Journal of Gastrointestinal Cancer

, Volume 43, Issue 1, pp 103–109 | Cite as

Metastatic Desmoplastic Small Round Cell Tumor Controlled by an Anthracycline-Based Regimen: Review of the Role of Chemotherapy

  • H. Mrabti
  • W. Kaikani
  • N. Ahbeddou
  • H. Abahssain
  • B. El Khannoussi
  • M. Amrani
  • H. Errihani
Case Report

Abstract

Desmoplastic small round cell tumors (DSRCT) are rare and aggressive malignant neoplasms that usually occur in young males. They usually present intraabdominally with widespread peritoneal involvement at diagnosis and are sometimes associated with lymphadenopathies and liver metastases. Intraabdominal DSRCT is usually disseminated; no curative outcome has yet been achieved. We report a case of metastatic intraabdominal DSRCT controlled by an anthracycline-based regimen. A literature review of the treatment options, mainly chemotherapy, available for this unusual neoplasm, will be discussed. In patients with unresectable or metastatic disease, symptom control is most important because treatment modalities minimally impact survival. Palliative chemotherapy (mainly monotherapy) is preferable.

Keywords

Desmoplastic small round cell tumor Chemotherapy Treatment 

References

  1. 1.
    Gerald WL, Rosai J. Case 2: desmoplastic small cell tumor with divergent differentiation. Pediatr Pathol. 1989;9:177–83.PubMedCrossRefGoogle Scholar
  2. 2.
    Biswas G, Laskar S, Banavali SD, Gujral S, Kurkure PA, Muckaden M, et al. Desmoplastic small round cell tumor: extra abdominal and abdominal presentations and the results of treatment. Indian J Cancer. 2005;42(2):78–84.PubMedCrossRefGoogle Scholar
  3. 3.
    Cao L, Ni J, Que R, Wu Z, Song Z. Desmoplastic small round cell tumor: a clinical, pathological, and immunohistochemical study of 18 Chinese cases. Int J Surg Pathol. 2008;16(3):257–62.CrossRefGoogle Scholar
  4. 4.
    Gerald WL, Ladanyi M, de Alava E, Cuatrecasas M, Kushner BH, LaQuaglia MP, 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.PubMedGoogle Scholar
  5. 5.
    Stuart-Buttle CE, Smart CJ, Pritchard S, Martin D, Welch IM. Desmoplastic small round cell tumour: a review of literature and treatment options. Surg Oncol. 2008;17(2):107–12.PubMedCrossRefGoogle Scholar
  6. 6.
    Chouli M, Viala J, Dromain C, Fizazi K, Duvillard P, Vanel D. Intra-abdominal desmoplastic small round cell tumors: CT findings and clinicopathological correlations in 13 cases. Eur J Radiol. 2005;54:438–42.PubMedCrossRefGoogle Scholar
  7. 7.
    Coulibalya B, Liprandia A, Le Hémon A, Fernandez C, Hardwigsen J, Berthet B, et al. Desmoplastic small round-cell tumor: two cases of diffuse abdominopelvic infiltration. Gastroentérol Clin Biol. 2008;32(3):278–81.PubMedCrossRefGoogle Scholar
  8. 8.
    Kim YS, Cha SJ, Choi YS, Kim BG, Park SJ, Chang IT. Retroperitoneal desmoplastic small round cell tumor: pediatric patient treated with multimodal therapy. World J Gastroenterol. 2009;15(33):4212–4.PubMedCrossRefGoogle Scholar
  9. 9.
    Li F, Li G, Chen Z, Bi C. Primary desmoplastic small round cell tumor of urinary bladder. J Clin Oncol. 2010;28(18):e291–4.PubMedCrossRefGoogle Scholar
  10. 10.
    Battisti S, Renaudin K, Rigaud J, Hetet JF, Braud G, Kapfer J, et al. Small round cell desmoplastic tumour presenting as a testicular mass. Prog Urol. 2005;15(2):333–6.PubMedGoogle Scholar
  11. 11.
    Rais H, Elmansouri F, Belaabidia B, Essadki O, Oussehal A, Sarf I. Paratesticular desmoplastic small round cell tumour: case report with literature review. Cancer Radiothér. 2010;14(2):111–4.PubMedCrossRefGoogle Scholar
  12. 12.
    Neuzillet C, Hammel P, Couvelard A, Msika S, Felce-Dachez M, Laé M, et al. Desmoplastic small round cell tumour of the pancreas with breast metastasis. Gastroentérol Clin Biol. 2009;33(3):217–24.PubMedCrossRefGoogle Scholar
  13. 13.
    Hiralal GS, Thulkar S, Rao SK. Desmoplastic round cell tumour of the abdomen. Singapore Med J. 2007;1:e19–21.Google Scholar
  14. 14.
