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1.
Thorac Cancer ; 12(3): 339-348, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-33386778

RESUMO

BACKGROUND: Thymic epithelial tumors constitute a morphologically and clinically diverse group of rare neoplasm of the anterior mediastinum. METHODS: Here, we present an analysis of 188 patients diagnosed with primary thymic tumors between 1995 and 2015. The prognostic value of selected clinical and morphological factors was assessed in relation to overall survival and recurrence-free survival. RESULTS: The risk of recurrence increased significantly in thymic carcinoma diagnosis (P = 0.0036), co-occurrence of other diseases, and weight loss (P = 0.0012 and 0.0348, respectively). Multivariate analysis showed that the most important independent risk factor for disease recurrence was clinical stage IV (P = 0.0036). A total of 63 patients (33.5%) died. In the univariate analysis, the following factors were considered as independent prognostic factors for overall survival: clinical stage (P < 0.0001), histological type (P < 0.0001), lymph node involvement (P < 0.001), WHO performance status 2 (P < 0.0001), anemia (Hb <9.5 g/dL; P = 0.0002), leucocytosis (>12.5 G/L; P = 0.0011), LDH level (>185 U/L; P < 0.0001), concomitant diseases (P = 0.0012) and weight loss (P < 0.0001).The strongest independent risk factor for death was stage IV disease (P < 0.001). CONCLUSIONS: The results confirmed a fairly good prognosis for patients with thymic epithelial tumors. Clinical stage was the most important prognostic factor, but, some additional clinical factors may also have prognostic value.

2.
Virchows Arch ; 478(1): 111-128, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33389148

RESUMO

Thymic carcinoma encompasses a diverse group of rare tumors that occur almost exclusively in the prevascular (anterior) mediastinum. Thymic carcinomas have a worse outcome than thymomas with a median time to death of under 3 years. These tumors lack the typical lobulation of thymomas, exhibit commonly more cytologic atypia, are associated with a desmoplastic stromal reaction, and lack thymocytes, features that distinguish them from thymomas. The most common thymic carcinoma is squamous cell carcinoma; other subtypes include mucoepidermoid carcinoma, NUT carcinoma, and adenocarcinoma, among others. Largely due to multi-institutional and global efforts and meta-analysis of case reports and series, some of the thymic carcinoma subtypes have been studied in more detail and molecular studies have also been performed. Morphology and immunophenotype for the vast majority of thymic carcinoma subtypes are similar to their counterparts in other organs. Therefore, the distinction between thymic carcinoma and metastatic disease, which is relatively common in the prevascular mediastinum, can be challenging and in general requires clinical and radiologic correlation. Although surgical resection is the treatment of choice, only 46 to 68% of patients with thymic carcinoma can undergo resection as many other tumors present at high stage with infiltration into vital neighboring organs. These patients are usually treated with chemotherapy and/or radiation. The search for better biomarkers for prognosis and treatment of thymic carcinomas is important for improved management of these patients and possible targeted therapy.


Assuntos
Adenocarcinoma/patologia , Carcinoma de Células Escamosas/patologia , Timoma/patologia , Neoplasias do Timo/patologia , Adenocarcinoma/diagnóstico , Carcinoma de Células Escamosas/diagnóstico , Humanos , Prognóstico , Timoma/diagnóstico , Neoplasias do Timo/diagnóstico
3.
Am J Pathol ; 2020 Dec 08.
Artigo em Inglês | MEDLINE | ID: mdl-33307035

RESUMO

Primary mediastinal large B-cell lymphoma (PMBL) cells depend on the constitutive activity of NFκB and STAT transcription factors, which drive expression of multiple molecules essential for their survival. We have previously showed that in a molecularly-related B-cell malignancy, classical Hodgkin lymphoma (cHL), tumor Reed-Sternberg (RS) cells overexpress oncogenic PIM1/2/3 kinases (PIMs) in a NFκB- and STAT-dependent manner, and that PIMs enhance survival and expression of immunomodulatory molecules. Given the multiple overlapping characteristics of RS and PMBL cells, we hypothesized that PIM kinases may be overexpressed in PMBL and be involved in PMBL pathogenesis. Herein, we assessed the expression of PIM kinases in PMBL diagnostic biopsies and determined their role in survival and immune escape of the tumor cells. PIMs were abundantly expressed in primary tumors and PMBL cell lines. Inhibition of PIM kinases was toxic to PMBL cells, attenuated protein translation and downregulated NFκB-/STAT-dependent transcription of pro-survival factors BCL2A1, BCL2L1 and FCER2. Furthermore, PIM inhibition decreased expression of molecules engaged in shaping the immunosuppressive microenvironment, including PD-L1/2 and CCL17. Taken together, our data demonstrate that PIMs support PMBL cell survival and immune escape, and identify PIMs as promising therapeutic targets in PMBL.

