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1.
Zhonghua Jie He He Hu Xi Za Zhi ; 47(4): 325-331, 2024 Apr 12.
Artigo em Chinês | MEDLINE | ID: mdl-38599807

RESUMO

Objective: To analyze the clinical characteristics and prognosis of 17 patients with pathologically confirmed SMARCA4-deficient chest tumors. Methods: Seventeen patients with SMARCA4-deficient thoracic tumors diagnosed by pathology in the Affiliated Hospital of Jining Medical University from September 2021 to January 2023 were collected through Results Query System of Pathology Department, and the patients' general conditions, clinical symptoms, tumor markers, imaging features, treatment and regression were retrospectively analyzed, and literature review was performed. Results: A total of 17 patients were included in this study. Their clinical characteristics were characterized as follows: male/female=16/1, age 42-74 years, mean (64.0±5.7)years. Only 1 female had no clear smoking history, and 16 males had a smoking history, of whom 1 had 5 smoking pack-years, and the remaining 15 case had a smoking history of 20-100 smoking pack-years, with a mean of (68.5±44.5) smoking pack-years. Clinical symptoms were mainly cough and sputum, followed by chest tightness, hemoptysis and chest pain. Tumor markers CYFRA19-9 was elevated in 9 cases (3.79-16.61 ng/ml), CEA was elevated in 8 cases (5.37-295.93 ng/ml), and NSE was elevated in 6 cases (17.18-70.37 ng/ml). Imaging manifestations were intrapulmonary or mediastinal mass shadows, and the tumor involved the mediastinum in 9 cases, the upper lobe of the right lung in 6 cases, the upper lobe of the left lung in 5 cases, the lower lobe of the right lung in 3 cases, the lower lobe of the left lung in 3 cases; cervical or supraclavicular lymph node metastasis in 8 cases, pleural metastasis in 4 cases, hepatic metastasis in 3 cases, cerebral metastasis in 3 cases, bone metastasis in 2 cases, and subcutaneous metastasis in 1 case. Combining immuno-histochemistry and pathology, there were 6 cases of SMARCA4-deficient NSCLC and 11 cases of SMARCA4-deficient undifferentiated tumor. Eight patients were treated with platinum-contained chemotherapy agents, four of which were combined with immune checkpoint inhibitors, and one was treated with enzatinib; only one of the 9 patients achieved partial remission after treatment, and the remaining eight had progression of the tumors on chest CT after treatment. Five patients abandoned the treatment, and died in 6-month of follow-up. Three patients underwent surgery for resection, and there was no significant progression in the three patients in the 6 months of follow-up. Conclusions: Clinically, middle-aged and elderly men with a history of heavy smoking should be given high priority, especially in patients whose imaging mostly showed intrapulmonary, especially in upper lobes, and/or mediastinal masses, rapid lesion progression, and early distant metastasis, and who should be alerted to the possibility of SMARCA4-deficient thoracic tumors. Late clinical stage is a high risk factor for poor overall patient survival, and platinum-containing chemotherapy agents combined with immune checkpoint inhibitor therapy may be effective, and early surgery may improve patient prognosis.


Assuntos
Carcinoma Pulmonar de Células não Pequenas , Neoplasias Pulmonares , Neoplasias Torácicas , Pessoa de Meia-Idade , Idoso , Humanos , Masculino , Feminino , Adulto , Estudos Retrospectivos , Platina , Prognóstico , Neoplasias Torácicas/patologia , Neoplasias Pulmonares/patologia , Biomarcadores Tumorais , DNA Helicases , Proteínas Nucleares , Fatores de Transcrição
2.
Lung Cancer ; 190: 107513, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38432027

RESUMO

OBJECTIVES: Cancer is a disease of old age; however, most studies usually included minority of patients fit elderly. The purpose is to investigate the clinical characteristics and genetic information of patients with thoracic tumors who are 80 years old or older compared to those under 80 years old. STUDY DESIGN AND METHODS: The Thoracic Tumor Registry (TTR) is a Spanish observational, prospective cohort study that included patients diagnosed with thoracic tumors. Data were collected from medical records related to sociodemographic, epidemiological, clinical, molecular/genetic, and treatment outcome variables. RESULTS: The total number of patients, recruited from August 2016 to April 2023, was 26.193 (93,1 % were younger than 80 years and 6,9 % were 80 years or older). In the group of older patients: the male ratio increased (72,9 % vs. 80 %); the number of elderly people who had never smoked or were ex-smokers increased (9,9 % vs. 21,1 % and 44,8 % vs. 61,3 %, respectively) and the number of current smokers decreased (43,3 % vs. 17,5 %); had higher ECOG performance status at diagnosis (for ECOG ≥ 2, 15 % vs. 32,9 %), and there were more patients with previous cancer (17,3 % vs. 28 %). The proportion of men is higher than that of women (73 % vs. 27 % in <80 years and 80 % vs. 20 % in ≥80 years). For all biomarkers, the proportion of patients who had a molecular determination was lower in older patients. There were no differences in terms of alterations in the biomarkers tested; except for EGFR, for which the positivity rate was higher in patients aged 80 years and older (25 % vs. 15,3 %). CONCLUSION: The proportion of older patients with targeted mutations is higher. So, at least at diagnosis, it should be proceeded in a standard way. Then, when it comes to treatment, comorbidities and patient's baseline situation should be considered. CLINICAL TRIAL REGISTRATION: NCT02941458.


