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Cytoreductive Surgery Followed by Hyperthermic Intraperitoneal Chemotherapy in DSRCT: Progress and Pitfalls

  • Sarcomas (SR Patel, Section Editor)
  • Published:
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Abstract

Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC) is an approach for local control of desmoplastic small round cell tumor (DSRCT). DSRCT is a rare sarcoma which presents with multiple intra-abdominal masses. Overall survival has been 15–30 %. Because of the poor prognosis of DSRCT, novel treatment strategies were necessary. Cytoreductive surgery (CRS) and HIPEC has been recently trialed as part of multimodality therapy in DSRCT. CRS and HIPEC allows complete resection of the sometimes hundreds of intra-abdominal tumor implants, followed by the delivery of hyperthermic cisplatin for 90 min at approximately 41 °C. HIPEC is thought to enhance microscopic control of abdominal DSRCT, after surgical resection, and prevent or prolong recurrence. Here, the background of DSRCT, the multimodal treatments available, and the progress and pitfalls of CRS and HIPEC in DSRCT are reviewed.

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References

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  1. Gerald WL, Ladanyi M, de Alava E, et al. Clinical, pathologic, and molecular spectrum of tumors associated with t(11;22)(p13;q12): desmoplastic small round-cell tumor and its variants. J Clin Oncol. 1998;16:3028–36.

    CAS  PubMed  Google Scholar 

  2. Park BJ, Alexander HR, Libutti SK, et al. Treatment of primary peritoneal mesothelioma by continuous hyperthermic peritoneal perfusion (CHPP). Ann Surg Oncol. 1999;6:582–90.

    Article  CAS  PubMed  Google Scholar 

  3. Ladanyi M, Gerald W. Fusion of the EWS and WT1 genes in the desmoplastic small round cell tumor. Cancer Res. 1994;54:2837–40.

    CAS  PubMed  Google Scholar 

  4. Arnold MA, Schoenfield L, Limketkai BN, Arnold CA. Diagnostic pitfalls of differentiating desmoplastic small round cell tumor (DSRCT) from Wilms tumor (WT): overlapping morphologic and immunohistochemical features. Am J Surg Pathol. 2014;38:1220–6.

    Article  PubMed  Google Scholar 

  5. 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;2014:680126.

    PubMed Central  PubMed  Google Scholar 

  6. 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.

    Article  PubMed  Google Scholar 

  7. Zhang WD, Li CX, Liu QY et al. CT, MRI, and FDG-PET/CT imaging findings of abdominopelvic desmoplastic small round cell tumors: correlation with histopathologic findings. Eur J Radiol 2010.

  8. 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.

    Article  CAS  PubMed  Google Scholar 

  9. Sugarbaker PH. A curative approach to peritoneal carcinomatosis from colorectal cancer. Semin Oncol. 2005;32:S68–73.

    Article  PubMed  Google Scholar 

  10. Sugarbaker PH, Stuart OA, Yoo D. Strategies for management of the peritoneal surface component of cancer: cytoreductive surgery plus perioperative intraperitoneal chemotherapy. J Oncol Pharm Pract. 2005;11:111–9.

    Article  PubMed  Google Scholar 

  11. Sugarbaker PH, Jablonski KA. Prognostic features of 51 colorectal and 130 appendiceal cancer patients with peritoneal carcinomatosis treated by cytoreductive surgery and intraperitoneal chemotherapy. Ann Surg. 1995;221:124–32.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  12. Sugarbaker PH, Welch LS, Mohamed F, Glehen O. A review of peritoneal mesothelioma at the Washington Cancer Institute. Surg Oncol Clin N Am. 2003;12:605–21.

    Article  PubMed  Google Scholar 

  13. Glehen O, Gilly FN, Sugarbaker PH. New perspectives in the management of colorectal cancer: what about peritoneal carcinomatosis? Scand J Surg. 2003;92:178–9.

    CAS  PubMed  Google Scholar 

  14. Gough DB, Donohue JH, Schutt AJ, et al. Pseudomyxoma peritonei. Long-term patient survival with an aggressive regional approach. Ann Surg. 1994;219:112–9.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  15. Glehen O, Mithieux F, Osinsky D, et al. Surgery combined with peritonectomy procedures and intraperitoneal chemohyperthermia in abdominal cancers with peritoneal carcinomatosis: a phase II study. J Clin Oncol. 2003;21:799–806.

    Article  CAS  PubMed  Google Scholar 

  16. Yan TD, Edwards G, Alderman R, et al. Morbidity and mortality assessment of cytoreductive surgery and perioperative intraperitoneal chemotherapy for diffuse malignant peritoneal mesothelioma—a prospective study of 70 consecutive cases. Ann Surg Oncol. 2007;14:515–25.

    Article  PubMed  Google Scholar 

  17. Yan TD, Black D, Savady R, Sugarbaker PH. Systematic review on the efficacy of cytoreductive surgery combined with perioperative intraperitoneal chemotherapy for peritoneal carcinomatosis from colorectal carcinoma. J Clin Oncol. 2006;24:4011–9.