4.
Transl Lung Cancer Res ; 9(3): 887-897, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32676354

RESUMO

A questionnaire on biomarker testing previously used in central European countries was extended and distributed in Western and Central European countries to the pathologists participating at the Pulmonary Pathology Society meeting 26-28 June 2019 in Dubrovnik, Croatia. Each country was represented by one responder. For recent biomarkers the availability and reimbursement of diagnoses of molecular alterations in non-small cell lung carcinoma varies widely between different, also western European, countries. Reimbursement of such assessments varies widely between unavailability and payments by the health care system or even pharmaceutical companies. The support for testing from alternative sources, such as the pharmaceutical industry, is no doubt partly compensating for the lack of public health system support, but it is not a viable or long-term solution. Ideally, a structured access to testing and reimbursement should be the aim in order to provide patients with appropriate therapeutic options. As biomarker enabled therapies deliver a 50% better probability of outcome success, improved and unbiased reimbursement remains a major challenge for the future.

6.
Adv Respir Med ; 88(2): 123-128, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32383463

RESUMO

INTRODUCTION: Transbronchial lung cryobiopsy (TBLC) is commonly used in diagnosing interstitial lung diseases (ILDs). Ageneral anesthesia with endotracheal intubation, balloon blockers and fluoroscopy control is the most common modality. Simplifying the procedure without decreasing it's safety could result in wider use. Prospective, observational study was conducted in three Polish pulmonology centers to evaluate safety and diagnostic yield of TBLC under conscious sedation, without intubation and bronchial blockers and with radial-EBUS guidance instead of fluoroscopy. MATERIAL AND METHODS: In patients suspected of ILD, in accordance with high resolution computer tomography (HRCT) selected lung segments were examined with radial-EBUS mini probe without aguide sheath. If the lung infiltrations were visible this locations were preferred. If not, specimens were taken from two different segments of the same lobe. Two to five biopsies with freezing time 5-8 seconds were performed. Moreover ultrasound examination was used to avoid injury of lung vessels. RESULTS: From March 2017 to September 2019 - 114 patients (M: 59, F: 55) of mean (SD) age 54 (14) years were included to the study on the basis of medical history and HRCT. Histopathology was conclusive in 90 (79%) patients and included 16 different diagnoses (sarcoidosis, EAA, COP predominantly). 24 inconclusive biopsies of unclassifiable pulmonary fibrosis were followed up. Complications included five cases (4.4%) of pneumothorax requiring achest tube drainage and aminor and moderate bleeding in few cases. There was no need for use of balloon bronchial blockers. CONCLUSIONS: TBLC under conscious sedation guided by radial EBUS mini-probe is novel, reasonable and safe technique for histological diagnosis of ILDs.

7.
Adv Respir Med ; 88(1): 41-93, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32153010

RESUMO

INTRODUCTION: This document presents the guidelines of the Polish Respiratory Society (PTChP, Polskie Towarzystwo Chorób Pluc) for diagnosis and treatment of idiopathic pulmonary fibrosis (IPF), developed by agroup of Polish experts. MATERIAL AND METHODS: The recommendations were developed in the form of answers to previously formulated questions concer-ning everyday diagnostic and therapeutic challenges. They were developed based on acurrent literature review using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. RESULTS: We formulated 28 recommendations for diagnosis (8), pharmacological treatment (12) as well as non-pharma-cological and palliative therapy (8). The experts suggest that surgical lung biopsy (SLB) not be performed in patients with the probable usual interstitial pneumonia (UIP) pattern, with an appropriate clinical context and unanimous opinion of a multidisciplinary team. The experts recommend using antifibrotic agents in IPF patients and suggest their use irrespective of the degree of functional impairment. As regards non-pharmacological and palliative treatment, strong re-commendations were formulated regarding pulmonary rehabilitation, oxygen therapy (in patients with chronic respiratory failure), preventive vaccinations as well as referring IPF patients to transplant centres. Table 1 presents an aggregate list of recommendations. CONCLUSIONS: The Polish Respiratory Society Working Group developed guidelines for IPF diagnosis and treatment.

