Skip to main content
SpringerLink
Log in

Desmoplastic small round cell tumors: Prognostic indicators and results of surgical management

  • Original Articles
  • Published:
Annals of Surgical Oncology Aims and scope Submit manuscript

Abstract

Background: Desmoplastic small round cell tumors (DSRCT or DSCT) are rare aggressive cancers of adolescence and early adulthood. There are few reported series to guide clinical therapy. This study correlates survival with treatment variables, including aggressive surgical debulking.

Methods: Thirty-two patients with documented DSRCT received treatment at our institution. Demographic, clinical, and treatment variables were correlated with progression-free survival using log-rank statistics.

Results: Thirty patients were male (96%), and two were female (4%), with a median age at diagnosis of 22 years. The primary site of disease in 97% of cases was the abdomen or pelvis. Twenty-nine patients (91%) had extensive disease involving peritoneal surfaces, lymph nodes, or discontinuous organs. All 32 patients received systemic chemotherapy. Fifteen (47%) underwent tumor debulking greater than 90% at diagnosis or during therapy. A complete or very good response to therapy occurred in 13 patients, and depended on surgical removal of bulk disease in all. Thirteen patients remained progression-free, but three of these patients died from treatment toxicity. Improved survival was correlated with a complete or very good partial response to multimodality therapy, surgical debulking of more than 90% either before or after chemotherapy, and use of the P6 protocol.

Conclusions: DSRCT is an aggressive cancer that occurs predominantly in young males. Improved survival is correlated with intense chemotherapy and aggressive resection.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Gerald WL, Rosai J. Case 2. Desmoplastic small cell tumor with divergent differentiation.Pediatr Pathol 1989;9:177–83.

    CAS  PubMed  Google Scholar 

  2. Gerald WL, Miller HK, Battifora H, Miettinen M, Silva EG, Rosai J. Intra-abdominal desmoplastic small round-cell tumor.Am J Surg Pathol 1991;15:499–513.

    CAS  PubMed  Google Scholar 

  3. Rodriguez E, Sreekantaiah C, Gerald W, Reuter VE, Motzer RJ, Chaganti RS. A recurring translocation, t(11; 22)(p13; q11.2), characterizes intra-abdominal desmoplastic small round-cell tumors.Cancer Genet Cytogenet 1993;69:17–21.

    Article  CAS  PubMed  Google Scholar 

  4. Biegel JA, Conard K, Brooks JJ. Translocation (11; 22)(p13; q12): primary change in intra-abdominal desmoplastic small round cell tumor.Genes Chromosom Cancer 1993;7:119–21.

    CAS  PubMed  Google Scholar 

  5. 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 

  6. Brodie SG, Stocker SJ, Wardlaw JC, Duncan MH, McConnell TS, Feddersen RM, Williams TM. EWS and WT-1 gene fusion in desmoplastic small round cell tumor of the abdomen.Hum Pathol 1995;26:1370–4.

    Article  CAS  PubMed  Google Scholar 

  7. Gerald WL, Rosai J, Ladanyi M. Characterization of the genomic breakpoint and chimeric transcripts in the EWS-WT1 gene fusion of desmoplastic small round cell tumor.Proc Natl Acad Sci USA 1995;92:1028–32.

    CAS  PubMed  Google Scholar 

  8. de Alava E, Ladanyi M, Rosai J, Gerald WL. Detection of chimeric transcripts in desmoplastic small round cell tumor and related developmental tumors by reverse transcriptase polymerase chain reaction. A specific diagnostic assay.Am J Pathol 1995;147:1584–91.

    PubMed  Google Scholar 

  9. Benjamin LE, Fredericks WJ, Barr FG, Rauscher FR. Fusion of the EWS1 and WT1 genes as a result of the t(11;22)(p13;q12) translocation in desmoplastic small round cell tumors.Med Pediatr Oncol 1996;27:434–9.

    Article  CAS  PubMed  Google Scholar 

  10. Gerald WL, Rosai J. Desmoplastic small cell tumor with multiphenotypic differentiation.Zentralbl Pathol 1993;139:141–51.

    CAS  PubMed  Google Scholar 

  11. Amato RJ, Ellerhorst JA, Ayala AG. Intraabdominal desmoplastic small cell tumor. Report and discussion of five cases.Cancer 1996;78:845–51.

    Article  CAS  PubMed  Google Scholar 

  12. Parkash V, Gerald WL, Parma A, Miettinen M, Rosai J. Desmoplastic small round cell tumor of the pleura.Am J Surg Pathol 1995;19:659–65.

    CAS  PubMed  Google Scholar 

  13. Tison V, Cerasoli S, Morigi F, Ladanyi M, Gerald WL, Rosai J. Intracranial desmoplastic small-cell tumor. Report of a case.Am J Surg Pathol 1996;20:112–7.

    Article  CAS  PubMed  Google Scholar 

  14. Ordonez NG, el-Naggar AK, Ro JY, Silva EG, Mackay B. Intra-abdominal desmoplastic small cell tumor: a light microscopic, immunocytochemical, ultrastructural, and flow cytometric study [see comments].Hum Pathol 1993;24:850–65.

