Skip to main content
SpringerLink
Log in

Systemic Therapy for Rare Tumours of the Skin and Soft Tissue Tumour

  • Chapter
  • First Online:
Skin Cancer

Part of the book series: Current Clinical Pathology ((CCPATH))

  • 5142 Accesses

Abstract

The role of systemic therapy in the management of soft tissue sarcoma and rare tumours of the skin is still extremely important. Even if the principal therapeutic approach in the localised disease is represented by surgery, in the metastatic setting, the role of surgery is still poorly defined, and a systemic approach becomes mandatory. Furthermore, the possible use of systemic therapies both in the adjuvant setting, with the aim of consolidating the results obtained through surgery, and in the neoadjuvant setting, in order to optimise the local control of the disease, has been recently assessed.

For years, standard chemotherapy has represented the only systemic approach available in the treatment of soft tissue sarcoma and rare tumours of the skin: however, given the poor results obtained up today, newer compounds and target therapies are currently under active investigation.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 89.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Ries LAG, Melbert D, Krapcho M, Stinchcomb DG, Howlader N, Horner MJ, Mariotto A, Miller BA, Feuer EJ, Altekruse SF, Lewis DR, Clegg L, Eisner MP, Reichman M, Edwards BK (editors). SEER Cancer Statistics Review, 1975–2005. Bethesda: National Cancer Institute; 2008. http://seer.cancer.gov/csr/1975_2005/, based on November 2007 SEER data submission, posted to the SEER web site.

  2. Cormier JN, Pollock RE. Soft tissue sarcomas. CA Cancer J Clin. 2004;54(2):94–109.

    Article  PubMed  Google Scholar 

  3. Fong Y, Coit DG, Woodruff JM, Brennan MF. Lymph node metastasis from soft tissue sarcoma in adults. Analysis of data from a prospective database of 1772 sarcoma patients. Ann Surg. 1993;217:72–7.

    Article  PubMed  CAS  Google Scholar 

  4. Criscione VD, Weinstock MA. Descriptive epidemiology of dermatofibrosarcoma protuberans in the United States, 1973 to 2002. J Am Acad Dermatol. 2007;56:968–73.

    Article  PubMed  Google Scholar 

  5. Rutgers EJ, Kroon BB, Albus-Lutter CE, Gortzak E. Dermatofibrosarcoma protuberans: treatment and prognosis. Eur J Surg Oncol. 1997;18:241–8.

    Google Scholar 

  6. Patel KU, Szabo SS, Hernandez VS, Prieto VG, Abruzzo LV, Lazar AJ, et al. Dermatofibrosarcoma protuberans COL1A1-PDGFB fusion is identified in virtually all dermatofibrosarcoma protuberans cases when investigated by newly developed multiplex reverse transcription polymerase chain reaction and fluorescence in situ hybridization assays, Hum Pathol. 2008;39(2):184–93.

    Article  PubMed  CAS  Google Scholar 

  7. Rollison DE, Giuliano AR, Becker JC. New virus associated with Merkel cell carcinoma development. J Natl Compr Canc Netw. 2010;8(8):874–80.

    PubMed  Google Scholar 

  8. Medina-Franco H, Urist MM, Fiveash J, Heslin MJ, Bland KI, Beenken SW. Multimodality treatment of Merkel cell carcinoma: case series and literature review of 1024 cases. Ann Surg Oncol. 2001;8(3):204–8.

    Article  PubMed  CAS  Google Scholar 

  9. Allen PJ, Bowne WB, Jaques DP, Brennan MF, Busam K, Coit DG. Merkel cell carcinoma: prognosis and treatment of patients from a single institution. J Clin Oncol. 2005;23(10):2300–9.

    Article  PubMed  Google Scholar 

  10. Lorigan P, Verweij J, Papai Z, Rodenhuis S, Le Cesne A, Leahy MG, et al. Phase III trial of two investigational schedules of ifosfamide compared with standard-dose doxorubicin in advanced or metastatic soft tissue sarcoma: a European Organisation for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group Study. J Clin Oncol. 2007;25(21):3144–50.

    Article  PubMed  CAS  Google Scholar 

  11. Frustaci S, Gherlinzoni F, De Paoli A, et al. Adjuvant chemotherapy for adult soft tissue sarcomas of the extremities and girdles: results of the Italian randomized cooperative trial. J Clin Oncol. 2001;19:1238–47.

