Contributions
Abstract: PB2248
Type: Publication Only
Background
Multiple myeloma (MM) is a clonal plasma cell neoplasm typically confined to the bone marrow and skeleton. In addition, a number of patients develop extramedullary disease, which may sometimes present as the leading clinical manifestation. The incidence of extramedullary plasmacytomas has been reported in the western literature as 7% - 18% at the time of multiple myeloma diagnosis and up to 20% at myeloma relapse. However, there is very little data regarding the same in the Indian scenario.
Aims
The present abstract reports the high incidence of bulky extramedullary soft-tissue plasmacytomas in newly diagnosed multiple myeloma patients hailing from the western parts of Uttar Pradesh and adjoining Haridwar district of Uttarakhand, who attended AIIMS Rishikesh for diagnosis & treatment.
Methods
The study included 32 newly diagnosed patients of multiple myeloma between March 2016 & December 2017. The investigations done for confirmation of myeloma diagnosis included bone marrow aspiration+biopsy, serum protein electrophoresis+immunofixation, serum free light chain assay, and X-ray skeletal survey. MRI was done for imaging of the soft-tissue masses. Diagnosis of extramedullary soft-tissue plasmacytoma was confirmed by core biopsy/excision biopsy & immunohistochemistry (IHC) in all cases. PET-CT imaging facilities were not available at the institution. All the six patients were treated with Bortezomib-containing triple-drug regimens. Local radiation therapy was administered for compressive symptoms.
Results
Six patients (18.7%) out of total 32 consecutive newly diagnosed myeloma patients had one or more extramedullary soft-tissue plasmacytomas at presentation. The median age of these patients was 61 years (range 39-75 years). The patient characteristics & treatment outcomes are given in Table 1.
Sl | Age/Sex | Diagnosis | Plasmacytoma site | Treatment & outcome |
1 | 68 y / M | IgA Kappa MM | Bulky retroperitoneal mass; Multiple left cervical & supraclavicular lymphadenopathy (Biopsy+IHC: Plasmacytoma) | CyBorD x 2 cycles: No response. Bortezomib+ Bendamustine+ Dexa x 4 cycles: CR |
2 | 62 y / F | Kappa Light Chain MM | Plasmacytoma involving left lung & adjacent thoracic wall (10.8 x 7.9 x 5.6 cm) | CyBorD x 4 cycles. Partial response. |
3 | 60 y / M | Kappa Light Chain MM | Soft-tissue plasmacytomas on chest wall. Sphenoid bone plasmacytoma, adjacent to sphenoid sinus (24x13mm). Right Facial nerve palsy | Refractory to CyBorD. RVD started. Lost to follow up. |
4 | 75 y / M | IgG Lambda MM | Multiple, large soft-tissue plasmacytomas in paraspinal region & on scapula | CyBorD x 3 cycle: Progressive disease. Lost to follow up. |
5 | 39 y / F | Non-secretory MM | Presented with Left lumbar region mass & paraplegia. Biopsy: plasmacytoma. (25 x 14 cm) with intra-abdominal & intra-spinal extension | Bortezomib+ PegLiposomal Doxorubicin + Dexa x 2 cycles. Radiation (40Gy). |
6 | 47 y / M | IgG Kappa MM, del17p13 | D3-D4 paraspinal soft-tissue plasmacytoma with intraspinal extension. Large soft-tissue plasmacytoma in sacral region | Bortezomib + Lenalidomide + Dexa (RVD) started - February 2018. |
Conclusion
The frequent finding of bulky extramedullary soft-tissue plasmacytomas in newly diagnosed multiple myeloma patients in the western districts of Uttar Pradesh & adjacent Uttarakhand state in India could be the manifestation of unique myeloma disease biology. Possible association of aggressive plasmacytomas with kappa light chain paraprotein also needs to be explored in larger number of patients.
