Myeloid sarcoma: An overview

Semin Diagn Pathol. 2023 May;40(3):129-139. doi: 10.1053/j.semdp.2023.04.009. Epub 2023 Apr 17.


Myeloid Sarcoma (MS) is a high grade, hematological malignancy defined as an extramedullary tumor mass of myeloid blasts with or without maturation that effaces tissue architecture. It is a highly heterogenous condition that represents a variety of myeloid neoplasms. This heterogeneity of MS, together with its rarity, have greatly hampered our understanding of the condition. Diagnosis requires tumor biopsy, which should be accompanied by bone marrow evaluation for medullary disease. It is presently recommended that MS be treated similar to AML. Additionally, ablative radiotherapy and novel targeted therapies may also be beneficial. Genetic profiling has identified recurrent genetic abnormalities including gene mutations associated with MS, supporting its etiology similar to AML. However, the mechanisms by which MS homes to specific organs is unclear. This review provides an overview of pathogenesis, pathological and genetic findings, treatment, and prognosis. Improving the management and outcomes of MS patients requires a better understanding of its pathogenesis and its response to various therapeutic approaches.

Keywords: Acute myeloid leukemia; Chemotherapy; Genetics; Immunophenotype; Myeloid sarcoma; Prognosis; Radiotherapy.

Publication types

  • Review

MeSH terms

  • Humans
  • Leukemia, Myeloid, Acute* / diagnosis
  • Mutation
  • Myeloproliferative Disorders*
  • Prognosis
  • Sarcoma, Myeloid* / diagnosis
  • Sarcoma, Myeloid* / genetics
  • Sarcoma, Myeloid* / therapy