    CAS  PubMed  Google Scholar 

  15. Kretschmar CS, Colbach C, Bhan I, Crombleholme TM. Desmoplastic small cell tumor: a report of three cases and a review of the literature.J Pediatr Hematol Oncol 1996;18:293–8.

    Article  CAS  PubMed  Google Scholar 

  16. Shintaku M, Baba Y, Fujiwara T. Intra-abdominal desmoplastic small cell tumor in a patient with Peutz-Jeghers syndrome.Virchows Arch 1994;425:211–5.

    Article  CAS  PubMed  Google Scholar 

  17. Resnick MB, Donovan M. Intra-abdominal desmoplastic small round cell tumor with extensive extra-abdominal involvement.Pediatr Pathol Lab Med 1995;15:797–803.

    CAS  PubMed  Google Scholar 

  18. Mead M, Jones MA, Decain M, Tarraza HM. Intra-abdominal desmoplastic small round-cell tumor in a postmenopausal female. Report of a case and review of the literature.Eur J Gynaecol Oncol 1994;15:267–71.

    CAS  PubMed  Google Scholar 

  19. Frappaz D, Bouffet E, Dolbeau D, et al. Desmoplastic small round cell tumors of the abdomen.Cancer 1994;73:1753–6.

    CAS  PubMed  Google Scholar 

  20. Crombleholme TM, Harris BH, Jacir NN, et al. The desmoplastic round cell tumor: a new solid tumor of childhood.J Pediatr Surg 1993;28:1023–5.

    Article  CAS  PubMed  Google Scholar 

  21. Basade MM, Vege DS, Nair CN, Kurkure PA, Advani SH. Intra-abdominal desmoplastic small round cell tumor in children: a clinicopathologic study.Pediatr Hematol Oncol 1996;13:95–9.

    CAS  PubMed  Google Scholar 

  22. Dorsey BV, Benjamin LE, Rauscher FR, Klencke B, Venook AP, Warren RS, Weidner N. Intra-abdominal desmoplastic small round-cell tumor: expansion of the pathologic profile.Mod Pathol 1996;9:703–9.

    CAS  PubMed  Google Scholar 

  23. Liau CT, Yang TS, Wang CH, Hsueh S. Intra-abdominal desmoplastic small cell tumors: report of two cases.Anticancer Drugs 1996;7:228–32.

    CAS  PubMed  Google Scholar 

  24. Murray JC, Minifee PK, Trautwein LM, Hicks MJ, Langston C, Morad AB. Intraabdominal desmoplastic small round cell tumor presenting as a gastric mural mass with hepatic metastases.J Pediatr Hematol Oncol 1996;18:289–92.

    CAS  PubMed  Google Scholar 

  25. 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 

  26. Kaplan EL, Meier P. Nonparametric estimation from incomplete observations.Am Stat Assoc J 1958;53:457–81.

    Google Scholar 

  27. Peto R, Pike MC. Conservatism in the approximation E(0-E)2/E in the log rank test for survival data or tumor incidence data.Biometrics 1973;29:579–84.

    CAS  PubMed  Google Scholar 

  28. Cox DR. Regression models and life tables.J R Stat Assoc 1972;29:187–220.

    Google Scholar 

  29. Farhat F, Culine S, Lhomme C, et al. Desmoplastic small round cell tumors: results of a four-drug chemotherapy regimen in five adult patients.Cancer 1996;77:1363–6.

    CAS  PubMed  Google Scholar 

  30. Schmidt D, Koster E, Harms D. Intraabdominal desmoplastic small-cell tumor with divergent differentiation: clinicopathological findings and DNA ploidy.Med Pediatr Oncol 1994;22:97–102.

    CAS  PubMed  Google Scholar 

  31. Min KW. Usefulness of electron microscopy in the diagnosis of “small” round cell tumors of the sinonasal region.Ultrastruct Pathol 1995;19:347–63.

    CAS  PubMed  Google Scholar 

Download references

Author information

Author notes

  1. Roderich E. Schwarz MD

    Present address: the Department of General Oncologic Surgery, City of Hope National Medical Center, 1500 East Duarte Rd., 91010-3000, Duarte, CA

Authors and Affiliations

  1. the Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, New York

    William L. Gerald MD

  2. Dept. of Surgery, Memorial Sloan-Kettering Cancer Center, 1275 York Ave., 10021, New York, NY

    Roderich E. Schwarz MD, Daniel G. Coit MD, Murray F. Brennan MD & Michael P. La Quaglia MD

Authors
  1. Roderich E. Schwarz MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. William L. Gerald MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Brian H. Kushner MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Daniel G. Coit MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Murray F. Brennan MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Michael P. La Quaglia MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Schwarz, R.E., Gerald, W.L., Kushner, B.H. et al. Desmoplastic small round cell tumors: Prognostic indicators and results of surgical management. Annals of Surgical Oncology 5, 416–422 (1998). https://doi.org/10.1007/BF02303860

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02303860

Key Words

Search

Navigation