Case Series of Myeloid Sarcoma from a Tertiary Center

Introduction: Myeloid sarcoma (MS) represents a rare neoplasm derived from myeloid precursor cells, typically manifesting as an extramedullary tumor mass. While frequently associated with acute myeloid leukemia (AML), chronic myeloid leukemia (CML), or myelodysplastic syndrome (MDS), it can also manifest independently as a solitary lesion. This phenomenon underscores the heterogeneous clinical presentations of myeloid sarcoma, necessitating thorough diagnostic evaluation and tailored management strategies. Management strategies are primarily guided by protocols established for AML, although MS exhibits considerable clinical variability and is associated with a generally unfavorable prognosis (Aldapt et al., 2022, Blood). Herein, we report six cases of myeloid sarcoma diagnosed at a tertiary care facility over the preceding decade.

Clinical Cases: The average age at presentation was 42 years old with average baseline hemoglobin 8.84, WBC 11.49 (range: 0.2 to 35.5), LDH 445. The average time to treatment was 32 days. 5 cases were associated with a diagnoses of MDS or AML while one case had no associated diagnosis.

Case 1 presented with gingival MS in the context of AML, achieving disease remission after multiple rounds of chemotherapy. Case 2, diagnosed with CML, was found to have a gastric mass on CT, which was later diagnosed as MS with AML transformation. Case 3, known for MDS, presented with bleeding and was diagnosed with MS involving an axillary lymph node. Case 4 presented with finger ulceration, diagnosed with MS and concurrent AML necessitating finger amputation and ongoing treatment. Case 5 presented with a rapidly growing eye mass, diagnosed with MS that metastasized and subsequently developed AML. Case 6 presented with neck and chest pain, diagnosed with solitary mediastinal myeloid sarcoma, experiencing persistent disease activity despite treatment.

The overall prognosis was poor, compounded by treatment complications and mass effect. Treatment approaches varied; two patients underwent surgical interventions due to mass-related complications. Immunohistochemical profiles differed across biopsies, with genetic testing for targeted therapies conducted only in the most recent patient (Case 4).

Conclusion: This case series aims to explore the diverse presentations and progression of myeloid sarcoma (MS). Extramedullary masses can vary in location and may present asymptomatically. MS should be considered in patients with a history of myeloid disorders or new masses. Delays in diagnosis and treatment are common as patients often seek care from multiple providers despite the disease's rapid progression. While management guidelines primarily align with those for AML, integrating surgical oncology guidelines could improve outcomes, especially in cases of isolated MS where mass-related complications and disease progression are concerns.

No relevant conflicts of interest to declare.