarizes what is currently known about the role of ECM during CXPA development.
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Objective: To report a case of pleomorphic adenoma that was surgically removed and to describe the surgical technique available to remove this lesion. Case Report: A male patient, caucasian, 46 years old, with a 4-year history of pleomorphic adenoma. He mentioned that he went to other services for treatment, but without success. He came at our service for treatment with tumoral excision. Local anesthesia was performed with mepivacaine with a vasoconstrictor and tumoral excision was performed with the overlying mucosa and the periosteal region to avoid recurrence. The patient has been under postoperative follow-up for two years without recurrence. Conclusion: Pleomorphic adenoma is a benign tumor and presents as a submucosal mass of slow growth. The ideal treatment for the Pleomorphic Adenoma tumor is the total excision of the lesion with the removal of the mucosa covering the region to avoid recurrence. (AU)
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Humanos , Masculino , Pessoa de Meia-Idade , Adenoma Pleomorfo , Palato Duro , Técnicas de Diagnóstico por Cirurgia , Glândulas Salivares , NeoplasiasRESUMO
OBJECTIVE: To analyze the proteomic profile of salivary pleomorphic adenoma (PA) and carcinoma ex pleomorphic adenoma (CXPA) samples and correlate them with the malignant transformation of the PA. MATERIALS AND METHODS: Thirty samples (10 PA, 16 CXPA, and 4 residual PA) were microdissected and submitted to liquid chromatography-tandem mass spectrometry (LC-MS/MS). The proteomic data and protein identification were analyzed through LC-MS/MS spectra using the MaxQuant software. RESULTS: The proteomic analysis identified and quantified a total of 240 proteins in which 135 were found in PA, residual PA, and CXPA. The shared proteins were divided into six subgroups, and the proteins that showed statistically significant differences (p > 0.05) and fold-change > or <2.5 in one subgroup to another subgroup were included. Seven proteins (Apolipoprotein A-I-APOA1, haptoglobin-HP, protein of the synaptonemal complex 1-SYCP1, anion transport protein of band 3-SLC4A1, subunit µ1 of AP-1 complex-AP1M1, beta subunit of hemoglobin-HBB, and dermcidin-DCD) were classified as potential protein signatures, being HP, AP1M1, and HBB with higher abundance for PA to residual PA, APOA1 with higher abundance for PA to CXPA, SLC4A1 with lower abundance in the PA to CXPA, SYCP1with lower abundance for residual PA to CXPA, and DCD with higher abundance in the CXPA with epithelial differentiation to myoepithelial differentiation. CONCLUSIONS: In this work, we demonstrated the comparative proteomic profiling of PA, residual PA, and CXPA, and seven were proposed as protein signatures, some of which may be associated with the malignant phenotype acquisition.
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Adenoma Pleomorfo , Neoplasias das Glândulas Salivares , Humanos , Adenoma Pleomorfo/genética , Adenoma Pleomorfo/metabolismo , Adenoma Pleomorfo/patologia , Neoplasias das Glândulas Salivares/patologia , Cromatografia Líquida , Proteômica , Espectrometria de Massas em TandemRESUMO
ABSTRACT Salivary gland tumors account for only 3%-5% of all tumors in the head and neck, 10%-15% of which originate from minor salivary glands. Pleomorphic adenoma is a benign lesion of the salivary gland, most commonly occurring in the region of the hard and soft palates. The treatment of choice for pleomorphic adenomas is with the partial or total function of the extension of the lesion, and placing palatal obturators are one method of reestablishing masticatory function and facial esthetics. This study aimed to rehabilitate a patient using a palatal implant following partial maxillectomy for the removal of a pleomorphic adenoma. A young patient with pleomorphic adenoma of the hard palate underwent a partial right-sided maxillectomy procedure which removed the hard palate and alveolar regions of the molars. Prior to surgery, the patient was assessed to make a surgical guide for resection of the tumor, as well as a provisional obturator plate using orthodontic wire clasps. After healing, the patient was rehabilitated using a palatal obturator which had been incorporated into a removable partial denture. The clinical sequence used to fabricate the final prosthesis was as follows: initial molding after surgery, prosthesis design, preparation of the mouth, work molding, structure testing and orientation planning, teeth testing and installation, and periodic maintenance. Thus, we can conclude that the palatal obturator is an excellent means of restoring a patient's oral function, facial esthetics, and overall quality of life.
