Mediastinal Gray Zone Lymphoma with Features Intermediate between Classical Hodgkin Lymphoma and Primary Mediastinal B-Cell Lymphoma

2016 ◽  
Vol 136 (3) ◽  
pp. 186-190 ◽  
Author(s):  
Haa-Na Song ◽  
Seok Jin Kim ◽  
Young Hyeh Ko ◽  
Won Seog Kim
Keyword(s):  
B Cell ◽  
Hodgkin Lymphoma ◽  
Systemic Chemotherapy ◽  
Cell Lymphoma ◽  
Progression Free Survival ◽  
B Cell Lymphoma ◽  
Gray Zone ◽  
Bulky Disease ◽  
Gray Zone Lymphoma ◽  
Frontline Therapy

Background: Mediastinal gray zone lymphoma (MGZL) shares clinical characteristics with primary mediastinal B-cell lymphoma (PMBCL) and nodular sclerosing Hodgkin lymphoma (NSHL). However, MGZL is extremely rare, and an appropriate treatment for it has not yet been established. Methods: We retrospectively analyzed 8 patients who were treated with systemic chemotherapy for MGZL between 2007 and 2014. Results: The patients with MGZL were predominantly young and male (median age 26 years), and 62.5% of patients had bulky disease. The overall response rate (ORR) and complete remission (CR) rate were both 75% (6/8) for all treated patients The median overall survival (OS) and progression-free survival (PFS) was 40.7 and 3.9 months, respectively. Most responders (4/6, 66.7%) were treated with R-CHOP (rituximab + cyclophosphamide, hydroxydaunorubicin, Oncovin and prednisolone) as the frontline therapy. The CR rate of patients who received R-CHOP and those who did not was 100% (4/4) and 50% (2/4), respectively. Particularly striking was the finding that the median PFS of patients who received R-CHOP frontline chemotherapy was 11.4 months, which was superior to the median PFS of patients who did not receive R-CHOP. Conclusions: Of the 8 patients with MGZL who were treated with systemic chemotherapy, superior treatment responses were observed in patients who received R-CHOP as the frontline therapy.

Expression pattern of PD-L1 and PD-L2 in classical Hodgkin lymphoma, primary mediastinal large B-cell lymphoma, and gray zone lymphoma

2018 ◽  
Vol 100 (5) ◽  
pp. 511-517 ◽  
Author(s):  
Yosuke Tanaka ◽  
Akiko Miyagi Maeshima ◽  
Junko Nomoto ◽  
Shinichi Makita ◽  
Suguru Fukuhara ◽  
...  
Keyword(s):  
B Cell ◽  
Hodgkin Lymphoma ◽  
Expression Pattern ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Gray Zone ◽  
Classical Hodgkin Lymphoma ◽  
Gray Zone Lymphoma ◽  
Large B Cell Lymphoma ◽  
Large B Cell

scholarly journals “Do Not Ignore Musculoskeletal Pain”—Initial Presentation of a Rare Malignant Disease: Case Report and Literature Review

2020 ◽  
Vol 8 ◽  
pp. 232470962094131
Author(s):  
Ishan Patel ◽  
Arda Akoluk ◽  
Vandan Upadhyaya ◽  
Shraddhadevi Makadia ◽  
Steven Douedi ◽  
...  
Keyword(s):  
Case Report ◽  
B Cell ◽  
Hodgkin Lymphoma ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Gray Zone ◽  
Classical Hodgkin Lymphoma ◽  
Gray Zone Lymphoma ◽  
Large B Cell Lymphoma ◽  
Large B Cell

Gray zone lymphoma, also known as B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma, is a rare malignancy with overlapping features of both diffuse large B-cell lymphoma and classical Hodgkin lymphoma. Most commonly mediastinal involvement is seen. Extranodal involvement is rare. In this case report, we present the case of a 59-year-old male who presented with stress-related left shoulder pain, ultimately diagnosed with gray zone lymphoma. The patient was treated with etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin-rituximab (EPOCH-R) regimen followed by consolidation radiotherapy resulting in complete response. We are highlighting this case as rare and atypical presentation of a rare disease.