Assuntos
Neoplasias Pulmonares , Neoplasias Torácicas , Idoso , Humanos , Masculino , Feminino , Idoso de 80 Anos ou mais , Neoplasias Pulmonares/epidemiologia , Neoplasias Pulmonares/terapia , Estudos Prospectivos , Neoplasias Torácicas/epidemiologia , Sistema de Registros , Biomarcadores , Análise de Dados
3.
Cancer Med ; 13(4): e6839, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38457231

RESUMO

BACKGROUND: Mannitol is exclusively recommended in the National Comprehensive Cancer Network guidelines for diuresis in cisplatin (CDDP)-based chemotherapy. The utility of furosemide, a widely used and convenient diuretic, thus requires clarification. METHODS: This is a prospective, single-centered, open-label, noninferiority phase II study. Patients with thoracic malignancies who planned to receive CDDP-based chemotherapy were randomly assigned to receive either mannitol (arm A) or furosemide (arm B). The primary end point was set as the proportion of patients who experienced any grade of "creatinine (Cr) increased" based on the upper limit of the normal range (ULN) during the first cycle as assessed by Common Terminology Criteria for Adverse Events Version 4.0. Secondary end points were Cr increased based on the baseline value during the first cycle, Cr increased after the completion of CDDP, and the proportion of patients with phlebitis. RESULTS: Between April 2018 and March 2022, 115 patients were enrolled and 106 were analyzed. Any grade of Cr increased based on the ULN during the first cycle was 17.3% (arm A) and 24.1% (arm B), respectively (p = 0.34). Therefore, the primary end point was not met. After completion of chemotherapy, any grade of Cr increased was observed in 23.1% (arm A) and 31.5% (arm B), respectively. However, the actual serum Cr level and Cr clearance during the courses were not different between the arms. Phlebitis occurred more frequently in arm A (28.8%) than arm B (16.7%). CONCLUSIONS: Mannitol should remain the standard diuresis in CDDP-based chemotherapy assessed by conventional CTCAE grading, but furosemide can be room for consideration when assessed by actual serum Cr level and Cr clearance.


Assuntos
Flebite , Neoplasias Torácicas , Humanos , Furosemida/efeitos adversos , Cisplatino/efeitos adversos , Manitol/efeitos adversos , Estudos Prospectivos , Flebite/induzido quimicamente , Flebite/tratamento farmacológico
4.
Curr Oncol ; 31(3): 1389-1399, 2024 Mar 07.
Artigo em Inglês | MEDLINE | ID: mdl-38534938

RESUMO

Although cancer care is often contextualized in terms of survival, there are other important cancer care outcomes, such as quality of life and cost of care. The ASCO Value Framework assesses the value of cancer therapies not only in terms of survival but also with consideration of quality of life and financial cost. Early palliative care for patients with advanced cancer is associated with improved quality of life, mood, symptoms, and overall survival for patients, as well as cost savings. While palliative care has been shown to have numerous benefits, the impact of real-world implementation of outpatient embedded palliative care on value-based metrics is not fully understood. We sought to describe the association between outpatient embedded palliative care in a multidisciplinary thoracic oncology clinic and inpatient value-based metrics. We performed a retrospective cohort study of 215 patients being treated for advanced thoracic malignancies with non-curative intent. We evaluated the association between outpatient embedded palliative care and inpatient clinical outcomes including emergency room visits, hospitalizations, intensive care unit admissions, hospital charges, as well as hospital quality metrics including 30-day readmissions, admissions within 30 days of death, inpatient mortality, and inpatient hospital charges. Outpatient embedded palliative care was associated with lower hospital charges per day (USD 3807 vs. USD 4695, p = 0.024). Furthermore, patients who received outpatient embedded palliative care had lower hospital admissions within 30 days of death (O.R. 0.45; 95% CI 0.29, 0.68; p < 0.001) and a lower inpatient mortality rate (IRR 0.67; 95% CI 0.48, 0.95; p = 0.024). Our study further supports that outpatient palliative care is a high-value intervention and alternative models of palliative care, including one embedded into a multidisciplinary thoracic oncology clinic, is associated with improved value-based metrics.


