scholarly journals Ectopic Anterior Mediastinal Pathology in the Chest: Radiologic-pathologic Correlation of Unexpected Encounters with the “Terrible Ts”

2016 ◽  
Vol 6 ◽  
pp. 49 ◽  
Author(s):  
Mahsan Rashidfarokhi ◽  
Jessica Gupta ◽  
Anatoly Leytin ◽  
Oleg Epelbaum
Keyword(s):  
Mediastinal Mass ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Lung Parenchyma ◽  
Anterior Mediastinum ◽  
Pleural Space ◽  
Anterior Mediastinal Mass ◽  
Primary Tumors ◽  
Primary Neoplasms ◽  
Mediastinal Pathology

The complex embryology of the anterior mediastinum makes it home to an array of primary neoplasms tied to the presence of the thyroid and thymus glands in that compartment. While the occurrence of ectopic thyroid deposits in the extramediastinal thorax has not been convincingly established, the other three “Ts” of the classic “4T” mnemonic for the differential diagnosis of an anterior mediastinal mass have occurred in the lung parenchyma, pleural space, and endobronchially as primary tumors. Finding any of the three lesions – thymoma, teratoma, or B-cell lymphoma – in the chest outside the mediastinum is very unusual, but that possibility exists. Herein, we illustrate examples of this rare phenomenon.

Pneumomediastinum as a complication to treatment of mediastinal (thymic) large b‐cell lymphoma

2005 ◽  
Vol 46 (4) ◽  
pp. 371-373
Author(s):  
G. Pavlisa ◽  
A. Planinc‐Peraica ◽  
P. Anic ◽  
I. Kardum‐Skelin ◽  
G. Pavlisa ◽  
...  
Keyword(s):  
B Cell ◽  
Mediastinal Mass ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Tracheobronchial Tree ◽  
Anterior Mediastinal Mass ◽  
Axial Diameter ◽  
Large B Cell Lymphoma ◽  
Fluid Levels ◽  
Large B Cell

Mediastinal (thymic) large B‐cell lymphoma (Med‐DLBCL) is a subtype of diffuse large B‐cell lymphomas (DLBCL) with a typical radiological appearance of bulky anterior mediastinal mass, often with areas of necrosis. We report a case of Med‐DLBCL with unusual radiological findings and clinical development. Computed tomography (CT) obtained at presentation revealed a huge anterior mediastinal tumor with an axial diameter of 180 mm. Nineteen days after the first cycle of chemotherapy, chest radiography and CT revealed large areas of tumor necrosis and pneumomediastinum with air‐fluid levels. To our knowledge, air‐fluid levels inside Med‐DLBCL have not been previously described. This finding, in combination with necrotic sputum, may indicate communication between the tracheobronchial tree and the tumor.

Persistent FDG Avid Anterior Mediastinal Mass in the Setting of Treated Diffuse Large B-Cell Lymphoma

CHEST Journal ◽  
2013 ◽  
Vol 144 (4) ◽  
pp. 634A
Author(s):  
Kevin Kane ◽  
Jennifer Toth ◽  
Christopher Gilbert ◽  
Michael Reed
Keyword(s):  
B Cell ◽  
Mediastinal Mass ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Anterior Mediastinal Mass ◽  
Large B Cell Lymphoma ◽  
Large B Cell

Ectopic Primary B Cell Lymphoma of the Thyroid Presenting as an Anterior Mediastinal Mass. A Case Report

2009 ◽  
Vol 109 (6) ◽  
pp. 802-804 ◽  
Author(s):  
F. Demirag ◽  
E. Cakir ◽  
E. Aydin ◽  
S. Kaya ◽  
N. Akyurek
Keyword(s):  
Case Report ◽  
B Cell ◽  
Mediastinal Mass ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Anterior Mediastinal Mass

An Interesting Case Report of A Concurrent Classic Hodgkin Lymphoma and Gray Zone Lymphoma in the Mediastinum

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S110-S110
Author(s):  
B Mai ◽  
J Huddin ◽  
Z Hu
Keyword(s):  
B Cell ◽  
Hodgkin Lymphoma ◽  
Mediastinal Mass ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Gray Zone ◽  
Large B Cell Lymphoma ◽  
Classic Hodgkin Lymphoma ◽  
Atypical Cells ◽  
Large B Cell

