Managing sarcoma: where have we come from and where are we going?

Abstract

Sarcomas are a heterogeneous group of neoplasms of mesenchymal origin. Approximately 80% arise from soft tissue and 20% originate from bone. To date more than 100 sarcoma subtypes have been identified and they vary in molecular characteristics, pathology, clinical presentation and response to treatment. While sarcomas represent <1% of adult cancers, they account for approximately 21% of paediatric malignancies and thus pose some of the greatest risks of mortality and morbidity in children and young adults. Metastases occur in one-third of all patients and approximately 10–20% of sarcomas recur locally. Surgery in combination with preoperative and postoperative therapies is the primary treatment for localized sarcoma tumours and is the most promising curative possibility. Metastasized sarcomas, on the other hand, are treated primarily with single-agent or combination chemotherapy, but this rarely leads to a complete and robust response and often becomes a palliative form of treatment. The heterogeneity of sarcomas results in variable responses to current generalized treatment strategies. In light of this and the lack of curative strategies for metastatic and unresectable sarcomas, there is a need for novel subtype-specific treatment strategies. With the more recent understanding of the molecular mechanisms underlying the pathogenesis of some of these tumours, the treatment of sarcoma subtypes with targeted therapies is a rapidly evolving field. This review discusses the current management of sarcomas as well as promising new therapies that are currently underway in clinical trials.

Treatment Patterns and Healthcare Resource Utilization among Patients with Advanced or Metastatic Soft Tissue Sarcoma in US Community Practices

“…Similar to what was reported by [11]. e observed diversity in treatment approaches observed in STS is likely due to both underlying differences in disease characteristics and lack of predominant guideline regimen recommendations [1,19,20]. Future studies should consider the impact of this treatment heterogeneity on clinical outcomes and how treatment algorithms may aid in provider decision-making.…”

Real-Time Molecular Diagnosis of Tumors Using Water-Assisted Laser Desorption/Ionization Mass Spectrometry Technology

“…Sarcoma is a uniquely complex cancer type because it has more than 50 histological subtypes and is thus probably the most diverse and heterogeneous of all cancers (Bleloch et al, 2017;Skubitz et al, 2008;Stiller et al, 2013). We developed a classification model for a collection of canine sarcomas using lipidomic molecular signatures collected by the MS analysis of histologically annotated biopsy specimens (Table S1).…”

Disruption of mammalian SWI/SNF and polycomb complexes in human sarcomas: mechanisms and therapeutic opportunities

“…Sarcoma subtypes possess diverse genetic profiles ranging from a single pathognomonic genetic event, such as chromosomal translocations in synovial sarcoma and Ewing sarcoma, to extensive mutational burden and complex genomes [1][2][3][4][5][6][7]. Sarcomas arise in all age groups, but are disproportionally present in children, accounting for a significant number of cancer-related mortalities in both pediatric and young adult patients [8]. In the absence of molecularly targeted therapies, treatment of sarcomas most often relies on cytotoxic chemotherapy in combination with radiation and surgery [9][10][11].…”