This case highlights an atypical presentation of myeloid sarcoma in a patient with acute myeloid leukemia (AML), focusing on diagnostic challenges, treatment decisions, and outcomes. The case emphasizes extramedullary involvement and therapeutic approaches for patients with poor performance status.

A 68-year-old woman presented with neck swelling. Ultrasound and CT imaging revealed multiple enlarged cervical lymph nodes, with the largest measuring 30 × 25 mm in the right submandibular region. A tru-cut biopsy confirmed myeloid sarcoma infiltration. Upon admission, she was not cytopenic, but peripheral blood smear revealed blasts. Bone marrow biopsy confirmed AML, and diffuse chloroma foci were noted on her face. Due to poor performance status, the 5+1 chemotherapy regimen (5 days cytarabine, 1 day anthracycline) was initiated. After achieving remission in bone marrow, HDAC (high-dose cytarabine, 1500 mg/day) was administered as consolidation therapy. Severe cytopenias during HDAC led to a switch to azacitidine (Vidaza, 75 mg/m²) and venetoclax. Allogeneic stem cell transplantation (AlloSCT) was recommended, but the patient declined.

This case illustrates the diagnostic challenges of myeloid sarcoma in rare locations like the neck, compounded by diffuse chloroma. For patients with poor performance status, low-intensity regimens such as azacitidine and venetoclax are viable alternatives to intensive chemotherapy. AlloSCT remains the preferred treatment for high-risk AML, but in this case, azacitidine and venetoclax provided an alternative therapeutic pathway.