Table 1.

Patients with extramedullary plasma cell tumors (ExPCTs)


Patient name

PC, %*medullary at diagnosis of MM

PC, %*medullary when ExMM diagnosed

ExPCT location

PC, %extramedullary

Time

Status§
Simultaneously diagnosed medullary and extramedullary disease       
ExPCT-1C   44   44   Orbit   100   4   Dead  
ExPCT-4   5   5   Pleura   4   12   Dead  
ExPCT-7C   7   7   Lymph node   100 M   63   Dead  
ExPCT-8   42   42   Tumor pelvis   6   48   PR  
ExPCT-10   8   8   Thorax, upper limb   NA   42   PR  
Medullary phase followed by extramedullary phase       
ExPCT-2C   10   19   Hypophysis   12   17   Dead  
ExPCT-5C   25   35   Soft tissue   13   71   Dead  
ExPCT-12   40   36   Intraspinal-lumbosacralcoluma   NA   27   Dead  
ExPCT-13C   32   2  Intraspinal, cervico-thoracal   100 M   7   Dead  
Extramedullary phase with less than 5%medullary PC       
ExPCT-3C   < 5   1.3  Ascites/peritoneum (lower limb, gastrointestinal tract, skin)  12 M   80   Dead  
ExPCT-6   0.5   0.5   Pleura   77   9   Dead  
ExPCT-10   1.2   < 5   (Mammae and skin)  NA   71   Dead  
ExPCT-12
 
1.1
 
0.5
 
Pleura (thorax wall)
 
0.6
 
12
 
CR
 

Patient name

PC, %*medullary at diagnosis of MM

PC, %*medullary when ExMM diagnosed

ExPCT location

PC, %extramedullary

Time

Status§
Simultaneously diagnosed medullary and extramedullary disease       
ExPCT-1C   44   44   Orbit   100   4   Dead  
ExPCT-4   5   5   Pleura   4   12   Dead  
ExPCT-7C   7   7   Lymph node   100 M   63   Dead  
ExPCT-8   42   42   Tumor pelvis   6   48   PR  
ExPCT-10   8   8   Thorax, upper limb   NA   42   PR  
Medullary phase followed by extramedullary phase       
ExPCT-2C   10   19   Hypophysis   12   17   Dead  
ExPCT-5C   25   35   Soft tissue   13   71   Dead  
ExPCT-12   40   36   Intraspinal-lumbosacralcoluma   NA   27   Dead  
ExPCT-13C   32   2  Intraspinal, cervico-thoracal   100 M   7   Dead  
Extramedullary phase with less than 5%medullary PC       
ExPCT-3C   < 5   1.3  Ascites/peritoneum (lower limb, gastrointestinal tract, skin)  12 M   80   Dead  
ExPCT-6   0.5   0.5   Pleura   77   9   Dead  
ExPCT-10   1.2   < 5   (Mammae and skin)  NA   71   Dead  
ExPCT-12
 
1.1
 
0.5
 
Pleura (thorax wall)
 
0.6
 
12
 
CR
 

C (in ExPCT-1C and similar constructions) indicates clonal lgH; M, RAS mutation in extramedullary but not intramedullary tumor cells; PR, partial remission; NA, not available; and CR, complete remission.

*

PC% determined morphologically.

For determination of PC% in extramedullary locations and when < 5% PCs were observed morphologically, aberrant PC percentages were determined by flow cytometry as described in “Patients, materials, and methods.”

Observation time in months after diagnosis.

§

Status at the observation time after diagnosis.

These patients were under treatment, which may result in low medullary tumor burden.

Multiple locations, the locations in parentheses were not available for analysis.

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