Desmoplastic Small Round Cell Tumor

Long-Term Complications After Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy

Zachary E. Stiles, Andrew J. Murphy, Doralina L. Anghelescu, Christina Lin Brown, Andrew M. Davidoff, Paxton V. Dickson, Evan Glazer, Michael W. Bishop, Wayne L. Furman, Alberto S. Pappo, John T. Lucas, Jeremiah Deneve

Research output: Contribution to journalArticle

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Abstract

Background: Desmoplastic small round cell tumor (DSRCT) is a rare intra-abdominal soft tissue sarcoma affecting adolescents and young adults. Cytoreduction, hyperthermic intraperitoneal chemotherapy (CRS/HIPEC), and adjuvant radiotherapy may improve local control. We review our experience with patients who undergo CRS/HIPEC and adjuvant radiotherapy for DSRCT. Methods: A retrospective review was performed for patients with DSRCT from 2013 to 2017 who underwent CRS/HIPEC. Clinicopathologic, operative, and outcome data were reviewed. Results: Ten CRS/HIPEC procedures were performed for nine patients (7 males, 6 Caucasian, median age 19 years (range 10–24)). Four patients presented with extra-abdominal disease; five had liver involvement. The median peritoneal cancer index was 16 (range 5–20). All received neoadjuvant chemotherapy. CCR 0/1 resection was possible in nine patients. Major complications occurred in four with no operative mortalities. All received adjuvant chemotherapy, seven received radiation therapy, and three received stem-cell transplant. All but one patient recurred after treatment. The median recurrence-free and overall survival (OS) were 12 and 45 months (95% confidence interval 35.1–54.9) respectively, with a 3-year OS of 55%. Long-term parenteral nutrition was required in eight for a median of 261 days (range 37–997). Clinically significant long-term complications requiring further surgery included gastroparesis (N = 1), small bowel obstruction (N = 3) and hemorrhagic cystitis (N = 2). Conclusions: Multimodal therapy for DSRCT consisting of multiagent neoadjuvant chemotherapy, CRS/HIPEC, adjuvant chemotherapy, and radiation therapy is associated with potential cumulative toxicity. Recurrence after resection is common. Prolonged parenteral nutrition may be necessary, and late gastrointestinal and genitourinary complications may require additional treatment.

Original languageEnglish (US)
JournalAnnals of Surgical Oncology
DOIs
StatePublished - Jan 1 2019

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Desmoplastic Small Round Cell Tumor
Drug Therapy
Adjuvant Radiotherapy
Parenteral Nutrition
Adjuvant Chemotherapy
Radiotherapy
Gastroparesis
Recurrence
Cystitis
Survival
Sarcoma
Young Adult
Stem Cells
Therapeutics
Confidence Intervals
Transplants
Mortality
Liver

All Science Journal Classification (ASJC) codes

  • Surgery
  • Oncology

Cite this

Desmoplastic Small Round Cell Tumor : Long-Term Complications After Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy. / Stiles, Zachary E.; Murphy, Andrew J.; Anghelescu, Doralina L.; Brown, Christina Lin; Davidoff, Andrew M.; Dickson, Paxton V.; Glazer, Evan; Bishop, Michael W.; Furman, Wayne L.; Pappo, Alberto S.; Lucas, John T.; Deneve, Jeremiah.

In: Annals of Surgical Oncology, 01.01.2019.

Research output: Contribution to journalArticle

Stiles, Zachary E. ; Murphy, Andrew J. ; Anghelescu, Doralina L. ; Brown, Christina Lin ; Davidoff, Andrew M. ; Dickson, Paxton V. ; Glazer, Evan ; Bishop, Michael W. ; Furman, Wayne L. ; Pappo, Alberto S. ; Lucas, John T. ; Deneve, Jeremiah. / Desmoplastic Small Round Cell Tumor : Long-Term Complications After Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy. In: Annals of Surgical Oncology. 2019.
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abstract = "Background: Desmoplastic small round cell tumor (DSRCT) is a rare intra-abdominal soft tissue sarcoma affecting adolescents and young adults. Cytoreduction, hyperthermic intraperitoneal chemotherapy (CRS/HIPEC), and adjuvant radiotherapy may improve local control. We review our experience with patients who undergo CRS/HIPEC and adjuvant radiotherapy for DSRCT. Methods: A retrospective review was performed for patients with DSRCT from 2013 to 2017 who underwent CRS/HIPEC. Clinicopathologic, operative, and outcome data were reviewed. Results: Ten CRS/HIPEC procedures were performed for nine patients (7 males, 6 Caucasian, median age 19 years (range 10–24)). Four patients presented with extra-abdominal disease; five had liver involvement. The median peritoneal cancer index was 16 (range 5–20). All received neoadjuvant chemotherapy. CCR 0/1 resection was possible in nine patients. Major complications occurred in four with no operative mortalities. All received adjuvant chemotherapy, seven received radiation therapy, and three received stem-cell transplant. All but one patient recurred after treatment. The median recurrence-free and overall survival (OS) were 12 and 45 months (95{\%} confidence interval 35.1–54.9) respectively, with a 3-year OS of 55{\%}. Long-term parenteral nutrition was required in eight for a median of 261 days (range 37–997). Clinically significant long-term complications requiring further surgery included gastroparesis (N = 1), small bowel obstruction (N = 3) and hemorrhagic cystitis (N = 2). Conclusions: Multimodal therapy for DSRCT consisting of multiagent neoadjuvant chemotherapy, CRS/HIPEC, adjuvant chemotherapy, and radiation therapy is associated with potential cumulative toxicity. Recurrence after resection is common. Prolonged parenteral nutrition may be necessary, and late gastrointestinal and genitourinary complications may require additional treatment.",
author = "Stiles, {Zachary E.} and Murphy, {Andrew J.} and Anghelescu, {Doralina L.} and Brown, {Christina Lin} and Davidoff, {Andrew M.} and Dickson, {Paxton V.} and Evan Glazer and Bishop, {Michael W.} and Furman, {Wayne L.} and Pappo, {Alberto S.} and Lucas, {John T.} and Jeremiah Deneve",
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T2 - Long-Term Complications After Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy

AU - Stiles, Zachary E.

AU - Murphy, Andrew J.

AU - Anghelescu, Doralina L.

AU - Brown, Christina Lin

AU - Davidoff, Andrew M.

AU - Dickson, Paxton V.

AU - Glazer, Evan

AU - Bishop, Michael W.

AU - Furman, Wayne L.

AU - Pappo, Alberto S.

AU - Lucas, John T.

AU - Deneve, Jeremiah

PY - 2019/1/1

Y1 - 2019/1/1

N2 - Background: Desmoplastic small round cell tumor (DSRCT) is a rare intra-abdominal soft tissue sarcoma affecting adolescents and young adults. Cytoreduction, hyperthermic intraperitoneal chemotherapy (CRS/HIPEC), and adjuvant radiotherapy may improve local control. We review our experience with patients who undergo CRS/HIPEC and adjuvant radiotherapy for DSRCT. Methods: A retrospective review was performed for patients with DSRCT from 2013 to 2017 who underwent CRS/HIPEC. Clinicopathologic, operative, and outcome data were reviewed. Results: Ten CRS/HIPEC procedures were performed for nine patients (7 males, 6 Caucasian, median age 19 years (range 10–24)). Four patients presented with extra-abdominal disease; five had liver involvement. The median peritoneal cancer index was 16 (range 5–20). All received neoadjuvant chemotherapy. CCR 0/1 resection was possible in nine patients. Major complications occurred in four with no operative mortalities. All received adjuvant chemotherapy, seven received radiation therapy, and three received stem-cell transplant. All but one patient recurred after treatment. The median recurrence-free and overall survival (OS) were 12 and 45 months (95% confidence interval 35.1–54.9) respectively, with a 3-year OS of 55%. Long-term parenteral nutrition was required in eight for a median of 261 days (range 37–997). Clinically significant long-term complications requiring further surgery included gastroparesis (N = 1), small bowel obstruction (N = 3) and hemorrhagic cystitis (N = 2). Conclusions: Multimodal therapy for DSRCT consisting of multiagent neoadjuvant chemotherapy, CRS/HIPEC, adjuvant chemotherapy, and radiation therapy is associated with potential cumulative toxicity. Recurrence after resection is common. Prolonged parenteral nutrition may be necessary, and late gastrointestinal and genitourinary complications may require additional treatment.

AB - Background: Desmoplastic small round cell tumor (DSRCT) is a rare intra-abdominal soft tissue sarcoma affecting adolescents and young adults. Cytoreduction, hyperthermic intraperitoneal chemotherapy (CRS/HIPEC), and adjuvant radiotherapy may improve local control. We review our experience with patients who undergo CRS/HIPEC and adjuvant radiotherapy for DSRCT. Methods: A retrospective review was performed for patients with DSRCT from 2013 to 2017 who underwent CRS/HIPEC. Clinicopathologic, operative, and outcome data were reviewed. Results: Ten CRS/HIPEC procedures were performed for nine patients (7 males, 6 Caucasian, median age 19 years (range 10–24)). Four patients presented with extra-abdominal disease; five had liver involvement. The median peritoneal cancer index was 16 (range 5–20). All received neoadjuvant chemotherapy. CCR 0/1 resection was possible in nine patients. Major complications occurred in four with no operative mortalities. All received adjuvant chemotherapy, seven received radiation therapy, and three received stem-cell transplant. All but one patient recurred after treatment. The median recurrence-free and overall survival (OS) were 12 and 45 months (95% confidence interval 35.1–54.9) respectively, with a 3-year OS of 55%. Long-term parenteral nutrition was required in eight for a median of 261 days (range 37–997). Clinically significant long-term complications requiring further surgery included gastroparesis (N = 1), small bowel obstruction (N = 3) and hemorrhagic cystitis (N = 2). Conclusions: Multimodal therapy for DSRCT consisting of multiagent neoadjuvant chemotherapy, CRS/HIPEC, adjuvant chemotherapy, and radiation therapy is associated with potential cumulative toxicity. Recurrence after resection is common. Prolonged parenteral nutrition may be necessary, and late gastrointestinal and genitourinary complications may require additional treatment.

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