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Blood Sep 2022Primary mediastinal large B-cell lymphoma (PMBCL) is a separate entity in the World Health Organization's classification, based on clinicopathologic features and a...
Primary mediastinal large B-cell lymphoma (PMBCL) is a separate entity in the World Health Organization's classification, based on clinicopathologic features and a distinct molecular signature that overlaps with nodular sclerosis classic Hodgkin lymphoma (cHL). Molecular classifiers can distinguish PMBCL from diffuse large B-cell lymphoma (DLBCL) using ribonucleic acid derived from paraffin-embedded tissue and are integral to future studies. However, given that ∼5% of DLBCL can have a molecular PMBCL phenotype in the absence of mediastinal involvement, clinical information remains critical for diagnosis. Studies during the past 10 to 20 years have elucidated the biologic hallmarks of PMBCL that are reminiscent of cHL, including the importance of the JAK-STAT and NF-κB signaling pathways, as well as an immune evasion phenotype through multiple converging genetic aberrations. The outcome of PMBCL has improved in the modern rituximab era; however, whether there is a single standard treatment for all patients and when to integrate radiotherapy remains controversial. Regardless of the frontline therapy, refractory disease can occur in up to 10% of patients and correlates with poor outcome. With emerging data supporting the high efficacy of PD1 inhibitors in PMBCL, studies are underway that integrate them into the up-front setting.
Topics: Hodgkin Disease; Humans; Lymphoma, Large B-Cell, Diffuse; Mediastinal Neoplasms; Mediastinum; Rituximab
PubMed: 34496020
DOI: 10.1182/blood.2020008376 -
International Heart Journal Sep 2018Hemoptysis is a rare complication of acute aortic dissection. A 77-year-old woman was admitted to our department with epigastralgia and hemoptysis. Computed tomography...
Hemoptysis is a rare complication of acute aortic dissection. A 77-year-old woman was admitted to our department with epigastralgia and hemoptysis. Computed tomography showed Stanford A acute aortic dissection and massive posterior mediastinal hematoma which extended along the right pulmonary artery. Hemoptysis is a lethal sign of aortic dissection, therefore, emergency ascending aortic replacement was performed with a good clinical outcome.
Topics: Acute Disease; Aged; Aortic Dissection; Aorta; Female; Hematoma; Hemoptysis; Humans; Mediastinal Diseases; Pulmonary Artery; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 30158389
DOI: 10.1536/ihj.18-263 -
Forensic Science, Medicine, and... Sep 2018Although death from food is not an uncommon finding in forensic facilities worldwide, the range of underlying lethal mechanisms and associated conditions that should be... (Review)
Review
Although death from food is not an uncommon finding in forensic facilities worldwide, the range of underlying lethal mechanisms and associated conditions that should be sought at the time of autopsy is quite disparate. Deaths may occur from i) infectious agents including bacteria, viruses, protozoa, cestodes, nematodes and prions; ii) natural toxins including amanita toxins, tetrodotoxin, ciguatera and scombroid; iii) anaphylaxis; iv) poisoning; v) mechanical issues around airway and gut obstruction and/or perforation; and vi) miscellaneous causes. Food-related deaths are important in terms of global mortality, and thus autopsies need to be comprehensive with full ancillary testing. Medicolegal matters may involve issues concerning likely exposure to infectious agents, possible foods ingested, the declared content and possible components of food, the significance of toxicological analyses, and aspects of duty of care in cases of café coronary syndrome and gastroenteritis while in care.
Topics: Airway Obstruction; Anaphylaxis; Esophageal Perforation; Food; Food Hypersensitivity; Food Microbiology; Foodborne Diseases; Forensic Medicine; Humans; Mediastinal Diseases; Obesity; Pica; Prader-Willi Syndrome
PubMed: 28710688
DOI: 10.1007/s12024-017-9899-9 -
The British Journal of Radiology Dec 2019Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis which can have a broad range of clinical and radiological presentations. Typically, ECD affects... (Review)
Review
Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis which can have a broad range of clinical and radiological presentations. Typically, ECD affects multiple organ systems, with skeletal involvement present in almost all ECD patients and cardiothoracic manifestations in more than half. Cardiac and thoracic involvement contributes significantly to morbidity and mortality in affected patients and may have prognostic implications. The diagnosis of ECD can be challenging due to its rarity and similarity to other systemic disease processes. Although the diagnosis can be suggested on imaging, histopathology and immunohistochemistry are required for confirmation. We describe the multimodal imaging features of mediastinal, cardiac, pleural and lung parenchymal ECD. This review identifies the most common radiological manifestations of cardiac and thoracic ECD on contrast-enhanced CT, fluorine-fludeoxyglucose positron emission tomography/CT and cardiac MRI, and highlights the role of these cross-sectional techniques in disease diagnosis.
