Extramedullary plasmacytoma was initially thought to be a rare feature of multiple myeloma, but now with the introduction of more advanced imaging techniques and wider use of PET-CT, now we’re recognizing EMD at diagnosis within 5 to 10 percent of patients and this incidence is increasing with the relapses and at later phases of more advanced patients, the incidence is even higher...
Extramedullary plasmacytoma was initially thought to be a rare feature of multiple myeloma, but now with the introduction of more advanced imaging techniques and wider use of PET-CT, now we’re recognizing EMD at diagnosis within 5 to 10 percent of patients and this incidence is increasing with the relapses and at later phases of more advanced patients, the incidence is even higher. Initially, extramedullary disease, if it happens as a single feature, what we call a solitary plasmacytoma, was regarded as a disease which is separate from multiple myeloma and was treated with a unique approach with surgery and radiotherapy without the systemic therapy. But with newer tools, if we measure in depth the M protein in the blood or in urine with free light chains, it’s possible that we may find dissemination of the disease, and that’s a sign that we need to give a systemic therapy.
And if a patient is diagnosed with extramedullary disease, it’s unfortunately a poor prognostic feature. For this purpose, we need to give our best available treatment possible, and that includes usually the quadruplets, and we need to give a PI-based regimen. In plus, one may consider using such as a lymphoma type of polychemotherapy, and this is usually more recommended for patients who are presenting with secondary extramedullary plasmacytoma. And for a newly diagnosed patient presenting with an extramedullary plasmacytoma, after the induction regimens, high-dose melphalan with the help of stem cell transplant and followed by a maintenance phase, which includes a PI and an IMiD, is the recommended approach. But any time during the course of this disease, we have to evaluate the response not only in the marrow, the traditional hematological response assessment, but we also need to use the imaging response criteria and that will allow us to look at the response in the EMD carefully with metabolic response, with complete response being the aimed final endpoint.
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