Table 1 List of cases with extramedullary multiple myeloma progression despite concomitant medullary response to multiple myeloma therapy
Case study/series | Number of cases | Multiple myeloma treatment | Bone marrow at extramedullary multiple myeloma relapse | Extramedullary multiple myeloma relapse course/response |
|---|---|---|---|---|
Bairey et al. [12] | 1 | Chemotherapy | No evidence of disease | eMM relapse in skin and subcutaneous areas, right eyebrow, right knee, sternum and right axilla. Failed chemotherapy. Died secondary to liver eMM. |
Iwasaki et al. [13] | 1 | Chemotherapy | No evidence of disease | Skin eMM relapse in 6 months which responded to chemotherapy. Retroperitoneal eMM relapse 2 years later causing death. |
Avigdor et al. [11] | 2 | Chemotherapy followed by ASCT. Relapse of MM was treated with thalidomide. | No evidence of disease | Patient 1 developed parasellar eMM after 3 months of thalidomide and died in 2 weeks. Patient 2 developed diffuse skin eMM which failed to respond to Allo SCT. |
Ah-Weng et al.[14] | 1 | Chemotherapy followed by ASCT | No evidence of disease | Multiple cutaneous eMM in 3 months. VAD salvage chemotherapy followed by localized RT and IFN-2a attempted but patient died in 2 weeks. |
Terpos et al. [10] | 15 | ASCT or Allo SCT | No evidence of disease | median time from ASCT to eMM was 24 months. eMM sites included skin, rectum, and testicles. Treated with local RT (n=5), combination of RT and chemotherapy or thalidomide (n=7), and chemotherapy +/- thalidomide (n=2), VAD-chemotherapy and local RT followed by a mini-allograft from the original donor (n=1). 11 patients died at a median of 10 months following diagnosis of eMM. 4 patients were still alive at 12–20 months after eMM relapse. |
Candoni et al. [15] | 3 | Thalidomide | No evidence of disease | Median time to eMM relapse was 3 months. eMM sites included cutaneous, soft tissue, parasellar. Salvage therapy attempted but poor clinical outcome. |
Cerny et al. [16] | 6 | IMiD and/or ASCT | n/a | Median time to progression and survival after eMM relapse was 29 months and 38 days respectively. |
Waterhouse et al. [17] | 1 | Thalidomide/melphalan followed by ASCT. MM relapse treated with bortezomib. | No evidence of disease | eMM relapse in the brain, pleural and paravertebral soft tissue in 1 month following bortezomib. |
Gozzetti et al.[18] | 1 | Chemotherapy followed by ASCT | No evidence of disease | eMM relapse in lung, mediastinum, pancreas, psoas muscle at 5 months post-ASCT. Failed hyper C-PAD. Disease stabilized on lenalidomide at 10 months from eMM relapse. |