Unmet Medical Needs and Future Perspectives for Leiomyosarcoma Patients—A Position Paper from the National LeioMyoSarcoma Foundation (NLMSF) and Sarcoma Patients EuroNet (SPAEN)

Kasper, Bernd; Achee, Annie; Schuster, Kathrin; Wilson, Roger; Oortmerssen, G. van; Gladdy, Rebecca A.; Hemming, Matthew L.; Huang, Paul H.; Ingham, Matthew; Jones, Robin L.; Pollack, Seth M.; Reinke, Denise K.; Sanfilippo, Roberta; Schuetze, Scott M.; Somaiah, Neeta; Tine, Brian A. Van; Wilky, Breelyn A.; Okuno, Scott H.; Trent, Jonathan C.

doi:10.3390/cancers13040886

Cited by 19 publications

(8 citation statements)

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“…Additionally, these results can be used to determine research priorities for specific groups or subtypes of sarcomas, which has already been done for leiomyosarcoma based on the results from this PSP. 27 The topics for advocacy that this PSP revealed can be used by patient advocacy organizations in the representation of sarcoma patients and carers. Patient advocacy organizations and health care professionals can use the requests for information that came forth to improve on information provision and materials to patients and carers.…”

Section: Discussionmentioning

confidence: 99%

Setting the international research agenda for sarcoma together with patients and carers: first results of the Sarcoma Patient EuroNet (SPAEN) priority setting partnership

Husson

Drabbe

Schuster³

et al. 2022

ESMO Open

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Section: Discussionmentioning

confidence: 99%

Setting the international research agenda for sarcoma together with patients and carers: first results of the Sarcoma Patient EuroNet (SPAEN) priority setting partnership

Husson

Drabbe

Schuster³

et al. 2022

ESMO Open

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“…Leiomyosarcoma (LMS) is one of the most common soft tissue sarcoma (STS) subtypes, accounting for up to 20% of STS diagnoses 1 . LMS are tumours of smooth muscle origin, most often developing in the extremities, retroperitoneum and uterus.…”

Section: Subtype Proportion (%) Clinical Features Biological Features...mentioning

confidence: 99%

Molecular subtypes of leiomyosarcoma: Moving toward a consensus

Burns

Jones

Huang

2022

Clinical and Translational Dis

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Leiomyosarcoma (LMS) is one of the most common soft tissue sarcoma (STS) subtypes, accounting for up to 20% of STS diagnoses. 1 LMS are tumours of smooth muscle origin, most often developing in the extremities, retroperitoneum and uterus. The 5-year recurrence rate of LMS varies from 10% to 43% dependent on anatomical site, and long-term patient follow-up shows that late recurrences (>10 years) can occur in extremity, abdominal and retroperitoneal patients. 2 In addition, only a subset of LMS patients respond to conventional chemotherapy and radiotherapy. Due to advancements in gene expression profiling technologies, our understanding of the molecular basis of LMS has improved over the past decade and supports the concept of LMS molecular subtypes to explain some of the extensive clinical heterogeneity observed across patients.In 2009, molecular subtypes of LMS were first documented by Beck et al., through gene expression microarray profiling in a cohort of 51 samples. 3 Following on from this pivotal report, several additional studies utilising transcriptomics have consistently identified 3 different molecular subtypes. [4][5][6][7][8] The different features of the molecular subtypes reported in each of these studies are summarised in Table 1. Whilst the relationship between the This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

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“…In another recently updated PD-1 inhibitor trial from National Cancer Institute (NCT02500797), nivolumab is used with and without ipilimumab (targeting CTLA-4); nivolumab prevented disease progression in 5/11 (46%) patients with OS during the first six weeks after treatment initiation but only endured in one patient (9%) after eight weeks of treatment, with only two patients expressing PD-L1 (1-3%) [ 35 ]. Ipilimumab showed disease control (DC) in 25% of OS patients, yet at a higher dose than the maximally tolerated dose, and caused grade III colitis in one of two patients with stable disease (SD) beyond the six weeks treatment [ 22 , 31 , 36 ].…”

Section: Reviewmentioning

confidence: 99%

A Deep Dive Into the Newest Avenues of Immunotherapy for Pediatric Osteosarcoma: A Systematic Review

Suri¹,

Soni²,

Okpaleke³

et al. 2021

Cureus

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Osteosarcoma (OS) is the most common primary bone cancer affecting children and young adults, most often occurring at the metaphysis of long bones. At present, treatment with combinations of surgery and chemotherapy for the localized OS has only brought minuscule improvements in prognosis. In comparison, the advanced, metastatic, or recurrent forms of OS are often non-responsive to chemotherapy, adding to the dire need to develop new and efficient therapies.The question of interest investigated in this systematic review is whether immunotherapy can play a meaningful role in improving the clinical outcomes of children with OS. This article aims to summarize the preclinical and clinical research conducted thus far on potential therapeutic avenues for pediatric OS using immunotherapy, including methods like checkpoint inhibition, adoptive cellular therapy with T-cells, chimeric antigen receptor T (CAR-T), and natural killer (NK) cells. It also highlights the influence of the innate and adaptive immune system on the tumor microenvironment, allowing for OS progression and metastasis.This systematic review contains 27 articles and analyses of multiple clinical trials employing immunotherapeutic drugs to 785 osteosarcoma participants and over 243 pediatric patients. The articles were obtained through PubMed, PubMed Central, and ClinicalTrials.gov and individually assessed for quality using the Assessment of Multiple Systematic Reviews (AMSTAR) checklist and the Cochrane risk-of-bias tool. The reviews reveal that immunotherapy's most significant impact on pediatric OS includes combining immune checkpoint blockers with traditional chemotherapy and surgery. However, due to the bimodal distribution of this aggressive malignancy, these studies cannot precisely estimate the overall effect and any potential life-threatening adverse events following therapy in children. Further research is required to fully assess the impact of these immunotherapies, including more extensive multinational clinical trials to focus on the pediatric population.

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Unmet Medical Needs and Future Perspectives for Leiomyosarcoma Patients—A Position Paper from the National LeioMyoSarcoma Foundation (NLMSF) and Sarcoma Patients EuroNet (SPAEN)

Cited by 19 publications

References 88 publications

Setting the international research agenda for sarcoma together with patients and carers: first results of the Sarcoma Patient EuroNet (SPAEN) priority setting partnership

Setting the international research agenda for sarcoma together with patients and carers: first results of the Sarcoma Patient EuroNet (SPAEN) priority setting partnership

Molecular subtypes of leiomyosarcoma: Moving toward a consensus

A Deep Dive Into the Newest Avenues of Immunotherapy for Pediatric Osteosarcoma: A Systematic Review

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