Abstract Casestudy A 52-year-old female presented with night sweats, chills, anorexia, and weight loss. Computed tomography and positron emission tomography showed a soft tissue infiltration in the anterior mediastinum and hypermetabolic bilateral supraclavicular, mediastinal, right hilar, and left internal mammary lymph nodes. An anterior mediastinal mass resection and thymectomy was subsequently performed. Results Sections of the mediastinal mass showed Hodgkin/Reed-Sternberg cells (HRS) admixed with small lymphocytes, histiocytes, plasma cells, and eosinophils. The HRS cells are positive for CD30, CD15, and MUM1, faintly positive for PAX5, and negative for CD20, CD45, CD79a, and BCL6. The morphology and immunophenotype is diagnostic of nodular sclerosis classic Hodgkin lymphoma (CHL). Sections of the thymectomy specimen showed similar morphology, however, in an area that represents 10-20% of the specimen, there are nodular and diffuse lymphoid infiltrates consisting of small lymphocytes, histiocytes, and large atypical cells. The large atypical cells are positive for CD20, CD23, CD30, CD45, CD79a, BCL2, BCL6, MUM-1, and PAX5, and negative for CD1a, CD3, CD57, and Cyclin D1. The background small CD3-positive lymphocytes form a rosette around most of the large atypical cells. CD21 and CD23 stains highlight residual follicular structures. In situ hybridization for EBV-encoded RNA (EBER) is negative. The presence of residual follicular meshwork with an immunophenotype of large B cell lymphoma supports a diagnosis of a gray zone lymphoma (GZL). Overall, CHL is involving 80-90% and GZL is involving 10-20% of the thymic tissue. The patient was subsequently placed on ABVD chemotherapy and achieved remission. Conclusion An accurate diagnosis of GZL is challenging. GZL is a rare type of lymphoma with morphological features between CHL and diffuse large B-cell lymphoma (DLBCL). It is even rarer to encounter a CHL concurrently present with a GZL. The optimal therapeutic approach for cases with concurrent lymphoma diagnosed with CHL and GZL needs further investigation.

Unclassifiable lymphoma in pregnancy

2021 ◽  
Vol 14 (2) ◽  
pp. e239462
Author(s):  
Mariola Hernández Martínez ◽  
César Lizán Tudela ◽  
Ana Saus Carreres
Keyword(s):  
Mediastinal Mass ◽  
Cell Lymphoma ◽  
Elective Caesarean Section ◽  
B Cell Lymphoma ◽  
Fetal Outcome ◽  
Large B Cell Lymphoma ◽  
Acute Complications ◽  
Chemotherapy Regime ◽  
Unusual Occurrence ◽  
Multidisciplinary Teamwork

A 28-year-old woman, 15 weeks pregnant, consulted for cervical ache and left laterocervical mass. Imaging scans revealed a large mediastinal mass that had spread to supraclavicular and left axillar spaces, including cervicobrachial plexus. Pathological anatomy confirmed an unclassifiable lymphoma, with intermediate features between diffuse large B-cell lymphoma and classical Hodgkin’s lymphoma. CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) therapy was started with partial response. But due to disease progression, an elective caesarean section was performed (30th week of pregnancy) to begin a chemotherapy regime incompatible with pregnancy. Despite acute complications related to prematurity, the newborn could be discharged from hospital at 45 days of life. The patient did not respond to the second treatment line and she is currently undergoing a third chemotherapy regime. Given the unusual occurrence of lymphoma during pregnancy, multidisciplinary teamwork between haematologists, neonatologists and obstetricians is essential to achieve the best maternal–fetal outcome.

