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Introduction. Primary pulmonary salivary gland-type carcinomas are rare malignancies arising from minor salivary gland tissue in the lower respiratory tract. Given their rarity, constituting <1% of all primary lung malignancies, their epidemiological features and outcomes remain poorly documented. This study analyzed data from the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) database to identify primary pulmonary salivary gland carcinomas, including the most prevalent tumor types. Methods. All patients diagnosed with mucoepidermoid carcinoma, adenoid cystic carcinoma, and epithelial-myoepithelial carcinoma, with the lung designated as the primary site between 1975 and 2019, were subject to analysis. Overall and disease-specific survival were calculated using Kaplan-Meier curves and Cox proportional hazards models. Results. The study identified 323 mucoepidermoid carcinoma, 284 adenoid cystic carcinoma, and 6 epithelial-myoepithelial carcinoma diagnosed as pulmonary salivary gland-type carcinoma. An analysis of age distribution revealed a unimodal pattern for both mucoepidermoid carcinoma and adenoid cystic carcinoma, with most patients diagnosed after age 40. Most patients were Caucasians (77% for mucoepidermoid carcinoma and 83% for adenoid cystic carcinoma). Both disease-specific and overall survival were worse for patients diagnosed at the age of 60 years or above. Race or sex did not significantly impact patient survival. High-grade mucoepidermoid carcinoma demonstrated a significantly worse prognosis than low or intermediate-grade mucoepidermoid carcinoma. Conclusion. A comprehensive review of clinical and epidemiological features of pulmonary salivary gland-type carcinomas reveals that the age of diagnosis and tumor grade are the most significant factors in determining patient survival.
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Asma , Proteínas de Fusão Oncogênica , Receptor alfa de Fator de Crescimento Derivado de Plaquetas , Fatores de Poliadenilação e Clivagem de mRNA , Humanos , Proteínas de Fusão Oncogênica/genética , Fatores de Poliadenilação e Clivagem de mRNA/genética , Asma/genética , Asma/patologia , Asma/complicações , Receptor alfa de Fator de Crescimento Derivado de Plaquetas/genética , Masculino , Eosinófilos/patologia , Eosinófilos/metabolismo , Eosinofilia/patologia , Eosinofilia/genética , Pessoa de Meia-Idade , FemininoRESUMO
Interferon É (IFNÉ) is a unique type I IFN that has been implicated in host defense against sexually transmitted infections. Zika virus (ZIKV), an emerging pathogen, can infect the female reproductive tract (FRT) and cause devastating diseases, particularly in pregnant women. How IFNÉ contributes to protection against ZIKV infection in vivo is unknown. In this study, we show that IFNÉ plays a critical role in host protection against vaginal ZIKV infection in mice. We found that IFNÉ was expressed not only by epithelial cells in the FRT but also by immune and stromal cells at baseline or after exposure to viruses or specific Toll-like receptor (TLR) agonists. IFNÉ-deficient mice exhibited abnormalities in the epithelial border and underlying tissue in the cervicovaginal tract, and these defects were associated with increased susceptibility to vaginal but not subcutaneous ZIKV infection. IFNÉ deficiency resulted in an increase in magnitude, duration, and depth of ZIKV infection in the FRT. Critically, intravaginal administration of recombinant IFNÉ protected IfnÉ-/- mice and highly susceptible Ifnar1-/- mice against vaginal ZIKV infection, indicating that IFNÉ was sufficient to provide protection even in the absence of signals from other type I IFNs and in an IFNAR1-independent manner. Our findings reveal a potentially critical role for IFNÉ in mediating protection against the transmission of ZIKV in the context of sexual contact.
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Interferon ε (IFNε) is a unique type I IFN that has been implicated in host defense against sexually transmitted infections (STIs). Zika virus (ZIKV), an emerging pathogen, can infect the female reproductive tract (FRT) and cause devastating diseases, particularly in pregnant women. How IFNε contributes to protection against ZIKV infection in vivo is unknown. Here, we show that IFNε plays a critical role in host protection against vaginal ZIKV infection in mice. We found that IFNε was expressed not only by epithelial cells in the FRT, but also by certain immune and other cells at baseline or after exposure to viruses or specific TLR agonists. IFNε-deficient mice exhibited abnormalities in the epithelial border and underlying tissue in the cervicovaginal tract, and these defects were associated with increased susceptibility to vaginal, but not subcutaneous ZIKV infection. IFNε-deficiency resulted in an increase in magnitude, duration, and depth of ZIKV infection in the FRT. Critically, intravaginal administration of recombinant IFNε protected Ifnε-/- mice and highly susceptible Ifnar1-/- mice against vaginal ZIKV infection, indicating that IFNε was sufficient to provide protection even in the absence of signals from other type I IFNs and in an IFNAR1-independent manner. Our findings reveal a potentially critical role for IFNε in mediating protection against transmission of ZIKV in the context of sexual contact.
