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The Journal of International Medical... Nov 2023Angiosarcoma is the most invasive and malignant cardiac tumor and most commonly originates from the right atrium. Early diagnosis is essential, and echocardiography has...
Angiosarcoma is the most invasive and malignant cardiac tumor and most commonly originates from the right atrium. Early diagnosis is essential, and echocardiography has an important role in diagnosis. This tumor grows aggressively, and metastases to other sites makes it difficult to control. Surgical treatment remains the best option for patients who do not respond to chemoradiotherapy. We herein report a case of a 17-year-old patient with cardiac angiosarcoma who presented with dyspnea, chest pain, dry cough, and fever. Although we considered the most probable diagnosis to be constrictive pericarditis, pathologic examination revealed a primary angiosarcoma originating from the pericardium. The patient underwent total pericardiectomy. However, despite receiving chemotherapy for 2 weeks postoperatively, she developed complications including leukopenia and eventually died of respiratory failure. Late diagnosis of angiosarcoma often occurs, resulting in progression to end-stage disease and a very poor prognosis. Therefore, a thorough understanding of this entity, knowledge of its pitfalls in management, and establishment of an accurate treatment guideline would help to develop a reliable and life-saving treatment approach for these patients.
Topics: Female; Humans; Adolescent; Hemangiosarcoma; Mediastinal Neoplasms; Thymus Neoplasms; Heart Neoplasms; Chemoradiotherapy
PubMed: 37987637
DOI: 10.1177/03000605231211772 -
Clinical Radiology Dec 2023Anterior mediastinal masses can be difficult to characterise on computed tomography (CT) due to the wide spectrum of normal appearances of thymic tissue as well as the... (Review)
Review
Anterior mediastinal masses can be difficult to characterise on computed tomography (CT) due to the wide spectrum of normal appearances of thymic tissue as well as the challenge of differentiating between benign and malignant pathologies. Additionally, attenuation of cystic mediastinal lesions can be misinterpreted on CT due to varying attenuation values. Anecdotally, non-vascular magnetic resonance imaging (MRI) of the thorax is underutilised across radiology departments in the UK, but has been shown to improve diagnostic certainty and reduce unnecessary surgical intervention. T2-weighted MRI is useful in confirming the cystic nature of lesions, whereas chemical shift techniques can be utilised to document the presence of macroscopic and intra-cellular fat and thus help distinguish between benign and malignant pathologies. In this review article, we present a practical approach to using MRI for the characterisation of anterior mediastinal lesions based on our clinical experience in a UK district general hospital.
Topics: Humans; Mediastinal Neoplasms; Magnetic Resonance Imaging; Thymus Gland; Tomography, X-Ray Computed; Mediastinum
PubMed: 37709611
DOI: 10.1016/j.crad.2023.08.015 -
Canadian Association of Radiologists... Nov 2023To evaluate the feasibility and usefulness of T1 and T2 mapping in characterization of mediastinal masses. From August 2019 through December 2021, 47 patients...
To evaluate the feasibility and usefulness of T1 and T2 mapping in characterization of mediastinal masses. From August 2019 through December 2021, 47 patients underwent 3.0-T chest MRI with T1 and post-contrast T1 mapping using modified look-locker inversion recovery sequences and T2 mapping using a T2-prepared single-shot shot steady-state free precession technique. Mean native T1, native T2, and post-contrast T1 values were measured by drawing the region of interest in the mediastinal masses, and enhancement index (EI) was calculated using these values. All mapping images were acquired successfully, without significant artifact. There were 25 thymic epithelial tumors (TETs), 3 schwannomas, 6 lymphomas, and 9 thymic cysts, and 4 other cystic tumors. TET, schwannoma, and lymphoma were grouped together as "solid tumor," to be compared with thymic cysts and other tumors ("cystic tumors"). The mean post-contrast T1 mapping ( < .001), native T2 mapping ( < .001), and EI ( < .001) values showed significant difference between these two groups. Among TETs, high risk TETs (thymoma types B2, B3, and thymic carcinoma) showed significantly higher native T2 mapping values (P = .002) than low risk TETs (thymoma types A, B1, and AB). For all measured variables, interrater reliability was good to excellent (intraclass coefficient [ICC]: .869∼.990) and intrarater reliability was excellent (ICC: .911∼.995). The use of T1 and T2 mapping in MRI of mediastinal masses is feasible and may provide additional information in the evaluation of mediastinal masses.
Topics: Humans; Thymoma; Mediastinal Cyst; Feasibility Studies; Reproducibility of Results; Thymus Neoplasms; Magnetic Resonance Imaging; Lymphoma
PubMed: 36882380
DOI: 10.1177/08465371231160052 -
Kyobu Geka. the Japanese Journal of... Sep 2023The number of elderly patients in thoracic surgery is increasing. The percentage of patients over the age of 80 in surgical cases of malignant diseases such as lung...
