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Chemotherapy in clear cell sarcoma

Abstract

Clear cell sarcoma is a rare translocation-related sarcoma. There have been few studies documenting the response rate and progression-free survival in clear cell sarcoma patients treated with palliative chemotherapy. The prospectively maintained databases of two referral centres were searched to identify clear cell sarcoma patients treated with chemotherapy. Twenty-four patients were treated with palliative first-line chemotherapy with a median age of 30 years at diagnosis. There were 18 men and 6 women. One (4%) achieved a partial response and 9 (38%) had stable disease. Fourteen patients (58%) progressed on therapy. The median progression-free survival was 11 weeks (95% CI, 3–20 weeks). The median overall survival from commencing first-line chemotherapy was 39 weeks (95% CI, 34–45 weeks). Second-line chemotherapy was administered to 12 patients, 11 (92%) of these progressed and one (8%) had stable disease. Of the 5 patients treated with third-line chemotherapy, 4 (80%) progressed and one (20%) had stable disease. One patient received fourth-line chemotherapy and maintained stable disease for 4 months. Conventional chemotherapy has minimal activity in clear cell sarcoma as documented by the response rate of 4% and median progression-free survival of 11 weeks in this retrospective series. These data provide a reference for response and outcome in the assessment of novel agents in this histological subtype.

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References

  1. Enzinger FM. Clear-cell sarcoma of tendons and aponeuroses. An analysis of 21 cases. Cancer. 1965;18:1163–74.

    PubMed  Article  CAS  Google Scholar 

  2. Chung EB, Enzinger FM. Malignant melanoma of soft parts. A reassessment of clear cell sarcoma. Am J Surg Pathol. 1983;7:405–13.

    PubMed  Article  CAS  Google Scholar 

  3. Clark MA, Johnson MB, Thway K, Fisher C, Thomas JM, Hayes AJ. Clear cell sarcoma (melanoma of soft parts): the Royal Marsden Hospital experience. Eur J Surg Oncol. 2008;34:800–4.

    PubMed  CAS  Google Scholar 

  4. Stenman G, Kindblom LG, Angervall L. Reciprocal translocation t(12;22)(q13;q13) in clear-cell sarcoma of tendons and aponeuroses. Genes Chromosomes Cancer. 1992;4:122–7.

    PubMed  Article  CAS  Google Scholar 

  5. Zucman J, Delattre O, Desmaze C, et al. EWS and ATF-1 gene fusion induced by t(12;22) translocation in malignant melanoma of soft parts. Nat Genet. 1993;4:341–5.

    PubMed  Article  CAS  Google Scholar 

  6. Hisaoka M, Ishida T, Kuo TT, et al. Clear cell sarcoma of soft tissue: a clinicopathologic, immunohistochemical, and molecular analysis of 33 cases. Am J Surg Pathol. 2008;32:452–60.

    PubMed  Article  Google Scholar 

  7. Eckardt JJ, Pritchard DJ, Soule EH. Clear cell sarcoma. A clinicopathologic study of 27 cases. Cancer. 1983;52:1482–8.

    PubMed  Article  CAS  Google Scholar 

  8. Kawai A, Hosono A, Nakayama R, et al. Clear cell sarcoma of tendons and aponeuroses: a study of 75 patients. Cancer. 2007;109:109–16.

    PubMed  Article  CAS  Google Scholar 

  9. Sara AS, Evans HL, Benjamin RS. Malignant melanoma of soft parts (clear cell sarcoma). A study of 17 cases, with emphasis on prognostic factors. Cancer. 1990;65:367–74.

    PubMed  Article  CAS  Google Scholar 

  10. Ferrari A, Casanova M, Bisogno G, et al. Clear cell sarcoma of tendons and aponeuroses in pediatric patients: a report from the Italian and German Soft Tissue Sarcoma Cooperative Group. Cancer. 2002;94:3269–76.

