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Hematology. American Society of... Dec 2017Primary mediastinal B-cell lymphoma (PMBCL) is recognized as a distinct clinicopathologic entity that predominantly affects adolescents and young adults and is more... (Review)
Review
Primary mediastinal B-cell lymphoma (PMBCL) is recognized as a distinct clinicopathologic entity that predominantly affects adolescents and young adults and is more common in female subjects. Although PMBCL is considered to be a subtype of diffuse large B-cell lymphoma, its clinical, morphologic, and biological characteristics overlap significantly with those of nodular sclerosing Hodgkin lymphoma (NSHL). Over the past few years, the shared biology of these 2 entities has been highlighted in several studies, and mediastinal gray zone lymphoma, with features intermediate between PMBCL and NSHL, has been recognized as a unique molecular entity. Although there is a lack of consensus about the optimal therapeutic strategy for adolescent and young adult patients newly diagnosed with PMCBL, highly curative strategies that obviate the need for mediastinal radiation are favored by most. Progress in understanding the biology of PMBCL and its close relationship to NSHL have helped pave the way for the investigation of novel approaches such as immune checkpoint inhibition. Other strategies such as adoptive T-cell therapy and targeting CD30 are also being studied.
Topics: Adolescent; Adoptive Transfer; Adult; Cell Cycle Checkpoints; Female; Humans; Ki-1 Antigen; Lymphoma, B-Cell; Male; Mediastinal Neoplasms; Neoplasm Proteins; T-Lymphocytes
PubMed: 29222270
DOI: 10.1182/asheducation-2017.1.298 -
Annals of the Royal College of Surgeons... May 2015Primary hyperparathyroidism is a relatively common problem encountered by any endocrine surgical unit. Ectopic parathyroid adenomas have been known to be a common cause... (Review)
Review
Primary hyperparathyroidism is a relatively common problem encountered by any endocrine surgical unit. Ectopic parathyroid adenomas have been known to be a common cause of persistent hyperparathyroidism after surgery. A common site of the missed ectopic gland will be that in the mediastinum. However, with the increasing improvement in available imaging, it is likely that this can be diagnosed preoperatively. The surgical approach to the mediastinal parathyroid has also changed vastly over the last decade from maximally invasive to minimally invasive with minimal complications. We provide a review on the entity of mediastinal parathyroid adenomas and their surgical implications.
Topics: Adenoma; Humans; Mediastinal Neoplasms; Parathyroid Neoplasms
PubMed: 26263931
DOI: 10.1308/003588415X14181254789088 -
Korean Journal of Radiology Jan 2021Magnetic resonance imaging (MRI) has become a crucial tool for evaluating mediastinal masses considering that several lesions that appear indeterminate on computed... (Review)
Review
Magnetic resonance imaging (MRI) has become a crucial tool for evaluating mediastinal masses considering that several lesions that appear indeterminate on computed tomography and radiography can be differentiated on MRI. Using a three-compartment model to localize the mass and employing a basic knowledge of MRI, radiologists can easily diagnose mediastinal masses. Here, we review the use of MRI in evaluating mediastinal masses and present the images of various mediastinal masses categorized using the International Thymic Malignancy Interest Group's three-compartment classification system. These masses include thymic hyperplasia, thymic cyst, pericardial cyst, thymoma, mediastinal hemangioma, lymphoma, mature teratoma, bronchogenic cyst, esophageal duplication cyst, mediastinal thyroid carcinoma originating from ectopic thyroid tissue, mediastinal liposarcoma, mediastinal pancreatic pseudocyst, neurogenic tumor, meningocele, and plasmacytoma.