Assuntos
Cuidados Paliativos , Neoplasias Torácicas , Humanos , Pacientes Ambulatoriais , Estudos Retrospectivos , Qualidade de Vida
5.
PeerJ ; 12: e16923, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38374950

RESUMO

Background: Thoracic SMARCA4-deficient undifferentiated tumor (SMARCA4-UT) is a distinct clinicopathological entity with an aggressive clinical course. Additionally, SMARCA4/BRG1 deficiency can be observed in a few patients with non-small cell lung cancer (NSCLC). We aimed to compare the clinicopathological, immunohistochemical and prognostic features of SMARCA4-deficient NSCLC (SMARCA4-dNSCLC) with those of thoracic SMARCA4-UT. Methods: Patients with BRG1-deficient tumors in the lung or thorax were enrolled in the study from the Department of Pathology of West China Hospital, Sichuan University, from January 2014 to June 2022. We retrospectively collected the clinicopathological and immunohistochemical features and outcomes of these patients. Results: Seventy-two patients had tumors in the lung or thorax with BRG1-deficient expression, including 52 patients with SMARCA4-dNSCLC and 20 patients with thoracic SMARCA4-UT. Among the patients with SMARCA4-dNSCLC, 98.1% were male, 85.7% were smokers, and 79.5% (35/44) had tumor-node-metas-tasis (TNM) III-IV tumors. Among the patients with thoracic SMARCA4-UT, all were males who smoked, and 93.75% (15/16) had TNM III-IV tumors. Pure solid architecture and necrosis were the predominant pathological features. Rhabdoid morphology was observed in some SMARCA4-dNSCLCs (10/52, 19.2%) and thoracic SMARCA4-UTs (11/20, 55%). In most patients with thoracic SMARCA4-UT, the tumors exhibited scattered weak expression or negative expression of epithelial markers, and positive expression of CD34 and Syn. Overall survival (OS) and progression-free survival (PFS) were not significantly different between patients with SMARCA4-dNSCLC and patients with thoracic SMARCA4-UT (p = 0.63 and p = 0.20, respectively). Conclusions: Thoracic SMARCA4-DTs include SMARCA4-dNSCLC and thoracic SMARCA4-UT. Both have overlapping clinicopathological features and poor prognosis. We hypothesize that thoracic SMARCA4-UT may be the undifferentiated or dedifferentiated form of SMARCA4-dNSCLC. However, further studies with larger cohorts and longer follow-up periods are needed.


Assuntos
Carcinoma Pulmonar de Células não Pequenas , Neoplasias Pulmonares , Neoplasias Torácicas , Humanos , Masculino , Feminino , Carcinoma Pulmonar de Células não Pequenas/genética , Estudos Retrospectivos , Neoplasias Pulmonares/diagnóstico por imagem , Biomarcadores Tumorais/genética , Neoplasias Torácicas/genética , DNA Helicases/genética , Proteínas Nucleares/genética , Fatores de Transcrição/genética
6.
Radiat Oncol ; 19(1): 18, 2024 Feb 05.
Artigo em Inglês | MEDLINE | ID: mdl-38317205