Limited Tissue Biopsies and Hematolymphoid Neoplasms

2019 ◽  
Vol 152 (6) ◽  
pp. 782-798
Author(s):  
Kimberly F Ingersoll ◽  
Yue Zhao ◽  
Grant P Harrison ◽  
Yang Li ◽  
Lian-He Yang ◽  
...  
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Excisional Biopsy ◽  
Needle Aspiration ◽  
High Rate ◽  
P Value ◽  
History Of ◽  
Primary Neoplasms ◽  
The Difference

Abstract Objectives Use of fine-needle aspiration/needle core biopsy (FNA/CNB) in evaluating hematolymphoid processes has been debated. We investigate its applicability in various clinicopathologic settings. Methods We retrospectively analyzed 152 cases of FNA/CNB. Results Of 152 FNA/CNBs, 124 (81.6%) resulted in diagnoses without excisional biopsies, while 28 required subsequent excisional biopsies. Of these, 43 FNA/CNBs performed for suspected lymphoma relapse demonstrated 95.4% diagnostic rate (41/43), which was significantly better than those without history of lymphoma (77/109, 71%; odds ratio [OR], 8.5; confidence interval, 1.9-37.4). Patients with immunodeficiency also showed a high rate of diagnosis by FNA/CNB (100%). When stratified by types of disease, diffuse large B-cell lymphoma/high-grade B-cell lymphoma demonstrated a higher success rate (92.7%) than small B-cell lymphoma (79.2%), though the difference was not statistically significant (OR, 3.3; P value = .07). A subsequent excisional biopsy was required in 28 cases, 23 of which resulted in diagnoses concordant with the FNA/CNB. Five cases showed diagnostic discordance, reflecting pitfalls of FNA/CNB in unusual cases with complex pathology. Conclusions FNA/CNB is practical in evaluating most hematolymphoid lesions, with high efficacy in recurrent disease and some primary neoplasms with homogeneous/ aggressive histology, or characteristic immunophenotype.

Loss of Tnk1/Kos1 in Mice Is Associated with B-Lymphomas Specifically DLBCL.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 3972-3972
Author(s):  
Kishalay Hoare ◽  
Mary K Reinhard ◽  
Sarasija Hoare ◽  
Tammy Flagg ◽  
W. Stratford May
Keyword(s):  
Tyrosine Kinase ◽  
B Cell ◽  
Knockout Mice ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Negative Regulator ◽  
Allelic Loss ◽  
B Cell Lymphomas ◽  
Primary Tumors ◽  
Cell Functions

Abstract Abstract 3972 Poster Board III-908 The ubiquitously expressed nonreceptor tyrosine kinase (NRPTK) Tnk1/Kos1 (Thirty-eight negative kinase/Kinase of the embryonic stem cell) functions as a negative regulator of growth in both murine and human cells by suppressing the Ras-Raf1-MapK growth pathway. Since Tnk1 requires its intrinsic protein tyrosine kinase activity to suppress Ras activity and cell growth, the kinase domain is critical for its function and deletion by targeted homologous recombination leads to spontaneous tumor development in mice. To date, Tnk1/Kos1 is the only reported NRPTK that functions as a tumor suppressor in vivo, while other tyrosine kinases may be oncogenic when mutated or activated. While Tnk1 knockout mice may develop primary tumors in different tissues/organs, mainly B-cell lymphomas develop in Tnk1-/- (80%, 47 of 60) and Tnk1+/- (57%, 31 of 54) mice with similar characteristics of Diffuse Large B-Cell Lymphoma (DLBCL) and Burkitt Lymphoma types. Typically in lymphomas from Tnk1+/- mice the intact wild type allele is epigenetically modified and silenced by promoter methylation. Importantly, the absence of Tnk1 occurs only in the tumor tissue but not in the adjacent uninvolved tissue. Now we find allelic loss with associated reduced expression of Tnk1 transcripts and protein in a cohort of human DLBCL patients. These data underscore the potential clinical relevance of Tnk1 in human hematological malignancies. Furthermore, the B-cell lymphomas that develop in the Tnk1 knockout mice express aberrantly high Ras activity indicating that unmutated Ras is a likely necessary effector of B-cell lymphoma development and survival. We also recently determined that the aberrantly high levels Ras activity in lymphoma (but not paired uninvolved lymphoid tissue) from mice results from a novel mechanism involving stabilization of the Grb2-Sos1 complex to maintain activated Ras in these tissues. Therefore, the Tnk1 knockout mouse provides a unique opportunity to test whether and how Tnk1 is involved in the development and/or maintenance of the B-cell lymphomas that develop in the absence of Ras mutation which may have clinical significance for patients with lymphoma. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Mutation analysis links angioimmunoblastic T-cell lymphoma to clonal hematopoiesis and smoking

