Abdominal desmoplastic small round cell tumor without extraperitoneal metastases: Is there a benefit for HIPEC after macroscopically complete cytoreductive surgeryReportar como inadecuado




Abdominal desmoplastic small round cell tumor without extraperitoneal metastases: Is there a benefit for HIPEC after macroscopically complete cytoreductive surgery - Descarga este documento en PDF. Documentación en PDF para descargar gratis. Disponible también para leer online.

Background

Desmoplastic Small Round Cell Tumor DSRCT is a rare disease affecting predominantly children and young adults and for which the benefit of hyperthermic intraperitoneal chemotherapy HIPEC after complete cytoreductive surgery CCRS remains unknown.

Methods

To identify patients with DSRCT without extraperitoneal metastases EPM who underwent CCRS between 1991 and 2015, a retrospective nation-wide survey was conducted by crossing the prospective and retrospective databases of the French Network for Rare Peritoneal Malignancies, French Reference Network in Sarcoma Pathology, French Sarcoma Clinical Network and French Pediatric Cancer Society.

Results

Among the 107 patients with DSRCT, 48 had no EPM and underwent CCRS. The median peritoneal cancer index PCI was 9 range: 2–27. Among these 48 patients, 38 79% had pre- and-or postoperative chemotherapy and 23 48% postoperative whole abdominopelvic radiotherapy WAP-RT. Intraperitoneal chemotherapy was administered to 11 patients 23%: two received early postoperative intraperitoneal chemotherapy EPIC and nine HIPEC. After a median follow-up of 30 months, the median overall survival OS of the entire cohort was 42 months. The 2-y and 5-y OS were 72% and 19%. The 2-y and 5-y disease-free survival DFS were 30% and 12%. WAP-RT was the only variable associated with longer peritoneal recurrence-free survival and DFS after CCRS. The influence of HIPEC-EPIC on OS and DFS was not statistically conclusive.

Conclusion

The benefit of HIPEC is still unknown and should be evaluated in a prospective trial. The value of postoperative WAP-RT seems to be confirmed.



Autor: C. Honoré , V. Atallah, O. Mir, D. Orbach, G. Ferron, C. LePéchoux, J. B. Delhorme, P. Philippe-Chomette, S. Sarnacki, S. Msika

Fuente: http://plos.srce.hr/



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