Session topic: 14. Myeloma and other monoclonal gammopathies - Clinical
Keyword(s): Multiple Myeloma
Abstract: PB2248
Type: Publication Only
Background
Multiple myeloma (MM) is a clonal plasma cell neoplasm typically confined to the bone marrow and skeleton. In addition, a number of patients develop extramedullary disease, which may sometimes present as the leading clinical manifestation. The incidence of extramedullary plasmacytomas has been reported in the western literature as 7% - 18% at the time of multiple myeloma diagnosis and up to 20% at myeloma relapse. However, there is very little data regarding the same in the Indian scenario.
Aims
The present abstract reports the high incidence of bulky extramedullary soft-tissue plasmacytomas in newly diagnosed multiple myeloma patients hailing from the western parts of Uttar Pradesh and adjoining Haridwar district of Uttarakhand, who attended AIIMS Rishikesh for diagnosis & treatment.
Methods
The study included 32 newly diagnosed patients of multiple myeloma between March 2016 & December 2017. The investigations done for confirmation of myeloma diagnosis included bone marrow aspiration+biopsy, serum protein electrophoresis+immunofixation, serum free light chain assay, and X-ray skeletal survey. MRI was done for imaging of the soft-tissue masses. Diagnosis of extramedullary soft-tissue plasmacytoma was confirmed by core biopsy/excision biopsy & immunohistochemistry (IHC) in all cases. PET-CT imaging facilities were not available at the institution. All the six patients were treated with Bortezomib-containing triple-drug regimens. Local radiation therapy was administered for compressive symptoms.
Results
Six patients (18.7%) out of total 32 consecutive newly diagnosed myeloma patients had one or more extramedullary soft-tissue plasmacytomas at presentation. The median age of these patients was 61 years (range 39-75 years). The patient characteristics & treatment outcomes are given in Table 1.
Sl | Age/Sex | Diagnosis | Plasmacytoma site | Treatment & outcome |
1 | 68 y / M | IgA Kappa MM | Bulky retroperitoneal mass; Multiple left cervical & supraclavicular lymphadenopathy (Biopsy+IHC: Plasmacytoma) | CyBorD x 2 cycles: No response. Bortezomib+ Bendamustine+ Dexa x 4 cycles: CR |
2 | 62 y / F | Kappa Light Chain MM | Plasmacytoma involving left lung & adjacent thoracic wall (10.8 x 7.9 x 5.6 cm) | CyBorD x 4 cycles. Partial response. |
3 | 60 y / M | Kappa Light Chain MM | Soft-tissue plasmacytomas on chest wall. Sphenoid bone plasmacytoma, adjacent to sphenoid sinus (24x13mm). Right Facial nerve palsy | Refractory to CyBorD. RVD started. Lost to follow up. |
4 | 75 y / M | IgG Lambda MM | Multiple, large soft-tissue plasmacytomas in paraspinal region & on scapula | CyBorD x 3 cycle: Progressive disease. Lost to follow up. |
5 | 39 y / F | Non-secretory MM | Presented with Left lumbar region mass & paraplegia. Biopsy: plasmacytoma. (25 x 14 cm) with intra-abdominal & intra-spinal extension | Bortezomib+ PegLiposomal Doxorubicin + Dexa x 2 cycles. Radiation (40Gy). |
6 | 47 y / M | IgG Kappa MM, del17p13 | D3-D4 paraspinal soft-tissue plasmacytoma with intraspinal extension. Large soft-tissue plasmacytoma in sacral region | Bortezomib + Lenalidomide + Dexa (RVD) started - February 2018. |
Conclusion
The frequent finding of bulky extramedullary soft-tissue plasmacytomas in newly diagnosed multiple myeloma patients in the western districts of Uttar Pradesh & adjacent Uttarakhand state in India could be the manifestation of unique myeloma disease biology. Possible association of aggressive plasmacytomas with kappa light chain paraprotein also needs to be explored in larger number of patients.
Session topic: 14. Myeloma and other monoclonal gammopathies - Clinical
Keyword(s): Multiple Myeloma