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PLoS One. 2017 Feb 24;12(2):e0171639. doi: 10.1371/journal.pone.0171639. eCollection 2017.

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

Author information

1
Department of Surgical Oncology, Gustave Roussy Cancer Campus, Villejuif, France.
2
Department of Radiotherapy, Bergonié Institute, Bordeaux, France.
3
Department of Medical Oncology, Gustave Roussy Cancer Campus, Villejuif, France.
4
Department of Pediatric, Adolescent and Young Adult Oncology, Curie Institute, Paris, France.
5
Department of Surgical Oncology, Claudius Régaud Institute, Toulouse, France.
6
Department of Radiotherapy, Gustave Roussy Cancer Campus, Villejuif, France.
7
Department of General and Digestive Surgery, Hautepierre Hospital, Strasbourg University, Strasbourg, France.
8
Department of Pediatric Surgery, Robert Debré Hospital, Paris, France.
9
Department of Pediatric Surgery, Necker Enfants Malades Hospital, Paris Descartes University, Paris, France.
10
of Digestive Surgery, Louis Mourier Hospital, Paris Diderot University, Colombes, France.
11
Department of Pathology, Gustave Roussy Cancer Campus, Villejuif, France.
12
Department of Surgical Oncology, Lyon Civil Hospices, South Lyon University Hospital Center, Lyon, France.
13
Department of Pediatric Surgery, Kremlin-Bicêtre Hospital, Paris 11 University, Kremlin-Bicêtre, France.
14
Department of Pediatric Oncology, Gustave Roussy Cancer Campus, Villejuif, France.
15
Department of Medical Oncology, Paoli-Calmettes Institute, Aix-Marseille University, Marseille, France.
16
Department of Medical Oncology, Leon Bérard Center, Lyon, France.
17
Department of Surgery, Curie Institute, Paris, France.

Abstract

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.

PMID:
28234908
PMCID:
PMC5325210
DOI:
10.1371/journal.pone.0171639
[Indexed for MEDLINE]
Free PMC Article

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