scholarly journals Gray-zone lymphoma. Examples of rare clinical manifestation

2019 ◽  
Vol 91 (4) ◽  
pp. 107-113
Author(s):  
A E Misyurina ◽  
S K Kravchenko ◽  
Ya K Mangasarova ◽  
A U Magomedova ◽  
A M Kovrigina ◽  
...  
Keyword(s):  
B Cell ◽  
Hodgkin Lymphoma ◽  
Clinical Manifestation ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Mediastinal Lymphadenopathy ◽  
Grey Zone ◽  
Gray Zone ◽  
Classical Hodgkin Lymphoma ◽  
Gray Zone Lymphoma

Mediastinal gray-zone lymphoma (MGZL, lymphoma with features intermediate between classical Hodgkin lymphoma and diffuse large B-cell lymphoma) was declared as a separate entity in WHO classification of Tumors of Haematopoetic and Lymphoid Tissues in 2008 and 2017 years. Despite of similar pathomorphological characteristics between primary mediastinal B-cell lymphoma and Hodgkin lymphoma, clinical features and optimal therapeutic approach to MGZL are not clearly defined. Usually MGZL manifests with mediastinal lymphadenopathy, although extranodal lesions often occur (grey-zone lymphoma, GZL). Patients with MGZL have unfavorable prognosis, taking into account high rate of relapse. This article describes two cases of MGZL. First case manifested by arrhythmias due to primary heart involvement. In spite of cardiac failure antracycline-containing chemotherapy (6 courses of R-DA-EPOCH) it allowed to achieve a complete remission and resolving of arrhythmias. Second case was represented by metachronous tumors: primary mediastinal B-cell lymphoma at the time of disease onset and classical Hodgkin lymphoma, NS II, diagnosed after disease progression. Thus, we demonstrated two examples of MGZL that differ by clinical manifestation, response to chemotherapy, which emphasizes an importance of pathogenesis studying, and using of new therapeutic approaches.

scholarly journals Gray Zone Lymphoma: Better Treated Like Hodgkin Lymphoma or Mediastinal Large B-Cell Lymphoma?

2012 ◽  
Vol 7 (3) ◽  
pp. 241-247 ◽  
Author(s):  
Kieron Dunleavy ◽  
Cliona Grant ◽  
Franziska C. Eberle ◽  
Stefania Pittaluga ◽  
Elaine S. Jaffe ◽  
...  
Keyword(s):  
B Cell ◽  
Hodgkin Lymphoma ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Gray Zone ◽  
Gray Zone Lymphoma ◽  
Large B Cell Lymphoma ◽  
Large B Cell

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.

Primary Mediastinal (Thymic) Large B-Cell Lymphoma: A Short Review With Brief Discussion of Mediastinal Gray Zone Lymphoma

2011 ◽  
Vol 135 (3) ◽  
pp. 394-398 ◽  
Author(s):  
Charles Blake Hutchinson ◽  
Endi Wang
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Short Review ◽  
Comparative Genomic ◽  
Gray Zone ◽  
Superior Mediastinum ◽  
Gray Zone Lymphoma ◽  
Large B Cell Lymphoma ◽  
Large B Cell

Abstract Primary mediastinal (thymic) large B-cell lymphoma (PMBCL) is a subtype of diffuse large B-cell lymphoma (DLBCL). It commonly presents as a bulky lesion in the anterior-superior mediastinum with symptoms related to local invasion or compression. Microscopic examination typically shows infiltration of medium-large cells surrounded by collagen fibrosis. The neoplastic cells express B-cell markers, and CD30 often shows heterogeneous staining. Comparative genomic hybridization has identified gains in loci of 9p24 and 2p15 as well as Xp11.4-21 and Xq24-26. Amplification of REL and BCL11A at 2p as well as elevated expression of JAK2, PDL1, and PDL2 at 9p has been demonstrated. Nodular sclerosis classic Hodgkin lymphoma needs to be differentiated from PMBCL and cases with overlapped features have been described as mediastinal gray zone lymphoma. Primary mediastinal (thymic) large B-cell lymphoma carries a favorable prognosis in comparison to conventional DLBCL.

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