RESUMO

PURPOSE: This study seeks to examine the influence of the heartbeat on the position, volume, and shape of the heart and its substructures during various breathing states. The findings of this study will serve as a valuable reference for dose-volume evaluation of the heart and its substructures in radiotherapy for treating thoracic tumors. METHODS: Twenty-three healthy volunteers were enrolled in this study, and cine four-dimensional magnetic resonance images were acquired during periods of end-inspiration breath holding (EIBH), end-expiration breath holding (EEBH), and deep end-inspiration breath holding (DIBH). The MR images were used to delineate the heart and its substructures, including the heart, pericardium, left ventricle (LV), left ventricular myocardium, right ventricle (RV), right ventricular myocardium (RVM), ventricular septum (VS), atrial septum (AS), proximal and middle portions of the left anterior descending branch (pmLAD), and proximal portion of the left circumflex coronary branch (pLCX). The changes in each structure with heartbeat were compared among different respiratory states. RESULTS: Compared with EIBH, EEBH increased the volume of the heart and its substructures by 0.25-3.66%, while the average Dice similarity coefficient (DSC) increased by - 0.25 to 8.7%; however, the differences were not statistically significant. Conversely, the VS decreased by 0.89 mm in the left-right (LR) direction, and the displacement of the RV in the anterior-posterior (AP) direction significantly decreased by 0.76 mm (p < 0.05). Compared with EIBH and EEBH, the average volume of the heart and its substructures decreased by 3.08-17.57% and 4.09-20.43%, respectively, during DIBH. Accordingly, statistically significant differences (p < 0.05) were observed in the volume of the heart, pericardium, LV, RV, RVM, and AS. The average DSC increased by 0-37.04% and - 2.6 to 32.14%, respectively, with statistically significant differences (p < 0.05) found in the right ventricular myocardium and interatrial septum. Furthermore, the displacements under DIBH decreased in the three directions (i.e.,- 1.73 to 3.47 mm and - 0.36 to 2.51 mm). In this regard, the AP displacement of the heart, LV, RV, RVM, LR direction, LV, RV, and AS showed statistically significant differences (p < 0.05). The Hausdorff distance (HD) of the heart and its substructures under the three breathing states are all greater than 11 mm. CONCLUSION: The variations in the displacement and shape alterations of the heart and its substructures during cardiac motion under various respiratory states are significant. When assessing the dose-volume index of the heart and its substructures during radiotherapy for thoracic tumors, it is essential to account for the combined impacts of cardiac motion and respiration.


Assuntos
Coração , Neoplasias Torácicas , Humanos , Frequência Cardíaca , Respiração , Ventrículos do Coração , Suspensão da Respiração , Planejamento da Radioterapia Assistida por Computador/métodos
7.
Thorac Cancer ; 15(10): 847-851, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38390699

RESUMO

SMARCA4-deficient undifferentiated thoracic tumors are a rare phenomenon. A 40-year-old male was newly diagnosed with SMARCA4-deficient undifferentiated non-small cell lung cancer. He had a history of heavy smoking and job-related exposure to metal dust and melted nickel. CT imaging showed numerous right-sided pleural masses and soft tissue plaques, but no metastases. CT-guided biopsy of a pleural mass confirmed the diagnosis. He was prescribed six cycles of carboplatin paclitaxel, and follow-up imaging showed largely stable disease. Treatment was changed to nivolumab due to shortness of breath, and he received one cycle of nivolumab without considerable side effects. Unfortunately, during the second cycle of his nivolumab, the patient presented with new weakness. Imaging showed spinal cord metastasis and he underwent a laminectomy; he was subsequently followed up as an outpatient. The objective of this publication was to explore SMARCA4-deficient undifferentiated thoracic tumors, other related SMARCA4-deficient tumors, and their overall pattern of presentation. The genetic aberrations of this case are compared to recent publications that also discuss genetic aberrations commonly occurring with this disease process, with an ultimate goal of hastening detection and adding to the library of treatment results.


Assuntos
Carcinoma Pulmonar de Células não Pequenas , Neoplasias Pulmonares , Sarcoma , Neoplasias Torácicas , Masculino , Humanos , Adulto , Nivolumabe , Neoplasias Pulmonares/genética , Neoplasias Pulmonares/diagnóstico , Sarcoma/patologia , Neoplasias Torácicas/patologia , Biomarcadores Tumorais/genética , DNA Helicases/genética , Proteínas Nucleares/genética , Fatores de Transcrição/genética
9.
Zhonghua Bing Li Xue Za Zhi ; 53(2): 143-148, 2024 Feb 08.
Artigo em Chinês | MEDLINE | ID: mdl-38281781

