Omar A Shahin1, Farhad Ravandi. 1. Department of Leukemia, University of Texas - MD Anderson Cancer Center, Houston, Texas, USA.
Abstract
PURPOSE OF REVIEW: Myeloid sarcoma; also known as granulocytic sarcoma and chloroma, often occurs concomitantly with AML, and rarely without bone marrow involvement. In this article, we review the recent literature on myeloid sarcoma, focusing on treatment approach for this rare disease, and addressing the prognostic and therapeutic role of molecular and cytogenetic aberrations. RECENT FINDINGS: Molecular testing and cytogenetics are important adjunct to conventional diagnostic methods. The significance of cytogenetic and molecular abnormalities in myeloid sarcoma is not completely established, but testing for targetable mutations on myeloid sarcoma cells is feasible, imperative, and may guide treatment decisions. Outcomes in myeloid sarcoma largely depend on the background of its development. Almost all patients with myeloid sarcoma eventually develop AML typically in a short period after its diagnosis; therefore, remission induction treatment using AML type chemotherapy has been the standard of care. Postremission therapy is controversial; allogenic SCT, radiotherapy or consolidation chemotherapy should be considered according to patient risk. SUMMARY: Further research is required to understand the nature of myeloid sarcoma, and inclusion of patients with this condition in clinical trials should be considered to better identify the best diagnostic, prognostic, and therapeutic approach in managing this rare disease.
PURPOSE OF REVIEW: Myeloid sarcoma; also known as granulocytic sarcoma and chloroma, often occurs concomitantly with AML, and rarely without bone marrow involvement. In this article, we review the recent literature on myeloid sarcoma, focusing on treatment approach for this rare disease, and addressing the prognostic and therapeutic role of molecular and cytogenetic aberrations. RECENT FINDINGS: Molecular testing and cytogenetics are important adjunct to conventional diagnostic methods. The significance of cytogenetic and molecular abnormalities in myeloid sarcoma is not completely established, but testing for targetable mutations on myeloid sarcoma cells is feasible, imperative, and may guide treatment decisions. Outcomes in myeloid sarcoma largely depend on the background of its development. Almost all patients with myeloid sarcoma eventually develop AML typically in a short period after its diagnosis; therefore, remission induction treatment using AML type chemotherapy has been the standard of care. Postremission therapy is controversial; allogenic SCT, radiotherapy or consolidation chemotherapy should be considered according to patient risk. SUMMARY: Further research is required to understand the nature of myeloid sarcoma, and inclusion of patients with this condition in clinical trials should be considered to better identify the best diagnostic, prognostic, and therapeutic approach in managing this rare disease.