title: Axial skeletal osteosarcoma: a 25-year monoinstitutional experience in children and adolescents.
abstract: The survival of patients with axial skeletal or pelvic osteosarcoma (OS) remains poor, and the management of these patients is challenging. The object of this study is a cohort of unselected patients aged < 19 years with primary high-grade pelvic/axial OS. Patients were treated with high-dose methotrexate, doxorubicin, cisplatin, ifosfamide followed or preceded by local treatment (surgery and/or radiotherapy). Twenty patients aged 3-19 years were treated. Eight patients had pelvic OS, 8 axial OS and 4 mandible/maxilla OS. All patients received chemotherapy, after which necrosis was evaluable in 9 patients (≥ 90% in 3). Sixteen patients underwent surgery. Radiotherapy was administered to 8 patients (total dose 34-60 Gy). The median follow-up was 35 months (8-276), and the 5-year disease-free survival and overall survival rates were 37 and 40%, respectively. Six patients were alive at the time of this report: 2 with pelvic OS (both responded well to chemotherapy, one underwent hemipelvectomy and the other had non-radical surgery plus radiotherapy); 1 with axial and multicentric OS (with a good histological response and radical surgery); 3 with mandible/maxilla OS. Two patients died of secondary tumors (one bone and one breast cancer). It is worth noting that 4 patients had a p53 mutation: 1 is alive, 2 died of their OS, 1 of breast cancer. Adequacy of local treatment and pathological response influenced the prognosis for axial OS, which remained dismal. A high incidence of p53 mutation emerged in our series of patients.
authors.affiliation: Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Via G. Venezian 1, 20133, Milan, Italy, cristina.meazza@istitutotumori.mi.it.
authors.forename: Cristina
authors.initials: C
authors.lastname: Meazza
authors.forename: Roberto
authors.initials: R
authors.lastname: Luksch
authors.forename: Primo
authors.initials: P
authors.lastname: Daolio
authors.forename: Marta
authors.initials: M
authors.lastname: Podda
authors.forename: Alessandro
authors.initials: A
authors.lastname: Luzzati
authors.forename: Alessandro
authors.initials: A
authors.lastname: Gronchi
authors.forename: Antonina
authors.initials: A
authors.lastname: Parafioriti
authors.forename: Lorenza
authors.initials: L
authors.lastname: Gandola
authors.forename: Paola
authors.initials: P
authors.lastname: Collini
authors.forename: Andrea
authors.initials: A
authors.lastname: Ferrari
authors.forename: Michela
authors.initials: M
authors.lastname: Casanova
authors.forename: Monica
authors.initials: M
authors.lastname: Terenziani
authors.forename: Filippo
authors.initials: F
authors.lastname: Spreafico
authors.forename: Daniela
authors.initials: D
authors.lastname: Polastri
authors.forename: Veronica
authors.initials: V
authors.lastname: Biassoni
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Schiavello
authors.forename: Emilia
authors.initials: E
authors.lastname: Pecori
authors.forename: Maura
authors.initials: M
authors.lastname: Massimino
mesh_terms: D000293:Adolescent; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001859:Bone Neoplasms / Q000188:drug therapy* / Q000401:mortality / Q000532:radiotherapy*; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D009336:Necrosis / Q000139:chemically induced; D020360:Neoadjuvant Therapy / Q000379:methods; D012516:Osteosarcoma / Q000188:drug therapy* / Q000401:mortality / Q000532:radiotherapy*; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000970:Antineoplastic Agents