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Clinicopathological characteristics, genetics and prognosis of patients with myeloid sarcoma: a single-center study
  1. Derya Demir1,
  2. Mine Hekimgil1,
  3. Emin Karaca2,
  4. Yusuf Ulusoy3,
  5. Hamiyet Hekimci Özdemir4,
  6. Güray Saydam5,
  7. Burak Durmaz2,
  8. Haluk Akın2,
  9. Nazan Çetingül4,
  10. Murat Tombuloğlu5,
  11. Nazan Özsan1
  1. 1Pathology, Ege University Faculty of Medicine, Izmir, Turkey
  2. 2Medical Genetics, Ege University Faculty of Medicine, Izmir, Turkey
  3. 3Internal Medicine, Division of Hematology, Basaksehir Cam and Sakura City Hospital, Istanbul, Turkey
  4. 4Pediatric Hematology-Oncology, Ege University Faculty of Medicine, Izmir, Turkey
  5. 5Internal Medicine, Division of Hematology, Ege University Faculty of Medicine, Izmir, Turkey
  1. Correspondence to Derya Demir, Pathology, Ege University Faculty of Medicine, Izmir, Turkey; derya.demir{at}ege.edu.tr

Abstract

Aim Myeloid sarcoma (MS) is a rare tumour comprising myeloid blasts occurring at an anatomical site other than the bone marrow. We sought to investigate both paediatric and adult patients with MS diagnosed at our institution and determine possible correlations among their clinicopathological, phenotypic, molecular and prognostic features.

Methods This study retrospectively evaluated the data of 45 patients diagnosed with MS at Ege University Faculty of Medicine Hospital, Turkey, over a 17-year period.

Results The male-to-female ratio was 1.5:1, and the median age was 39.12 years. The most commonly involved sites were the skin, lymph nodes, soft tissues and bone. Immunohistochemically, CD68-KP1 was the most commonly expressed marker, followed by CD33, myeloperoxidase, CD117, lysozyme, CD68-PGM1 and CD34. Of the patients, 26 (57.7%) presented with de novo MS, 7 (15.5%) had simultaneous acute myeloid leukaemia and 12 (26.8%) had a previous history of haematological disorders. Kaplan-Meier survival analysis revealed that the 2-year and 5-year overall survival (OS) rates were 46.4% and 39.8%, respectively; the median OS duration was 11 months. Increasing age had a negative prognostic relationship with survival (p = 0.04). Chromosomal abnormalities were detected in approximately 6/10 (60%) of paediatric patients and 6/9 (66.7%) of adult patients. t(8;21)(q22;q22) translocation was identified in 20% of paediatric patients.

Conclusions MS diagnosis is usually challenging; an expanded immunohistochemical panel should be used for an accurate diagnosis. Although MS generally has a poor prognosis, increasing age appears to be associated with a worse outcome.

  • myeloid sarcoma
  • chloroma
  • granulocytic sarcoma
  • genetic
  • prognosis

Data availability statement

All data relevant to the study are included in the article or uploaded as supplemental information. Data are available in online supplemental tables.

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Data availability statement

All data relevant to the study are included in the article or uploaded as supplemental information. Data are available in online supplemental tables.

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Footnotes

  • Handling editor Tahir S Pillay.

  • Twitter @DeryaDemirDr

  • Contributors DD, MH and NÖ contributed to the research study design. DD, NÖ, MH, EK, BD, HA, YU, HHÖ, GS, MT and NÇ contributed to data acquisition and investigation. DD, NÖ, MH, YU and HHÖ contributed to data analysis and interpretation. DD, YU and HHÖ contributed to statistical analysis. DD, EK, BD, GS, MT, NÇ, HHÖ and YU contributed to data analysis and data interpretation. DD contributed as guarantor. All authors critically reviewed and approved the final version of the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests The authors have no conflicts of interest, including specific financial interests, relationships, and/or affiliations, relevant to the subject matter or materials included.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.