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Pathology & Oncology Research

, Volume 20, Issue 1, pp 161–168 | Cite as

Analysis of Clinicopathological Features and Prognostic Factors of Desmoplastic Small Round Cell Tumor

  • Jinnan Zhang
  • Haiyan Xu
  • Fang Ren
  • Yijin Yang
  • Bin Chen
  • Fengchun Zhang
Research

Abstract

Desmoplastic small round cell tumor (DSRCT) is a relatively uncommon and highly aggressive malignancy in young males. It is associated with a poor outcome, due in part to missed diagnosis. To characterize the clinical pathological features of DSRCT in Chinese patients and to find out the characteristics of treatment and prognostic factors, the authors collected and analyzed the clinical information of 48 cases. A total of 48 cases of DSRCT between March 1995 and March 2012 were retrospectively reviewed and analyzed. The clinical information, histological results and survival data of the patients were collected. Median age was 26.96 ± 14.09 years with a range of 6–66 years. Thirty-three patients (68.75 %) were seen before 30 years old, and 15 patients (31.25 %) were diagnosed after 30 years old. The male-to-female ratio is 3.36 :1. Among them, 37 cases presented with tumors in the abdominal or pelvic cavity; the other 11 cases had extra-abdominal tumors. The most common symptoms were abdominal pain (19/48, 39.58 %) and palpable mass (12/48, 25.00 %). The percentage of patients received surgery, complete surgery, and chemotherapy was 79.17 %, 37.50 %, and 52.08 %, respectively. Median follow-up duration was 2.67 years. Median overall survival for all patients was 24.33 months (95 % CI: 9.74–38.92 months) and median event-free survival for all patients was 8.00 months (95 % CI: 5.13–10.89 months). Univariate analysis revealed that surgery, effective debulking surgery, chemotherapy and any two or more combined therapeutics were significant prognostic factors for longer overall survival (p < 0.05). Cox regression analysis showed complete surgery was an independent prognostic factor. Standard therapy for DSRCT consists of combination of surgical resection and postoperative chemotherapy. Complete surgery is an independent prognostic factor and should be further investigated.

Keywords

Desmoplastic small round cell tumor Prognosis 

Notes

Acknowledgments

We thank Dr Viive Howell in the Kolling Institute of RNS Hospital of Austria for paper revision.

Source of Support

This work was supported by National Key Basic Research Program (973 Project) (No. 2010CB933901) and the National Natural Science Foundation of China (No.81172522).

