Extraskeletal osteosarcoma: A European Musculoskeletal Oncology Society study on 266 patients.
Prognosis of extraskeletal osteosarcoma (ESOS) is reported to be poorer than that of skeletal osteosarcoma. This multicenter retrospective study aimed to evaluate factors influencing ESOS prognosis.



Members of the European Musculoskeletal Oncology Society (EMSOS) submitted institutional data on patients with ESOS.



Data from 274 patients treated from 1981 to 2014 were collected from 16 EMSOS centres; 266 patients were eligible. Fifty (18.7%) had metastases at diagnosis. Of 216 patients with localised disease, 211 (98%) underwent surgery (R0 = 70.6%, R1 = 27%). Five-year overall survival (OS) for all 266 patients was 47% (95% CI 40-54%). Five-year OS for metastatic patients was 27% (95% CI 13-41%). In the analysis restricted to the 211 localised patients who achieved complete remission after surgery 5-year OS was 51.4% (95% CI 44-59%) and 5-year disease-free survival (DFS) was 43% (95% CI 35-51%). One hundred twenty-one patients (57.3%) received adjuvant or neoadjuvant chemotherapy and 80 patients (37.9%) received radiotherapy. A favourable trend was seen for osteosarcoma-type chemotherapy versus soft tissue sarcoma-type (doxorubicin ± ifosfamide) regimens. For the 211 patients in complete remission after surgery, patient age, tumour size, margins and chemotherapy were positive prognostic factors for DFS and OS by univariate analysis. At multivariate analysis, patient age (≤40 years versus >40 years) (P = 0.05), tumour size (P = 0.0001) and receipt of chemotherapy (P = 0.006) were statistically significant prognostic factors for survival.



Patient age and tumour size are factors influencing ESOS prognosis. Higher survival was observed in patients who received perioperative chemotherapy with a trend in favour of multiagent osteosarcoma-type regimen which included doxorubicin, ifosfamide and cisplatin.
Istituto Ortopedico Rizzoli, Bologna, Italy. Electronic address: alessandra.longhi@ior.it.
A
A
Longhi
Klinikum Stuttgart, Olgahospital, Cooperative Osteosarcoma Study Group (COSS), Stuttgart, Germany.
S S
SS
Bielack
Royal Orthopaedic Hospital, Birmingham, UK.
R
R
Grimer
London Sarcoma Service, University College Hospital, UK.
J
J
Whelan
Department of Orthopaedics, Medical University of Vienna, Vienna General Hospital, Vienna, Austria.
R
R
Windhager
Orthopaedic Surgery, Medical University Graz, Graz, Austria.
A
A
Leithner
Chirurgia dei Sarcomi, Istituto Nazionale Tumori, Milano, Italy.
A
A
Gronchi
Chirurgie Orthopédique, Hôpital Cochin, Université Paris-Descartes, Paris, France.
D
D
Biau
University Medical Center, Groningen, The Netherlands.
P
P
Jutte
Paediatric Orthopaedic Department, University Childrens Hospital (UKBB), Basel, Switzerland.
A H
AH
Krieg
INSELSPITAL, University Hospital, Bern, Switzerland.
F M
FM
Klenke
Candiolo Cancer Institute - FPO, IRCCS, Italy.
G
G
Grignani
Istituto Ortopedico Rizzoli, Bologna, Italy.
D M
DM
Donati
CTO, Careggi, Firenze, Italy.
R
R
Capanna
Orthopedic University Hospital, University of Coimbra, Coimbra, Portugal.
J
J
Casanova
Freeman Hospital, Newcastle upon Tyne, UK.
C
C
Gerrand
Oncoematologia Pediatrica, University of Padova, Padova, Italy.
G
G
Bisogno
Klinikum Stuttgart, Olgahospital, Cooperative Osteosarcoma Study Group (COSS), Stuttgart, Germany.
S
S
Hecker-Nolting
London Sarcoma Service, University College Hospital, UK.
M
M
De Lisa
Candiolo Cancer Institute - FPO, IRCCS, Italy.
L
L
D'Ambrosio
Department of Orthopaedics, Medical University of Vienna, Vienna General Hospital, Vienna, Austria.
M
M
Willegger
CTO, Careggi, Firenze, Italy.
G
G
Scoccianti
Istituto Ortopedico Rizzoli, Bologna, Italy.
S
S
Ferrari
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D059248:Chemoradiotherapy / Q000379:methods* / Q000401:mortality; D002648:Child; D018572:Disease-Free Survival; D005060:Europe / Q000453:epidemiology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D020360:Neoadjuvant Therapy; D009362:Neoplasm Metastasis; D009364:Neoplasm Recurrence, Local / Q000401:mortality / Q000473:pathology / Q000628:therapy; D012516:Osteosarcoma / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D012189:Retrospective Studies; D012307:Risk Factors; D012983:Soft Tissue Neoplasms / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D047368:Tumor Burden; D055815:Young Adult
EMSOS; Extraskeletal osteosarcoma; Localised osteosarcoma