    PubMed  CAS  Google Scholar 

  12. Pervaiz N, Colterjohn N, Farrokhyar F, Tozer R, Figueredo A, Ghert M. A systematic meta-analysis of randomized controlled trials of adjuvant chemotherapy for localized resectable soft-tissue sarcoma. Cancer. 2008;113(3):573–81.

    Article  PubMed  Google Scholar 

  13. Le Cesne A, Van Glabbeke M, Woll PJ, Bramwell VH, Casali PG, Hoekstra HJ, Reichardt P, Hogendoorn PC, Hohenberger P, Blay JY. The end of adjuvant chemotherapy (adCT) era with doxorubicin-based regimen in resected high-grade soft tissue sarcoma (STS): Pooled analysis of the two STBSG-EORTC phase III clinical trials. J Clin Oncol (ASCO Annual Meeting). 2008;20(15S):10525.

    Google Scholar 

  14. Shapira J, Gotfried M, Lishner M, Ravid M. Reduced cardiotoxicity of doxorubicin by a 6-hour infusion regimen: a prospective randomized evaluation. Cancer. 1990;65:870–3.

    Article  PubMed  CAS  Google Scholar 

  15. Judson I, Radford JA, Harris M, Blay JY, van Hoesel Q, le Cesne A, et al. Randomised phase II trial of pegylated liposomal doxorubicin (DOXIL/CAELYX) versus doxorubicin in the treatment of advanced or metastatic soft tissue sarcoma: a study by the EORTC Soft Tissue and Bone Sarcoma Group. Eur J Cancer. 2001;37(7):870–7.

    Article  PubMed  CAS  Google Scholar 

  16. Edmonson JH, Ryan LM, Blum RH, Brooks JS, Shiraki M, Frytak S, et al. Randomized comparison of doxorubicin alone versus ifosfamide plus doxorubicin or mitomycin, doxorubicin, and cisplatin against advanced soft tissue sarcomas. J Clin Oncol. 1993;11(7):1269–75.

    PubMed  CAS  Google Scholar 

  17. Garcia-Carbonero R, Supko JG, Manola J, et al. Phase II and pharmacokinetic study of ecteinascidin 743 in patients with progressive sarcomas of soft tissues refractory to chemotherapy. J Clin Oncol. 2004;22:1480–90.

    Article  PubMed  CAS  Google Scholar 

  18. Le Cesne A, Blay JY, Judson I, et al. Phase II study of ET-743 in advanced soft tissue sarcomas: a European Organisation for the Research and Treatment of Cancer (EORTC) Soft Tissue and Bone Sarcoma Group Trial. J Clin Oncol. 2005;23:576–84.

    Article  PubMed  Google Scholar 

  19. Yovine A, Riofrio M, Blay JY, et al. Phase II study of ecteinascidin-743 in advanced pretreated soft tissue sarcoma patients. J Clin Oncol. 2004;22:890–9.

    Article  PubMed  CAS  Google Scholar 

  20. Grosso F, Dileo P, Sanfilippo R, Stacchiotti S, Bertulli R, Piovesan C, et al. Steroid premedication markedly reduces liver and bone marrow toxicity of trabectedin in advanced sarcoma. Eur J Cancer. 2006;42(10):1484–90.

    Article  PubMed  CAS  Google Scholar 

  21. Maki RG, Wathen JK, Patel SR, Priebat DA, Okuno SH, Samuels B, et al. Randomized phase II study of gemcitabine and docetaxel compared with gemcitabine alone in patients with metastatic soft tissue sarcomas: results of sarcoma alliance for research through collaboration study 002. J Clin Oncol. 2007;25(19):2755–63.

    Article  PubMed  CAS  Google Scholar 

  22. Hensley ML, Maki R, Venkatraman E, Geller G, Lovegren M, Aghajanian C, et al. Gemcitabine and docetaxel in patients with unresectable leiomyosarcoma: results of a phase II trial. J Clin Oncol. 2002;20(12):2824–31.