The number of elderly patients in thoracic surgery is increasing. The percentage of patients over the age of 80 in surgical cases of malignant diseases such as lung cancer and mediastinal tumors is increasing every year. It is also true that the indications for surgery have been expanding as surgery itself has become less invasive, such as thoracoscopic and robotic surgery. However, it is not uncommon for patients over 80 years of age to have some organ dysfunction and many comorbidities. Therefore, when performing surgery for lung cancer and other diseases, it is important to assess the patient's ability to tolerate surgery, including respiratory and cardiac functions, and to perform risk management. To prevent postoperative complications and improve the accuracy of perioperative management, respiratory rehabilitation should be conducted before and after surgery, and not only smoking cessation instruction but also inhalation training using incentive spirometry( IS), breathing exercises, and the use of inhalers such as long-acting β2 agonist (LABA)/long-acting muscaring antagonist (LAMA) for patients with chronic obstructive pulmonary disease( COPD) are useful.
Topics: Humans; Aged, 80 and over; Aged; Muscarinic Antagonists; Adrenergic beta-2 Receptor Agonists; Administration, Inhalation; Respiratory Therapy; Pulmonary Disease, Chronic Obstructive; Lung Neoplasms; Drug Therapy, Combination
PubMed: 38056855
DOI: No ID Found -
Magnetic Resonance in Medical Sciences... Oct 2023The anterior mediastinum is the most common location of mediastinal tumors, and thymic epithelial tumors are the most common mediastinal tumors. It is important to...
The anterior mediastinum is the most common location of mediastinal tumors, and thymic epithelial tumors are the most common mediastinal tumors. It is important to differentiate thymic epithelial tumors from malignant lymphomas and malignant germ cell tumors because of the different treatment strategies. Dynamic contrast-enhanced MRI and diffusion-weighted imaging can provide additional information on the differential diagnosis. Chemical shift imaging can detect tiny fat tissues in the lesion and is useful in differentiating thymic hyperplasia from other solid tumors such as thymomas. MRI findings reflect histopathological features of mediastinal tumors, and a comprehensive evaluation of MRI sequences is important for estimation of the histopathological features of the tumor. In this manuscript, we describe the MRI findings of anterior mediastinal solid tumors and the role of MRI in the differential diagnosis.
Topics: Humans; Mediastinum; Mediastinal Neoplasms; Diagnosis, Differential; Thymus Neoplasms; Magnetic Resonance Imaging; Neoplasms, Glandular and Epithelial
PubMed: 35296589
DOI: 10.2463/mrms.rev.2021-0098 -
Respiratory Medicine Sep 2023Paragangliomas are rarely found in the mediastinum, where they account for a small proportion of mediastinal masses. This study aimed to better characterize the...
BACKGROUND
Paragangliomas are rarely found in the mediastinum, where they account for a small proportion of mediastinal masses. This study aimed to better characterize the presenting features and relevant aspects in optimizing the diagnosis and treatment of mediastinal paragangliomas.
METHODS
A computer-assisted search of electronic health records was performed to identify adult patients (≥18 years) who underwent evaluation for a primary mediastinal paraganglioma at Mayo Clinic between January 2000 and April 2022. Medical charts, laboratory tests and radiology images were reviewed to collect data.
RESULTS
The study included 51 patients, each with a single mediastinal paraganglioma. The median age was 47 years (IQR: 39-67), 67% females. Symptoms of catecholamine excess were manifest in 39% of patients, and 14% presented with mass effect, while the remaining 47% had no paraganglioma-related symptoms. Genetic testing was performed in 35 patients; 66% harbored a pathogenic variant in the succinate dehydrogenase enzyme complex. Most paragangliomas (71%) were in the middle mediastinum and showed uptake of intravenous contrast on chest imaging. Biopsies were performed in 30 (59%) patients; 27% were inconclusive and 10% resulted in major complications. Surgical resection occurred in 75%, primarily for relief of symptoms (50%) followed by proximity to critical structures (45%). Perioperative complications were common (66%), but there were no cases of local tumor recurrence during the follow-up period (median 8 years; IQR: 4-13).
CONCLUSION
Mediastinal paragangliomas are most located in the middle mediastinum and can often be diagnosed noninvasively using a combination of clinical, biochemical, and radiological features.
Topics: Adult; Female; Humans; Middle Aged; Male; Retrospective Studies; Paraganglioma, Extra-Adrenal; Paraganglioma; Mediastinal Neoplasms; Mediastinum
PubMed: 37257785
DOI: 10.1016/j.rmed.2023.107296 -
Journal of Visualized Experiments : JoVE Aug 2023Lung cancer is the leading cause of cancer mortality globally. To ensure the correct diagnosis and staging in relation to treatment options, it is crucial to obtain...
Lung cancer is the leading cause of cancer mortality globally. To ensure the correct diagnosis and staging in relation to treatment options, it is crucial to obtain valid biopsies from suspected tumors and mediastinal lymph nodes and accurate identification of the mediastinal lymph nodes regarding the Tumor-Node-Metastasis (TNM)-classification. Flexible bronchoscopy combined with endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is essential in the workup and diagnosis of patients suspected of lung cancer. EBUS-TBNA from mediastinal lymph nodes is a technically difficult procedure and has been identified as one of the most important procedures that should be integrated into a simulation-based training program for invasive pulmonologists. More specific guidelines that govern training in EBUS-TBNA are needed to meet this demand. We hereby propose a systematic, stepwise approach with specific attention to six landmarks that support the endoscopist when navigating through the bronchial maze. The stepwise approach relying on the six landmarks is used in the EBUS-certified training program offered by the European Respiratory Society (ERS).
