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.
|Data di pubblicazione:||2007|
|Titolo:||Myeloid sarcoma: clinico-pathologic, phenotypic and cytogenetic analysis of 92 adult patients.|
|Autori:||PILERI SA; ASCANI S; COX MC; CAMPIDELLI C; BACCI F; PICCIOLI M; PICCALUGA; PP; AGOSTINELLI C; ASIOLI S; NOVERO D; BISCEGLIA M; PONZONI M; GENTILE A; RINALDI P; FRANCO V; VINCELLI D; JR AP; GASBARRA R; FALINI B; ZINZANI PL; BACCARANI M|
|Tipologia:||Articolo su rivista|
|Citazione:||PILERI SA, ASCANI S, COX MC, CAMPIDELLI C, BACCI F, PICCIOLI M, et al. (2007). Myeloid sarcoma: clinico-pathologic, phenotypic and cytogenetic analysis of 92 adult patients. LEUKEMIA, 21, 340-350.|
|Digital Object Identifier (DOI):||10.1038/sj.leu.2404491|
|Appare nelle tipologie:||01 - Articolo su rivista|