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ICML 2025 | The LYSA guidelines for the diagnosis and management of primary mediastinal B-cell lymphoma

Vincent Camus, MD, Centre Henri Becquerel & LYSA Group Member, Rouen, France, highlights the recent publication of the Lymphoma Study Association (LYSA) guidelines for the diagnosis and management of primary mediastinal B-cell lymphoma (PMBCL), and comments on the importance of molecular testing in this disease. He emphasizes the need for combining biopsy and molecular genotyping to ensure an accurate diagnosis of PMBCL, as this influences the therapeutic strategy that should be selected for patients. This interview took place during the 18th International Conference on Malignant Lymphoma (18-ICML) in Lugano, Switzerland.

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Transcript

This year the LYSA group published the LYSA guidelines for primary mediastinal B-cell lymphoma in the European Journal of Cancer. It was a very collaborative work of the LISA group. More than 40 experts, pathologists, molecular biologists, also PET scan experts and clinicians have worked together to cover all the features of the primary mediastinal B-cell lymphoma, diagnosis, follow-up, and treatment...

This year the LYSA group published the LYSA guidelines for primary mediastinal B-cell lymphoma in the European Journal of Cancer. It was a very collaborative work of the LISA group. More than 40 experts, pathologists, molecular biologists, also PET scan experts and clinicians have worked together to cover all the features of the primary mediastinal B-cell lymphoma, diagnosis, follow-up, and treatment. 

And one of the main challenges is the diagnosis pathway because it’s a hard to diagnose lymphoma, a hard to biopsy anatomical site, the mediastinum. And we observe that there are difficulties for the pathologist to identify with only the biopsy, diagnosis of primary mediastinal B-cell lymphoma, so we suggest adding molecular testing, molecular genotyping for all cases of suspicion of primary mediastinal B-cell lymphoma, because there is a specific genotype landscape, a mutational landscape, that is very useful to confirm the diagnosis of primary mediastinal lymphoma. So we suggest that the pathologist can help, can do a first pathology report with probably this is a primary mediastinal lymphoma, but then to confirm with molecular testing with either next-generation sequencing or gene expression profiling to be certain, absolutely sure of the diagnosis of PMBL, because the consequences for the patient are important. The treatment is a little bit different with a dose intensity that is very important for primary mediastinal B-cell lymphoma, and this is different from DLBCL, for example. So the dose intensity, the treatment at the first line is quite different. So it’s important to know if this is a PMBL or just a DLBCL with a secondary mediastinal lymphoma, because you will not treat the patient the same way. And also in the relapse setting, the PMBL is very sensitive to checkpoint inhibitor anti-PD-1 antibodies, so it’s very important to do a re-biopsy again when there is a suspicion of relapse and to be sure that this is still a PMBL histology, and so you can decide between CAR T-cells and anti-PD-1 checkpoint inhibitor in the relapse setting.

 

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