Clinical entities of EMM reported in the myeloma literature
| EMM entities . | Definition . | Clinical presentation . |
|---|---|---|
| Bone-related plasmacytomas | Plasmacytomas developed from the bone, arising in continuity with the bone marrow. | Tumor masses affecting the axial skeleton: ribs, vertebrae, skull, sternum, pelvis. |
| Extramedullary disease | Soft-tissue plasmacytoma or PC infiltration of an anatomical site distant from the bone marrow. Secondary to a hematogenous spread. | Mainly affect the liver, skin, CNS, pleural effusion, kidneys, lymph nodes, pancreas. May be triggered by invasive procedures (ie, catheter insertion, surgical scars). |
| PCL | Aggressive variant of myeloma characterized by the presence of circulating plasma cells (>20% and/or absolute count >2 × 109/L). | Could be considered as EMM because of blood involvement. Extramedullary disease is also very common in PCL patients. |
| SP | Localized bone or extramedullary infiltration by clonal plasma cells without systemic tumor dissemination. | Bone marrow and skeletal survey are both normal. CRAB symptoms are absent. Focal radiotherapy is the treatment of choice. |
| EMM entities . | Definition . | Clinical presentation . |
|---|---|---|
| Bone-related plasmacytomas | Plasmacytomas developed from the bone, arising in continuity with the bone marrow. | Tumor masses affecting the axial skeleton: ribs, vertebrae, skull, sternum, pelvis. |
| Extramedullary disease | Soft-tissue plasmacytoma or PC infiltration of an anatomical site distant from the bone marrow. Secondary to a hematogenous spread. | Mainly affect the liver, skin, CNS, pleural effusion, kidneys, lymph nodes, pancreas. May be triggered by invasive procedures (ie, catheter insertion, surgical scars). |
| PCL | Aggressive variant of myeloma characterized by the presence of circulating plasma cells (>20% and/or absolute count >2 × 109/L). | Could be considered as EMM because of blood involvement. Extramedullary disease is also very common in PCL patients. |
| SP | Localized bone or extramedullary infiltration by clonal plasma cells without systemic tumor dissemination. | Bone marrow and skeletal survey are both normal. CRAB symptoms are absent. Focal radiotherapy is the treatment of choice. |
CNS, central nervous system; CRAB, hypercalcemia, renal failure, anemia, bone lesions; SP, solitary plasmacytoma.