    PubMed  Article  Google Scholar 

  11. Pavlidis NA, Fisher C, Wiltshaw E. Clear-cell sarcoma of tendons and aponeuroses: a clinicopathologic study. Presentation of six additional cases with review of the literature. Cancer. 1984;54:1412–7.

    PubMed  Article  CAS  Google Scholar 

  12. Radstone DJ, Revell PA, Mantell BS. Clear cell sarcoma of tendons and aponeuroses treated with bleomycin and vincristine. Br J Radiol. 1979;52:238–41.

    PubMed  Article  CAS  Google Scholar 

  13. Deenik W, Mooi WJ, Rutgers EJ, Peterse JL, Hart AA, Kroon BB. Clear cell sarcoma (malignant melanoma) of soft parts: a clinicopathologic study of 30 cases. Cancer. 1999;86:969–75.

    PubMed  Article  CAS  Google Scholar 

  14. Finley JW, Hanypsiak B, McGrath B, Kraybill W, Gibbs JF. Clear cell sarcoma: the Roswell Park experience. J Surg Oncol. 2001;77:16–20.

    PubMed  Article  CAS  Google Scholar 

  15. Lucas DR, Nascimento AG, Sim FH. Clear cell sarcoma of soft tissues. Mayo Clinic experience with 35 cases. Am J Surg Pathol. 1992;16:1197–204.

    PubMed  Article  CAS  Google Scholar 

  16. Montgomery EA, Meis JM, Ramos AG, Frisman DM, Martz KL. Clear cell sarcoma of tendons and aponeuroses: a clinicopathologic study of 58 cases with analysis of prognostic factors. Int J Surg Pathol. 1993;1:89–100.

    Article  Google Scholar 

  17. Steger GG, Wrba F, Mader R, Schlappack O, Dittrich C, Rainer H. Complete remission of metastasised clear cell sarcoma of tendons and aponeuroses. Eur J Cancer. 1991;27:254–6.

    PubMed  Article  CAS  Google Scholar 

  18. Lauro S, Bordin F, Trasatti L, Lanzetta G, Della Rocca C, Frati L. Concurrent chemoimmunotherapy in metastatic clear cell sarcoma: a case report. Tumori. 1999;85:512–4.

    PubMed  CAS  Google Scholar 

  19. Goldberg J, Demetri GD, Choy E, Rosen L, Pappo A, Dubois S, Geller J, Chai F, Ferrari D, Wagner AJ. Preliminary results from a phase II study of ARQ 197 in patients with microphthalmia transcription factor family (MiT)-associated tumors. J Clin Oncol 2009;27:Abstract 10502.

  20. Liu S, Cheng H, Kwan W, Lubieniecka JM, Nielsen TO. Histone deacetylase inhibitors induce growth arrest, apoptosis, and differentiation in clear cell sarcoma models. Mol Cancer Ther. 2008;7:1751–61.

    PubMed  Article  CAS  Google Scholar 

  21. Yokoyama S, Feige E, Poling LL, et al. Pharmacologic suppression of MITF expression via HDAC inhibitors in the melanocyte lineage. Pigment Cell Melanoma Res. 2008;21:457–63.

    PubMed  Article  CAS  Google Scholar 

  22. Segal NH, Pavlidis P, Noble WS, et al. Classification of clear-cell sarcoma as a subtype of melanoma by genomic profiling. J Clin Oncol. 2003;21:1775–81.

    PubMed  Article  CAS  Google Scholar 

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Correspondence to Robin L. Jones.

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Jones, R.L., Constantinidou, A., Thway, K. et al. Chemotherapy in clear cell sarcoma. Med Oncol 28, 859–863 (2011). https://doi.org/10.1007/s12032-010-9502-7

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  • DOI: https://doi.org/10.1007/s12032-010-9502-7

Keywords

  • Clear cell sarcoma
  • Chemotherapy
  • Response rate
  • Progression-free survival