References

  1. 1.
    Gerald WL, Rosai J (1989) Case 2: desmoplastic small round cell tumor with divergent differentiation. Pediatr Pathol 9:177–183PubMedCrossRefGoogle Scholar
  2. 2.
    Dufresne A, Cassier P, Couraud L, Marec-Bérard P, Meeus P, Alberti L, Blay JY (2012) Desmoplastic small round cell tumor: current management and recent findings. Sarcoma 2012:714986PubMedCentralPubMedCrossRefGoogle Scholar
  3. 3.
    Rekhi B, Ahmed S, Basak R, Qureshi SS, Desai SS, Ramadwar M, Desai SB, Kurkure P, Jambhekar NA (2012) Desmoplastic small round cell tumor-clinicopathological spectrum, including unusual features and immunohistochemical analysis of 45 tumors diagnosed at a tertiary cancer referral centre, with molecular results t(11; 22) (p13; q12) (EWS-WT1) in select cases. Pathol Oncol Res 18:917–927PubMedCrossRefGoogle Scholar
  4. 4.
    Gerald WL, Ladanyi M, de Alava E , Cuatrecasas M, Kushner BH, LaQuaglia MP, Rosai J (1998) 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 16:3028–3036Google Scholar
  5. 5.
    Lal DR, Su WT, Wolden SL, Loh KC, Cuatrecasas M, Kushner BH, LaQuaglia MP, Rosai J (2005) Results of multimodal treatment for desmoplastic small round cell tumors. J Pediatr Surg 40:251–255PubMedCrossRefGoogle Scholar
  6. 6.
    Gil A, Gomez Portilla A, Brun EA, Sugarbaker PH (2004) Clinical perspective on desmoplastic small round-cell tumor. Oncology 67:231–242PubMedCrossRefGoogle Scholar
  7. 7.
    Perna MJ, Streck CJ (2012) A large solitary desmoplastic small round cell tumor. Am Surg 78:316–317Google Scholar
  8. 8.
    Jordan AH, Pappo A (2012) Management of desmoplastic small round-cell tumors in children and young adults. J Pediatr Hematol Oncol 34:S73–S75PubMedCrossRefGoogle Scholar
  9. 9.
    Chang F (2006) Desmoplastic small round cell tumors: cytologic, histologic, and immunohistochemical features. Arch Pathol Lab Med 130:728–732PubMedGoogle Scholar
  10. 10.
    Harmon RL, Sugarbaker PH (2005) Prognostic indicators in peritoneal carcinomatosis from gastrointestinal cancer. Int Semin Surg Oncol 2:3PubMedCentralPubMedCrossRefGoogle Scholar
  11. 11.
    Kallianpur AA, Shukla NK, Deo SV, Yadav P, Mudaly D, Yadav R, Palaniappan RM (2012) Updates on the multimodality management of desmoplastic small round cell tumor. J Surg Oncol 105:617–621PubMedCrossRefGoogle Scholar
  12. 12.
    Biswas G, Laskar S, Banavali SD, Gujral S, Kurkure PA, Muckaden M, Parikh PM, Nair CN (2005) Desmoplastic small round cell tumour: extra abdominal and abdominal presentations and the results of treatment. Indian J Cancer 42:78–84PubMedCrossRefGoogle Scholar
  13. 13.
    Hayes-Jordan A, Anderson PM (2011) The diagnosis and management of desmoplastic small round cell tumor: a review. Curr Opin Oncol 23:385–389PubMedCrossRefGoogle Scholar
  14. 14.
    Kushner BH, LaQuaglia MP, Wollner N, Meyers PA, Lindsley KL, Ghavimi F, Merchant TE, Boulad F, Cheung NK, Bonilla MA, Crouch G, Kelleher JF Jr, Steinherz PG, Gerald WL (1996) Desmoplastic small round cell tumor: prolonged progression-free survival with aggressive multimodality therapy. J Clin Oncol 14:1515–1531Google Scholar
  15. 15.
    Bertuzzi A, Castagna L, Nozza A, Quagliuolo V, Siracusano L, Balzarotti M, Compasso S, Alloisio M, Soto Parra H, Santoro A (2002) High-dose chemotherapy in poor-prognosis adult small round cell tumours: clinical and molecular results from a prospective study. J Clin Oncol 20:2181–2188PubMedCrossRefGoogle Scholar
  16. 16.
    Schwarz RE, Gerald WL, Kushner BH, Coit DG, Brennan MF, La Quaglia MP (1998) Desmoplastic small round cell tumors: prognostic indicators and results of surgical management. Ann Surg Oncol 5:416–422PubMedCrossRefGoogle Scholar
  17. 17.
    Ordóñez NG (1998) Desmoplastic small round cell tumor: I: a histopathologic study of 39 cases with emphasis on unusual histological patterns. Am J Surg Pathol 22:1303–1313PubMedCrossRefGoogle Scholar
  18. 18.
    Watanabe T, Miyamoto S, Kitagori K, Horimatsu T, Morita S, Mashimo Y, Ezoe Y, Muto M, Chiba T (2012) A case of long-term survival of metastatic desmoplastic small round cell tumor treated with multimodal therapy. Oncol Lett 3:30–34PubMedCentralPubMedGoogle Scholar
  19. 19.
    Quaglia MP, Brennan MF (2000) The clinical approach to desmoplastic small round cell tumor. Surg Oncol 9:77–81PubMedCrossRefGoogle Scholar

Copyright information

© Arányi Lajos Foundation 2013

Authors and Affiliations

  • Jinnan Zhang
    • 1
  • Haiyan Xu
    • 2
  • Fang Ren
    • 2
  • Yijin Yang
    • 2
  • Bin Chen
    • 2
  • Fengchun Zhang
    • 2
    • 3
  1. 1.Department of Neurosurgery, China-Japan Union HospitalJilin UniversityChangchunChina
  2. 2.Department of Oncology, Suzhou Kowloon HospitalShanghai Jiaotong University School of MedicineSuzhouChina
  3. 3.Department of Oncology, Ruijin HospitalShanghai Jiaotong University School of MedicineShanghaiChina

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