Clinicobiological features and outcome of acute promyelocytic leukemia occurring as a second tumor: the GIMEMA experience.
We analyzed the clinicobiological features and treatment outcome of a series of acute promyelocytic leukemias (APLs) occurring as a second tumor (APL-st's, n = 51) and compared these with a large group of de novo APL cases (n = 641), both observed by the Italian cooperative group GIMEMA. In the APL-st group, 37 patients had received radiotherapy and/or chemotherapy for their primary malignancy (PM), while 14 had been treated by surgery alone. Compared with de novo APL patients, APL-st patients were characterized by a predominance of females (P <.003), higher median age (P <.05), and worse performance status (P <.005). The median time elapsed between PM and APL-st was 36 months, with a longer latency for patients treated with surgery alone. No significant differences were found with regard to karyotypic lesions or type of promyelocytic leukemia/retinoic acid receptor alpha (PML/RARalpha) fusion in the 2 cohorts. A high prevalence of PMs of the reproductive system was observed among the female APL-st population (24 [71%] of 34 patients in this group had suffered from breast, uterine, or ovarian cancer). Thirty-one APL-st and 641 de novo APL patients received homogeneous APL therapy according to the all-trans retinoic acid (ATRA) and idarubicin regimen (the AIDA regimen). The complete remission (CR), 4-year event-free survival (EFS), and 4-year overall survival (OS) rates were 97% and 93%, 65% and 68%, and 85% and 78% in the APL-st and de novo APL groups, respectively. In spite of important clinical differences (older age and poorer performance status), the APL-st group responded as well as the de novo APL group to upfront ATRA plus chemotherapy, probably reflecting genetic similarity.
Pulsoni
Alessandro
A
Cellular Biotechnology and Hematology Department, La Sapienza University, Via Benevento 6, 00161 Rome, Italy. pulsoni@bce.uniroma1.it
Pagano
Livio
L
Lo Coco
Francesco
F
Avvisati
Giuseppe
G
Mele
Luca
L
Di Bona
Eros
E
Invernizzi
Rosangela
R
Leoni
Franco
F
Marmont
Filippo
F
Mele
Alfonso
A
Melillo
Lorella
L
Nosari
Anna Maria
AM
Pogliani
Enrico Maria
EM
Vignetti
Marco
M
Visani
Giuseppe
G
Zagonel
Vittorina
V
Leone
Giuseppe
G
Mandelli
Franco
F
D000293:Adolescent; D000328:Adult; D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D015255:Idarubicin / Q000627:therapeutic use; D015994:Incidence; D007223:Infant; D015473:Leukemia, Promyelocytic, Acute / Q000453:epidemiology* / Q000401:mortality / Q000628:therapy; D008297:Male; D008875:Middle Aged; D016609:Neoplasms, Second Primary / Q000188:drug therapy / Q000453:epidemiology* / Q000401:mortality; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis; D016896:Treatment Outcome; D014212:Tretinoin / Q000627:therapeutic use
D000970:Antineoplastic Agents; D014212:Tretinoin; D015255:Idarubicin