Myeloid sarcoma: clinico-pathologic, phenotypic and cytogenetic analysis of 92 adult patients.

摘要: Myeloid sarcoma (MS) is a rare neoplasm whose knowledge 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 also studied by fluorescent situ hybridization tissue sections conventional karyotyping bone marrow or peripheral blood. Histologically, 50% of the tumors blastic type, 43.5% either monoblastic myelomonocytic 6.5% corresponded to different histotypes. CD68/KP1 was most commonly expressed marker (100%), followed 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 T cells (2.2%), CD30 (2.2%) CD4 (1.1%). Foci plasmacytoid monocyte differentiation observed intestinal carrying inv16. Chromosomal aberrations detected about 54% cases: monosomy 7(10.8%), trisomy 8(10.4%) mixed lineage leukemia-splitting (8.5%) commonest abnormalities, whereas t(8;21) (2.2%). The behavior dramatic irrespective presentation, age, sex, phenotype cytogenetics. Most if not all, long survivors received bone-marrow transplantation. present report expands spectrum our showing that MS has frequent monoblastic/myelomonocytic differentiation, displays distinctive phenotypic profile, carries chromosomal other than t(8;21), requires supra-maximal therapy.