    Lae ME, Roche PC, Jin L, Lloyd RV, Nascimento AG. Desmoplastic small round cell tumor: a clinicopathologic, immunohistochemical, and molecular study of 32 tumors. Am J Surg Pathol. 2002;26:823–35.PubMedCrossRefGoogle Scholar
  15. 15.
    Chang F. Desmoplastic small round cell tumours: cytologic, histologic, and immunochemical features. Arch Pathol Lab Med. 2005;130(5):728–32.Google Scholar
  16. 16.
    Fizazi K, Farhat F, Theodore C, Rixe O, Le Cesne A, Comoy E, et al. Ca125 and neuron-specific enolase (NSE) as tumor markers for intra-abdominal desmoplastic small round-cell tumors. Br J Cancer. 1997;75:76–8.PubMedCrossRefGoogle Scholar
  17. 17.
    Gerald WL, Haber DA. The EWS–WT1 gene fusion in desmoplastic small round cell tumor. Semin Cancer Biol. 2005;15:197–205.PubMedCrossRefGoogle Scholar
  18. 18.
    Sawyer JR, Tryka AF, Lewis JM. A novel reciprocal chromosome translocation in an intraabdominal t(11;22)(p13;q12) desmoplastic small round cell tumour. Am J Surg Pathol. 1992;16(4):411–6.PubMedCrossRefGoogle Scholar
  19. 19.
    Zhang J, Dalton J, Fuller C. Epithelial marker-negative desmoplastic small round cell tumor with atypical morphology: definitive classification by fluorescence in situ hybridization. Arch Pathol Lab Med. 2007;131(4):646–9.PubMedGoogle Scholar
  20. 20.
    Liping Cao, Jun Ni, Risheng Que, Zhengrong Wu, Zhenya Song. desmoplastic small round cell tumor: a clinical, pathological, and immunohistochemical study of 18 Chinese cases. Int J Surg Pathol. 2008;16(3):257–62.CrossRefGoogle Scholar
  21. 21.
    Hassan I, Shyyan R, Donohue JH, Edmonson JH, Gunderson LL, Moir CR, et al. Intraabdominal desmoplastic small round cell tumours: a diagnostic and therapeutic challenge. Cancer. 2005;104(6):1264–70.PubMedCrossRefGoogle Scholar
  22. 22.
    Lal DR, Su WT, Wolden SL, Loh KC, Modak S, La Quaglia MP. Results of multimodal treatment for desmoplastic small round cell tumour. J Pediatr Surg. 2005;40(1):251–5.PubMedCrossRefGoogle Scholar
  23. 23.
    Livaditi E, Mavridis G, Soutis M, Papandreou E, Moschovi M, Papadakis V, et al. Diffuse intraabdominal desmoplastic small round cell tumour: a ten-year experience. Eur J Pediatr Surg. 2006;16(6):423–7.PubMedCrossRefGoogle Scholar
  24. 24.
    Goodman KA, Wolden SL, La Quaglia MP, Kushner BH. Whole abdominopelvic radiotherapy for desmoplastic small round cell tumour. Int J Radiat Oncol Biol Phys. 2002;54(1):170–6.PubMedCrossRefGoogle Scholar
  25. 25.
    Schwarz RE, Gerald WL, Kushner BH, Coit DG, Brennan MF, La Quaglia MP. Desmoplastic small round cell tumors: prognostic indicators and results of surgical management. Ann Surg Oncol. 1998;5:416–22.PubMedCrossRefGoogle Scholar
  26. 26.
    LaQuaglia MP, Brennan MF. The clinical approach to desmoplastic small round cell tumor. Surg Oncol. 2000;9:77–81.CrossRefGoogle Scholar
  27. 27.
    Kushner BH, LaQuaglia MP, Wollner N, Meyers PA, Lindsley KL, Ghavimi F, et al. Desmoplastic small round cell tumor prolonged progression free survival with aggressive multimodality therapy. J Clin Oncol. 1996;14:1526–31.PubMedGoogle Scholar
  28. 28.
    Farhat F, Culine S, Lhomme C, Duvillard P, Terrierlacombe MJ, Michel G, et al. Desmoplastic small round cell tumour result of a four-drug chemotherapy regimen in five adult patients. Cancer. 1996;77:1363–6.PubMedCrossRefGoogle Scholar
  29. 29.
    Bisogno G, Riccardi R, Ruggiero A, et al. Phase II study of a protracted irinotecan schedule in children with refractory or recurrent soft tissue sarcoma. Cancer. 2006;106(3):703–7.PubMedCrossRefGoogle Scholar
  30. 30.
    Rosoff PM, Bayliff S. Successful clinical response to irinotecan in desmoplastic round blue cell tumor. Med Pediatr Oncol. 1999;33(5):500–3.PubMedCrossRefGoogle Scholar
  31. 31.
    Ferrari A, Grosso F, Stacchiotti S, Meazza C, Zaffignani E, Marchianò A, et al. Response to vinorelbine and low-dose cyclophosphamide chemotherapy in two patients with desmoplastic small round cell tumor. Pediatr Blood Cancer. 2007;49(6):864–6.PubMedCrossRefGoogle Scholar
  32. 32.