Topics: Humans; Image Interpretation, Computer-Assisted; Lymphoma; Magnetic Resonance Imaging; Mediastinal Cyst; Mediastinal Neoplasms; Mediastinum; Societies, Medical; Thymoma; Thymus Hyperplasia
PubMed: 32783412
DOI: 10.3348/kjr.2019.0897 -
British Journal of Haematology Apr 2019Primary mediastinal B-cell lymphoma (PMBCL) is a distinct disease closely related to classical nodular sclerosing Hodgkin lymphoma. Conventional diagnostic paradigms... (Review)
Review
Primary mediastinal B-cell lymphoma (PMBCL) is a distinct disease closely related to classical nodular sclerosing Hodgkin lymphoma. Conventional diagnostic paradigms utilising clinical, morphological and immunophenotypical features can be challenging due to overlapping features with other B-cell lymphomas. Reliable diagnostic and prognostic biomarkers that are applicable to the conventional diagnostic laboratory are largely lacking. Nuclear factor kappa B (NF-κB) and Janus kinase/signal transducers and activators of transcription (JAK-STAT) signalling pathways are characteristically dysregulated in PMBCL and implicated in several aspects of disease pathogenesis, and the latter pathway in host immune evasion. The tumour microenvironment is manipulated by PMBCL tumours to avoid T-cell mediated destruction via strategies that include loss of tumour cell antigenicity, T-cell exhaustion and activation of suppressive T-regulatory cells. R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone) and DA-EPOCH-R (dose-adjusted etoposide, prednisolone, vincristine, cyclophosphamide, doxorubicin, rituximab) are the most common first-line immunochemotherapy regimens. End of treatment positron emission tomography scans are the recommended imaging modality and are being evaluated to stratify patients for radiotherapy. Relapsed/refractory disease has a relatively poor outcome despite salvage immunochemotherapy and subsequent autologous stem cell transplantation. Novel therapies are therefore being developed for treatment-resistant disease, targeting aberrant cellular signalling and immune evasion.
Topics: Adult; Antigens, Neoplasm; Clonal Anergy; Female; Humans; Immunotherapy; Janus Kinases; Lymphoma, B-Cell; Male; Mediastinal Neoplasms; Middle Aged; NF-kappa B; STAT Transcription Factors; Signal Transduction; T-Lymphocytes; Tumor Microenvironment; Young Adult
PubMed: 30740662
DOI: 10.1111/bjh.15778 -
Korean Journal of Radiology Jan 2023As the majority of incidentally detected lesions in the anterior mediastinum is small nodules with soft tissue appearance, the differential diagnosis has typically... (Review)
Review
As the majority of incidentally detected lesions in the anterior mediastinum is small nodules with soft tissue appearance, the differential diagnosis has typically included thymic neoplasm and prevascular lymph node, with benign cyst. Overestimation or misinterpretation of these lesions can lead to unnecessary surgery for ultimately benign conditions. nonsurgical anterior mediastinal lesions. The pitfalls of MRI evaluation for anterior mediastinal cystic lesions are as follows: first, we acknowledge the limitation of T2-weighted images for evaluating benign cystic lesions. Due to variable contents within benign cystic lesions, such as hemorrhage, T2 signal intensity may be variable. Second, owing to extensive necrosis and cystic changes, the T2 shine-through effect may be seen on diffusion-weighted images (DWI), and small solid portions might be missed on enhanced images. Therefore, both enhancement and DWI with apparent diffusion coefficient values should be considered. An algorithm will be suggested for the diagnostic evaluation of anterior mediastinal cystic lesions, and finally, a management strategy based on MRI features will be suggested.
