Systemic treatment in advanced soft tissue sarcoma: what is standard, what is new

Abstract

For metastatic soft tissue sarcoma (STS) patients not eligible for surgery, systemic treatments, including standard chemotherapy and newer biological compounds, still play the most relevant role in the management of the disease. An anthracycline and alkylating agent combination has formed the cornerstone of chemotherapy in STS for more than 30 years, with its value over that of administration of anthracycline as a single agent still being debated. Efforts have been made to improve the activity and minimise the toxicity of the combination, as well as to explore the upfront efficacy of agents known to be active in sarcoma and to develop new biological compounds. Nevertheless, beyond the first line, evidence for medical treatment in STS is less robust and all the more driven by histology. Thus, the introduction of kinases and small molecule inhibitors in the treatment armamentarium for STS is a major achievement in this setting. Preliminary data on immunotherapy are also available and discussed in this review.

Call for improved design and reporting in soft tissue sarcoma studies: A systematic review and meta‐analysis of chemotherapy and survival outcomes in resectable STS

“…These unfavorable results coupled with a lack of robust evidence from randomized trials and known toxicity data suggest that chemotherapy in STS should be reserved for specific patients, such as those with particular histologic subtypes or poor operative candidates. Further evidence pertaining to particular populations that may derive benefit from systemic therapy is needed, especially as chemotherapeutic agents evolve and the role of immunotherapeutic adjuncts is being elucidated …”

“…With respect to the treatment, we recommend the uniform reporting of a proportion of subjects receiving RT or chemotherapy, RT modality, dose, fractionation, and sequence, chemotherapy agent(s), dose, number of cycles, sequence, and the use of combination immunotherapy. Such granular reporting becomes especially important when we consider the burgeoning role of immunotherapy in systemic therapy regimens for STS . Surgical pathology findings including lymph node positivity, lymphovascular invasion, and neurovascular invasion should also be reported where possible.…”

“…Further evidence pertaining to particular populations that may derive benefit from systemic therapy is needed, especially as chemotherapeutic agents evolve and the role of immunotherapeutic adjuncts is being elucidated. 27…”

“…Such granular reporting becomes especially important when we consider the burgeoning role of immunotherapy in systemic therapy regimens for STS. 27,36,37 Surgical pathology findings including lymph node positivity, lymphovascular invasion, and neurovascular invasion should also be reported where possible.…”

A Case of Unresectable Pulmonary Artery Intimal Sarcoma with Prolonged Survival by Chemotherapy

“…In addition, several cytotoxic agents were studied and reported to be useful for soft tissue sarcomas. Of these, paclitaxel has also been shown to be effective for soft tissue sarcoma [10, 11, 12]. We selected paclitaxel as the first-line chemotherapy in the present case because of her poor PS and increased risk of pulmonary hypertension due to preexisting severe pulmonary artery obstruction.…”