SELNET clinical practice guidelines for soft tissue sarcoma and GIST

Blay, Jean‐Yves; Hindi, Nadia; Bollard, Julien; Aguiar, Samuel; Ángel, Martín; Araya, Birhane Desta; Badilla, R.; Bernabeu, Daniel; Campos, Fernando Augusto Batista; Caro-Sánchez, Claudia Haydeé Saraí; Carvajal, B.; Montoya, A. Carvajal; Casavilca‐Zambrano, Sandro; Castro, Victor; Chacón, Matías; Clara, M.; Collini, Paola; Genoroso, R. Correa; Costa, Felipe D’Almeida; Cuellar, M.; Tos, Angelo Paolo Dei; Malagon, H. R. Dominguez; Donati, Davide María; Dufresne, Armelle; Eriksson, Mikael; Farias-Loza, M; Fernández, Piga; Frezza, Anna Maria; Frisoni, Tommaso; García-Ortega, Dorian Yarih; Gelderblom, Hans; Gouin, F.; Gómez-Mateo, M. Carmen; Gronchi, Alessandro; Haro, Juan C; Huanca, Lourdes; Jiménez, N. V.; Karanian, Marie; Kasper, Bernd; David, Bruna Bianca Lopes; López‐Pousa, Antonio; Lutter, Georg; Saíd, Héctor Martínez; Martínez-Tláhuel, Jorge Luis; Mello, Celso Abdon Lopes de; Pérez, José Garcı́a; Moura, David S.; Nascimento, Antonio G.; Ortiz‐Cruz, Eduardo J.; Palmerini, Emanuela; Patel, Shreyaskumar; Pflüger, Yanina; Provenzano, Salvatore; Righi, Alberto; Rodríguez, Angélica Rodríguez; Salas, R.; Santos, TO; Scotlandi, Katia; Soule, Tomas; Stacchiotti, Silvia; Valverde, Claudia; Waisberg, Federico; Estrada, E. Zamora; Martin‐Broto, Javier

doi:10.1016/j.ctrv.2021.102312

Cited by 43 publications

(23 citation statements)

References 133 publications

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“…The other factor that may have impacted our RR is the predominance of leiomyosarcoma in the cohort (36%). Since beginning of last decade, evidence emerged showing that this subtype of sarcoma has limited sensitivity to ifosfamide combinations [ 23 ], and based on more recent data [ 24 ] the current guidelines consider ifosfamide to be less preferential in the treatment of patients with metastatic non-uterine leiomyosarcoma [ 3 , 25 , 26 ]. Data shown in our study reinforce this, as seen by the higher progression disease rate in the leiomyosarcoma group when compared to the other sarcoma histologies, although not statistically significant (58.3% × 28.6%, p = 0.14), with a median PFS of 1 month versus 4 months in the others ( p = 0.3).…”

Section: Discussionmentioning

confidence: 99%

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Combination of ifosfamide and etoposide as a salvage regimen for previously treated soft tissue sarcomas: a retrospective single centre study

Ribeiro¹,

Campos²,

Mello³

2022

ecancer

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Introduction: Systemic treatment for metastatic soft tissue sarcoma (STS) results in modest activity in second and further lines. The aim of this study was to evaluate the efficacy of ifosfamide and etoposide (IE) as a salvage regimen for patients with metastatic STS. Methods:A retrospective, single centre study included patients with STS treated with IE from 2010 to 2018. The primary endpoint was progression-free survival (PFS). Secondary endpoints were toxicity, response rate (RR) and overall survival (OS). Survival was estimated by the Kaplan-Meier method and log-rank test used to compare the groups.Results: A total of 33 patients were identified, median age was 43 years, 60% were female, 12 had leiomyosarcoma. IE was used in second line in 51.5% and in >third line in 30.3% of patients. Median number of cycles was four and treatment discontinuation due to grade 3/4 toxicity occurred in 30.3%. The objective RR was 9% and the disease control rate was 60.6%. Median PFS was 4 months (95% CI, 2.1-5.9) and the median OS was 15 months (95% CI, 7.1-22.9). In the univariate analysis, smoking history, line of therapy and prior response to previous chemotherapy were prognostic factors for PFS. Conclusion:IE showed activity in previously treated STS, but with a non-negligible toxicity profile, worse than that with other available therapies. The use of the IE combination is not supported by our findings outside a clinical trial for soft part sarcomas.