    Article  PubMed  Google Scholar 

  18. de Bree E, Romanos J, Michalakis J, et al. Intraoperative hyperthermic intraperitoneal chemotherapy with docetaxel as second-line treatment for peritoneal carcinomatosis of gynaecological origin. Anticancer Res. 2003;23:3019–27.

    PubMed  Google Scholar 

  19. Sugarbaker PH, Alderman R, Edwards G, et al. Prospective morbidity and mortality assessment of cytoreductive surgery plus perioperative intraperitoneal chemotherapy to treat peritoneal dissemination of appendiceal mucinous malignancy. Ann Surg Oncol. 2006;13:635–44.

    Article  PubMed  Google Scholar 

  20. Farma JM, Pingpank JF, Libutti SK, et al. Limited survival in patients with carcinomatosis from foregut malignancies after cytoreduction and continuous hyperthermic peritoneal perfusion. J Gastrointest Surg. 2005;9:1346–53.

    Article  PubMed  Google Scholar 

  21. Kunisaki C, Shimada H, Akiyama H, et al. Therapeutic outcomes of continuous hyperthermic peritoneal perfusion against advanced gastric cancer with peritoneal carcinomatosis. Hepatogastroenterology. 2006;53:473–8.

    PubMed  Google Scholar 

  22. Feldman AL, Libutti SK, Pingpank JF, et al. Analysis of factors associated with outcome in patients with malignant peritoneal mesothelioma undergoing surgical debulking and intraperitoneal chemotherapy. J Clin Oncol. 2003;21:4560–7.

    Article  CAS  PubMed  Google Scholar 

  23. 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 2014. This article is a summary of DSRCT patients treated by one group in France. They show in a large series, that the multimodal combination of chemotherapy, complete surgical excision and radiation are ALL components necessary to extend survival in DSRCT patients

  24. Hayes-Jordan A, Anderson P, Curley S, et al. Continuous hyperthermic peritoneal perfusion for desmoplastic small round cell tumor. J Pediatr Surg. 2007;42:E29–32.

    Article  PubMed  Google Scholar 

  25. Hayes-Jordan A, Green H, Ludwig J, Anderson P. Toxicity of hyperthermic intraperitoneal chemotherapy (HIPEC) in pediatric patients with sarcomatosis/carcinomatosis: early experience and phase 1 results. Pediatr Blood Cancer. 2012;59:395–7. This article describes the outcome of the largest cohort of DSRCT patients, who underwent HIPEC. The best outcomes are in a select group of patients with disease limited to the abdominal cavity, who had a partial response to chemotherapy, and who had a complete cytoreduction (NOT debulking), and postoperative whole abdominal radiation. The median survival with complete cytoreduction and HIPEC is 63 months, compared to 26 months with incomplete surgical resection.

    Article  CAS  PubMed  Google Scholar 

  26. Green H, Lin H, Owusu-Agyemang P et al. Perioperative renal protective treatment avoids renal toxicity in pediatric and adult patients undergoing HIPEC with cisplatin. J Pediatr Oncology 2014. This article highlights one of the pitfalls of HIPEC using Cisplatin, renal failure. The Hayes-Jordan group describes, in over 20 variables evaluated in DSRCT patients undergoing HIPEC, which were statistically significant in predicting renal failure these patients. By modifying the perioperative treatment in these patients, they were able to reduce the renal failure rate post HIPEC from 27% to 0%.

  27. 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.

    Article  PubMed  Google Scholar 

  28. 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.

    CAS  PubMed  Google Scholar 

  29. Aguilera D, Hayes-Jordan A, Anderson P, et al. Outpatient and home chemotherapy with novel local control strategies in desmoplastic small round cell tumor. Sarcoma. 2008;2008:261589.

    Article  PubMed Central  PubMed  Google Scholar 

  30. Frezza AM, Benson C, Judson IR, et al. Pazopanib in advanced desmoplastic small round cell tumours: a multi-institutional experience. Clin Sarcoma Res. 2014;4:7.

    Article  PubMed Central  PubMed  Google Scholar 

  31. Brunetti AE, Delcuratolo S, Lorusso V, et al. Third-line trabectedin for a metastatic desmoplastic small round cell tumour treated with multimodal therapy. Anticancer Res. 2014;34:3683–8.

    CAS  PubMed  Google Scholar 

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Acknowledgments

This study was supported by the NIH/NCI under award number P30CA016672.

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No financial disclosure.

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ᅟ

Conflict of Interest

Andrea Hayes-Jordan declares that she has no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

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Authors and Affiliations

  1. Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Unit 1484 1400 Pressler Street, Houston, TX, 77030, USA

    Andrea Hayes-Jordan

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  1. Andrea Hayes-Jordan
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Correspondence to Andrea Hayes-Jordan.

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This article is part of the Topical Collection on Sarcomas

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Hayes-Jordan, A. Cytoreductive Surgery Followed by Hyperthermic Intraperitoneal Chemotherapy in DSRCT: Progress and Pitfalls. Curr Oncol Rep 17, 38 (2015). https://doi.org/10.1007/s11912-015-0461-1

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  • DOI: https://doi.org/10.1007/s11912-015-0461-1

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