Topics: Contrast Media; Erdheim-Chester Disease; Fluorodeoxyglucose F18; Heart Diseases; Humans; Lung Diseases; Magnetic Resonance Imaging; Mediastinal Diseases; Multimodal Imaging; Pleural Diseases; Positron Emission Tomography Computed Tomography; Radiopharmaceuticals; Tomography, X-Ray Computed
PubMed: 31386554
DOI: 10.1259/bjr.20190473 -
Lakartidningen Jan 2018A previously healthy 21-year old man presented to the emergency department with sudden onset central chest pain exacerbated by breathing. A plain chest X-ray showed air...
A previously healthy 21-year old man presented to the emergency department with sudden onset central chest pain exacerbated by breathing. A plain chest X-ray showed air within the mediastinum and pericardium confirmed by a CT scan with contrast. The patient history did not raise suspicion of any concomitant disease and the diagnosis of spontaneous pneumomediastinum with pneumopericardium was made. The patient recovered completely over the next few days with bed rest and analgesics. Spontaneous pneumomediastinum is an uncommon disease caused by rupture of perivascular alveoli causing air leakage to the mediastinum. The condition is benign and self-resolving in most cases. Pneumopericardium, a rare complication to spontaneous pneumomediastinum, is also usually self-resolving but may cause cardiac tamponade requiring intervention. Spontaneous pneumomediastinum must be differentiated from secondary pneumomediastinum caused by an underlying condition, such as esofageal rupture, trauma or infection, which may require specific treatment.
Topics: Acute Pain; Algorithms; Chest Pain; Humans; Male; Mediastinal Emphysema; Pneumopericardium; Radiography; Tomography, X-Ray Computed; Young Adult
PubMed: 29319833
DOI: No ID Found -
Medicine Dec 2017Mediastinal masses are usually assessed by computer tomography (CT) and magnetic resonance imaging (MRI). Transthoracic ultrasonography (TUS) can also provide useful... (Review)
Review
Mediastinal masses are usually assessed by computer tomography (CT) and magnetic resonance imaging (MRI). Transthoracic ultrasonography (TUS) can also provide useful information concerning prevascular and posterior mediastinal masses abutting the thoracic wall, but is underused for mediastinal pathology. Moreover, it provides a valuable and safe method for guiding interventional procedures in those areas, even in cases when other approaches are difficult or impossible. Considering TUS a very useful imagistic method for diagnosing mediastinal masses, we present a pictorial essay of various mediastinal diseases which can be assessed by this method.
Topics: Diagnosis, Differential; Humans; Mediastinal Diseases; Ultrasonography
PubMed: 29245326
DOI: 10.1097/MD.0000000000009082 -
Internal Medicine (Tokyo, Japan) Jan 2022
Topics: Esophageal Diseases; Esophageal Perforation; Humans; Mediastinal Diseases; Rupture, Spontaneous
PubMed: 34219113
DOI: 10.2169/internalmedicine.7807-21 -
CMAJ : Canadian Medical Association... Sep 2021
Topics: Alcohol Drinking; Esophageal Perforation; Humans; Hydropneumothorax; Male; Mediastinal Diseases; Mediastinal Emphysema; Middle Aged; Vomiting
PubMed: 34580144
DOI: 10.1503/cmaj.202893 -
Ugeskrift For Laeger Jan 2016Spontaneous mediastinal emphysema, also known as spontaneous pneumomediastinum, is defined as radiologically detected free air in the mediastinum, without preceding... (Review)
Review
Spontaneous mediastinal emphysema, also known as spontaneous pneumomediastinum, is defined as radiologically detected free air in the mediastinum, without preceding trauma. It is a rare condition, mainly affecting young adults. It can be caused by coughing, strenuous sports or cocaine inhalation, however, 40% are idiopatic. Common symptoms are chest pain and dyspnoea. 75-90% can be diagnosed with a chest X-ray, and 100% with a computed tomography. Treatment is symptomatic and complications are rare, however, pneumothorax and pneumorrachis have been reported.
Topics: Humans; Mediastinal Emphysema; Radiography; Tomography, X-Ray Computed; Young Adult
PubMed: 26750190
DOI: No ID Found -
Blood Aug 2018The World Health Organization now recognizes primary mediastinal B-cell lymphoma (PMBCL) as a unique clinical and biologic entity. PMBCL is distinct from other B-cell... (Review)
Review
The World Health Organization now recognizes primary mediastinal B-cell lymphoma (PMBCL) as a unique clinical and biologic entity. PMBCL is distinct from other B-cell non-Hodgkin lymphoma subtypes and has features that overlap with classical Hodgkin lymphoma, including a peak incidence in the adolescent and young adult population, mediastinal presentation of disease, and molecular alterations in JAK2 and programmed death ligands. Because PMBCL is rare, there are few prospective clinical trials to guide therapy, resulting in no single standard of care. Given the long life expectancy of survivors of PMBCL, treatment approaches must balance maximizing cure while minimizing long-term toxicity. In this article, I review my approach to the treatment of PMBCL, incorporating data from adult and pediatric studies, as well as recent advances in our understanding of the molecular basis of PMBCL.
Topics: Humans; Lymphoma, B-Cell; Mediastinal Neoplasms
PubMed: 29976557
DOI: 10.1182/blood-2018-04-791566