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 S. Glazer
  • Michael W. Bishop
  • Wayne L. Furman
  • Alberto S. Pappo
  • John T. LucasJr.
  • Jeremiah L. DeneveEmail author
Peritoneal Surface Malignancy



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.


A retrospective review was performed for patients with DSRCT from 2013 to 2017 who underwent CRS/HIPEC. Clinicopathologic, operative, and outcome data were reviewed.


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).


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.



The authors thank Lynn Wynn, Amy Kimble, and Dina Darby for the care of all patients treated in this series.


This work was supported in part by the R25CA23944 Grant from the National Cancer Institute.


All authors declare no conflicts of interest.


  1. 1.
    Gerald WL, Rosai J. Case 2. Desmoplastic small cell tumor with divergent differentiation. Pediatr Pathol. 1989;9:177–83.CrossRefGoogle Scholar
  2. 2.
    Lettieri CK, Garcia-Filion P, Hingorani P. Incidence and outcomes of desmoplastic small round cell tumor: results from the surveillance, epidemiology, and end results database. J Cancer Epidemiol. 2014;2014:1–5.CrossRefGoogle Scholar
  3. 3.
    Gerald WL, Miller HK, Battifora H, et al. Intra-abdominal desmoplastic small round-cell tumor. Report of 19 cases of a distinctive type of high-grade polyphenotypic malignancy affecting young individuals. Am J Surg Pathol. 1991;15:499–513.CrossRefGoogle Scholar
  4. 4.
    Schwarz RE, Gerald WL, Kushner BH, et al. Desmoplastic small round cell tumors: prognostic indicators and results of surgical management. Ann Surg Oncol. 1998;5:416–22.CrossRefGoogle Scholar
  5. 5.
    Osborne EM, Briere TM, Hayes-Jordan A, et al. Survival and toxicity following sequential multimodality treatment including whole abdominopelvic radiotherapy for patients with desmoplastic small round cell tumor. Radiother Oncol. 2016;119:40–4.CrossRefGoogle Scholar
  6. 6.
    Kushner BH, LaQuaglia MP, Wollner N, et al. Desmoplastic small round-cell tumor: prolonged progression-free survival with aggressive multimodality therapy. J Clin Oncol. 1996;14:1526–31.CrossRefGoogle Scholar
  7. 7.
    Lal DR, Su WT, Wolden SL, et al. Results of multimodal treatment for desmoplastic small round cell tumors. J Pediatr Surg. 2005;40:251–5.CrossRefGoogle Scholar
  8. 8.
    Atallah V, Honore C, Orbach D, et al. Role of adjuvant radiation therapy after surgery for abdominal desmoplastic small round cell tumors. Int J Radiat Oncol Biol Phys. 2016;95:1244–53.CrossRefGoogle Scholar
  9. 9.
    Pinnix CC, Fontanilla HP, Hayes-Jordan A, et al. Whole abdominopelvic intensity-modulated radiation therapy for desmoplastic small round cell tumor after surgery. Int J Radiat Oncol Biol Phys. 2012;83:317–26.CrossRefGoogle Scholar
  10. 10.
    Hayes-Jordan A, Green H, Fitzgerald N, et al. Novel treatment for desmoplastic small round cell tumor: hyperthermic intraperitoneal perfusion. J Pediatr Surg. 2010;45:1000–6.CrossRefGoogle Scholar
  11. 11.
    Hayes-Jordan AA, Coakley BA, Green HL, et al. Desmoplastic small round cell tumor treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy: results of a Phase 2 trial. Ann Surg Oncol. 2018;25(4):872–7.CrossRefGoogle Scholar
  12. 12.
    Scalabre A, Philippe-Chomette P, Passot G, et al. Cytoreductive surgery and hyperthermic intraperitoneal perfusion with chemotherapy in children with peritoneal tumor spread: a French nationwide study over 14 years. Pediatr Blood Cancer. 2018;65(4):1–7.CrossRefGoogle Scholar
  13. 13.
    Zmora O, Hayes-Jordan A, Nissan A, et al. Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) for disseminated intraabdominal malignancies in children—a single-institution experience. J Pediatr Surg. 2018;53(7):1381–6.CrossRefGoogle Scholar