RESUMO

Objective: To investigate the clinicopathological characteristics of SMARCA4-deficient thoracic undifferentiated tumors, and the diagnostic value of the cells in serous effusion. Methods: Eleven cases of SMARCA4-deficient tumor were collected from the Affiliated Hospital of Hebei University, China from January 2018 to July 2023, which were diagnosed using cell block of serous effusion. The clinical, histopathological, immunohistochemical and molecular genetic features were reviewed, along with related literature. Results: All the 11 patients were males with ages ranging from 54 to 77 years (median 64 years). Nine patients were smokers and two had an unknown smoking history. Most of them complained of cough and dyspnea with pleural effusion. The primary tumor sites included lung (9 cases), thoracic wall (1 case), and mediastinum (1 case), while 3 patients had a history of lung surgery. Histologically, tumor cells were large and pleomorphic, with increased nuclear-cytoplasmic ratio. They also showed round nuclei, conspicuous nucleoli, and basophilic cytoplasm in serous effusion. Immunohistochemically, tumor cells in all cases were negative for SMARCA4/BRG1, CKpan and CK7, but positive for SMARCB1/INI1. Some of the cases were positive for CD34 (7/11), synaptophysin (4/11) and SALL4 (2/11). Histologically, monotonous tumor cells formed solid sheets or anastomosing islands with poor cell adhesion and rhabdoid morphology. Brisk mitotic figures were accompanied by large areas of necrosis. Some cases focally exhibited syncytia, and some had bright cytoplasm and vesicular chromatin. The immunohistochemical profiles in the tumor tissues were consistent with those of cytology. Six cases were negative for PD-L1 (22c3). Among the 6 cases analyzed by targeted next generation sequencing, concurrent SMARCA4 and TP53 mutations were detected in all 6 cases. Some of the 6 tumors showed mutations of STK11, CDKN2A, and MET, and amplification of ERBB2, exon deletion of BRCA2, etc. Follow-up information was available in all cases and ranged from 2 to 24 months. The patients showed metastases to various sites, including lymph node, liver, kidney, adrenal gland, brain, bone and other sites. Four patients died of the tumor. The survival time of 4 patients who underwent radical resection or radiofrequency ablation was more than 13 months. Conclusions: SMARCA4-deficient thoracic sarcoma is a rare but highly aggressive tumor with dismal prognosis and rhabdomyoid features. It is difficult to diagnose this disease using only serous effusion samples. This tumor thus warrants careful consideration. Accurate diagnosis can greatly improve early diagnosis and treatment of these tumors.


Assuntos
Neoplasias Torácicas , Humanos , Masculino , Biomarcadores Tumorais/genética , Núcleo Celular/metabolismo , DNA Helicases/genética , Mutação , Proteínas Nucleares/genética , Prognóstico , Fatores de Transcrição/genética , Pessoa de Meia-Idade , Idoso , Neoplasias Torácicas/patologia
11.
Pediatr Pulmonol ; 59(2): 371-378, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37975500

RESUMO

OBJECTIVE: Endobronchial ultrasound-guided transbronchial biopsy and needle aspiration (EBUS-TBB/EBUS-TBNA) are first line investigative modalities for lung and mediastinal pathology in adults. We aimed to characterize and assess the diagnostic yield of EBUS and virtual CT navigation guided biopsies in children. STUDY DESIGN: This single center, retrospective cohort study included patients who underwent radial or linear EBUS procedures (+/- CT navigation) for biopsy of mediastinal lymph nodes, tumors, and pulmonary nodules. Demographic, procedural, and outcome were collected. RESULTS: Sixty procedures were performed in 56 patients aged 2-22 years of age between January 2015 and May 2023. The most common indications for biopsy were pulmonary nodules (45%) and hilar/mediastinal lymphadenopathy (33%). For cases in which a final diagnosis was ascertained by any means, the diagnostic yield for linear EBUS (mediastinal pathology) was 76% and the diagnostic yield from radial EBUS (pulmonary nodules and lung masses) was 85%. The most common diagnoses were infection (45%), malignancy (17%), and sarcoidosis (11%). Among patients in whom infection was the final diagnosis, a total of 31 pathogens were identified. Eighteen were identified on bronchoalveolar lavage and an additional 14 pathogens identified on EBUS-TBB, representing an increase of 77% (p < .005). The sensitivity, specificity, negative and positive predictive values for malignancy detection were 73%, 100%, 94%, and 100%, respectively. CONCLUSION: EBUS-TBB/TBNA is a safe and effective way to diagnose lung and mediastinal pathology in children. Pediatric interventional pulmonology is a growing field offering minimally-invasive diagnostic opportunities for children in whom more invasive procedures were previously the only option.


Assuntos
Neoplasias Pulmonares , Linfadenopatia , Doenças do Mediastino , Neoplasias Torácicas , Adulto , Criança , Humanos , Broncoscopia/métodos , Estudos Retrospectivos , Mediastino/patologia , Linfonodos/diagnóstico por imagem , Linfonodos/patologia , Doenças do Mediastino/diagnóstico , Neoplasias Torácicas/patologia , Aspiração por Agulha Fina Guiada por Ultrassom Endoscópico/métodos , Sensibilidade e Especificidade , Neoplasias Pulmonares/diagnóstico por imagem , Neoplasias Pulmonares/patologia
12.
Radiother Oncol ; 190: 110034, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38030080

