1509P - Chemotherapy is effective in primitive neuroectodermal tumor (pNET) / Ewing sarcoma (EWS) of the kidney

Date 29 September 2012
Event ESMO Congress 2012
Session Poster presentation I
Topics Anticancer agents
Soft Tissue Sarcomas
Therapy
Biological therapy
Presenter Emanuela Risi
Authors E. Risi1, R. Iacovelli2, A. Palazzo1, A. Passaro3, G. Campennì4, F. Morano5, K. Truscelli4, P. Trenta4, S. Mezi4, E. Cortesi4
  • 1Dipartimento Di Medicina Sperimentale, Sapienza University of Rome, 00161 - Rome/IT
  • 2Dipartimento Di Scienze Radiologiche, Oncologiche Ed Anatomo-patologiche, Sapienza University of Rome, 00161 - Rome/IT
  • 3Medical Oncology Division, Sapienza University of Rome, 00161 - Rome/IT
  • 4Department Of Radiology, Oncology And Human Pathology, Sapienza University of Rome, 00161 - Rome/IT
  • 5Oncologia B, Sapienza University of Rome, 00161 - Rome/IT

Abstract

Background

PNET/EWS is a rare tumor that occurs most commonly in bone and soft tissues of children and young. PNET/EWS arise rarely as a primary renal neoplasm and exhibits highly aggressive biological behavior. Since its first description in 1975, less than 100 cases of EWS/PNET have been published in medical literature. We aim to collect and to analyze clinical and pathological features of this tumor.

Patient and methods

All cases reports of PNET/EWS published from 1975 to February 2012 were collected. When available, clinical and pathological data were extracted for each case such as previously reported (Iacovelli et al. BJU Int 2012). Survivals were estimated with Kaplan-Meier method and compared with log-rank test with 95% CI.

Results

A total of 117 cases were found, 55% were male and the median age was 28.0 y (IQR: 20.0 – 42.0). All patients (pts) had clinical symptoms as first presentation of disease such as pain (54%), hematuria (29%) and bulky renal mass (28%); whereas dysuria, fever, weight loss fatigue and abdomen swelling were reported rarely. 66% of pts had stage IV at diagnosis and the main sites of metastases were lung (60%), liver (37%), abdominal lymph-nodes (20%), bone and local relapse (<20%). The median DFS was 5.0 months (95%CI, 2.4 – 7.6). The probability to be alive at 18 mos was 60% and 85% for patients with metastatic disease (M1) or not (M0) at diagnosis. In the M1 pts the median OS was 24 months (95%CI, 4.5 – 15.1) while the median was not reached in M0 pts (p < 0.001). In M0 pts: 50% received neo/adjuvant chemotherapy and the 12mos-OS was 93% compared to 75% of untreated pts (p = 0.092). In M1 treated pts, the median PFS was 22.0 mos (95%CI, 17.9 – 26.1) with a clinical benefit in 74% of cases. The presence of translocation (11:22) between the genes EWS-FLI-1 was found in 72% of cases and it was related to the tumor expression of the FLI-1 (p = 0.03). A positive relationship between expression of EMA, FLI and M1 status was also detected (p < 0.001 for both).

Conclusions

PNET/EWS is a rare aggressive tumor with a well defined genetic profile. It affects principally young people with a poor prognosis for M1 pts; chemotherapy is an effective strategy in M1 and probably also in M0 pts.

Disclosure

All authors have declared no conflicts of interest.