Annals of Surgical Oncology

, Volume 22, Issue 4, pp 1073–1079 | Cite as

Abdominal Desmoplastic Small Round Cell Tumor: Multimodal Treatment Combining Chemotherapy, Surgery, and Radiotherapy is the Best Option

  • Charles Honoré
  • Koceila Amroun
  • Laurence Vilcot
  • Olivier Mir
  • Julien Domont
  • Philippe Terrier
  • Axel Le Cesne
  • Cecile Le Péchoux
  • Sylvie Bonvalot
Bone and Soft Tissue Sarcomas

Abstract

Background

With nearly 450 cases reported since 1991, desmoplastic small round cell tumor (DSRCT) is a rare abdominal tumor typically arising in adolescent and young adult white men. With no large series described, the best therapeutic strategy remains unclear.

Methods

All consecutive patients treated in our tertiary care center between January 1991 and December 2013 for a DSRCT were retrospectively studied.

Results

Thirty-eight patients with a median age of 27 years (range 13–57 years) were identified; 71 % were men. At the time of diagnosis, 47.4 % patients had extraperitoneal metastases (EPM): 78 % were located in the liver and 11 % were located in the lungs. Fourteen patients (37 %) were treated exclusively with systemic chemotherapy, with a median survival of 21.1 months. Twenty-three patients underwent surgery, 12 (52 %) experienced complete removal of all macroscopic disease, 5 (21.7 %) received additional intraperitoneal chemotherapy, and 7 (30 %) received postoperative whole abdominopelvic radiotherapy (WAP RT). With a median follow-up of 59.9 months, the median survival was 37.7 months, and the median disease-free survival was 15.5 months. The factors predictive of 3-year overall survival were the absence of EPM, complete surgical resection, postoperative WAP RT, and postoperative chemotherapy. The intraperitoneal chemotherapy had no impact on overall survival.

Conclusions

DSRCT is a rare and aggressive disease. In patients without EPM, a multimodal treatment combining systemic chemotherapy, complete macroscopic resection, and postoperative WAP RT could enable prolonged survival. No benefit of surgery was demonstrated for patients with EPM. The value of associated hyperthermic intraperitoneal chemotherapy remains unproven.

