Optimization of the Therapeutic Approach to Patients with Sarcoma: Delphi Consensus

Álvarez, Rosa; Jurado, Josefina Cruz; Soláns, Xavier; Giner, Javier Lavernia; Pousa, Antonio López; Martin‐Broto, Javier; Valverde, Claudia

doi:10.1155/2019/4351308

Cited by 4 publications

(4 citation statements)

References 70 publications

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“…There is also a need to revisit standards for adoption of new agents in other rare conditions with high unmet clinical need where a traditional clinical development pathway is unfeasible. Finally, consensus should be undertaken in other areas of sarcoma management where data and guidance are lacking 36,67,72 . Our survey of Canadian sarcoma community revealed an openness to consider use of targeted therapy in target‐matched population.…”

Section: Implementation and Future Workmentioning

confidence: 95%

“…Finally, consensus should be undertaken in other areas of sarcoma management where data and guidance are lacking. 36,67,72 Our survey of Canadian sarcoma community revealed an openness to consider use of targeted therapy in target-matched population. In the absence of widely-accepted guidelines, our consensus serves as an important step toward the adoption of a new systemic therapy treatment option for molecularly-selected subsets of relapsed/refractory sarcoma as well as a step toward new ways of thinking about clinical development pathways for novel targeted therapies in an era of precision medicine.…”

Section: Implementation and Future Workmentioning

confidence: 99%

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Canadian consensus on TRK‐inhibitor therapy for NTRK fusion‐positive sarcoma

Simmons

Deyell

MacNeill

et al. 2021

Intl Journal of Cancer

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Malignant sarcomas are rare accounting for <1% of all adult solid malignancies and approximately 11% to 13% of all pediatric malignancies. TRK‐inhibitors have demonstrated robust and long‐lasting responses in patients with NTRK fusion‐positive solid tumors, including sarcoma. Access to these agents in many jurisdictions such as Canada remains limited. We undertook a modified Delphi consensus to articulate and convey the clinical importance of these agents for the Canadian sarcoma community. A systematic search of published and presented literature was conducted to identify clinical trials reporting outcomes on the use of TRK‐inhibitors in relapsed/refractory NTRK fusion‐positive sarcoma. Three main consensus questions were identified: (a) is there currently an unmet clinical need for systemic therapy options in relapsed/refractory sarcoma? (b) do TRK‐inhibitors confer a clinical benefit to patients with NTRK fusion‐positive sarcoma? (c) do phase I/II basket trials provide sufficient evidence to justify funding of TRK‐inhibitors in NTRK fusion‐positive sarcoma? Response rates to the first and second surveys were 57% (n = 30) and 42% (n = 22), respectively. There was strong agreement among the Canadian sarcoma community that there was unmet clinical need for effective systemic therapy options in relapsed/refractory sarcoma, that TRK‐inhibitors are a safe and effective treatment option for patients with NTRK fusion‐positive sarcoma, and that available phase I/II basket trials provide sufficient evidence to support funding of these agents in relapsed/refractory NTRK fusion‐positive sarcoma. TRK‐inhibitors are a safe and effective systemic therapy option for patients with relapsed/refractory NTRK fusion‐positive sarcoma.

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Section: Implementation and Future Workmentioning

confidence: 95%

Section: Implementation and Future Workmentioning

confidence: 99%

Canadian consensus on TRK‐inhibitor therapy for NTRK fusion‐positive sarcoma

Simmons

Deyell

MacNeill

et al. 2021

Intl Journal of Cancer

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“…These tumors are characterized by high relapse rates, chemoresistance, and rapid progression, making them challenging to treat ( 1 ). Therefore, they generally warrant multidisciplinary intervention by surgeons, and medical and radiation oncologists ( 2 ).…”

Section: Introductionmentioning

confidence: 99%

Case Report: Adjuvant image-guided radiation therapy reduces surgical invasiveness in malignant peripheral nerve sheath tumors

Harikar

Ferini²,

Palmisciano

et al. 2023

Front. Oncol.