9.
J Thorac Dis ; 11(6): 2498-2511, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31372287

RESUMO

Background: This study was an epidemiological analysis of all primary mediastinal neoplasms (PMNs) diagnosed between 2000 and 2016 at the National Tuberculosis and Lung Diseases Research Institute, Poland. Methods: All patients with any mediastinal abnormality were included in the analysis. The patients' age and gender were obtained from the institutional database. Results: From a cohort of 5,108 patients, 3,691 primary mediastinal lesions were found, including 1,005 (19%) PMNs: lymphomas (533, 53% of PMNs), thymomas (215, 21%), neurogenic tumors (NTs) (100, 10%), germ cell tumors (GCTs) (62, 6%), soft tissue tumors (STTs) (47, 5%) and thymic carcinomas/thymic neuroendocrine tumors (TCs/TNETs) (37 in total, 4%). The most frequent lymphomas were classical Hodgkin lymphomas [256] and primary mediastinal large B-cell lymphomas [163]. Type AB [73] predominated in thymomas and squamous cell carcinomas [9] and carcinoids [10] in TCs/TNETs. NTs encompassed mainly schwannomas [49], ganglioneuromas [21] and neurofibromas [10]. The most frequent STTs were hemangiomas [13] and lymphangiomas [11]. Lymphomas, thymomas and NT were more often in women, TCs/TNETs in men (P<0.001). Lymphomas predominated between the 2nd and 4th decade of life, NTs under the 3rd decade and thymic epithelial tumors between the 6th and 8th decade (P<0.001). There was no correlation between the subtypes of thymomas and the patients' gender (P=0.389) but it was found between histology and patients' age: in patients <30 years of age type B2 and B3 thymomas and >70 years of age AB type and micronodular thymomas with lymphoid stroma (P<0.001) predominated. In the group of GCTs half of them were malignant and these were noted exclusively in men. No correlation between subtypes of NTs or TCs/TNETs and patients' age and gender was found (P>0.05). Conclusions: PMNs are rare conditions thus awareness of basic epidemiology may be very helpful for final diagnosis.

11.
Adv Respir Med ; 87(2): 114-117, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31038723

RESUMO

Primary lung cancer manifesting as a thin-walled solitary cavity, occurs relatively infrequently. The most common histologic type presenting such a pattern is squamous cell cancer, followed by adenocarcinoma, and finally - large cell cancer. Cavitation is typically not seen in small cell lung cancer. Entities indicating malignancy of such lesions include irregular cystic wall, wall nodule formation, nodular septa or increased standard uptake on positron emission tomography (PET). We are presenting a case of a squamous cell lung cancer manifesting on chest CT as a thin-walled septated cavity with irregular margins mimicking a cyst. The lesion was reported unchanged in a follow-up computed tomography after 3 months. A follow-up scan obtained 2 years after initial examination showed thickening of a cyst wall, solid structures within its lumen and thoracic lymph nodes enlargement.


Assuntos
Carcinoma de Células Escamosas/diagnóstico por imagem , Neoplasias Pulmonares/diagnóstico por imagem , Nódulo Pulmonar Solitário/diagnóstico por imagem , Idoso , Carcinoma de Células Escamosas/patologia , Humanos , Neoplasias Pulmonares/patologia , Masculino , Estadiamento de Neoplasias , Nódulo Pulmonar Solitário/patologia , Tomografia Computadorizada por Raios X
15.
Nat Commun ; 9(1): 3927, 2018 09 25.
Artigo em Inglês | MEDLINE | ID: mdl-30254314

RESUMO

Lung cancer has several genetic associations identified within the major histocompatibility complex (MHC); although the basis for these associations remains elusive. Here, we analyze MHC genetic variation among 26,044 lung cancer patients and 20,836 controls densely genotyped across the MHC, using the Illumina Illumina OncoArray or Illumina 660W SNP microarray. We impute sequence variation in classical HLA genes, fine-map MHC associations for lung cancer risk with major histologies and compare results between ethnicities. Independent and novel associations within HLA genes are identified in Europeans including amino acids in the HLA-B*0801 peptide binding groove and an independent HLA-DQB1*06 loci group. In Asians, associations are driven by two independent HLA allele sets that both increase risk in HLA-DQB1*0401 and HLA-DRB1*0701; the latter better represented by the amino acid Ala-104. These results implicate several HLA-tumor peptide interactions as the major MHC factor modulating lung cancer susceptibility.


Assuntos
Mapeamento Cromossômico , Predisposição Genética para Doença/genética , Neoplasias Pulmonares/genética , Complexo Principal de Histocompatibilidade/genética , Grupo com Ancestrais do Continente Asiático/genética , Grupo com Ancestrais do Continente Europeu/genética , Feminino , Frequência do Gene , Predisposição Genética para Doença/etnologia , Genótipo , Antígenos HLA/genética , Humanos , Neoplasias Pulmonares/etnologia , Masculino , Pessoa de Meia-Idade , Peptídeos/genética , Polimorfismo de Nucleotídeo Único
16.
Adv Respir Med ; 86(3)2018.
Artigo em Inglês | MEDLINE | ID: mdl-29960280