RESUMO Os tumores de glândulas salivares representam apenas 3%-5% de todos os tumores em região de cabeça e pescoço, dentre eles 10%-15% se originam de glândulas salivares menores. O adenoma pleomórfico é uma lesão benigna de glândula salivar, tendo uma maior prevalência em região de palato. O tratamento dessas lesões se dá por método excisional ou maxilectomia dependendo da extensão da lesão e, uma das formas de reestabelecimento da estética e função do paciente é com um obturador palatino. O objetivo deste trabalho foi relatar uma reabilitação oral através da utilização de um obturador palatino em um paciente submetido a maxilectomia parcial para remoção de adenoma pleomórfico. Paciente de 22 anos, com adenoma pleomórfico em região de palato, foi submetido a maxilectomia parcial, removendo região de palato duro e região alveolar dos molares do lado direito. Foi realizada moldagem da maxila previamente a cirurgia para confecção de um guia cirúrgico, o guia foi utilizado na ressecção do tumor e, serviu também, para confecção de uma placa obturadora provisória associada a fios ortodônticos. Após cicatrização completa, foi acordado que a reabilitação do paciente seria com uma prótese parcial removível do tipo obturador palatino. A sequência clínica para a realização da prótese foi: moldagem inicial após a cirurgia, delineamento, preparo de boca, moldagem de trabalho, prova da infraestrutura e plano de orientação, prova dos dentes, instalação e manutenções periódicas. Com isso, podemos concluir que o obturador palatino é um excelente meio de reestabelecer função e estética do paciente, melhorando sua qualidade de vida.
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Salivary gland neoplasms comprise a heterogeneous group of lesions with multiple histological subtypes, each with distinct growth patterns, resulting in a spectrum of tumor-specific prognoses; pleomorphic adenoma (PA) and mucoepidermoid carcinoma (MEC) are the most common representatives of these neoplasms. Many studies have associated specific profiles of membrane and adhesion molecules in salivary gland tissues; these profiles appear to be relevant in tumor biology as well as be interpreted as fingerprints for tumor classification, diagnostic prognostic and therapeutic targets. One of these membrane molecule complexes are the tight junctions, composed by various proteins, in which claudins are protagonists. The aim of this study was to evaluate the expressions of genes that encode tight junction proteins (CLDN-1, -3, -4, -5, -7, and -11, occludin [OCLN], zonula occludens [TJP1, TJP2, and TJP3] and junctional adhesion molecule A [F11R]) in MEC and PA using real time RT-PCR. We observed high expression of CLDN-1 and -7 and low expression of CLDN-3, -11 and TJP2 in MEC compared to PA. PA samples demonstrated high OCLN expression when compared to MEC. CRTC1::MAML2 fusion was detected in 12 of 20 (60.0%) MEC samples and was associated with CLDN7 expression, while the absence of fusion was associated with high histological grade. Increased CLDN5 expression was associated with submandibular gland tumors. This study demonstrated differential expressions of genes encoding tight junction constituent proteins and their associations with tumor characteristics, suggesting their potential future role as diagnostic and prognostic markers.
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O Sarcoma de partes moles é um tumor maligno raro, correspondendo a aproximadamente 1% dos tumores malignos em adultos. Objetivamos descrever um raro caso de Lipossarcoma pleomórfico em paciente jovem, sem história familiar, que representa 0,3% dos sarcomas da mama. Relatamos o caso de uma paciente do sexo feminino, 39 anos, com nodulação em prolongamento axilar de mama esquerda, cujas mamografia e ecografia caracterizaram nodulação regular, de limites definidos, sem fluxo central ou periférico e ecotextura heterogênea. A Core-Biopsy diagnosticou neoplasia maligna fusocelular. Realizada ressecção segmentar, cujos laudos histopatológico e de imuno-histoquímica concluíram como sendo Lipossarcoma pleomórfico de alto grau. O sarcoma pleomórfico é um subtipo raro e agressivo entre os lipossarcomas. O seu tratamento, assim como dos sarcomas de mama, é a ressecção cirúrgica com margens de segurança; a terapia adjuvante com radioterapia é indicada devido à taxa de recorrência deste tumor. A quimioterapia ainda tem papel indefinido nesses tipos de tumores. Concluímos que, por ser o Lipossarcoma pleomórfico uma neoplasia maligna rara, o diagnóstico e a conduta adequados se fazem necessários para prover terapêutica precisa e conter metastatização e recidiva local.
