Review Article

Multiple Myeloma of the Central Nervous System: 13 Cases and Review of the Literature

Table 1

Patient characteristics.

Patient At diagnosis Prior treatment CNS presentation Survival (days)
AgeSexISS/PCLFISH/karyotypeM-proteinLinesThalBorLenASCTAgeSymptomsDiagnosisSystemic relapseISSNew FISHTreatmentResponseOS from CNSOS from DGPFS from CNSDG to CNS

169M31q ampLC lambda1110069Double visionCT, CSF flowyes3NDEDAP, IT, Mel-ASCT, Len maintPR144335130191
265M11q ampLC kappa1010065ParaplegiaCSF flowyes1NDVTD, Mel-ASCT, Len maintPR427897124470
369F1NDIgG lambda2110074Double visionCSF IFXno1NDLenDexNR56202501969
441F31q ampLC lambda1110041Headache, hypoglossus, and abducent paresisClinical (unspecific MRI abnormalities)yes317pVRD-PACE, IT, EDAP, IRD, Mel-Benda-ASCTPR18029063110
552M3del 13qIgA kappa3110Allo55Abducent paresis, unable to swallowCSF cytospin, MRIno1NDThal + IT + craniocaudal irrad + discontinue GVHD prophylaxisCR1341103126969
656F3hyperdIgA kappa3110160HeadacheMRIno1NDThal + craniocaudal irrad, benda-VTDPR77621911041415
754M3del 13qIgA kappa2110156Oculomotor nerve paresisClinical (MRI orbit neg, CSF ND)yes31qampVTDNR93103332940
853M3complex, hypodiplIgG lambda3110257Abducent paresisClinical (MRI neg, CSF ND)yes3samePAD-ThalMR21315632101350
943M3, PCLcomplex, del 13qLC lambda1010044Paresis, cannot swallowCSF cytospin, MRI: tumorsyes3sameHD MTX, AraC, ITPD44140096
1066M31q ampIgG lambda2110067Abducent paresisMRIyes3sameIRDPD143860372
1134M2t(4;14)IgG lambda3111136Left leg paresisMRI, CSF flowyes1sameThio, Car, Thal, DexPR3855738519
1260F3, PCLt(11;14), del 17pIgG kappa2111160SeizuresCSF cytospinyes3sameVTD-PACE + ith triplet 4x, Mel-TBI alloPD1103250215
1372M2normalLC kappa4111073Back painCSF flow, CTyesND1qampDVd + ITPR6069560675

Mean56.58/12 HR7/13 lambda258.212589763715

Allo: allogeneic stem cell transplantation; amp: amplification; ASCT: autologous stem cell transplantation; Bor: bortezomib; Car: carfilzomib; CI: cranial irradiation; CSF: cerebrospinal fluid; CTD: cyclophosphamide, thalidomide, and dexamethasone; CyBorDex: cyclophosphamide, bortezomib, and dexamethasone; Dara: daratumumab; del: deletion; DVd: daratumumab, bortezomib, and dexamethasone; flow: flow cytometry; hyperd: hyperdiploid; IFX: immunofixation; IRD: ixazomib, revlimid, and dexamethasone; IT: intrathecal chemotherapy; KTD: carfilzomib, thalidomide, and dexamethasone; LC: light chain; Len: lenalidomide; maint: maintenance; MPV: melphalan, prednisolone, and bortezomib; ND: not done; PACE: cisplatin, doxorubicin, cyclophosphamide, and etoposide; PAD: bortezomib, doxorubicin, and dexamethasone; PCL: plasma cell leukemia; PomD: pomalidomide and dexamethasone; RT: radiotherapy; T-CED: thalidomide, cyclophosphamide, etoposide, and dexamethasone; Thal: thalidomide; Thio: thiotepa; VAD: vincristine, doxorubicin, and dexamethasone; VTD: bortezomib, thalidomide, and dexamethasone.