Myeloid sarcoma: clinico-pathologic, phenotypic and cytogenetic analysis of 92 adult patients.
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.
Pileri
S A
SA
Institute of Hematology and Clinical Oncology 'L and A Seràgnoli, University of Bologna, Bologna, Italy. pileri@med.unibo.it
Ascani
S
S
Cox
M C
MC
Campidelli
C
C
Bacci
F
F
Piccioli
M
M
Piccaluga
P P
PP
Agostinelli
C
C
Asioli
S
S
Novero
D
D
Bisceglia
M
M
Ponzoni
M
M
Gentile
A
A
Rinaldi
P
P
Franco
V
V
Vincelli
D
D
Pileri
A
A
Gasbarra
R
R
Falini
B
B
Zinzani
P L
PL
Baccarani
M
M
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
D015703:Antigens, CD; D005819:Genetic Markers