Topics: Humans; Mediastinum; Magnetic Resonance Imaging; Thymus Neoplasms; Diffusion Magnetic Resonance Imaging; Diagnosis, Differential; Mediastinal Neoplasms
PubMed: 36606621
DOI: 10.3348/kjr.2022.0606 -
Cancer Journal (Sudbury, Mass.) 2020Diffuse large B-cell lymphoma (DLBCL) encompasses a group of aggressive B-cell non-Hodgkin lymphomas with striking genetic heterogeneity and variable clinical... (Review)
Review
Diffuse large B-cell lymphoma (DLBCL) encompasses a group of aggressive B-cell non-Hodgkin lymphomas with striking genetic heterogeneity and variable clinical presentations. Among these is primary mediastinal B-cell lymphoma (PMBL), which has unique clinical and molecular features resembling Hodgkin lymphoma. Treatment of DLBCL is usually curative, but identifiable subsets at highest risk for treatment failure may benefit from intensified chemotherapy regimens and/or targeted agents added to frontline therapy. Recent comprehensive genomic analyses have identified distinct genetic subtypes of DLBCL with characteristic genetic drivers and signaling pathways that are targetable. Immune therapy with chimeric antigen receptor T cells and checkpoint inhibitors has revolutionized the treatment of relapsed or refractory disease, and antibody drug conjugates have weaponized otherwise intolerable cytotoxic agents. Ongoing clinical trials are further refining the specificity of these approaches in different genetic subtypes and moving them from the setting of recurrent disease to frontline treatment in high-risk patient populations.
Topics: Antineoplastic Combined Chemotherapy Protocols; Clinical Decision-Making; Clinical Trials as Topic; Disease-Free Survival; Drug Resistance, Neoplasm; Humans; Immune Checkpoint Inhibitors; Immunoconjugates; Immunotherapy, Adoptive; Lymphoma, Large B-Cell, Diffuse; Mediastinal Neoplasms; Molecular Targeted Therapy; Neoplasm Recurrence, Local; Progression-Free Survival; Receptors, Chimeric Antigen
PubMed: 32496453
DOI: 10.1097/PPO.0000000000000450 -
Journal of Medical Case Reports Aug 2023Mediastinal Liposarcoma (ML) is an exceedingly rare neoplasm, accounting for less than 1% of all liposarcomas. Surgical resection is the most effective therapeutic... (Review)
Review
BACKGROUND
Mediastinal Liposarcoma (ML) is an exceedingly rare neoplasm, accounting for less than 1% of all liposarcomas. Surgical resection is the most effective therapeutic modality, while adjuvant radiation therapy may be recommended for unresectable tumors.
CASE PRESENTATION
This case report presents a rare case of a 52-year-old Syrian male patient with well-differentiated mediastinal liposarcoma (ML) who presented with exertional dyspnea, cough, and weight loss. Imaging studies revealed a large tumor causing extrinsic compression on the right lung, as well as compression of the heart and great vessels. CT-guided biopsy confirmed a diagnosis of well-differentiated liposarcoma, and the patient underwent surgical resection. The patient had a rapid postoperative recovery and was discharged on the fourth day post-operation, displaying an excellent postoperative status. The patient was followed up for 3 months postoperatively with no recurrence.
CONCLUSION
This report highlights the significance of incorporating ML into the differential diagnosis of mediastinal masses, particularly in individuals presenting with exertional dyspnea and weight loss. Furthermore, this report provides a comprehensive review of previously reported cases of ML in the medical literature.
Topics: Male; Humans; Middle Aged; Mediastinal Neoplasms; Liposarcoma; Heart; Mediastinum; Dyspnea; Lipoma
PubMed: 37649065
DOI: 10.1186/s13256-023-04121-7 -
Haematologica Apr 2011
Topics: Humans; Lymphoma; Mediastinal Neoplasms
PubMed: 21454881
DOI: 10.3324/haematol.2011.043026 -
Endocrine Journal Oct 2022Papillary thyroid carcinoma (PTC) is a relatively indolent disease, despite the high incidence of lymph node metastases. Although less frequent, some upper mediastinal...