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IntroductionMalignant peripheral nerve sheath tumors (MPNSTs) are a group of rare soft tissue sarcomas of mesenchymal origin. These tumors generally require extensive local excision owing to their aggressive potential. Though the role of radiotherapy is controversial, in this report, we present the case of an MPNST in the forearm that was treated with microsurgery followed by image-guided radiation therapy to achieve complete tumor disappearance at the 18-month follow-up.Case reportA 69-year-old woman with underlying paranoid schizophrenia was referred to our department with pain, severe swelling, and ecchymosis of her right forearm. Physical examination showed hypoesthesia in the segments innervated by the median nerve and reduced motor strength of her right hand. A gadolinium-enhanced MRI showed a large malignant peripheral nerve sheath tumor (13 x 8 x 7 cm) of the median nerve in the forearm. She underwent microsurgical en-bloc tumor resection with sparing of the median nerve. Thirty-five days postoperatively, she underwent image-guided radiotherapy (IGRT) using volumetric modulated arc therapy (VMAT). Serial MRI scans of the forearm with Gadolinium and whole-body CT scan with contrast enhancement at 30 days, 6 months, 1 year, and 18 months postoperatively documented no tumor recurrence, remnants, or metastases.ConclusionsIn this report, we demonstrate the successful use of advanced radiotherapy techniques such as IGRT while avoiding demolitive surgery for MPNST. Though a longer follow-up is necessary, at the 18-month follow-up, the patient demonstrated good outcomes from surgical resection followed by adjuvant RT for MPNST in the forearm.

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“…Soft tissue sarcoma (STS) is a malignant tumour originating from mesenchymal tissue that has a very low incidence, accounting for only 1.0% of all adult malignant tumours, while the incidence of STS in children is slightly higher than that in adults. 1 – 3 There are numerous pathological types of STS and alveolar soft part sarcoma (ASPS) is a rare pathological type of STS, accounting for 1.0% of all STS. 4 , 5 ASPS was first reported and named in 1952; and the name was based on the fact that the tumour cells are arranged in a pseudoalveolar or organ-like manner.…”

Section: Introductionmentioning

confidence: 99%

Treatment of a case of primary alveolar soft part sarcoma of the lung: Case report and literature review

Zhao

et al. 2023

J Int Med Res

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Alveolar soft part sarcoma (ASPS) is an extremely rare type of soft tissue sarcoma. The primary sites of ASPS are mostly located in the extremities and trunk. Primary pulmonary ASPS is extremely rare. A search of the PubMed® database identified only five cases of primary pulmonary ASPS. This current case report describes the sixth case of ASPS in a 15-year-old male that presented with recurrent headaches. Head computed tomography showed space-occupying lesions in the left parietal lobe. Positron emission tomography–computed tomography confirmed the space-occupying lesions in the left parietal lobe and showed multiple nodules and masses in the two lungs and pleura, which were considered to be low-grade malignant mesenchymal tumours. The case report presents the clinical characteristics, diagnosis and treatment process. Programmed cell death protein 1 monoclonal antibody (sintilimab) combined with a tyrosine kinase inhibitor (anlotinib hydrochloride) achieved a good therapeutic effect, indicating that this combination therapy is worth exploring further. Large-scale prospective studies are needed to explore and develop standardized treatments for ASPS.

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Optimization of the Therapeutic Approach to Patients with Sarcoma: Delphi Consensus

Cited by 4 publications

References 70 publications

Canadian consensus on TRK‐inhibitor therapy for NTRK fusion‐positive sarcoma

Canadian consensus on TRK‐inhibitor therapy for NTRK fusion‐positive sarcoma

Case Report: Adjuvant image-guided radiation therapy reduces surgical invasiveness in malignant peripheral nerve sheath tumors

Treatment of a case of primary alveolar soft part sarcoma of the lung: Case report and literature review

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