Datasets for reporting of soft‐tissue sarcoma: recommendations from the International Collaboration on Cancer Reporting (<scp>ICCR</scp>)

Tos, Angelo Paolo Dei; Webster, Fleur; Agaimy, Abbas; Bovée, Judith V.M.G.; Dickson, Brendan C.; Doyle, Leona; Dry, Sarah M.; Gronchi, Alessandro; Hameed, Meera; Hemmings, Chris; Liegl‐Atzwanger, Bernadette; Thway, Khin; Wagner, Andrew J.; Wang, Jian; Yoshida, Akihiko; Fletcher, Christopher D.�M.

doi:10.1111/his.14862

Cited by 4 publications

(2 citation statements)

References 14 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Management of localized osteosarcoma and Ewing sarcoma requires the integration of multi-agent chemotherapy along with surgery and radiotherapy. Taken together, a multidisciplinary approach remains the most crucial element in optimizing the clinical outcomes of patients with sarcoma [1,2,5].…”

Section: Introductionmentioning

confidence: 99%

Germline Genetic Mutations in Adult Patients with Sarcoma: Insight into the Middle East Genetic Landscape

Abu-Hijlih,

Sharaf,

Salah

et al. 2024

Cancers

View full text Add to dashboard Cite

Data on germline mutations in soft tissue and bone sarcomas are scarce. We sought to identify the prevalence of germline mutations in adult sarcoma patients treated at a tertiary cancer center. Newly diagnosed patients were offered germline genetic testing via an 84-gene panel. The prevalence of pathogenic germline variants (PGVs) and their association with disease-, and patient- related factors are reported. A total of 87 patients were enrolled, the median age was 48 (19–78) years, and 47 (54%) were females. Gastrointestinal stromal tumors (n = 12, 13.8%), liposarcoma (n = 10, 11.5%), and Ewing sarcoma (n = 10, 11.5%) were the main subtypes. A total of 20 PGVs were detected in 18 (20.7%) patients. Variants of uncertain significance, in the absence of PGVs, were detected in 40 (45.9%) patients. Young age (p = 0.031), presence of a second primary cancer (p = 0.019), and female gender (p = 0.042) were correlated with the presence of PGVs. All identified PGVs have potential clinical actionability and cascade testing, and eight (44.44%) suggested eligibility for a targeted therapy. Almost one in five adult patients with soft tissue and bone sarcomas harbor pathogenic or likely pathogenic variants. Many of these variants are potentially actionable, and almost all have implications on cancer screening and family counselling. In this cohort from the Middle East, younger age, presence of a second primary tumor, and female gender were significantly associated with higher PGVs rates. Larger studies able to correlate treatment outcomes with genetic variants are highly needed.

show abstract

Section: Introductionmentioning

confidence: 99%

Germline Genetic Mutations in Adult Patients with Sarcoma: Insight into the Middle East Genetic Landscape

Abu-Hijlih,

Sharaf,

Salah

et al. 2024

Cancers

View full text Add to dashboard Cite

show abstract

“…Soft tissue sarcomas (STSs) are rare malignancies, accounting for only approximately 1% of all malignancies ( Bhatt et al, 2016 ; Yang et al, 2019 ). There are more than 70 histological subtypes, and the clinical characteristics and prognoses of these subtypes greatly vary ( Amadeo et al, 2020 ; Parikh et al, 2018 ; von Mehren et al, 2022 ; Tos et al, 2023 ). Despite the low incidence of STSs, hundreds of thousands of new STS cases are diagnosed annually worldwide, and approximately half of them eventually progress to advanced stages ( Corey et al, 2014 ; Bhatt et al, 2016 ; Hung et al, 2019 ; Yang et al, 2019 ).…”

Section: Introductionmentioning

confidence: 99%

Chemotherapeutic drugs for soft tissue sarcomas: a review

Tian

Yao

2023

Front. Pharmacol.

View full text Add to dashboard Cite

Despite the low incidence of soft tissue sarcomas (STSs), hundreds of thousands of new STS cases are diagnosed annually worldwide, and approximately half of them eventually progress to advanced stages. Currently, chemotherapy is the first-line treatment for advanced STSs. There are difficulties in selecting appropriate drugs for multiline chemotherapy, or for combination treatment of different STS histological subtypes. In this study, we first comprehensively reviewed the efficacy of various chemotherapeutic drugs in the treatment of STSs, and then described the current status of sensitive drugs for different STS subtypes. anthracyclines are the most important systemic treatment for advanced STSs. Ifosfamide, trabectedin, gemcitabine, taxanes, dacarbazine, and eribulin exhibit certain activities in STSs. Vinca alkaloid agents (vindesine, vinblastine, vinorelbine, vincristine) have important therapeutic effects in specific STS subtypes, such as rhabdomyosarcoma and Ewing sarcoma family tumors, whereas their activity in other subtypes is weak. Other chemotherapeutic drugs (methotrexate, cisplatin, etoposide, pemetrexed) have weak efficacy in STSs and are rarely used. It is necessary to select specific second- or above-line chemotherapeutic drugs depending on the histological subtype. This review aims to provide a reference for the selection of chemotherapeutic drugs for multi-line therapy for patients with advanced STSs who have an increasingly long survival.

show abstract