What is the prognosis of myeloid sarcoma?

Updated: Jul 28, 2020
  • Author: Amandeep Aneja, MD; Chief Editor: Aliyah R Sohani, MD  more...
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The overall survival of patients with extramedullary myeloid sarcoma does not appear to be influenced by age, sex, site(s) of involvement, history of prior therapy, or pathologic features including morphology, immunophenotype, or cytogenetics, although the probability of prolonged survival or cure seems higher for patients who undergo allogeneic or autologous bone marrow transplantation. [6, 7, 15]

The presence of myeloid sarcoma in pediatric patients with acute acute myeloid leukemia (AML) at diagnosis appears to be a poor prognostic indicator, particularly in association with KMT2A rearrangements. [16]  Stem cell transplantation may also not be beneficial for children with AML and myeloid sarcoma.

The revised 4th edition (2016/2017 revision) of the World Health Organization (WHO) classification recommends comprehensive investigation in patients without evidence of marrow disease to classify these neoplasms into the most precise disease category possible. [17]  This may necessitate cytogenetic analysis by conventional karyotype or fluorescence in situ hybridization (FISH), as well as mutational analysis to identify recurrent genetic abnormalities that may impact prognosis and choice of therapy (see the section on Molecular/Genetic Features and Methods). For example, in the case of AML with inv(16)(p13.1q22)/CBFB-MYH11 rearrangement illustrated previously, analysis for mutations involving the KIT gene is particularly important, because the presence of such mutations confers a worse prognosis in CBFB-rearranged AML.

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