Myeloid Sarcoma: Clinico-Pathologic, Phenotypic and Cytogenetic Analysis of 92 Adult Patients

Leukemia. 2007 Feb;21(2):340-50. doi: 10.1038/sj.leu.2404491. Epub 2006 Dec 14.

Abstract

Myeloid sarcoma (MS) is a rare neoplasm whose knowledge is largely based on case reports and/or technically dated contributions. Ninety-two MSs in adulthood with clinical data available were evaluated both morphologically and immunohistochemically. Seventy-four cases were also studied by fluorescent in situ hybridization on tissue sections and/or conventional karyotyping on bone marrow or peripheral blood. Histologically, 50% of the tumors were of the blastic type, 43.5% either monoblastic or myelomonocytic and 6.5% corresponded to different histotypes. CD68/KP1 was the most commonly expressed marker (100%), followed by myeloperoxidase (83.6%), CD117 (80.4%), CD99 (54.3%), CD68/PG-M1 (51%), CD34 (43.4%), terminal-deoxy-nucleotidyl-transferase (31.5%), CD56 (13%), CD61/linker for activation of T cells (2.2%), CD30 (2.2%) and CD4 (1.1%). Foci of plasmacytoid monocyte differentiation were observed in intestinal cases carrying inv16. Chromosomal aberrations were detected in about 54% of cases: monosomy 7(10.8%), trisomy 8(10.4%) and mixed lineage leukemia-splitting (8.5%) were the commonest abnormalities, whereas t(8;21) was rare (2.2%). The behavior was dramatic irrespective of presentation, age, sex, phenotype and cytogenetics. Most if not all, long survivors received bone-marrow transplantation. The present report expands the spectrum of our knowledge showing that MS has frequent monoblastic/myelomonocytic differentiation, displays distinctive phenotypic profile, carries chromosomal aberrations other than t(8;21), and requires supra-maximal therapy.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Antigens, CD / genetics
  • Chromosome Aberrations*
  • Female
  • Genetic Markers
  • Humans
  • Immunohistochemistry
  • In Situ Hybridization, Fluorescence
  • Leukemia, Myeloid / genetics*
  • Leukemia, Myeloid / pathology*
  • Lymphoma / genetics
  • Male
  • Middle Aged
  • Phenotype
  • Sarcoma / genetics*
  • Sarcoma / pathology*
  • Translocation, Genetic

Substances

  • Antigens, CD
  • Genetic Markers