title: Myeloid sarcoma: clinico-pathologic, phenotypic and cytogenetic analysis of 92 adult patients.
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.
authors.affiliation: Institute of Hematology and Clinical Oncology 'L and A Seràgnoli, University of Bologna, Bologna, Italy. pileri@med.unibo.it
authors.forename: S A
authors.initials: SA
authors.lastname: Pileri
authors.forename: S
authors.initials: S
authors.lastname: Ascani
authors.forename: M C
authors.initials: MC
authors.lastname: Cox
authors.forename: C
authors.initials: C
authors.lastname: Campidelli
authors.forename: F
authors.initials: F
authors.lastname: Bacci
authors.forename: M
authors.initials: M
authors.lastname: Piccioli
authors.forename: P P
authors.initials: PP
authors.lastname: Piccaluga
authors.forename: C
authors.initials: C
authors.lastname: Agostinelli
authors.forename: S
authors.initials: S
authors.lastname: Asioli
authors.forename: D
authors.initials: D
authors.lastname: Novero
authors.forename: M
authors.initials: M
authors.lastname: Bisceglia
authors.forename: M
authors.initials: M
authors.lastname: Ponzoni
authors.forename: A
authors.initials: A
authors.lastname: Gentile
authors.forename: P
authors.initials: P
authors.lastname: Rinaldi
authors.forename: V
authors.initials: V
authors.lastname: Franco
authors.forename: D
authors.initials: D
authors.lastname: Vincelli
authors.forename: A
authors.initials: A
authors.lastname: Pileri
authors.forename: R
authors.initials: R
authors.lastname: Gasbarra
authors.forename: B
authors.initials: B
authors.lastname: Falini
authors.forename: P L
authors.initials: PL
authors.lastname: Zinzani
authors.forename: M
authors.initials: M
authors.lastname: Baccarani
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015703:Antigens, CD / Q000235:genetics; D002869:Chromosome Aberrations; D005260:Female; D005819:Genetic Markers; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D007951:Leukemia, Myeloid / Q000235:genetics* / Q000473:pathology*; D008223:Lymphoma / Q000235:genetics; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D012509:Sarcoma / Q000235:genetics* / Q000473:pathology*; D014178:Translocation, Genetic
chemical_list: D015703:Antigens, CD; D005819:Genetic Markers