-
Journal of Cancer Research and... 2021Mediastinal masses span a wide histopathological and radiological spectrum. Apart from primary thymic/thyroid masses and lymphomas, all other mediastinal masses can be... (Review)
Review
Mediastinal masses span a wide histopathological and radiological spectrum. Apart from primary thymic/thyroid masses and lymphomas, all other mediastinal masses can be considered rare tumors. Chest radiography and Computed tomography (CT) are helpful to characterize the mass and can reach a diagnosis or a close differential diagnosis. MRI in special situations can depict the pericardial/vascular invasion better, and diffusion studies can recognize benign from the malignant mass. The imaging details of 15 histopathologically proven cases of rare mediastinal tumors are described. Neuroblastoma (NB) (n = 3) was the most common among the rare masses. Three were sarcomas, one liposarcoma, one synovial sarcoma, one spindle-cell tumor and one Hemangiopericytoma (HPC). Lymphoma presenting as a single mass, neuroendocrine tumor (NET) of the anterior mediastinum, paraganglioma of the posterior mediastinum (one each) were seen.The imaging features of these rare mediastinal masses have to be kept in mind for appropriate diagnosis.
Topics: Humans; Lymphoma; Magnetic Resonance Imaging; Mediastinal Neoplasms; Neuroblastoma; Thymus Neoplasms; Thyroid Neoplasms; Tomography, X-Ray Computed
PubMed: 33723126
DOI: 10.4103/jcrt.JCRT_587_19 -
Blood Jun 2023
Topics: Humans; Brentuximab Vedotin; Nivolumab; Lymphoma, B-Cell; Mediastinal Neoplasms; Mediastinum
PubMed: 37261857
DOI: 10.1182/blood.2023020396 -
Mediastinum (Hong Kong, China) 2020Synovial sarcoma is a malignant mesenchymal neoplasm that accounts for approximately 10% of all soft tissue sarcomas. These tumors most commonly occur in the extremities... (Review)
Review
Synovial sarcoma is a malignant mesenchymal neoplasm that accounts for approximately 10% of all soft tissue sarcomas. These tumors most commonly occur in the extremities of young adults but are not restricted to this site and can arise in virtually any organ system. Synovial sarcomas originating in the mediastinum are exceptionally rare and are often mistaken for other, more common neoplasms in this location, especially since there are no specific imaging characteristics or clinical manifestations. Contrary to synovial sarcomas of the extremities, mediastinal tumors more commonly affect male patients. Histologically, these tumors can be divided into monophasic, biphasic and poorly differentiated variants, further complicating the diagnostic process. Recent advances in immunohistochemical and molecular techniques have provided useful tools to confirm the diagnosis and distinguish these tumors from other mediastinal neoplasms. The treatment of mediastinal synovial sarcomas often requires multimodal therapy, including surgery, chemotherapy and radiation. Despite this, the prognosis for synovial sarcomas in this location appears to be worse than for their analogues in the soft tissue, likely related to the often large size of the lesions and close proximity to critical anatomic structures making complete surgical resection difficult to achieve. This review summarizes the clinicopathological, immunohistochemical and molecular characteristics of these rare neoplasms.
PubMed: 35118281
DOI: 10.21037/med-20-19 -
Virchows Archiv : An International... Dec 2023Session 3 of the 2021 European Association for Haematopathology/Society for Hematopathology Workshop focused on mediastinal large B cell lymphomas and surrounding gray... (Review)
Review
Session 3 of the 2021 European Association for Haematopathology/Society for Hematopathology Workshop focused on mediastinal large B cell lymphomas and surrounding gray areas. One half of the session was dedicated to primary mediastinal large B cell lymphoma (PMBL) and included cases with classic clinicopathologic features, as well as cases with either morphologic or immunophenotypic variation, and PMBL-like cases with primary extramediastinal disease. The role of additional immunophenotyping and/or molecular testing to aid in the diagnosis of PMBL was discussed. The second half of the session focused on mediastinal and non-mediastinal gray zone lymphomas (GZL) with features intermediate between diffuse large B cell lymphoma (DLBCL) and classic Hodgkin lymphoma (CHL). Several cases illustrating the current challenges in separating this entity from PMBL/DLBCL and CHL were presented. There was discussion regarding the clinical and genetic differences between mediastinal and non-mediastinal GZLs. Rare cases of PMBL and GZL associated with EBV or follicular lymphoma were reviewed. Finally, several cases included in the session highlighted composite or sequential CHL and PMBL/DLBCL and/or GZL, highlighting challenges in separating such cases from GZL.
