Table 1.
The clinical characteristics of cases of myeloid sarcoma that were successfully treated with azacitidine.
| Case/reported year | Age/sex | Diagnosis at development of MS | Sites of origin | Treatment for MS | Cycles of AZA for MS | Maximum response of AZA for MS | Outcome (cause of death) | Reference |
|---|---|---|---|---|---|---|---|---|
| 1 (2013) | 62/M | CMML type-2 | Right humerus | AZA (75 mg/m2 × 7 days) + RT (25 Gy) | 6∗ | Decreased in size | Alive | [8] |
| 2 (2013) | 34/M | Monoblastic leukemia cutis | Multiple skin nodules, groin mass | IDR + Ara C followed by allo-HSCT ⟶ CLAG followed by DLI and RT ⟶ a cycle of AZA (32 mg/m2 × 5 days) ⟶ 4 cycles of AZA (75 mg/m2 × 7 days) ⟶ RT | 5 | Decreased in size | Died (multiorgan failure) | [9] |
| 3 (2013) | 39/F | Relapse of AML-M2 after allo-HSCT | Gingiva | FLAG with GO ⟶ FLAG and DLI ⟶ AZA (75 mg/m2 × 7 days) | 17∗ | Clinical resolution | Alive | [9] |
| 4 (2015) | 72/M | MDS EB-2 | Disseminated skin papules (LC) | 36 cycles of AZA (75 mg/m2 × 5 days) for MDS ⟶ 41 cycles of the same dose of AZA after the onset of LC | 41∗ | Pathological resolution | Alive | [10] |
| 5 (2015) | 78/M | MDS MLD | Multiple skin lesions (LC) | AZA (75 mg/m2 × 7 days) ⟶ 6-MP | 7 | Clinical resolution | Died (N/A) | [10] |
| 6 (2015) | 76/M | CMML type-1 | Multiple skin lesions (LC) | 9 cycles of AZA (75 mg/m2 × 5 days) for CMML ⟶ 4 cycles of AZA (75 mg/m2 × 7 days) after the onset of LC | 4 | Regression of LC | Died (N/A) | [10] |
| 7 (2016) | 80/F | MDS ⟶ AML | Left eyelid | CAG ⟶ AZA (75 mg/m2 × 7 days) | At least 1 | Decreased in size | Died (AML progression) | [11] |
| 8 (2017) | 71/F | MDS EB-2 | Nasopharynx, tonsil, spleen, multiple lymph nodes, skin | DNR + Ara C ⟶ AZA (100 mg/body × 7 days, as maintenance therapy) | 20∗ | Skin: clinical and radiological resolution; others: a marked reduction in the FDG uptake on PET-CT | Alive | [12] |
| 9 (2018) | 70/F | Therapy-related MDS (MDS-RS-MLD) | Multiple skin lesions (LC) | A cycle of AZA (75 mg/m2) ⟶ 6 cycles of biomodulatory therapy: AZA (75 mg/body × 7 days), pioglitazone and ATRA | 7 | Clinical resolution | Died (AML progression) | [13] |
| 10 (present case) | 89/M | MDS EB-2 | Skin | AZA (37.5 mg/m2 × 7 days) | 16 | Clinical resolution | Died (AML progression) | N/A |
F: female; M: male; MS: myeloid sarcoma; CMML: chronic myelomonocytic leukemia; MDS: myelodysplastic syndrome; AML: acute myeloid leukemia; EB: excess blasts; AZA: azacitidine; RT: radiotherapy; Ara C: cytarabine; CAG: cytarabine, aclarubicin, and granulocyte colony-stimulating factor (G-CSF); DNR: daunorubicin; FDG: fluorodeoxyglucose; PET-CT: positron emission tomography-computed tomography; N/A: not available; LC: leukemia cutis; MLD: multilineage dysplasia; IDR: idarubicin; allo-HSCT: allogeneic hematopoietic stem-cell transplantation; CLAG: cladribine, high-dose cytarabine, and G-CSF; DLI: donor lymphocyte infusion; FLAG: fludarabine, high-dose cytarabine, and G-CSF; GO: gemtuzumab ozogamicin; 6-MP: 6-mercaptopurine; MDS-RS: MDS with ring sideroblasts; ATRA: all-trans-retinoic acid. ∗“Ongoing.”