References

  1. 1.
    Gerald WL, Miller HK, Battifora H, Miettinen M, Silva EG, Rosai J. 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.CrossRefPubMedGoogle Scholar
  2. 2.
    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
  3. 3.
    Nathan JD, Gingalewsik C, Salem RR. Intra-abdominal desmoplastic small round cell tumor. Yale J Biol Med. 1999;72:287–93.PubMedCentralPubMedGoogle Scholar
  4. 4.
    Bisogno G, Roganovich J, Sotti G, et al. Desmoplastic small round cell tumour in children and adolescents. Med Pediatr Oncol. 2000;34:338–42.CrossRefPubMedGoogle Scholar
  5. 5.
    Quaglia MP, Brennan MF. The clinical approach to desmoplastic small round cell tumor. Surg Oncol. 2000;9:77–81.CrossRefPubMedGoogle Scholar
  6. 6.
    Lae ME, Roche PC, Jin L, et al. Desmoplastic small round cell tumor: a clinicopathologic, immunohistochemical, and molecular study of 32 tumors. Am J Surg Pathol. 2002;26:823–35.CrossRefPubMedGoogle Scholar
  7. 7.
    Lal DR, Su WT, Wolden SL, Loh KC, Modak S, La Quaglia MP. Results of multimodal treatment for desmoplastic small round cell tumors. J Pediatr Surg. 2005;40:251–5.CrossRefPubMedGoogle Scholar
  8. 8.
    Biswas G, Laskar S, Banavali SD, et al. Desmoplastic small round cell tumor: extra abdominal and abdominal presentations and the results of treatment. Indian J Cancer. 2005;42:78–84.CrossRefPubMedGoogle Scholar
  9. 9.
    Hassan I, Shyyan R, Donohue JH, et al. Intraabdominal desmoplastic small round cell tumors: a diagnostic and therapeutic challenge. Cancer. 2005;104:1264–70.CrossRefPubMedGoogle Scholar
  10. 10.
    Saab R, Khoury JD, Krasin M, Davidoff AM, Navid F. Desmoplastic small round cell tumor in childhood: the St Jude Children’s Research Hospital experience. Pediatr Blood Cancer. 2007;49:274–9.CrossRefPubMedGoogle Scholar
  11. 11.
    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.CrossRefPubMedGoogle Scholar
  12. 12.
    Dufresne A, Cassier P, Couraud L, et al. Desmoplastic small round cell tumor: current management and recent findings. Sarcoma. 2012;2012:714986.CrossRefPubMedCentralPubMedGoogle Scholar
  13. 13.
    Chan AS, MacNeill S, Thorner P, Squire J, Zielenska M. Variant EWS-WT1 chimeric product in the desmoplastic small round cell tumor. Pediatr Dev Pathol. 1999;2:188–92.CrossRefPubMedGoogle Scholar
  14. 14.
    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
  15. 15.
    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
  16. 16.
    Armstrong JF, Pritchard-Jones K, Bickmore WA, Hastie ND, Bard JB. The expression of the Wilms’ tumour gene, WT1, in the developing mammalian embryo. Mech Dev. 1993;40:85–97.CrossRefPubMedGoogle Scholar
  17. 17.
    Jacquet P, Sugarbaker PH. Clinical research methodologies in diagnosis and staging of patients with peritoneal carcinomatosis. In: Sugarbaker PH, editor. Peritoneal carcinomatosis: principles of management. Boston: Kluwer Academic; 1996. pp. 359–74.CrossRefGoogle Scholar
  18. 18.
    Therasse P, Arbuck SG, Eisenhauer EA, et al. New guidelines to evaluate the response to treatment in solid tumors. European Organization for Research and Treatment of Cancer, National Cancer Institute of the United States, National Cancer Institute of Canada. J Natl Cancer Inst. 2000;92:205–16.CrossRefPubMedGoogle Scholar
  19. 19.
    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.CrossRefPubMedCentralPubMedGoogle Scholar
  20. 20.
    Assi H, Missenard G, Terrier P, et al. Intensive induction chemotherapy without methotrexate in adult patients with localized osteosarcoma: results of the Institut Gustave-Roussy phase II trial. Curr Oncol. 2010;17:23–31.CrossRefPubMedCentralPubMedGoogle Scholar
  21. 21.
    Paulussen M, Ahrens S, Dunst J, et al. Localized Ewing tumor of bone: final results of the cooperative Ewing’s sarcoma. Study CESS 86. J Clin Oncol. 2001;19:1818–29.PubMedGoogle Scholar
  22. 22.
    Le Deley MC, Paulussen M, Lewis I, et al. Cyclophosphamide compared with ifosfamide in consolidation treatment of standard-risk ewing sarcoma: results of the randomized noninferiority Euro-EWING99-R1 trial. J Clin Oncol. 2014;32:2440–8.CrossRefPubMedGoogle Scholar
  23. 23.
    Watanabe T, Miyamoto S, Kitagori K, et al. A case of long-term survival of metastatic desmoplastic small round cell tumor treated with multimodal therapy. Oncol Lett. 2012;3:30–4.PubMedCentralPubMedGoogle Scholar
  24. 24.
    Desai NB, Stein NF, LaQuaglia MP, et al. Reduced toxicity with intensity modulated radiation therapy (IMRT) for desmoplastic small round cell tumor (DSRCT): an update on the whole abdominopelvic radiation therapy (WAP-RT) experience. Int J Radiat Oncol Biol Phys. 2013;85:e67–72.CrossRefPubMedGoogle Scholar
  25. 25.
    Chao J, Budd GT, Chu P, et al. Phase II clinical trial of imatinib mesylate in therapy of KIT and/or PDGFRalpha-expressing Ewing sarcoma family of tumors and desmoplastic small round cell tumors. Anticancer Res. 2010;30:547–52.PubMedGoogle Scholar
  26. 26.
    Thijs AM, van der Graaf WT, van Herpen CM. Temsirolimus for metastatic desmoplastic small round cell tumor. Pediatr Blood Cancer. 2010;55:1431–2.CrossRefPubMedGoogle Scholar
  27. 27.
    López-González A, Cantos B, Tejerina E, Provencio M. Activity of trabectidin in desmoplastic small round cell tumor. Med Oncol. 2011;28:S644–6.CrossRefPubMedGoogle Scholar
  28. 28.
    Naing A, LoRusso P, Fu S, et al. Insulin growth factor-receptor (IGF-1R) antibody cixutumumab combined with the mTOR inhibitor temsirolimus in patients with refractory Ewing’s sarcoma family tumors. Clin Cancer Res. 2012;18:2625–31.CrossRefPubMedGoogle Scholar
  29. 29.
    Tap WD, Demetri G, Barnette P, et al. Phase II study of ganitumab, a fully human anti-type-1 insulin-like growth factor receptor antibody, in patients with metastatic Ewing family tumors or desmoplastic small round cell tumors. J Clin Oncol. 2012;30:1849–56.CrossRefPubMedGoogle Scholar
  30. 30.
    Wong HH, Hatcher HM, Benson C, et al. Desmoplastic small round cell tumour: characteristics and prognostic factors of 41 patients and review of the literature. Clin Sarcoma Res. 2013;3:14.CrossRefPubMedCentralPubMedGoogle Scholar
  31. 31.
    Bertuzzi A, Castagna L, Nozza A, et al. High-dose chemotherapy in poor-prognosis adult small round-cell tumors: clinical and molecular results from a prospective study. J Clin Oncol. 2002;20:2181–8.CrossRefPubMedGoogle Scholar
  32. 32.
    Lauridant-Philippin G, Ledem N, Lemoine F, et al. Optimal treatment with systemic chemotherapy, complete surgical excision and hyperthermic intraperitoneal chemotherapy for a desmoplastic small round cell tumor in an adult male patient. Gastroenterol Clin Biol. 2010;34:321–4.CrossRefPubMedGoogle Scholar
  33. 33.
    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.CrossRefPubMedGoogle Scholar
  34. 34.
    Philippe-Chomette P, Kabbara N, Andre N, et al. Desmoplastic small round cell tumors with EWS-WT1 fusion transcript in children and young adults. Pediatr Blood Cancer. 2012;58:891–7.CrossRefPubMedGoogle Scholar
  35. 35.
    Bonvalot S, Cavalcanti A, Le Péchoux C, et al. Randomized trial of cytoreduction followed by intraperitoneal chemotherapy versus cytoreduction alone in patients with peritoneal sarcomatosis. Eur J Surg Oncol. 2005;31:917–23.CrossRefPubMedGoogle Scholar
  36. 36.
    Lim SJ, Cormier JN, Feig BW, et al. Toxicity and outcomes associated with surgical cytoreduction and hyperthermic intraperitoneal chemotherapy (HIPEC) for patients with sarcomatosis. Ann Surg Oncol. 2007;14:2309–18.CrossRefPubMedGoogle Scholar
  37. 37.
    Rossi CR, Deraco M, De Simone M, et al. Hyperthermic intraperitoneal intraoperative chemotherapy after cytoreductive surgery for the treatment of abdominal sarcomatosis: clinical outcome and prognostic factors in 60 consecutive patients. Cancer. 2004;100:1943–50.CrossRefPubMedGoogle Scholar
  38. 38.
    Elias D, Goéré D, Dumont F, et al. Role of hyperthermic intraoperative peritoneal chemotherapy in the management of peritoneal metastases. Eur J Cancer. 2014;50:332–40.CrossRefPubMedGoogle Scholar
  39. 39.
    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
  40. 40.
    Goodman KA, Wolden SL, La Quaglia MP, Kushner BH. Whole abdominopelvic radiotherapy for desmoplastic small round-cell tumor. Int J Radiat Oncol Biol Phys. 2002;54:170–6.CrossRefPubMedGoogle Scholar
  41. 41.
    Casey DL, Wexler LH, Laquaglia MP, Meyers PA, Wolden SL. Favorable outcomes after whole abdominopelvic radiation therapy for pediatric and young adult sarcoma. Pediatr Blood Cancer. 2014;61:1565–9.CrossRefPubMedGoogle Scholar

Copyright information

© Society of Surgical Oncology 2014

Authors and Affiliations

  • Charles Honoré
    • 1
  • Koceila Amroun
    • 1
  • Laurence Vilcot
    • 2
  • Olivier Mir
    • 3
  • Julien Domont
    • 3
  • Philippe Terrier
    • 4
  • Axel Le Cesne
    • 3
  • Cecile Le Péchoux
    • 5
  • Sylvie Bonvalot
    • 1
  1. 1.Department of Surgical OncologyGustave Roussy Cancer CenterVillejuifFrance
  2. 2.Department of RadiologyGustave Roussy Cancer CenterVillejuifFrance
  3. 3.Department of Medical OncologyGustave Roussy Cancer CenterVillejuifFrance
  4. 4.Department of PathologyGustave Roussy Cancer CenterVillejuifFrance
  5. 5.Department of Radiation OncologyGustave Roussy Cancer CenterVillejuifFrance

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