Topics: Humans; Biomarkers, Tumor; Mediastinal Neoplasms; Hodgkin Disease; Lymphoma, Large B-Cell, Diffuse; Lymphoma, Follicular
PubMed: 37530791
DOI: 10.1007/s00428-023-03550-5 -
Thoracic Cancer Nov 2021Primary mediastinal large B cell lymphoma (PMBCL) is an aggressive large B cell lymphoma originating in the mediastinum, that mainly expresses B cell surface molecules,... (Review)
Review
Primary mediastinal large B cell lymphoma (PMBCL) is an aggressive large B cell lymphoma originating in the mediastinum, that mainly expresses B cell surface molecules, such as CD19, CD20, CD22, andCD79a. Clinically, they are characterized by rapidly increasing anterior mediastinal masses, which can cause compression of the surrounding tissues. The diagnosis of PMBCL mainly depends on the pathological features, imaging examination and clinical features. Currently, the most commonly used therapeutic regimens are R-CHOP and R-EPOCH. Radiotherapy is beneficial in some patients, but it can also lead to long-term toxicity. The research and development of novel therapies are ongoing, and some studies have achieved encouraging results, including those conducted on chimeric antigen receptor-modified T (CAR-T) cell therapy and anti-PD-1 drugs. However, randomized controlled trials with larger sample sizes are still needed. Positron emission tomography-computed tomography (PET-CT) is mainly used to assess the curative effect after treatment and to guide the subsequent treatment strategy.
Topics: Diagnosis, Differential; Humans; Lymphoma, Large B-Cell, Diffuse; Mediastinal Neoplasms; Positron Emission Tomography Computed Tomography
PubMed: 34590432
DOI: 10.1111/1759-7714.14155 -
Panminerva Medica Sep 2019Endobronchial ultrasound (EBUS) has revolutionized the field of bronchoscopy because it allows to observe peribronchial structures and distal peripheral lung lesions.... (Review)
Review
Endobronchial ultrasound (EBUS) has revolutionized the field of bronchoscopy because it allows to observe peribronchial structures and distal peripheral lung lesions. The use of EBUS was first described by Hurte and Hanrath in 1992. EBUS technology exists in two forms: radial and convex transducer probes. The radial EBUS probe has a 20-MHZ (12-30 MHz available) rotating transducer that can be inserted together with or without a guide sheath through the working channel (2.0-2.8 mm) of a standard flexible bronchoscope. The transducer rotates and produces a 360-degree circular image around the central position of the probe. There are two types of radial EBUS probes: "peripheral" probes, used to identify parenchymal lung lesions, and "central" probes, with balloon sheaths, used for the assessment of airway walls and peribronchial lymph nodes.
Topics: Artifacts; Bronchoscopes; Bronchoscopy; Clinical Competence; Endosonography; Equipment Design; Humans; Lung Diseases; Lung Neoplasms; Lymph Nodes; Mediastinal Neoplasms; Mediastinum; Pulmonary Medicine; Sensitivity and Specificity; Ultrasonography, Doppler; Virtual Reality
PubMed: 30421897
DOI: 10.23736/S0031-0808.18.03570-X -
Nagoya Journal of Medical Science May 2020Thoracic surgery has evolved drastically in recent years. Although thoracic surgeons mainly deal with tumorous lesion in the lungs, mediastinum, and pleura, they also... (Review)
Review
Thoracic surgery has evolved drastically in recent years. Although thoracic surgeons mainly deal with tumorous lesion in the lungs, mediastinum, and pleura, they also perform lung transplantation surgery in patients with end-stage lung disease. Herein, we introduce various major current topics in thoracic surgery. Minimally invasive surgical procedures include robot-assisted thoracic surgery and uniportal video-assisted thoracic surgery. Novel techniques for sublobar resection include virtual-assisted lung mapping, image-guided video-assisted thoracic surgery, and segmentectomy using indocyanine green. Three-dimensional (3D) computed tomography (CT) simulation consists of surgeon-friendly 3D-CT image analysis systems and new-generation, dynamic 3D-CT imaging systems. Updates in cadaveric lung transplantation include use of marginal donors, including donation after circulatory death, and lung perfusion for such donors. Topics in living donor lobar lung transplantation include size matching, donor issues, and new surgical techniques. During routine clinical practice, thoracic surgeons encounter various pivotal topics related to thoracic surgery, which are described in this report.
Topics: Humans; Imaging, Three-Dimensional; Living Donors; Lung Neoplasms; Lung Transplantation; Mediastinal Neoplasms; Organ Preservation; Pleural Neoplasms; Pneumonectomy; Robotic Surgical Procedures; Surgery, Computer-Assisted; Thoracic Surgery, Video-Assisted; Thoracic Surgical Procedures; Tissue and Organ Procurement; Tomography, X-Ray Computed
PubMed: 32581397
DOI: 10.18999/nagjms.82.2.161 -
Journal of Clinical Oncology : Official... Aug 2023Resectable non-small-cell lung cancer (NSCLC) with a high probability of mediastinal nodal involvement requires mediastinal staging by endosonography and, in the absence... (Randomized Controlled Trial)
Randomized Controlled Trial
PURPOSE
Resectable non-small-cell lung cancer (NSCLC) with a high probability of mediastinal nodal involvement requires mediastinal staging by endosonography and, in the absence of nodal metastases, confirmatory mediastinoscopy according to current guidelines. However, randomized data regarding immediate lung tumor resection after systematic endosonography versus additional confirmatory mediastinoscopy before resection are lacking.