eLife ◽  
2021 ◽  
Vol 10 ◽  
Author(s):  
Shuhua Cheng ◽  
Wei Zhang ◽  
Giorgio Inghirami ◽  
Wayne Tam
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Primary Tumors ◽  
Angioimmunoblastic T Cell Lymphoma ◽  
Clonal Hematopoiesis ◽  
Not Otherwise Specified ◽  
Smoking Exposure ◽  
Hematopoietic Precursor

Background:Although advance has been made in understanding the pathogenesis of mature T-cell neoplasms, the initiation and progression of angioimmunoblastic T cell lymphoma (AITL) and peripheral T cell lymphoma, not otherwise specified (PTCL-NOS), remains poorly understood. A subset of AITL/PTCL-NOS patients develop concomitant hematologic neoplasms (CHN), and a biomarker to predict this risk is lacking. Methods:We generated and analyzed the mutation profiles through 537-gene targeted sequencing of the primary tumors and matched bone marrow/peripheral blood samples in 25 patients with AITL and 2 with PTCL-NOS. Results:Clonal hematopoiesis (CH)-associated genomic alterations, found in 70.4% of the AITL/PTCL-NOS patients, were shared among CH and T-cell lymphoma, as well as concomitant myeloid neoplasms or diffuse large B-cell lymphoma (DLBCL) that developed before or after AITL. Aberrant AID/APOBEC activity-associated and tobacco smoking-associated mutational signatures were respectively enriched in the early CH-associated mutations and late non-CH associated mutations during AITL/PTCL-NOS development. Moreover, analysis showed that the presence of CH harboring ≥ 2 pathogenic TET2 variants with ≥ 15% of allele burden conferred higher risk for CHN (P = 0.0006, hazard ratio = 14.01, PPV=88.9%, NPV=92.1%). Conclusion:We provided genetic evidence that AITL/PTCL-NOS, CH, CHN can frequently arise from common mutated hematopoietic precursor clones. Our data also suggests smoking exposure as a potential risk factor for AITL/PTCL-NOS progression. These findings provide insights into the cell origin and etiology of AITL and PTCL-NOS and provide a novel stratification biomarker for CHN risk in AITL patients. Funding:R01 grant (CA194547) from the National Cancer Institute to WT.

Mutational Landscape of Grey Zone Lymphoma

Blood ◽  
2020 ◽  
Author(s):  
Clementine Sarkozy ◽  
Stacy S. Hung ◽  
Elizabeth A. Chavez ◽  
Gerben Duns ◽  
Katsuyoshi Takata ◽  
...  
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Anterior Mediastinal Mass ◽  
Grey Zone ◽  
Cell Of Origin ◽  
Disease Evolution ◽  
Mutational Landscape ◽  
Large B Cell Lymphoma ◽  
Large B Cell

The mutational landscape of grey zone lymphoma (GZL) has not yet been established and differences to related entities are largely unknown. Here, we studied coding sequence mutations of 50 EBV-negative GZL and 20 polymorphic EBV-positive DLBCL NOS (poly-EBV-L) in comparison to classical Hodgkin lymphoma (cHL), primary mediastinal large B cell lymphoma (PMBCL), and diffuse large B cell lymphoma (DLBCL). Exomes of 21 GZL and 7 poly-EBV-L cases along with paired normals 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) displayed 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, enriched in mutations related to apoptosis defects (TP53 (39%), BCL2 (28%), BIRC6 (22%)) and depleted in GNA13, XPO1or NFKB signaling pathway mutations (TNFAIP3, NFKBIE, IKBKB, NFKBIA). They also presented more BCL2/BCL6 rearrangements as opposed to thymic GZL. Poly-EBV-L cases presented a distinct mutational profile including STAT3 mutations and a significantly lower coding-mutation load in comparison to EBV-negative GZL. Our study highlights characteristic mutational patterns in GZL associated with presentation in the thymic niche suggesting a common cell of origin with disease evolution overlapping with related anterior mediastinal lymphomas.

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