RESUMO

BACKGROUND/PURPOSE: Central/ultra-central thoracic tumors are challenging to treat with stereotactic radiotherapy due potential high-grade toxicity. Stereotactic MR-guided adaptive radiation therapy (SMART) may improve the therapeutic window through motion control with breath-hold gating and real-time MR-imaging as well as the option for daily online adaptive replanning to account for changes in target and/or organ-at-risk (OAR) location. MATERIALS/METHODS: 26 central (19 ultra-central) thoracic oligoprogressive/oligometastatic tumors treated with isotoxic (OAR constraints-driven) 5-fraction SMART (median 50 Gy, range 35-60) between 10/2019-10/2022 were reviewed. Central tumor was defined as tumor within or touching 2 cm around proximal tracheobronchial tree (PBT) or adjacent to mediastinal/pericardial pleura. Ultra-central was defined as tumor abutting the PBT, esophagus, or great vessel. Hard OAR constraints observed were ≤ 0.03 cc for PBT V40, great vessel V52.5, and esophagus V35. Local failure was defined as tumor progression/recurrence within the planning target volume. RESULTS: Tumor abutted the PBT in 31 %, esophagus in 31 %, great vessel in 65 %, and heart in 42 % of cases. 96 % of fractions were treated with reoptimized plan, necessary to meet OAR constraints (80 %) and/or target coverage (20 %). Median follow-up was 19 months (27 months among surviving patients). Local control (LC) was 96 % at 1-year and 90 % at 2-years (total 2/26 local failure). 23 % had G2 acute toxicities (esophagitis, dysphagia, anorexia, nausea) and one (4 %) had G3 acute radiation dermatitis. There were no G4-5 acute toxicities. There was no symptomatic pneumonitis and no G2 + late toxicities. CONCLUSION: Isotoxic 5-fraction SMART resulted in high rates of LC and minimal toxicity. This approach may widen the therapeutic window for high-risk oligoprogressive/oligometastatic thoracic tumors.


Assuntos
Neoplasias Pulmonares , Lesões por Radiação , Radiocirurgia , Neoplasias Torácicas , Humanos , Planejamento da Radioterapia Assistida por Computador/métodos , Recidiva Local de Neoplasia , Radiocirurgia/métodos , Neoplasias Torácicas/radioterapia , Imageamento por Ressonância Magnética/métodos , Neoplasias Pulmonares/diagnóstico por imagem , Neoplasias Pulmonares/radioterapia , Neoplasias Pulmonares/patologia
13.
Pediatr Blood Cancer ; 71(3): e30821, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38148490

RESUMO

BACKGROUND AND AIMS: Nuclear protein of the testis (NUT) carcinoma (NC) is a rare and highly aggressive tumor defined by the presence of a somatic NUTM1 rearrangement, occurring mainly in adolescents and young adults. We analyzed the clinical and biological features of German pediatric patients (≤18 years) with NC. METHODS: This study describes the characteristics and outcome of 11 children with NC registered in the German Registry for Rare Pediatric Tumors (STEP). RESULTS: Eleven patients with a median age of 13.2 years (range 6.6-17.8) were analyzed. Malignant misdiagnoses were made in three patients. Thoracic/mediastinal tumors were found to be the primary in six patients, head/neck in four cases; one patient had multifocal tumor with an unknown primary. All patients presented with regional lymph node involvement, eight patients (72.7%) with distant metastases. Seven patients underwent surgery, eight radiotherapy with curative intent; polychemotherapy was administered in all patients. Novel treatment strategies including immunotherapy, targeted therapies, and virotherapy were applied in three patients. Median event-free survival and overall survival were 1.5 and 6.5 months, respectively. CONCLUSIONS: Every undifferentiated or poorly differentiated carcinoma should undergo testing for the specific rearrangement of NUTM1, in order to initiate an intense therapeutic regimen as early as possible. As in adults, only few pediatric patients with NC achieve prolonged survival. Thus, novel therapeutic strategies should be included and tested in clinical trials.


Assuntos
Carcinoma , Neoplasias Torácicas , Masculino , Adulto Jovem , Adolescente , Humanos , Criança , Proteínas de Neoplasias , Fatores de Transcrição , Testículo/patologia
14.
Jpn J Clin Oncol ; 54(3): 265-270, 2024 Mar 09.
Artigo em Inglês | MEDLINE | ID: mdl-38117955

RESUMO

Thoracic SMARCA4-deficient undifferentiated tumor is a newly recognized disease entity characterized as a high-grade malignant neoplasm with an undifferentiated or rhabdoid phenotype. The tumor was initially identified as a subtype of thoracic sarcoma with SMARCA4 loss, but further investigation resulted in its classification as a subtype of epithelial malignancies in the current World Health Organization classification. SMARCA4-deficient undifferentiated tumor is highly aggressive and has a poor prognosis. Because of its rarity, an optimal treatment strategy has not yet been identified. In this review, we summarize the literature on SMARCA4-deficient undifferentiated tumor in terms of its clinical characteristics, diagnosis, treatment strategy and future perspectives.


Assuntos
Sarcoma , Neoplasias de Tecidos Moles , Neoplasias Torácicas , Humanos , Sarcoma/diagnóstico , Biomarcadores Tumorais/genética , Neoplasias Torácicas/genética , Neoplasias Torácicas/diagnóstico , Neoplasias Torácicas/patologia , DNA Helicases/genética , Proteínas Nucleares/genética , Fatores de Transcrição/genética
15.
Histopathology ; 84(1): 86-101, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37873676

RESUMO

NUT carcinoma and thoracic SMARCA4-deficient undifferentiated tumour are unique entities in the 5th edition of the World Health Organisation (WHO) Classification of Thoracic Tumours, whose definitions include molecular genetic abnormalities. These aggressive tumours require rapid work-ups on biopsies, but a broad list of differential diagnoses poses challenges for practising pathologists. This review provides an update on their key clinicopathological and molecular characteristics, as well as controversies regarding tumour classification and diagnostic strategy. Phenotypical assessment plays a substantial role in diagnosis because recurrent and predictable clinicopathological findings exist, including robust immunohistochemical phenotypes. Accurate diagnosis is crucial for appropriate management and a clearer understanding of the disease.


Assuntos
Carcinoma , Neoplasias Torácicas , Humanos , Fatores de Transcrição/genética , Proteínas Nucleares/genética , DNA Helicases/genética , Biomarcadores Tumorais , Carcinoma/diagnóstico , Carcinoma/patologia , Neoplasias Torácicas/diagnóstico , Neoplasias Torácicas/genética , Neoplasias Torácicas/patologia
16.
Histopathology ; 84(1): 3-5, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38086737
17.
Khirurgiia (Mosk) ; (10): 60-70, 2023.
Artigo em Russo | MEDLINE | ID: mdl-37916559

RESUMO

OBJECTIVE: To analyze the outcomes after different methods of post-resection chest wall defect reconstruction. MATERIAL AND METHODS: The study included 41 patients aged 22-73 years who underwent chest wall repair with local tissues and synthetic materials. Twelve (29.3±7.1%) patients had sarcoma, 9 (21.9±5.9%) - non-small cell lung cancer (NSCLC) with invasion of the chest, 9 (21.9±5.9%) - metastatic lesions, 8 (19.5±6.2%) - benign tumors, 2 (4.8±3.4%) - breast cancer with invasion of the chest wall, 1 (2.4±2.4%) - desmoid tumor. Seven patients were diagnosed with T3N0M0, 1 - T3N2M0, 1 - T2N0M1b (oss). Among patients with NSCLC with invasion into the chest wall, squamous cell cancer was verified in 4 (44.4±16.6%) patients, adenocarcinoma - in 4 (44.4±16.6%), neuroendocrine tumor - in 1 (11.2±10.5%) patient. Stages of surgeries are presented. RESULTS: We analyzed treatment outcomes in 41 patients. Five (12.2%) patients had seroma, hemothorax, thoracopleural fistula, subcutaneous emphysema and fatal asystole. There were no postoperative complications associated with paradoxical breathing. CONCLUSION: Accurate morphological verification prior to treatment is valuable to determine the stages of combined treatment of chest wall tumors. Chest wall defect closure with own tissues and synthetic materials is necessary after extensive resections. A multidisciplinary approach involving thoracic and plastic surgeons is needed.


Assuntos
Carcinoma Pulmonar de Células não Pequenas , Neoplasias Pulmonares , Procedimentos de Cirurgia Plástica , Neoplasias Torácicas , Parede Torácica , Humanos , Parede Torácica/cirurgia , Carcinoma Pulmonar de Células não Pequenas/cirurgia , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/cirurgia , Neoplasias Pulmonares/patologia , Resultado do Tratamento , Neoplasias Torácicas/diagnóstico , Neoplasias Torácicas/cirurgia
19.
Lung Cancer ; 186: 107423, 2023 12.
Artigo em Inglês | MEDLINE | ID: mdl-37995456