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Section: Discussionmentioning

confidence: 99%

“…One important action is to debate the best options for the patients and make formal statement such as the guidelines for treatment of sarcoma. The recently published Sarcoma European-Latin American Network (SELNET) guideline [ 26 ] was reviewed and validated by members from European and Latin American countries.…”

Section: Discussionmentioning

confidence: 99%

Combination of ifosfamide and etoposide as a salvage regimen for previously treated soft tissue sarcomas: a retrospective single centre study

Ribeiro¹,

Campos²,

Mello³

2022

ecancer

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“…The risk is determined considering the size of the tumor, mitotic index and location, in addition, the tumor rupture must be assessed in the pathology studies to stratify the risk (Table 1). 2,3 Surgery is an opportune treatment in small and accessible lesions, with resection of the lesion without lymph node dissection in case of negative nodes. Even gastric lesions smaller than 2 cm may benefit from follow-up.…”

Section: Discussionmentioning

confidence: 99%

Gastrointestinal stromal tumor as cause of acute abdominal pain in a patient with neurofibromatosis type 1: case report and literature review

Blaz-Zavala

Villa-Santiago²,

Flores-García³

et al. 2022

Int J Res Med Sci

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Acute abdomen is a common and sometimes dramatic clinical condition, which can be fatal if diagnosis is not made in time. There are many etiologies for acute abdominal pain; therefore, the diagnostic approach should be based on clinical assessment, including laboratory and image studies. Neurofibromatosis 1 (NF1) is an autosomal dominant condition, characterized by cutaneous pigmentation and tumor formation along nerves in the brain, skin and other organs, the gastrointestinal stromal tumors are rare mesenchymal neoplasms associated with NF1. The close correlation between both pathologies is well known, and the clinical relevance relies on the different pathogenesis from sporadic gastrointestinal stromal tumor (GIST), with important therapeutic implications as the use of imatinib prior or after surgery, regarding the individual context of the patient. This case report illustrates the management of an NF1 patient presenting with acute abdomen to the emergency room and follow-up.

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“…Indeed, different histologic types have different presentations, behaviors, and outcomes, with 50% being fully malignant and often metastatic and 50% being locally aggressive with some but limited metastatic potential [ 2 ]. According to European Society for Medical Oncology (ESMO) and National Comprehensive Cancer Network (NCCN) guidelines, chemotherapy is the cornerstone of traditional treatment for advanced and metastatic sarcoma [ 3 – 6 ]. However, even front-line chemotherapy is associated with limited objective response rates (ORRs, averaging ~ 18%) [ 7 , 8 ].…”

Section: Introductionmentioning

confidence: 99%

The immune subtypes and landscape of sarcomas

Weng

et al. 2022

BMC Immunol

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Background Considering the molecular heterogeneity of sarcomas and their immunologically quiet character, immunotherapy (e.g., immune checkpoint inhibitors) plays a viable role in only a subset of these tumors. This study aimed to determine the immune subtypes (IMSs) of sarcomas for selecting suitable patients from an extremely heterogeneous population. Results By performing consensus clustering analysis of the gene expression profiles of 538 patients with sarcomas in online databases, we stratified sarcomas into three IMSs characterized by different immune cell features, tumor mutational burdens (TMBs), gene mutations, and clinical outcomes. IMS1 showed an immune “hot” and immunosuppressive phenotype, the highest frequencies of CSMD3 mutation but the lowest frequencies of HMCN1 and LAMA2 mutations; these patients had the worst progression-free survival (PFS). IMS2 was defined by a high TMB and more gene mutations, but had the lowest frequency of MND1 mutations. IMS3 displayed the highest MDN1 expression level and an immune “cold” phenotype, these patients had the worst PFS. Each subtype was associated with different expression levels of immunogenic cell death modulators and immune checkpoints. Moreover, we applied graph learning-based dimensionality reduction to the immune landscape and identified significant intra-cluster heterogeneity within each IMS. Finally, we developed and validated an immune gene signature with good prognostic performance. Conclusions Our results provide a conceptual framework for understanding the immunological heterogeneity of sarcomas. The identification of immune-related subtypes may facilitate optimal selection of sarcoma patients who will respond to appropriate therapeutic strategies.

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SELNET clinical practice guidelines for soft tissue sarcoma and GIST

Cited by 43 publications

References 133 publications

Combination of ifosfamide and etoposide as a salvage regimen for previously treated soft tissue sarcomas: a retrospective single centre study

Combination of ifosfamide and etoposide as a salvage regimen for previously treated soft tissue sarcomas: a retrospective single centre study

Gastrointestinal stromal tumor as cause of acute abdominal pain in a patient with neurofibromatosis type 1: case report and literature review

The immune subtypes and landscape of sarcomas

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