Author(s): Pileri SA, Ascani S, Cox MC, Campidelli C, Bacci F,
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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.
This article was published in Leukemia
and referenced in Journal of Antivirals & Antiretrovirals