Soft tissue sarcoma is a rare malignant tumor, accounting for approximately 1% of malignant tumors in adults. We aim to describe a rare case of pleomorphic liposarcoma in a young patient with no family history, representing 0.3% of breast of breast sarcomas. We report the case of a 39-year-old female patient with nodulation in the axillary extension of the left breast, whose mammogram and ultrasonography characterized a regular nodulation, with defined limits, without central or peripheral flow, and heterogeneous echotexture. The Core-Biopsy diagnosed malignant fusocellular neoplasm. Segmental resection was performed. The histopathological and immunohistochemistry reports concluded that it was a high grade pleomorphic liposarcoma. Pleomorphic sarcoma is a rare and aggressive subtype among the liposarcomas. Its treatment, as with breast sarcomas, is surgical resection with safety margins; adjuvant therapy with radiotherapy is indicated due to the recurrence rate of this tumor. Chemotherapy still has an undefined role in these types of tumors. We conclude that, because pleomorphic liposarcoma is a rare malignant neoplasm, the diagnosis and management are necessary to provide an accurate therapy and contain metastasis and local recurrence.
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SarcomaRESUMO
INTRODUCTION: This study investigates the relationship between cellularity and capsular characteristics of pleomorphic adenoma and its influence on operative strategies. MATERIAL AND METHODS: The capsular characteristics and clinical data of patients with pleomorphic adenomas were reviewed according to Seifert's definition: (1) classic type with balanced amount of cells and stroma, (2) myxoid type with abundant ground substance, interspersed spindle cells, and (3) cellular type with predominance of ductal trabecular structures and little stroma. The immunoreactivity of cellular proliferation (Ki-67) was semi-quantitatively measured using immunohistochemistry. Variables were analyzed using Fisher's test and one-way ANOVA, with (p < 0.05) considered statistically significant. RESULTS: The duration of presence was associated with cellularity (p = 0.01). In terms of capsular characteristics, satellite nodules and positive resection margins were not related to cellularity, except for incomplete capsules (p = 0.03). There was no difference in the staining scores of Ki-67 (p = 0.12). CONCLUSION: Lower cellularity reflects higher probability of an incomplete capsule, requiring more consideration for operative strategies to prevent recurrence.
INTRODUCCIÓN: Este estudio investiga la relación entre la celularidad y las características capsulares del adenoma pleomórfico y su influencia en las estrategias operativas. MATERIAL Y MÉTODOS: Se revisaron las características capsulares y los datos clínicos de los pacientes con adenomas pleomórficos según la definición de Seifert: 1) tipo clásico con cantidad equilibrada de células y estroma, 2) tipo mixoide con abundante sustancia fundamental, células fusiformes intercaladas y 3) tipo celular con predominio de estructuras trabeculares ductales y poco estroma. La inmunorreactividad de la proliferación celular (Ki-67) se midió semicuantitativamente usando inmunohistoquímica. Las variables se analizaron mediante la prueba de Fisher y ANOVA de una vía, considerándose significativo un valor de p inferior a 0.05. RESULTADOS: La duración de la presencia se asoció con la celularidad (p = 0.01). En cuanto a las características capsulares, los nódulos satélites y los márgenes de resección positivos no se relacionaron con la celularidad, a excepción de las cápsulas incompletas (p = 0.03). No hubo diferencia en las puntuaciones de tinción de Ki-67 (p = 0.12). CONCLUSIONES: La celularidad más baja refleja una mayor probabilidad de una cápsula incompleta, lo que requiere una mayor consideración de las estrategias quirúrgicas para prevenir la recurrencia.
Assuntos
Adenoma Pleomorfo , Neoplasias Parotídeas , Adenoma Pleomorfo/cirurgia , Humanos , Antígeno Ki-67 , Margens de Excisão , Neoplasias Parotídeas/cirurgiaRESUMO
BACKGROUND: Carcinoma ex-pleomorphic adenoma (Ca ex-PA) comprises 0.5% of head and neck neoplasms. Transoral robotic surgery (TORS) is an approach being used to treat a variety of benign and malignant head and neck neoplasms. Recently, this technique has gained popularity as an alternative for parapharyngeal space (PPS) tumor resection. To our knowledge, this is the first case of Ca ex-PA managed successfully by TORS of the PPS. CASE: Fifty-nine-year-old male with incidental mass in PPS, initial diagnosis of pleomorphic adenoma, who underwent transoral robotic resection. The histopathology diagnosis with minimally invasive Ca ex-PA findings and malignant component of high-grade epithelial/myoepithelial carcinoma and salivary duct carcinoma. Patient discharged on the fifth post-operative day without complications. CONCLUSION: Based on our findings, TORS may be a safe procedure to remove selected Ca ex-PA from the PPS; however, further research is needed.