Extraskeletal osteosarcoma has a favourable prognosis when treated like conventional osteosarcoma.
OBJECTIVE
The aims of this analysis were to investigate the clinical features of extraskeletal osteosarcoma (ESOS) and examine the outcome after multi-modal therapy.


METHODS
The co-operative osteosarcoma study-group database was searched for patients with extraskeletal osteosarcoma. Eligible patients were included in a retrospective analysis of patient, tumour and treatment related variables and outcome. As for conventional osteosarcoma, scheduled treatment included surgery and multi-agent chemotherapy.


RESULTS
Seventeen eligible patients were identified with a median age of 44 years (range, 3-65 years). The thigh was the commonest tumour site. Two patients had a history of previous malignancies and two had primary metastases. Median follow-up was 3.2 years (range: 0.6-7.4 years) and at last follow-up, 11 patients were alive in complete remission, 3 patients were alive with disease and 3 patients had died of their disease. Three-year overall actuarial and event-free survival rates were 77% and 56%, respectively. Patients with macroscopically complete surgical remission had an improved overall survival (P = 0.0004).


CONCLUSIONS
The patients in this retrospective study had a surprisingly good survival rate. This may be due to the combination of multi-agent chemotherapy with surgery, and we recommend this approach in the treatment of ESOS.
Goldstein-Jackson
Sing Yu
SY
Klinik und Poliklinik für Kinder- und Jugendmedizin, Pädiatrische Hämatologie und Onkologie, Universitätsklinikum Münster, Münster, Albert-Schweitzer-Str. 33, 48149, Germany.
Gosheger
Georg
G
Delling
Günter
G
Berdel
Wolfgang E
WE
Exner
G Ulrich
GU
Jundt
Gernot
G
Machatschek
Jan-Nicolas
JN
Zoubek
Andreas
A
Jürgens
Heribert
H
Bielack
Stefan S
SS
D000293:Adolescent; D000328:Adult; D001859:Bone Neoplasms / Q000175:diagnosis / Q000628:therapy; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012516:Osteosarcoma / Q000175:diagnosis* / Q000188:drug therapy / Q000601:surgery / Q000628:therapy*; D011379:Prognosis; D012189:Retrospective Studies; D016019:Survival Analysis