Annals of Surgical Oncology

, Volume 5, Issue 5, pp 416–422 | Cite as

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

  • Roderich E. Schwarz
  • William L. Gerald
  • Brian H. Kushner
  • Daniel G. Coit
  • Murray F. Brennan
  • Michael P. La Quaglia
Original Articles

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.

Key Words

Desmoplastic small round cell tumor Chemotherapy Resection Surgery 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Gerald WL, Rosai J. Case 2. Desmoplastic small cell tumor with divergent differentiation.Pediatr Pathol 1989;9:177–83.PubMedGoogle Scholar
  2. 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.PubMedGoogle Scholar
  3. 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.CrossRefPubMedGoogle Scholar
  4. 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.PubMedGoogle Scholar
  5. 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.PubMedGoogle Scholar
  6. 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.CrossRefPubMedGoogle Scholar
  7. 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.PubMedGoogle Scholar
  8. 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.PubMedGoogle Scholar
  9. 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.CrossRefPubMedGoogle Scholar
  10. 10.
    Gerald WL, Rosai J. Desmoplastic small cell tumor with multiphenotypic differentiation.Zentralbl Pathol 1993;139:141–51.PubMedGoogle Scholar
  11. 11.
    Amato RJ, Ellerhorst JA, Ayala AG. Intraabdominal desmoplastic small cell tumor. Report and discussion of five cases.Cancer 1996;78:845–51.CrossRefPubMedGoogle Scholar
  12. 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.PubMedGoogle Scholar
  13. 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.CrossRefPubMedGoogle Scholar
  14. 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.PubMedGoogle Scholar
  15. 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.CrossRefPubMedGoogle Scholar
  16. 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.CrossRefPubMedGoogle Scholar
  17. 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.PubMedGoogle Scholar
  18. 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.PubMedGoogle Scholar
  19. 19.
    Frappaz D, Bouffet E, Dolbeau D, et al. Desmoplastic small round cell tumors of the abdomen.Cancer 1994;73:1753–6.PubMedGoogle Scholar
  20. 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.CrossRefPubMedGoogle Scholar
  21. 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.PubMedGoogle Scholar
  22. 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.PubMedGoogle Scholar
  23. 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.PubMedGoogle Scholar
  24. 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.PubMedGoogle Scholar
  25. 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.PubMedGoogle Scholar
  26. 26.
    Kaplan EL, Meier P. Nonparametric estimation from incomplete observations.Am Stat Assoc J 1958;53:457–81.Google Scholar
  27. 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.PubMedGoogle Scholar
  28. 28.
    Cox DR. Regression models and life tables.J R Stat Assoc 1972;29:187–220.Google Scholar
  29. 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.PubMedGoogle Scholar
  30. 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.PubMedGoogle Scholar
  31. 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.PubMedGoogle Scholar

Copyright information

© The Society of Surgical Oncology, Inc. 1998

Authors and Affiliations

  • Roderich E. Schwarz
    • 2
  • William L. Gerald
    • 1
  • Brian H. Kushner
  • Daniel G. Coit
    • 2
  • Murray F. Brennan
    • 2
  • Michael P. La Quaglia
    • 2
  1. 1.the Department of PathologyMemorial Sloan-Kettering Cancer CenterNew York
  2. 2.Dept. of SurgeryMemorial Sloan-Kettering Cancer CenterNew York

Personalised recommendations