Maintenance therapy and drug holiday in sarcoma patients: systematic review

Acta Oncol. 2020 Sep;59(9):1084-1090. doi: 10.1080/0284186X.2020.1759825. Epub 2020 May 13.


Purpose: Overall prognosis of advanced sarcoma remains poor, optimization of systemic treatment is urgently needed in this setting.Materials and methods: We systematically reviewed fully published English-speaking literature about maintenance therapy and drug holiday in sarcoma patients management.Results: We found that switch maintenance therapy with cyclophosphamide/vinorelbine improves the outcome of localized high-risk rhabdomyosarcoma. There is no other maintenance therapy recommended in sarcoma patients. After classical chemotherapy, maintenance therapy with immune-stimulating agents for localized osteosarcoma, bevacizumab for advanced angiosarcoma or pediatric advanced sarcoma, or mTOR inhibitors for metastatic sarcoma does not improve the outcome. Drug holiday has been assessed for metastatic gastrointestinal stromal tumor treated with imatinib as the first-line therapy or for metastatic soft-tissue sarcoma treated with trabectedin. Drug holiday has been found to lead to rapid disease progression and should be avoided.Conclusions: Data about both maintenance and drug holiday are spare in sarcoma management.

Publication types

  • Systematic Review

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / administration & dosage*
  • Antineoplastic Combined Chemotherapy Protocols / adverse effects
  • Bevacizumab / administration & dosage
  • Bevacizumab / adverse effects
  • Cyclophosphamide / administration & dosage
  • Cyclophosphamide / adverse effects
  • Disease-Free Survival
  • Drug Administration Schedule
  • Drug Substitution
  • Hemangiosarcoma / diagnosis
  • Hemangiosarcoma / drug therapy*
  • Hemangiosarcoma / mortality
  • Humans
  • Maintenance Chemotherapy / adverse effects
  • Maintenance Chemotherapy / methods*
  • Osteosarcoma / diagnosis
  • Osteosarcoma / drug therapy*
  • Osteosarcoma / mortality
  • Prognosis
  • Rhabdomyosarcoma / diagnosis
  • Rhabdomyosarcoma / drug therapy*
  • Rhabdomyosarcoma / mortality
  • Survival Rate
  • Trabectedin / administration & dosage
  • Trabectedin / adverse effects
  • Vinorelbine / administration & dosage
  • Vinorelbine / adverse effects


  • Bevacizumab
  • Cyclophosphamide
  • Trabectedin
  • Vinorelbine