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The majority of neoplasms of the breast are derived from epithelial components and give rise to carcinoma, namely invasive ductal and lobular carcinoma of the breast. Unlike carcinomas, primary hematolymphoid malignancies of the breast are a rare group of malignant neoplasms. Due to their rarity, these patients' epidemiological features and outcomes have not been studied well. A few limited case series and case reports suggest that this group of heterogeneous neoplasms has female predominance and poor prognosis. However, no systematic study exists to date. In order to bridge this knowledge gap, the National Cancer Institute's Surveillance, Epidemiology, and End Results databases have been quarried and analyzed to investigate the epidemiological and outcome features of primary hematolymphoid malignancies of the breast. This study is one of the first efforts to establish a systematic understanding of the demographic characteristics and the survival features of this rare group of malignancies.
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OBJECTIVES: Neoplasms arising from the vulva are uncommon and comprise various subtypes. Given the recent advancements in the molecular aspects of oncologic pathology and how they have impacted cancer treatment, an understanding of recent innovations in the molecular features of vulvar lesions is important. MATERIALS AND METHODS: Systematic literature search was performed on PubMed, Google Scholar, and Scopus databases for molecular and genetic characteristics of vulvar neoplasms. Peer-reviewed literature published in English is included. RESULTS: Squamous cell carcinoma (SCC) and its precursors are the predominant neoplasm at this site. Human papillomavirus (HPV) plays a crucial role in the pathogenesis of some of these lesions. Human papillomavirus-associated SCC follows the carcinogenic pathway driven by viral proteins E6 and E7 while HPV-independent SCC shows a high incidence of mutation of TP53 and CDKN2A genes. Mutations in the genes involving the PI3K-Akt pathway play an important role in the pathogenesis of both types of SCC. Among other vulvar malignancies, melanoma, and vulvar Paget disease (VPD) pose a significant clinical challenge and have unique molecular characteristics. Compared with dermal cutaneous melanoma, vulvar melanoma shows a higher rate of mutation of cKIT and NRAS genes and a lower rate of mutations in BRAF . Less than 20% of VPD shows amplification of ERBB2 and seldom shows mutation in genes involving the PI3K-Akt pathway. CONCLUSIONS: Several potentially targetable molecular pathways have emerged as they have been shown to be involved in the tumorigenesis of SCC, melanoma, and VPD.
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Carcinoma de Células Escamosas , Melanoma , Infecções por Papillomavirus , Neoplasias Cutâneas , Neoplasias Vulvares , Feminino , Humanos , Carcinoma de Células Escamosas/patologia , Papillomavirus Humano , Fosfatidilinositol 3-Quinases , Proteínas Proto-Oncogênicas c-akt , Neoplasias Vulvares/patologiaRESUMO
Introduction. Pleomorphic rhabdomyosarcoma (RMS) is an aggressive and rare malignant neoplasm with a poor prognosis. As its name suggests, this tumor exhibits extensive pleomorphism with features of skeletal muscle differentiation. Due to its rarity, its diagnosis is often a clinical and pathological challenge. Since only small case series and a few scattered case reports exist in the literature, the impact of different demographic features, tumor site, and/or treatment modality on patient outcomes has yet to be extensively studied. Methods. We report a case of a pleomorphic RMS presenting atypically as an abdominal wall mass. We have also analyzed the National Cancer Institute's Surveillance, Epidemiology and End Results (SEER) database to determine the factors affecting the outcome of this neoplasm. Moreover, we present a review and summary of pleomorphic RMS cases arising from the abdominal wall reported in the English language literature. Results. We found two hundred and forty-two cases of pleomorphic RMS in the SEER database. The majority of the patients were diagnosed after the age of 40, with the age of diagnosis showing a unimodal distribution. The majority of the patients were Caucasian (82%) and male (59%). Age of diagnosis, tumor stage, and surgical management significantly affected the patients' outcome, while patients' ethnicity, sex, or tumor site did not affect the outcome. We only found five previously reported cases of pleomorphic RMS arising from the abdominal wall. Conclusions. Pleomorphic RMS arising from the abdominal wall is extremely rare. Our data sheds light on the factors affecting the outcome of pleomorphic RMS. We have also discussed the challenges involving the histopathological diagnosis of this rare neoplasm and how to best approach this task.
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Parede Abdominal , Rabdomiossarcoma , Humanos , Masculino , Parede Abdominal/cirurgia , Parede Abdominal/patologia , Rabdomiossarcoma/diagnóstico , Rabdomiossarcoma/cirurgia , Rabdomiossarcoma/patologia , Diagnóstico DiferencialRESUMO
Endometrial stromal sarcoma (ESS) is the second most common uterine mesenchymal neoplasm. ESS can arise from extrauterine locations without any uterine involvement and is called extrauterine ESS (EESS). The epidemiological features of EESS are not well-known. Moreover, the factors affecting its outcome have not been systemically studied. The treatment of EESS closely follows that of uterine ESS, comprised of different combinations of surgical management, hormone therapy, chemotherapy, and radiation therapy. However, the effectiveness of different treatment protocols for EESS has not been studied. Here, we have performed a systematic review of all reported cases of EESS in the English literature. We further performed a meta-analysis of the outcome data and investigated how the patients' age, tumor site, tumor size, and management affect the overall and progression-free survival of the patients. We found that tumor site and mode of treatment significantly affected the overall survival and progression-free survival of the patients. Tumor size significantly affected overall survival but not progression-free survival, while the age at diagnosis did not affect patient outcome. As far as we know, ours is the first systematic study of this rare malignancy with an emphasis on outcome analysis.