    Article  PubMed  CAS  Google Scholar 

  23. Tai PT, Yu E, Winquist E, Hammond A, Stitt L, Tonita J, et al. Chemotherapy in neuroendocrine/Merkel cell carcinoma of the skin: case series and review of 204 cases. J Clin Oncol. 2000;18(12):2493–9.

    PubMed  CAS  Google Scholar 

  24. Poulsen M, Rischin D, Walpole E, Harvey J, Macintosh J, Ainslie J, et al. Analysis of toxicity of Merkel cell carcinoma of the skin treated with synchronous carboplatin/etoposide and radiation: a Trans-Tasman Radiation Oncology Group Study. Int J Radiat Oncol Biol Phys. 2001;51(1):156–63.

    Article  PubMed  CAS  Google Scholar 

  25. Pectasides D, Pectasides M, Psyrri A, Koumarianou A, Xiros N, Pectasides E, et al. Cisplatin-based chemotherapy for Merkel cell carcinoma of the skin. Cancer Invest. 2006;24(8):780–5.

    Article  PubMed  CAS  Google Scholar 

  26. Labropoulos SV, Razis ED. Imatinib in the treatment of dermatofibrosarcoma protuberans. Biologics. 2007;1(4):347–53.

    PubMed  CAS  Google Scholar 

  27. McArthur GA, Demetri GD, van Oosterom A, Heinrich MC, Debiec-Rychter M, Corless CL, et al. Molecular and clinical analysis of locally advanced dermatofibrosarcoma protuberans treated with imatinib: Imatinib Target Exploration Consortium Study B2225. J Clin Oncol. 2005;23(4):866–73.

    Article  PubMed  CAS  Google Scholar 

  28. Okuno S. Mammalian target of rapamycin inhibitors in sarcomas. Curr Opin Oncol. 2006;18:360–2.

    Article  PubMed  CAS  Google Scholar 

  29. Magenau JM, Schuetze SM. New targets for therapy of sarcoma. Curr Opin Oncol. 2008;20:400–6.

    Article  PubMed  CAS  Google Scholar 

  30. Campistol JM, Schena FP. Kaposi’s sarcoma in renal transplant recipients—the impact of proliferation signal inhibitors. Nephrol Dial Transplant. 2007;22 Suppl 1:i17–22.

    Article  PubMed  CAS  Google Scholar 

  31. Montaner S. Akt/TSC/mTOR activation by the KSHV G protein-coupled receptor: emerging insights into the molecular oncogenesis and treatment of Kaposi’s sarcoma. Cell Cycle. 2007;6:438–43.

    Article  PubMed  CAS  Google Scholar 

  32. Guenova E, Metzler G, Hoetzenecker W, Berneburg M, Rocken M. Classic Mediterranean Kaposi’s sarcoma regression with sirolimus treatment. Arch Dermatol. 2008;144:692–3.

    Article  PubMed  Google Scholar 

  33. Merimsky O, Jiveliouk I, Sagi-Eisenberg R. Targeting mTOR in HIV-negative classic Kaposi’s sarcoma. Sarcoma. 2008;2008:825093.

    Article  PubMed  Google Scholar 

  34. Chawla SP. Results of the phase III, placebo-controlled trial (SUCCEED) evaluating the mTOR inhibitor ridaforolimus (R) as maintenance therapy in advanced sarcoma patients (pts) following clinical benefit from prior standard cytotoxic chemotherapy (CT). J Clin Oncol (ASCO Annual Meeting). 2011;29(15 suppl):10005 (abstract).

    Google Scholar 

  35. Sleijfer S, Ray-Coquard I, Papai Z, Le Cesne A, Scurr M, Schöffski P, et al. Pazopanib, a multikinase angiogenesis inhibitor, in patients with relapsed or refractory advanced soft tissue sarcoma: a phase II study from the European Organisation for Research and Treatment of Cancer-soft Tissue and Bone Sarcoma Group (EORTC study 62043). J Clin Oncol. 2009;27(19):3126–32.

    Article  PubMed  CAS  Google Scholar 

  36. Wilhelm SM, Carter C, Tang L, et al. BAY43-9006 exhibits broad spectrum oral antitumor activity and targets the RAF/MEK/ERK pathway and receptor tyrosine kinases involved in tumor progression and angiogenesis. Cancer Res. 2004;64:7099–109.