    Bertuzzi A, Castagna L, Nozza A, Quagliuolo V, Siracusano L, Balzarotti M, et al. High-dose chemotherapy in poor-prognosis adult small round cell tumours: clinical and molecular results from a prospective study. J Clin Oncol. 2002;20(8):2181–8.PubMedCrossRefGoogle Scholar
  33. 33.
    Bertuzzi A, Castagna L, Quagliuolo V, Ginanni V, Compasso S, Magagnoli M, et al. Prospective study of high-dose chemotherapy and autologous peripheral stem cell transplantation in adult patients with advanced desmoplastic small round cell tumour. Br J Cancer. 2003;89(7):1159–61.PubMedCrossRefGoogle Scholar
  34. 34.
    Mazuryk M, Paterson AH, Temple W, Arthur K, Crabtree T, Stewart DA. Benefit of aggressive multimodality therapy with autologous stem cell support for intra abdominal desmoplastic small round cell tumor. Bone Marrow Transplant. 1998;21:961–3.PubMedCrossRefGoogle Scholar
  35. 35.
    Hayes-Jordan A, Anderson P, Curley S, Herzog C, Lally KP, Green HL, et al. Continuous hyperthermic peritoneal perfusion for desmoplastic small round cell tumor. J Pediatr Surg. 2007;42(8):E29–32.PubMedCrossRefGoogle Scholar
  36. 36.
    Gil A, Paortilla A, Brun E, et al. Clinical perspective on desmoplastic small round cell tumour. Oncology. 2004;67:231–42.PubMedCrossRefGoogle Scholar
  37. 37.
    Aguilera D, Hayes-Jordan A, Anderson P, Woo S, Pearson M, Green H. Outpatient and home chemotherapy with novel local control strategies in desmoplastic small round cell tumor. Sarcoma. 2008;2008:261589.PubMedCrossRefGoogle Scholar
  38. 38.
    Lippe P, Berardi R, Cappelletti C, Massacesi C, Mattioli R, Latini L, et al. Desmoplastic small round cell tumour: a description of two cases and review of the literature. Oncology. 2003;64:14–7.PubMedCrossRefGoogle Scholar
  39. 39.
    Adamson PC, Blaney SM, Widemann BC, Kitchen B, Murphy RF, Hannah AL, et al. Pediatric phase I trial and pharmacokinetic study of the platelet-derived growth factor (PDGF) receptor pathway inhibitor SU101. Cancer Chemother Pharmacol. 2004;53:482–8.PubMedCrossRefGoogle Scholar
  40. 40.
    Bond M, Bernstein ML, Pappo A, Schultz KR, Krailo M, Blaney SM, et al. A phase II study of imatinib mesylate in children with refractory or relapsed solid tumors: a Children’s Oncology Group study. Pediatr Blood Cancer. 2008;50(2):254–8.PubMedCrossRefGoogle Scholar
  41. 41.
    R. Benjamin, L. Gore, C. Dias, et al., “Activity of R1507, a fully humanized monoclonal antibody IGF-1R (insulin-like growth factor receptor)antagonist, in patients with Ewing’s sarcoma noted in a phase I study,” in Proceedings of the 13th Annual Meeting of the Connective Tissue Oncology Society (CTOS ’07), Seattle, Washington, USA, November 2007, abstract # 932:49Google Scholar
  42. 42.
    Modak S, Kramer K, Gultekin SH, Guo HF, Cheung NK. Monoclonal antibody 8H9 targets a novel cell surface antigen expressed by a wide spectrum of human solid tumors. Cancer Res. 2001;61(10):4048–54.PubMedGoogle Scholar
  43. 43.
    Modak S, Gerald W, Cheung NK. Disialoganglioside GD2 and a novel tumor antigen: potential targets for immunotherapy of desmoplastic small round cell tumor. Med Pediatr Oncol. 2002;39(6):547–51.PubMedCrossRefGoogle Scholar
  44. 44.
    Cheung NK, Kushner BH, Cheung IY, Kramer K, Canete A, Gerald W, et al. Anti-G(D2) antibody treatment of minimal residual stage IV neuroblastoma diagnosed at more than 1 year of age. J Clin Oncol. 1998;16(9):3053–60.PubMedGoogle Scholar
  45. 45.
    Fine RL, Shah SS, Moulton TA, et al. Androgen and c-Kit receptors in desmoplastic small round cell tumors resistant to chemotherapy: novel targets for therapy. Cancer Chemother Pharmacol. 2007;59:429–37.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  • H. Mrabti
    • 1
  • W. Kaikani
    • 1
  • N. Ahbeddou
    • 1
  • H. Abahssain
    • 1
  • B. El Khannoussi
    • 2
  • M. Amrani
    • 2
  • H. Errihani
    • 1
  1. 1.Department of Medical Oncology, National Institute of OncologyUniversity HospitalRabatMorocco
  2. 2.Department of Histopathology, National Institute of OncologyUniversity HospitalRabatMorocco

Personalised recommendations