Myeloid Sarcoma: Current Approach and Treatment Strategies

Abstract

Myeloid sarcoma (MS) is a rare extramedullary tumor of immature myeloid cells with associated tissue damage. Although MS can manifest as isolated form without blood and marrow involvement, in the majority of the cases it is associated with acute myeloid leukemia (AML). Rarely, it may present in other myeloid disorders. It should, therefore, be considered in a differential diagnosis of any atypical soft tissue mass. MS can develop at any site, resulting in varied clinical manifestations. Identification and diagnosis of MS is highly challenging and depends on a high index of suspicion as well as on imaging, histopathology, immunophenotyping, and genetic analyses. Owing to the lack of prospective clinical trials, there is not enough data in the field to develop a consensus therapeutic regimen for MS. Majority of the patients with MS, including isolated MS, respond to upfront systemic chemotherapy using AML-like regimens that should be commenced early. Surgical resection and/or radiation therapy can be employed for symptomatic lesions or tumors causing local organ dysfunction or obstruction. Allogeneic hematopoietic stem cell transplantation has demonstrated promising results as consolidation therapy after complete remission with induction chemotherapy. Recent development in sequencing analysis has provided significant insights into the development of novel targeted therapies. At present, there are no validated prognostic factors for MS which can help in risk stratification of patients and treatment planning. As there is limited knowledge regarding the clinical approach and management strategies of MS, this review aims to update current knowledge about MS.