Metastatic Ewing sarcoma/PNET of bone at diagnosis: Prognostic factors?a report from Saudi Arabia

✍ Scribed by Jenkin, R. Derek ;Al-Fawaz, Ibrahim ;Al-Shabanah, Mohammed O. ;Allam, Ayman ;Ayas, Mouhab ;Memon, Muhammad ;Rifai, Samira ;Schultz, Henrik P.

• Publisher: John Wiley and Sons
• Year: 2001
• Tongue: English
• Weight: 97 KB
• Volume: 37
• Category: Article
• ISSN: 0098-1532
• DOI: 10.1002/mpo.1214

✦ Synopsis

Background:

To evaluate outcome and prognostic factors in saudi arabian patients with metastatic ewing sarcoma and pnet of bone (pmes) at diagnosis.

Procedure:

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.