  14. 14.
    Honore C, Atallah V, Mir O, et al. Abdominal desmoplastic small round cell tumor without extraperitoneal metastases: is there a benefit for HIPEC after macroscopically complete cytoreductive surgery? PLoS One. 2017;12:e0171639.CrossRefGoogle Scholar
  15. 15.
    Msika S, Gruden E, Sarnacki S, et al. Cytoreductive surgery associated to hyperthermic intraperitoneal chemoperfusion for desmoplastic round small cell tumor with peritoneal carcinomatosis in young patients. J Pediatr Surg. 2010;45:1617–21.CrossRefGoogle Scholar
  16. 16.
    Jacquet P, Sugarbaker PH. Clinical research methodologies in diagnosis and staging of patients with peritoneal carcinomatosis. Cancer Treat Res. 1996;82:359–74.CrossRefGoogle Scholar
  17. 17.
    Glehen O, Kwiatkowski F, Sugarbaker PH, et al. Cytoreductive surgery combined with perioperative intraperitoneal chemotherapy for the management of peritoneal carcinomatosis from colorectal cancer: a multi-institutional study. J Clin Oncol. 2004;22:3284–92.CrossRefGoogle Scholar
  18. 18.
    Dindo D, Demartines N, Clavien PA. Classification of surgical complications: a new proposal with evaluation in a cohort of 6336 patients and results of a survey. Ann Surg. 2004;240:205–13.CrossRefGoogle Scholar
  19. 19.
    Dufresne A, Cassier P, Couraud L, et al. Desmoplastic small round cell tumor: current management and recent findings. Sarcoma. 2012;2012:714986.CrossRefGoogle Scholar
  20. 20.
    Honore C, Amroun K, Vilcot L, et al. Abdominal desmoplastic small round cell tumor: multimodal treatment combining chemotherapy, surgery, and radiotherapy is the best option. Ann Surg Oncol. 2015;22:1073–9.CrossRefGoogle Scholar
  21. 21.
    Saab R, Khoury JD, Krasin M, et al. Desmoplastic small round cell tumor in childhood: the St. Jude Children’s Research Hospital experience. Pediatr Blood Cancer. 2007;49:274–9.CrossRefGoogle Scholar
  22. 22.
    Hayes-Jordan A, Green H, Lin H, et al. Cytoreductive surgery and Hyperthermic Intraperitoneal Chemotherapy (HIPEC) for children, adolescents, and young adults: the first 50 cases. Ann Surg Oncol. 2015;22:1726–32.CrossRefGoogle Scholar
  23. 23.
    Hayes-Jordan A, Green HL, Lin H, et al. Complete cytoreduction and HIPEC improves survival in desmoplastic small round cell tumor. Ann Surg Oncol. 2014;21:220–4.CrossRefGoogle Scholar
  24. 24.
    Green H, Lin H, Owusu-Agyemang K, et al. Perioperative renal protective treatment avoids renal toxicity in pediatric and adult patients undergoig HIPEC with cisplatin. J Pediatric Oncol. 2014;2:10–6.CrossRefGoogle Scholar
  25. 25.
    Stiles ZE, Dickson PV, Glazer ES, et al. Desmoplastic small round cell tumor: a nationwide study of a rare sarcoma. J Surg Oncol. 2018;117(8):1759–67.CrossRefGoogle Scholar

Copyright information

© Society of Surgical Oncology 2019

Authors and Affiliations

  • Zachary E. Stiles
    • 1
  • Andrew J. Murphy
    • 1
    • 2
  • Doralina L. Anghelescu
    • 3
  • Christina-Lin Brown
    • 3
  • Andrew M. Davidoff
    • 1
    • 2
  • Paxton V. Dickson
    • 1
  • Evan S. Glazer
    • 1
  • Michael W. Bishop
    • 4
  • Wayne L. Furman
    • 4
  • Alberto S. Pappo
    • 4
  • John T. LucasJr.
    • 5
  • Jeremiah L. Deneve
    • 1
    Email author
  1. 1.Division of Surgical Oncology, Department of SurgeryUniversity of Tennessee Health Science CenterMemphisUSA
  2. 2.Department of SurgerySt Jude Children’s Research HospitalMemphisUSA
  3. 3.Division of Anesthesiology, Department of Pediatric MedicineSt Jude Children’s Research HospitalMemphisUSA
  4. 4.Department of Medical OncologySt Jude Children’s Research HospitalMemphisUSA
  5. 5.Department of Radiation OncologySt Jude Children’s Research HospitalMemphisUSA

Personalised recommendations