Topics: Humans; Lung Neoplasms; Bronchi; Bronchoscopy; Computer Simulation; Endoscopic Ultrasound-Guided Fine Needle Aspiration
PubMed: 37590526
DOI: 10.3791/65551 -
International Journal of Surgical... Oct 2023Thoracic SMARCA4-deficient undifferentiated tumor (SMARCA4-UT) is a recently described aggressive neoplasm of young smokers defined by inactivating mutations and...
CONTEXT
Thoracic SMARCA4-deficient undifferentiated tumor (SMARCA4-UT) is a recently described aggressive neoplasm of young smokers defined by inactivating mutations and characterized by cells with rhabdoid morphology, high mitotic activity, and abundant necrosis.
OBJECTIVE
Describe and compare 3 unusual presentations of SMARCA4-UT in older adults, including one presenting as a metastatic lesion mimicking a primary bone sarcoma. Discuss the molecular characteristics of SMARCA4-UT and their relationship to nonsmall-cell lung carcinomas with .
DESIGN
Three patients with SMARCA4-UTs were identified utilizing a natural language search in CoPath. hematoxylin and eosin sections from all patients as well as Papanicolaou-stained slides and Diff-Quik-stained slides for the first patient were examined. A broad range of immunostains, including BRG1/SMARCA4, were evaluated. Molecular testing was performed via next-generation sequencing.
RESULTS
The 3 patients were aged 58, 70, and 70 years. All had a significant smoking history. The first patient presented with an iliac bone mass and mediastinal lymphadenopathy, the second with mediastinal adenopathy, and the third with a paratracheal mass. All 3 tumors showed a diffuse proliferation of pleomorphic, rhabdoid cells with high mitotic activity and tumor necrosis. SMARCA4 was lost in all 3 tumors by immunohistochemistry. Molecular testing revealed alterations in the first 2.
CONCLUSIONS
Thoracic SMARCA4-UT should be considered in the differential diagnosis of pleomorphic rhabdoid tumors in older adults with a smoking history. Although most present as lung and/or mediastinal masses, they may occasionally present as a metastasis and mimic an undifferentiated sarcoma, representing a potential diagnostic pitfall.
PubMed: 37903457
DOI: 10.1177/10668969231206350 -
The Journal of Thoracic and... Sep 2023
Topics: Humans; Heart; Heart Neoplasms; Mediastinal Neoplasms; Thymus Neoplasms; Sarcoma
PubMed: 34862054
DOI: 10.1016/j.jtcvs.2021.11.010 -
Blood Advances Sep 2023Patients with relapsed/refractory primary mediastinal large B-cell lymphoma (R/R PMBL) have poor responses to salvage therapy. Nivolumab and brentuximab vedotin (BV)...
Patients with relapsed/refractory primary mediastinal large B-cell lymphoma (R/R PMBL) have poor responses to salvage therapy. Nivolumab and brentuximab vedotin (BV) showed promising early efficacy in patients with R/R PMBL in the phase 1/2 open-label, multicenter CheckMate 436 study; we report safety and efficacy findings from the 3-year follow-up. Patients who were eligible were aged ≥15 years with R/R PMBL previously treated with either high-dose chemotherapy plus autologous hematopoietic cell transplantation (HCT) or ≥2 prior multiagent chemotherapies, and had Eastern Cooperative Oncology Group performance status scores of 0 to 1 and CD30 expression of ≥1%. Patients were treated with nivolumab 240 mg and BV 1.8 mg/kg once every 3 weeks until disease progression or unacceptable toxicity. Primary end point was objective response rate (ORR); secondary end points included complete response rate, duration of response, progression-free survival (PFS), and overall survival (OS). Safety was monitored throughout. At final database lock (30 March 2022), 29 patients had received nivolumab plus BV; median follow-up was 39.6 months. Investigator-assessed ORR was 73.3%; median time to response was 1.3 months (range, 1.1-4.8). Median PFS was 26.0 months; median OS was not reached. PFS and OS rates at 24 months were 55.5% (95% confidence interval [CI], 32.0-73.8) and 75.5% (95% CI, 55.4-87.5), respectively. The most frequently occurring grade 3/4 treatment-related adverse event was neutropenia. Consolidative HCT was received by 12 patients, with a 100-day complete response rate of 100.0%. This 3-year follow-up showed long-term efficacy for nivolumab plus BV in R/R PMBL, with no new safety signals. This trial was registered at www.clinicaltrials.gov as #NCT02581631.
Topics: Adult; Humans; Brentuximab Vedotin; Nivolumab; Follow-Up Studies; Hodgkin Disease; Neoplasm Recurrence, Local; Lymphoma, B-Cell
PubMed: 37352266
DOI: 10.1182/bloodadvances.2023010254