Metastatic Ewing sarcoma/PNET of bone at diagnosis: prognostic factors--a report from Saudi Arabia.
BACKGROUND
To evaluate outcome and prognostic factors in Saudi Arabian patients with metastatic Ewing sarcoma and PNET of bone (PMES) at diagnosis.


METHODS
Ninety-nine of 304 (33%) consecutive patients with Ewing sarcoma and PNET of bone registered at our centre from 1975 to 1998, had metastatic disease at registration and 93 were available for analysis. The maximum x-axis diameter of the primary tumor was used as the measure of primary tumor size. Usually a trial of systemic treatment was undertaken before a decision was made on local treatment. Standard chemotherapy regimens were used in all treated patients. Forty-one (44%) patients did not receive radical local treatment due to an inadequate response to chemotherapy, or a decision not to undertake more than palliative treatment. Radical treatment of the primary site was radiation alone 41 (79%), resection alone 7 (13%), and resection and radiation 4 (8%).


RESULTS
The 5-year survival rates were 9% for all 93 evaluable patients, 16% for 52 patients who received chemotherapy and radical local treatment, 0% for 41 patients who received lesser treatment, 19% for 43 patients with lung metastases alone, and 0% (P = 0.002) for 50 patients with other sites involved. For 60 patients with imaging data, 5-year survivals were 34 and 0% when the maximum transverse diameter of the primary tumor was < 10 cm (N = 20) and > or = 10 cm (N = 40), respectively.


CONCLUSIONS
Small primary tumor size and the presence of lung metastases alone were the only significant favorable prognostic factors. Earlier diagnosis will be the basis for better results.
Jenkin
R D
RD
Department of Oncology, Section of Radiation Oncology, King Faisal Specialist Hospital and Research Center, PO Box 3354, Riyadh, Saudi Arabia 11211. derek.jenkin@sci.kfshrc.edu.sa
Al-Fawaz
I
I
Al-Shabanah
M O
MO
Allam
A
A
Ayas
M
M
Memon
M
M
Rifai
S
S
Schultz
H P
HP
D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D001859:Bone Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000401:mortality / Q000556:secondary; D008297:Male; D009367:Neoplasm Staging; D017599:Neuroectodermal Tumors / Q000175:diagnosis* / Q000401:mortality / Q000556:secondary* / Q000628:therapy; D019637:Orthopedic Procedures / Q000379:methods*; D011379:Prognosis; D012042:Registries; D012512:Sarcoma, Ewing / Q000175:diagnosis* / Q000401:mortality / Q000556:secondary* / Q000628:therapy; D012529:Saudi Arabia; D015996:Survival Rate; D016896:Treatment Outcome