Mediastinal gray zone lymphoma: clinico-pathological characteristics and outcomes of 99 patients from the Lymphoma Study Association

Abstract

Mediastinal gray zone lymphoma, B-cell lymphomas with intermediate features between classical Hodgkin lymphoma and primary mediastinal B-cell lymphoma, have not been well described in the literature. We report the clinical characteristics and outcomes of a large retrospective series of 99 cases centrally reviewed by a panel of hematopathologists, with a consensus established for the diagnosis. Cases were defined as classical Hodgkin lymphoma-like morphology (64.6%) with primary mediastinal B-cell lymphoma immunophenotype, primary mediastinal B-cell lymphoma-like morphology (30.3%) with classical Hodgkin lymphoma or composite (5.1%) (synchronous occurrence of classical Hodgkin lymphoma and primary mediastinal B-cell lymphoma). The median age was 32 years (13-83 years); 55% were women. Thirteen of 81 evaluable cases (16%) were Epstein-Barr virus-positive. Twenty-eight percent of patients presented primary refractory disease (progression under first-line treatment or relapse within one year). The 3-year event-free and overall survival rates were 63% and 80%, respectively. Patients treated with a standard regimen (RCHOP/ABVD) had worse event-free survival (P=0.003) and overall survival (P=0.02) than those treated with a dose-intensive chemotherapy (high-dose RCHOP/escalated BEACOPP). Rituximab added to chemotherapy was not associated with better event-free survival (P=0.55) or overall survival (P=0.88). Radiotherapy for patients in complete remission had no impact on eventfree survival. In multivariate prognostic analysis, ECOG-PS and anemia were the strongest factors associated with a shorter event-free survival and overall survival, respectively. In conclusion, this report describes the largest series of mediastinal gray zone lymphoma. Our data suggest that a dose-intensive treatment might improve the outcome of this rare and aggressive disease.

Clinicopathologic consensus study of gray zone lymphoma with features intermediate between DLBCL and classical HL

“…A recent French series of GZL reported improved outcomes for patients treated with more intensive therapeutic regimens (ie, methylprednisolone, doxorubicin, cyclophosphamide, procarbazine, etoposide, bleomycin, vincristine (escBEACOPP) or doxorubicin, methylprednisolone, cyclophosphamide, bleomycin, vindesine. 29 It is unclear whether these more dose-intensive regimens are needed for therapy of GZL. It should be highlighted that 3-year event-free survival rates for these regimens were 73% and 70%, respectively, which compares with the 71% PFS rate for patients who received CHOP6R in the current series.…”

Biology and therapy of primary mediastinal B‐cell lymphoma: current status and future directions

“…The cytological appearance may be discordant with the immunophenotype, although this alone should not lead to a MGZL diagnosis (Swerdlow et al , ). Like PMBCL, MGZL presents with symptoms of localised compression and is more common in women, with a median age of 30 years (Sarkozy et al , ; Swerdlow et al , ).…”

Management of children and adolescents with gray zone lymphoma: A case series

“…sclerosing subtype). [1][2][3][4][5][6][7][8][9] Both entities may appear simultaneously in one sample (cHL-like, LBCL-like, and composite GZL subtype) or recur sequentially as one of either entity. 9 Therefore, GZL constitutes a "missing link" along a continuum between the two conditions.…”

“…4,5,7,9 GZL shows a male predominance, [3][4][5]7,8,11,12 usually involves the mediastinum, 2,3,5,7,8 and appears to have a higher tendency toward primary resistance, relapse, as well as toward inferior outcome as compared with PMLBCL or nodular-sclerosing cHL per se. 3,4,[7][8][9]12 Given the rarity of the disease and the fact that cHL and PML-BCL may occur simultaneously as a composite disease or sequentially but are usually treated differently, standardized therapeutic concepts are lacking, especially in pediatrics. 3,[6][7][8] Rituximab-based B-cell non-Hodgkin lymphoma (B-NHL)-directed regimens such as DA-EPOCH-R (dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, and rituximab) appear to be preferable for first-line treatment.…”