Table I.
Localized skin-limited blastic plasmacytoid dendritic cell neoplasm: current study and review of cases reported since 2008
| Author (year)/no. of patients | Sex/age at dx (y) | Time to dx from symptom onset/from initial evaluation by a clinician (mo) | Lesion location/morphology/size (cm) | Initial treatment and response | Relapse-free survival (mo)/therapy at relapse | Transplant | Overall survival (mo) | Outcome |
|---|---|---|---|---|---|---|---|---|
| Kaune et al.3 2009/1 | F/66 | 12/NS | Inner ankle/violaceous tumor | 3 cycles of cyclophosphamide, hydroxydaunomycin, vincristine, prednisone + RT (34 Gy)-PR | NS/6 weeks after completion of chemotherapy PD in skin, paranasal sinus and LN. Received 2 followed by 3 cycles of liposomal doxorubicin – NR. One month later, fulminant leukemia, treated with etoposide combined with fludarabine/cyclophosphamide and dexamethasone | No | 14 | DOD |
| Dalle et al.4 2010/9 | M/37 | NS | Leg/nodule | Lomustine, doxorubicin, cytarabine-PR | 3/ifosfamide, etoposide, cytarabine, MTX | Allo-SCT | 54 | DCD |
| M/70 | Cheek/nodule | Cytarabine, idarubicin, lomustine-PD | PD/Mini-CHA | No | 19 | DCD | ||
| M/73 | Shoulder/nodule | RT-PD | PD/Idarubicin, cytarabine, lomustine | No | 36 | DCD | ||
| M/75 | Arm/nodule | Refused treatment | NS | No | 12 | DCD | ||
| M/83 | Cheek/nodule | RT-CR | 8/Refused treatment | No | 8 | Alive | ||
| F/25 | Leg/tumor | Hyper-CVAD-CR | 6/Autologous BMT | Autologous BMT | 38 | Alive | ||
| M/82 | Scalp/tumor | RT-CR | 9/Etoposide | No | 19 | DCD | ||
| M/78 | Leg/tumor | CHOP-CR | 12/NS | No | 12 | Alive | ||
| F/60 | Cheek/nodule | MTX, asparaginase, RT-CR | NS | No | 11 | LTF | ||
| Dohm et al.5 2011/1 | M/32 | NS | Arm/nodule/6 × 4 | CHOEP-14 × 6, +RT (36 Gy) – CR | 11/First relapse: 4 cycles of ifosfamide, carboplatin and etoposide + RT (36 Gy): Second relapse: 4 cycles of fludarabine and cyclophosphamide. Consolidating allo-SCT. Third relapse: RT | Allo-SCT | 66 | DOD |
| Hashikawa et al.6 2012/2 | M/46 | NS | Arm/nodule/2 | AML-type-PD | PD | No | 1 | DOD |
| F/5 | NS | Arm | ALL-type-CR | No relapse during follow-up | No | 12 | Alive | |
| Tsunoda et al.7 2012/1 | M/74 | 0.5/immediate diagnosis | Postauricular region/red skin tumor/3.6 × 1.9 | RT (27 Gy)-CR | 2/AdVP | No | NS | DOD |
| An et al.8 2013/1 | F/18 | NS | Thigh/nodule | VPDL-CR | No relapse during follow-up | No | 18 | Alive |
| Sugimoto et al.9 2013/1 | M/74 | At least 6/3 | Shoulder/soft, erythematous mass with vessel dilatation, irreg. borders/5 × 3 | 2/3-dose DeVIC+RT-CR | No relapse during follow-up | No | 12 | NED |
| Heinicke et al.10 2015/1 | F/62 | 6/NS | Lower leg/papule surrounded by diffuse erythema | Vincristine and prednisolone, then CHOP ×6+RT (40 Gy)-CR | 4/1 cycle of FLAG-Ida, 1 cycle of MTX, asparaginase, high-dose cytarabine-CR | Allo-SCT | 41 from initial diagnosis | NED |
| Nomura et al.11 2015/1 | F/7 | 5/NS | Forearm/purplish macule/4.6 × 2.6 | Vincristine, prednisolone, cyclophosphamide, daunorubicin and L-asparaginase-CR | No relapse during follow-up. Received early intensification phase, a prophylaxis phase for the CNS, and a re-induction phase-CR. Maintenance therapy with MTX and 6-mercaptopurine | No | 12 (from beginning of therapy) | NED |
| Sheng et al.12 2015/1 | F/6 | 4/NS | Temple/red infiltrated plaques/1-2 | Topical Chinese medicine | NR | No | 16 | DOD |
| Present report, 2016/2 | Pt. 1 M/38 |
3/immediate diagnosis | Forearm/dusky, purple-colored plaque/5 × 6 | Hyper-CVAD (A+B)+RT (36Gy)-CR | No relapse during follow-up | No | 72 | NED |
| Pt. 2 F/23 |
7/5 | Forehead/bulky, violet-colored, ulcerated tumor/7 × 8 | Hyper-CVAD (A+B)+RT (36Gy)-CR | No relapse during follow-up | No | 108 | NED |
AdVP, Adriamycin/vincristine/prednisone; Allo-SCT, allogeneic stem cell transplantation; BMT, bone marrow transplantation; CHA, mitoxantrone, cytarabine; CHOP, cyclophosphamide/doxorubicin/vincristine, and prednisone; CR, complete response; DCD, decreases, could be dead; DeVIC, dexamethasone, VP16, ifosfamide, carboplatin; DOD, died of disease; dx, diagnosis; FLAG-Ida, fludarabine, cytarabine, idarubicin, and granulocyte colony-stimulating factor; LF, lost to follow-up; LN, lymph nodes; MTX, methotrexate; NED, no evidence of disease; NR, no response; NS, not stated; OS, overall survival; PD, progressive disease; PR, partial response; RFS, relapse-free survival; RT, radiation therapy; VPDL, vincristine, methylprednisolone, daunorubicin, L-asparaginase.