RESUMO

BACKGROUND: Patients with thoracic malignancies who develop COVID-19 infection have a higher hospitalization rate compared to the general population and to those with other cancer types, but how this outcome differs by race and ethnicity is relatively understudied. METHODS: The TERAVOLT database is an international, multi-center repository of cross-sectional and longitudinal data studying the impact of COVID-19 on individuals with thoracic malignancies. Patients from North America with thoracic malignancies and confirmed COVID-19 infection were included for this analysis of racial and ethnic disparities. Patients with missing race data or races and ethnicities with fewer than 50 patients were excluded from analysis. Multivariable analyses for endpoints of hospitalization and death were performed on these 471 patients. RESULTS: Of the 471 patients, 73% were White and 27% were Black. The majority (90%) were non-Hispanic ethnicity, 5% were Hispanic, and 4% were missing ethnicity data. Black patients were more likely to have an Eastern Cooperative Oncology Group (ECOG) Performance Status ≥ 2 (p-value = 0.04). On multivariable analysis, Black patients were more likely than White patients to require hospitalization (Odds Ratio (OR): 1.69, 95% CI: 1.01-2.83, p-value = 0.044). These differences remained across different waves of the pandemic. However, no statistically significant difference in mortality was found between Black and White patients (OR 1.29, 95% CI: 0.69-2.40, p-value = 0.408). CONCLUSIONS: Black patients with thoracic malignancies who acquire COVID-19 infection are at a significantly higher risk of hospitalization compared to White patients, but there is no significant difference in mortality. The underlying drivers of racial disparity among patients with thoracic malignancies and COVID-19 infection require ongoing investigation.


Assuntos
COVID-19 , Disparidades nos Níveis de Saúde , Neoplasias Torácicas , Humanos , COVID-19/epidemiologia , COVID-19/etnologia , Estudos Transversais , América do Norte/epidemiologia , Neoplasias Torácicas/epidemiologia , Neoplasias Torácicas/etnologia , Brancos , Negro ou Afro-Americano
20.
Med J Malaysia ; 78(6): 751-755, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-38031216

RESUMO

INTRODUCTION: Ultrasound guided lung biopsy (USLB) is a minimally invasive diagnostic tool with short examination time and real-time monitoring conducted bedside for accurate diagnosis in order to provide the best treatment. However, it is not widely performed by pulmonologists. We aim to explicate the efficacy and safety of USLB led by pulmonologists. The objective of this study is to assess safety and efficacy of USLB performed by pulmonologists in an outpatient setting. MATERIALS AND METHODS: We retrospectively enrolled patients who underwent the procedure from January 2018 to April 2022. Under real time ultrasound (Hitachi Medical ProSound F37), thoracic lesions adjacent to the chest wall were sampled with a full-core biopsy needle (CT Core Single Action Biopsy Device, 18G × 15 cm, Vigeo, Italy). Chest x-ray was performed 30 minutes post procedure ruling out pneumothorax. Patients were discharged home 1-2 hours post biopsy. Data was analysed using Microsoft Excel 2010 and Statistical Package for Social Science (SPSS) Version 26. RESULTS: A total of 18 patients (14 males, 4 females) underwent USLB for lung tumours. Biopsies were histologically deemed adequate with an overall diagnostic yield of 77.8% (14/18). A total of 57% were positive for thoracic malignancy (21% squamous cell carcinoma, 21% adenocarcinoma, 15% small cell carcinoma) and another 43% were positive for extra thoracic malignancy (1 hepatocellular carcinoma, 2 DLBCL, 1 Hodgkin's lymphoma, 1 seminoma, 1 thymoma). Four patients had inconclusive results but managed to get positive results from surgical or lymph node biopsy (thymoma and adenocarcinoma). Statistical analysis showed more than two passes are needed to achieve a positive HPE yield (p value<0.05). There were nil complications to all the cases done. CONCLUSIONS: USLB can safely and effectively be performed by trained pulmonologists with excellent accuracy and low complication rate in outpatients.


Assuntos
Adenocarcinoma , Neoplasias Pulmonares , Neoplasias Torácicas , Timoma , Neoplasias do Timo , Masculino , Feminino , Humanos , Estudos Retrospectivos , Pneumologistas , Timoma/patologia , Malásia , Tomografia Computadorizada por Raios X , Pulmão/diagnóstico por imagem , Pulmão/patologia , Biópsia Guiada por Imagem/efeitos adversos , Biópsia Guiada por Imagem/métodos , Neoplasias Pulmonares/diagnóstico por imagem , Neoplasias Pulmonares/patologia , Adenocarcinoma/patologia , Neoplasias do Timo/patologia , Ultrassonografia de Intervenção
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