Papillary thyroid carcinoma (PTC) is a relatively indolent disease, despite the high incidence of lymph node metastases. Although less frequent, some upper mediastinal metastases of PTC cannot be removed without sternal resection. In this study, we investigated the prognostic impact of upper mediastinal dissection (UMD) by sternotomy on patients with mediastinal metastases of PTC. Charts of patients with PTC who underwent surgical treatment at our institution between 2006 and 2018 were retrospectively reviewed. Fifty-eight patients with upper mediastinal metastases were enrolled. Kaplan-Meier survival curves were compared, and Cox hazard regression models were used for analyses. Of the 58 patients with mediastinal metastasis, 12 (20.7%) underwent dissection of the prevascular nodes, 51 (87.9%) underwent dissection of the upper paratracheal nodes, and 14 (24.1%) underwent dissection of the lower paratracheal node. The preferred site of mediastinal metastasis was the upper paratracheal nodes. The 5 and 10-year disease-specific survival rates for patients after UMD were 74.6% and 58.7%, respectively. Among 25 patients (43.1%) with locoregional recurrence, 12 (20.7%) had mediastinal recurrence and 7 were eligible for additional UMD. Although distant metastasis was the predominant poor prognostic factor, mediastinal recurrences were more frequently unresectable than cervical recurrences, suggesting that mediastinal recurrence is a poor prognostic factor. Mediastinal metastases larger than 30 mm or metastases to the lower paratracheal nodes are considered a risk factor for mediastinal recurrence. UMD by sternotomy for patient with upper mediastinal metastases which are difficult to resect via transcervical approach is an effective treatment option to improve patient prognosis.
Topics: Humans; Thyroid Cancer, Papillary; Carcinoma, Papillary; Thyroid Neoplasms; Sternotomy; Thyroidectomy; Retrospective Studies; Lymph Node Excision; Neoplasm Recurrence, Local; Neck Dissection; Lymph Nodes; Mediastinal Neoplasms
PubMed: 35650025
DOI: 10.1507/endocrj.EJ22-0033 -
American Journal of Respiratory and... Nov 2013The current management of lymphoma requires accurate diagnosis and subtyping of de novo lymphoma and of relapsed or refractory lymphoma in known cases. The role of... (Clinical Trial)
Clinical Trial
RATIONALE
The current management of lymphoma requires accurate diagnosis and subtyping of de novo lymphoma and of relapsed or refractory lymphoma in known cases. The role of endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) in the clinical management of lymphomas is unclear.
OBJECTIVES
To investigate the use of EBUS-TBNA in the diagnosis of de novo and relapsed mediastinal lymphomas.
METHODS
A total of 2,256 consecutive patients who underwent EBUS-TBNA in a tertiary center between February 2008 and April 2013 were prospectively evaluated. The diagnostic accuracy and clinical use of EBUS-TBNA in 100 cases of de novo or suspected relapsed mediastinal lymphoma was investigated by comparing EBUS-TBNA diagnosis with the final diagnosis.
MEASUREMENTS AND MAIN RESULTS
De novo mediastinal lymphoma was correctly diagnosed by EBUS-TBNA in 45 (88%) of 51 and relapsed lymphoma in 15 (100%) of 15 lymphoma cases. EBUS-TBNA accurately established a diagnosis other than lymphoma in 32 (97%) of 33 patients with suspected lymphoma relapse. Sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of EBUS-TBNA in the diagnosis of mediastinal lymphoma were 89%, 97%, 98%, 83%, and 91%, respectively. Sensitivity of EBUS-TBNA in subtyping lymphomas into high-grade non-Hodgkin lymphoma, low-grade non-Hodgkin lymphoma, and Hodgkin lymphoma was 90%, 100%, and 79%, respectively. EBUS-TBNA diagnosis was adequate for clinical management in 84 (84%) of 100 cases.
CONCLUSIONS
Multimodality evaluation of EBUS-TBNA can be successful in the diagnosis of de novo mediastinal lymphomas and is ideally suited in distinguishing lymphoma relapse from alternative pathologies; it is least sensitive in subtyping Hodgkin lymphoma.
Topics: Adult; Aged; Aged, 80 and over; Diagnosis, Differential; Endoscopic Ultrasound-Guided Fine Needle Aspiration; Female; Humans; Lymphoma; Male; Mediastinal Neoplasms; Middle Aged; Neoplasm Recurrence, Local; Predictive Value of Tests; Prospective Studies; Sensitivity and Specificity
PubMed: 24047336
DOI: 10.1164/rccm.201303-0462OC