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Neoplasias do Endométrio , Sarcoma do Estroma Endometrial , Neoplasias do Endométrio/patologia , Feminino , Humanos , Sarcoma do Estroma Endometrial/patologia , Sarcoma do Estroma Endometrial/terapiaRESUMO
Primary uterine angiosarcoma is an extremely rare neoplasm. Due to its rarity, knowledge regarding this malignancy is limited to a few scattered case reports. To better understand the prognostic factors and optimal management of these neoplasms, we have performed a systematic analysis of the disease. A systematic literature search of this entity yielded 25 reported cases of this entity. In addition, we searched the National Cancer Institute Surveillance, Epidemiology, and End Results database to find 4 additional cases and added a case diagnosed in our institution. We analyzed the patients' demographic characteristics and the different treatment protocols utilized to treat this malignancy. Patients were primarily treated by surgery with or without adjuvant chemotherapy and radiotherapy. Survival analysis was performed to examine the role of various factors in the outcome for the patients. There was no correlation with age of diagnosis, or treatment modality used, however, better outcomes were seen in patients presenting with smaller sized tumors. Our study is the first attempt to systematically study this rare malignancy in hopes of leading to a more standardized, evidence-based, and improved treatment protocol.
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Hemangiossarcoma , Neoplasias Uterinas , Quimioterapia Adjuvante , Feminino , Hemangiossarcoma/tratamento farmacológico , Hemangiossarcoma/terapia , Humanos , Análise de Sobrevida , Neoplasias Uterinas/tratamento farmacológico , Neoplasias Uterinas/terapia , Útero/patologiaRESUMO
Erosive pustular dermatosis of the scalp is an uncommon neutrophilic process representing a diagnostic and therapeutic challenge. It often occurs in older patients with prior sun exposure and manifests with hyperkeratosis and crust that may be difficult to distinguish from other inflammatory or neoplastic processes. Although erosive pustular dermatosis of the scalp may respond effectively to high potency topical steroids or other antiinflammatory regimens, caution is advised to avoid overlooking differential diagnoses that may not be mutually exclusive, especially squamous cell carcinoma.
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Dermatoses do Couro Cabeludo , Couro Cabeludo , Idoso , Anti-Inflamatórios/uso terapêutico , Diagnóstico Diferencial , Humanos , Dermatoses do Couro Cabeludo/diagnóstico , Dermatoses do Couro Cabeludo/tratamento farmacológicoRESUMO
Epstein-Barr virus (EBV) is a member of the herpes virus family and affects people worldwide. EBV-infected colitis can occur in patients in immunocompromised states. However, EBV-induced colitis in immunocompetent patients is extremely rare. A 34-year-old man with a prior history of possible intussusception, and who underwent a right hemicolectomy, presented with abdominal pain and rectal bleeding. Laboratory investigations were unremarkable, and the patient underwent a colonoscopy, which showed EBV-infected colitis. Both infectious and inflammatory bowel disease (IBD) workups were negative. The patient's symptoms were resolved with supportive therapy. A repeat colonoscopy showed normal colonic mucosa with an absence of EBV infection. Without evidence of IBD or infectious aetiologies, EBV-associated colitis is a rare finding in an immunocompetent patient.
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Colite , Infecções por Vírus Epstein-Barr , Doenças Inflamatórias Intestinais , Adulto , Colite/diagnóstico , Infecções por Vírus Epstein-Barr/complicações , Herpesvirus Humano 4 , Humanos , Doenças Inflamatórias Intestinais/complicações , MasculinoRESUMO
Endometrioid type of endometrial carcinoma is the most common form of uterine malignancy. The majority of patients in the developed world present with the low-grade, low-stage type of this malignancy. The current treatment of early-stage endometrioid carcinoma provides most patients with a favorable outcome. One of the important factors that determine the outcome of early-stage endometrial carcinoma is the involvement of cervical stroma. One of the very rare forms of cervical stromal involvement by endometrioid carcinoma is termed "adenoma malignum type" invasion due to its similarity to the infamously deceptive type of cervical adenocarcinoma called adenoma malignum. Since adenoma malignum is often discovered incidentally, finding adenoma malignum type of myoinvasion may deceive a pathologist to diagnose the simultaneous presence of endometrial carcinoma and adenoma malignum in the same patient as 2 separate entities. Also, this type of myoinvasion may be missed altogether for its subtle nature. In this article, we report a case of low-grade, low-stage endometrioid carcinoma with adenoma malignum type of myoinvasion. We have pointed out the subtle nature of this lesion and the important features to remember to successfully identify it.