RESUMO

Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary haemangiomatosis (PCH) are rare disorders, with the estimated prevalence of less than 1 case per million inhabitants. The vascular pathology in PVOD/PCH involves pre-septal and septal veins, alveolar capillaries and small pulmonary arteries. According to the ERS/ESC classification of pulmonary hypertension (PH) from 2015, PVOD/PCH have been included in the subgroup 1' of pulmonary arterial hypertension (PAH). Recent data indicate, however, the possibility of PVOD/PCH pathology in the patients diagnosed in the group 1. The problem may concern PAH associated with scleroderma, drug- induced PAH, PAH due to HIV infection and up to 10% of patients with idiopathic PAH (IPAH). Recently, bi-allelic EIF2AK4 mutations were found in the cases with heritable form of PVOD/PCH and in about 9% of sporadic cases. Moreover, an association between occupational exposure to organic solvents and PVOD/PCH was proved. The present review is an attempt to summarise the current data on pathogenesis, risk factors, clinical features and diagnostic algorithm for PVOD/PCH.


Assuntos
Pneumopatia Veno-Oclusiva/diagnóstico , Pneumopatia Veno-Oclusiva/etiologia , Algoritmos , Humanos , Proteínas Serina-Treonina Quinases/genética , Pneumopatia Veno-Oclusiva/genética , Fatores de Risco
18.
Adv Respir Med ; 86(2): 92-96, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29709049

RESUMO

Differentiation between pulmonary tuberculosis and lung cancer is often challenging for clinicians, especially that both conditions can coexist. This is due to the fact that the clinical and radiological symptoms of both diseases can be similar. Our case report presents a patient who was treated for advanced lung cancer 10 years earlier and currently has been hospitalized again because of a strong clinical and radiological suspicion of the cancer progression, but whose final diagnosis was tuberculosis.


Assuntos
Antituberculosos/administração & dosagem , Escarro/microbiologia , Tuberculose Pulmonar/diagnóstico por imagem , Tuberculose Pulmonar/microbiologia , Adulto , Diagnóstico Diferencial , Feminino , Humanos , Neoplasias Pulmonares/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Tuberculose Pulmonar/tratamento farmacológico
19.
J Thorac Dis ; 9(9): E808-E814, 2017 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-29221349

RESUMO

An atypical type A thymoma is a newly added entity to the last World Health Organization (WHO) histological classification [2015] of uncertain prognosis. The conventional type A and AB thymomas are usually locally aggressive neoplasms that rarely metastasize with distant metastases to the central nervous system (CNS) occurring extremely exceptionally. We present a history of a woman with a mediastinal tumor originally considered to be a Masaoka-Koga stage II "mixed thymoma with well-differentiated thymic carcinoma component" according to the historic Müller-Hermelink nomenclature. By applying the criteria of the new WHO classification the tumor should be reclassified as an AB thymoma with an atypical A component. The patient developed metastases to the lung and brain 10 and 15 years after the original diagnosis, respectively. All metastases morphologically corresponded to an atypical A component of primary thymoma. Molecular study revealed GTF2I mutations in the primary and one of the metastatic tumors. To our knowledge, this is the first description of a GTF2I mutation in AB thymoma with atypical A component and its metastases. The presented case highlights the necessity of an accurate microscopic search for atypical areas in A or AB thymomas because of their potentially negative impact on prognosis.

20.
J Vis Surg ; 3: 2, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-29078565

RESUMO

BACKGROUND: To present the technique of minimally invasive extended thymectomy performed through the subxiphoid-bilateral subcostal video-assisted thoracoscopic surgery (VATS) approach, with double elevation of the sternum for nonthymomatous myasthenia gravis (MG). METHODS: The whole dissection was performed through the 4-7 cm transverse subxiphoid incision with single 10 mm extra-long bariatric laparoscopy ports inserted subcostally to the right and left chest cavities for videothoracoscope and subsequently for chest tubes. The sternum was elevated with two hooks connected to the sternal frame (Rochard bar, Aesculap-Chifa, Nowy Tomysl, Poland). The lower hook was inserted through the subxiphoid incision and the superior hook was inserted percutaneously, after the mediastinal tissue including the major mediastinal vessels were dissected from the inner surface of the sternum. The fatty tissue of the anterior mediastinum and the aorta-pulmonary window was completely removed. RESULTS: There were 147 patients (62 patients operated on for thymomas, 75 patients for nonthymomatous MG and 10 for rethymectomies) in the period 1.1.2009-30.3.2016. There was no mortality and morbidity. The mean operative time was 109.1 min (range, 75-150 min). CONCLUSIONS: The subxiphoid approach combined with bilateral single port subcostal VATS and double elevation of the sternum enables very extensive thymectomy in case of nonthymomayous MG.

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