    Article  PubMed  CAS  Google Scholar 

  37. Levy AP, Pauloski N, Braun D, et al. Analysis of transcription and protein expression changes in the 786-O human renal cell carcinoma tumor xenograft model in response to treatment with the multi-kinase inhibitor sorafenib (BAY 43–9006). Proc Am Assoc Cancer Res. 2006;47:213–4.

    Google Scholar 

  38. Maki RG, D’Adamo DR, Keohan ML, Saulle M, Schuetze SM, Undevia SD, et al. Phase II study of sorafenib in patients with metastatic or recurrent sarcomas. J Clin Oncol. 2009;27(19):3133–40.

    Article  PubMed  CAS  Google Scholar 

  39. Leahy M, Ray-Coquard I, Verweij J, Le Cesne A, Duffaud F, Hogendoorn PC, et al. Brostallicin, an agent with potential activity in metastatic soft tissue sarcoma: a phase II study from the European Organisation for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group. Eur J Cancer. 2007;43(2):308–15.

    Article  PubMed  CAS  Google Scholar 

  40. Krasagakis K, Fragiadaki I, Metaxari M, Krüger-Krasagakis S, Tzanakakis GN, Stathopoulos EN, et al. KIT receptor activation by autocrine and paracrine stem cell factor stimulates growth of Merkel cell carcinoma in vitro. J Cell Physiol. 2011;226(4):1099–109. doi:10.1002/jcp.22431.

    Article  PubMed  CAS  Google Scholar 

  41. Samlowski WE, Moon J, Tuthill RJ, Heinrich MC, Balzer-Haas NS, Merl SA, et al. A phase II trial of imatinib mesylate in Merkel cell carcinoma (neuroendocrine carcinoma of the skin): a Southwest Oncology Group Study (S0331). Am J Clin Oncol. 2010;33(5):495–9.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Medical Oncology, University Campus Bio-Medico, 200 Via Alvaro del Portillo, Rome, 00128, Italy

    Bruno Vincenzi, Anna Maria Frezza, Daniele Santini & Giuseppe Tonini

Authors
  1. Bruno Vincenzi
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Anna Maria Frezza
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Daniele Santini
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Giuseppe Tonini
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Giuseppe Tonini .

Editor information

Editors and Affiliations

  1. and Pharmaceutical Sciences and Technologies, Second University of Naples Department of Environmental, Biological, Naples, Italy

    Alfonso Baldi

  2. Department of Dermatology, Pius Hospital de Valls, Valls, Tarragona, Spain

    Paola Pasquali

  3. S.A.F.U. Department, Regina Elena Cancer Institute, Rome, Italy

    Enrico P Spugnini

Glossary

Adjuvant chemotherapy

Additional cancer treatment given after the primary treatment to lower the risk that the cancer will come back.

Neoadjuvant chemotherapy

Treatment given as a first step to shrink a tumour before the main treatment, which is usually surgery, is given.

Overall survival

The percentage of people in a study or treatment group who are alive for a certain period of time after they were diagnosed with or treated for a disease, such as cancer. The overall survival rate is often stated as a 5-year survival rate, which is the percentage of people in a study or treatment group who are alive 5 years after diagnosis or treatment.

Progression-free survival

The length of time during and after treatment in which a patient is living with a disease that does not get worse.

Soft tissues

Non-epithelial extraskeletal tissue, including muscle, fat and fibrous supporting structures, arising mainly from embryonic mesoderm, with some neuroectodermal contribution.

Rights and permissions

Reprints and permissions

Copyright information

© 2014 Springer Science+Business Media New York

About this chapter

Cite this chapter

Vincenzi, B., Frezza, A.M., Santini, D., Tonini, G. (2014). Systemic Therapy for Rare Tumours of the Skin and Soft Tissue Tumour. In: Baldi, A., Pasquali, P., Spugnini, E. (eds) Skin Cancer. Current Clinical Pathology. Humana Press, New York, NY. https://doi.org/10.1007/978-1-4614-7357-2_31

Download citation

  • DOI: https://doi.org/10.1007/978-1-4614-7357-2_31

  • Published:

  • Publisher Name: Humana Press, New York, NY

  • Print ISBN: 978-1-4614-7356-5

  • Online ISBN: 978-1-4614-7357-2

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation