Complete cytoreduction and HIPEC improves survival in desmoplastic small round cell tumor

Andrea Hayes-Jordan, Holly L. Green, Heather Lin, Pascal Owusu-Agyemang, Nancy Fitzgerald, Radha Arunkumar, Rodrigo Mejia, Regina Okhuysen-Cawley, Rizalina Mauricio, Keith Fournier, Joseph Ludwig, Peter Anderson

Research output: Contribution to journalArticle

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Abstract

Background: Desmoplastic small round cell tumor (DSRCT) is a rare tumor of adolescents and young adults. Less than 100 cases per year are reported in North America. Extensive peritoneal metastases are characteristic of this disease. We performed cytoreductive surgery and hyperthermic peritoneal perfusion with chemotherapy (HIPEC) using cisplatin (CDDP) for DSRCT. Methods: A retrospective cohort study was performed on 26 pediatric and adult patients who underwent cytoreduction/HIPEC using CDDP for DSRCT at a single cancer center. Neoadjuvant chemotherapy, adjuvant chemotherapy, and postoperative enteral nutrition were given to all patients. Postoperative radiation therapy was given to most patients. Follow-up was from 6 months to 6 years. Outcome variables were evaluated for disease-free and overall survival (OS). Results: Five patients (19 %) were less than 12 years of age at surgery. Patients who had disease outside the abdomen at surgery had a larger risk of recurrence or death than those who did not (p = 0.0158, p = 0.0393 time from surgery to death respectively). Age, liver metastasis, and peritoneal cancer index level did not significantly predict disease-free or OS. Patients who had CR0 or CR1 and HIPEC had significantly longer median survival compared with patients who had HIPEC and CR2 cytoreduction (63.4 vs. 26.7 months). Conclusions: HIPEC may be an effective therapy for children and young adults with DSRCT. Patients with DSRCT require complete cytoreduction before HIPEC to optimize outcome. Patients with DSRCT and disease outside the abdomen at the time of surgery do not benefit from HIPEC.

Original languageEnglish (US)
Pages (from-to)220-224
Number of pages5
JournalAnnals of surgical oncology
Volume21
Issue number1
DOIs
StatePublished - Jan 1 2014

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Desmoplastic Small Round Cell Tumor
Perfusion
Drug Therapy
Survival
Abdomen
Young Adult
Neoplasm Metastasis
Neoplasms
Enteral Nutrition
Adjuvant Chemotherapy
North America
Cisplatin
Disease-Free Survival
Cohort Studies
Radiotherapy
Retrospective Studies

ASJC Scopus subject areas

  • Surgery
  • Oncology

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Complete cytoreduction and HIPEC improves survival in desmoplastic small round cell tumor. / Hayes-Jordan, Andrea; Green, Holly L.; Lin, Heather; Owusu-Agyemang, Pascal; Fitzgerald, Nancy; Arunkumar, Radha; Mejia, Rodrigo; Okhuysen-Cawley, Regina; Mauricio, Rizalina; Fournier, Keith; Ludwig, Joseph; Anderson, Peter.

In: Annals of surgical oncology, Vol. 21, No. 1, 01.01.2014, p. 220-224.

Research output: Contribution to journalArticle

Hayes-Jordan, Andrea ; Green, Holly L. ; Lin, Heather ; Owusu-Agyemang, Pascal ; Fitzgerald, Nancy ; Arunkumar, Radha ; Mejia, Rodrigo ; Okhuysen-Cawley, Regina ; Mauricio, Rizalina ; Fournier, Keith ; Ludwig, Joseph ; Anderson, Peter. / Complete cytoreduction and HIPEC improves survival in desmoplastic small round cell tumor. In: Annals of surgical oncology. 2014 ; Vol. 21, No. 1. pp. 220-224.
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T1 - Complete cytoreduction and HIPEC improves survival in desmoplastic small round cell tumor

AU - Hayes-Jordan, Andrea

AU - Green, Holly L.

AU - Lin, Heather

AU - Owusu-Agyemang, Pascal

AU - Fitzgerald, Nancy

AU - Arunkumar, Radha

AU - Mejia, Rodrigo

AU - Okhuysen-Cawley, Regina

AU - Mauricio, Rizalina

AU - Fournier, Keith

AU - Ludwig, Joseph

AU - Anderson, Peter

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N2 - Background: Desmoplastic small round cell tumor (DSRCT) is a rare tumor of adolescents and young adults. Less than 100 cases per year are reported in North America. Extensive peritoneal metastases are characteristic of this disease. We performed cytoreductive surgery and hyperthermic peritoneal perfusion with chemotherapy (HIPEC) using cisplatin (CDDP) for DSRCT. Methods: A retrospective cohort study was performed on 26 pediatric and adult patients who underwent cytoreduction/HIPEC using CDDP for DSRCT at a single cancer center. Neoadjuvant chemotherapy, adjuvant chemotherapy, and postoperative enteral nutrition were given to all patients. Postoperative radiation therapy was given to most patients. Follow-up was from 6 months to 6 years. Outcome variables were evaluated for disease-free and overall survival (OS). Results: Five patients (19 %) were less than 12 years of age at surgery. Patients who had disease outside the abdomen at surgery had a larger risk of recurrence or death than those who did not (p = 0.0158, p = 0.0393 time from surgery to death respectively). Age, liver metastasis, and peritoneal cancer index level did not significantly predict disease-free or OS. Patients who had CR0 or CR1 and HIPEC had significantly longer median survival compared with patients who had HIPEC and CR2 cytoreduction (63.4 vs. 26.7 months). Conclusions: HIPEC may be an effective therapy for children and young adults with DSRCT. Patients with DSRCT require complete cytoreduction before HIPEC to optimize outcome. Patients with DSRCT and disease outside the abdomen at the time of surgery do not benefit from HIPEC.

AB - Background: Desmoplastic small round cell tumor (DSRCT) is a rare tumor of adolescents and young adults. Less than 100 cases per year are reported in North America. Extensive peritoneal metastases are characteristic of this disease. We performed cytoreductive surgery and hyperthermic peritoneal perfusion with chemotherapy (HIPEC) using cisplatin (CDDP) for DSRCT. Methods: A retrospective cohort study was performed on 26 pediatric and adult patients who underwent cytoreduction/HIPEC using CDDP for DSRCT at a single cancer center. Neoadjuvant chemotherapy, adjuvant chemotherapy, and postoperative enteral nutrition were given to all patients. Postoperative radiation therapy was given to most patients. Follow-up was from 6 months to 6 years. Outcome variables were evaluated for disease-free and overall survival (OS). Results: Five patients (19 %) were less than 12 years of age at surgery. Patients who had disease outside the abdomen at surgery had a larger risk of recurrence or death than those who did not (p = 0.0158, p = 0.0393 time from surgery to death respectively). Age, liver metastasis, and peritoneal cancer index level did not significantly predict disease-free or OS. Patients who had CR0 or CR1 and HIPEC had significantly longer median survival compared with patients who had HIPEC and CR2 cytoreduction (63.4 vs. 26.7 months). Conclusions: HIPEC may be an effective therapy for children and young adults with DSRCT. Patients with DSRCT require complete cytoreduction before HIPEC to optimize outcome. Patients with DSRCT and disease outside the abdomen at the time of surgery do not benefit from HIPEC.

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