METHODS
Patients with (suspected) resectable NSCLC and an indication for mediastinal staging after negative systematic endosonography were randomly assigned to immediate lung tumor resection or confirmatory mediastinoscopy followed by tumor resection. The primary outcome in this noninferiority trial (noninferiority margin of 8% that previously showed to not compromise survival, < .0250) was the presence of unforeseen N2 disease after tumor resection with lymph node dissection. Secondary outcomes were 30-day major morbidity and mortality.
RESULTS
Between July 17, 2017, and October 5, 2020, 360 patients were randomly assigned, 178 to immediate lung tumor resection (seven dropouts) and 182 to confirmatory mediastinoscopy first (seven dropouts before and six after mediastinoscopy). Mediastinoscopy detected metastases in 8.0% (14/175; 95% CI, 4.8 to 13.0) of patients. Unforeseen N2 rate after immediate resection (8.8%) was noninferior compared with mediastinoscopy first (7.7%) in both intention-to-treat (Δ, 1.03%; UL 95% CIΔ, 7.2%; = .0144) and per-protocol analyses (Δ, 0.83%; UL 95% CIΔ, 7.3%; = .0157). Major morbidity and 30-day mortality was 12.9% after immediate resection versus 15.4% after mediastinoscopy first ( = .4940).
CONCLUSION
On the basis of our chosen noninferiority margin in the rate of unforeseen N2, confirmatory mediastinoscopy after negative systematic endosonography can be omitted in patients with resectable NSCLC and an indication for mediastinal staging.
Topics: Humans; Lung Neoplasms; Carcinoma, Non-Small-Cell Lung; Mediastinoscopy; Endosonography; Neoplasm Staging; Lymph Nodes
PubMed: 37018653
DOI: 10.1200/JCO.22.01728 -
Blood Apr 2021The mutational landscape of gray zone lymphoma (GZL) has not yet been established, and differences from related entities are largely unknown. Here, we studied coding...
The mutational landscape of gray zone lymphoma (GZL) has not yet been established, and differences from related entities are largely unknown. Here, we studied coding sequence mutations of 50 Epstein-Barr virus (EBV)-negative GZLs and 20 polymorphic EBV+ diffuse large B-cell lymphoma (DLBCL) not otherwise specified (poly-EBV-L) in comparison with classical Hodgkin lymphoma (cHL), primary mediastinal large B-cell lymphoma (PMBCL), and DLBCL. Exomes of 21 GZL and 7 poly-EBV-L cases, along with paired constitutional DNA, were analyzed as a discovery cohort, followed by targeted sequencing of 217 genes in an extension cohort of 29 GZL and 13 poly-EBV-L cases. GZL cases with thymic niche involvement (anterior mediastinal mass) exhibited a mutation profile closely resembling cHL and PMBCL, with SOCS1 (45%), B2M (45%), TNFAIP3 (35%), GNA13 (35%), LRRN3 (32%), and NFKBIA (29%) being the most recurrently mutated genes. In contrast, GZL cases without thymic niche involvement (n = 18) had a significantly distinct pattern that was enriched in mutations related to apoptosis defects (TP53 [39%], BCL2 [28%], BIRC6 [22%]) and depleted in GNA13, XPO1, or NF-κB signaling pathway mutations (TNFAIP3, NFKBIE, IKBKB, NFKBIA). They also exhibited more BCL2/BCL6 rearrangements compared with thymic GZL. Poly-EBV-L cases presented a distinct mutational profile, including STAT3 mutations and a significantly lower coding mutation load in comparison with EBV- GZL. Our study highlights characteristic mutational patterns in GZL associated with presentation in the thymic niche, suggesting a common cell of origin and disease evolution overlapping with related anterior mediastinal lymphomas.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Epstein-Barr Virus Infections; Female; Hodgkin Disease; Humans; Lymphoma, Large B-Cell, Diffuse; Male; Mediastinal Neoplasms; Middle Aged; Mutation; Thymus Gland; Young Adult
PubMed: 32961552
DOI: 10.1182/blood.2020007507 -
European Respiratory Review : An... Dec 2021The diagnosis of a mediastinal mass may be challenging for clinicians, since lesions arising within the mediastinum include a variety of disease entities, frequently... (Review)
Review
The diagnosis of a mediastinal mass may be challenging for clinicians, since lesions arising within the mediastinum include a variety of disease entities, frequently requiring a multidisciplinary approach. Age and sex represent important information, which need to be integrated with imaging and laboratory findings. In addition, the location of the mediastinal lesion is fundamental; indeed, we propose to illustrate mediastinal diseases based on the compartment of origin. We consider that this structured approach may serve as hint to the diagnostic modalities and management of mediastinal diseases. In this review, we present primary mediastinal tumours in the evolving context of new diagnostic and therapeutic tools, with recently described entities, based on our own experience with >900 cases encountered in the past 10 years.
Topics: Diagnostic Imaging; Humans; Mediastinal Neoplasms; Mediastinum
PubMed: 34615701
DOI: 10.1183/16000617.0309-2020