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Optimal Delivery of Follow-Up Care Following Treatment for Adults Treated for Ewing Sarcoma.

ABSTRACT: Ewing sarcoma (ES) is a rare, highly malignant sarcoma. It usually presents in the second decade of life; however, patients can be diagnosed as early as newborns and as late as in their seventies. ES is most frequently found in the long bones of the extremities and the pelvis. In older patients, ES can also arise in the soft tissues. Currently, there is no standard schedule for surveillance of adult patients with ES after their initial treatment for localised disease, not only for the early detection of recurrence but also for long-term side effects. Follow-up is based on group recommendations using extrapolated data obtained primarily from studies with paediatric patients. The main objective of this review is to summarise the data available on treatment-associated complications in long-term survivors. Furthermore, we provide a set of recommendations for optimising the follow-up of adults ES survivors, as well as for managing the sequelae that result from intensive multimodal treatment.

SUBMITTER: Digklia A

PROVIDER: S-EPMC10284160 | biostudies-literature | 2023

REPOSITORIES: biostudies-literature

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Optimal Delivery of Follow-Up Care Following Treatment for Adults Treated for Ewing Sarcoma.

Digklia Antonia A Dolcan Ana A Kucharczyk Monika A MA Jones Robin L RL Napolitano Andrea A

Cancer management and research 20230617

Ewing sarcoma (ES) is a rare, highly malignant sarcoma. It usually presents in the second decade of life; however, patients can be diagnosed as early as newborns and as late as in their seventies. ES is most frequently found in the long bones of the extremities and the pelvis. In older patients, ES can also arise in the soft tissues. Currently, there is no standard schedule for surveillance of adult patients with ES after their initial treatment for localised disease, not only for the early dete ...[more]

PMID: 37351338

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Project description:BackgroundAchondroplasia is a genetic condition that can cause complications across the lifespan. While complications in childhood are well documented, the natural history of achondroplasia in adults has, until recently, been relatively lacking, and little is known about the care they receive or how they access it. The European Achondroplasia Forum undertook two exploratory surveys, one for healthcare professionals (HCPs) and one for patient advocacy group (PAG) representatives, to gain an understanding of current practices of the transition process of individuals with achondroplasia from paediatric to adult services and how adults perceive their care.ResultsMost HCP respondents followed up more children than adults, and 8/15 responded that individuals did not transition to an adult multidisciplinary team (MDT) after paediatric care. Of 10 PAG respondents, none considered the experience of transition to adult services as good or very good and 50% considered it to be poor or very poor. A total of 64% (7/11) described the coordination of transition to adult services as "Not satisfactory" or "Poor". HCPs and PAG representatives largely agreed on the core specialists involved in adult care (orthopaedic surgeons, physiotherapists, rehabilitation specialists, rheumatologists, clinical geneticists). However, there was a discrepancy in the understanding of healthcare needs outside of this, with PAG representatives selecting neurosurgeons and genetic counsellors, while HCPs selected pulmonologists and obstetricians/gynaecologists. There was agreement between HCP and PAG respondents on the key barriers to effective care of adults with achondroplasia, with lack of an adult MDT, lack of interest from individuals in accessing care, and less experience in adult than paediatric MDTs ranking highly.ConclusionsThis study indicates that the care and follow up of adults with achondroplasia is challenging. Individuals are often lost to, or decline, follow up as they leave paediatric care, and it is largely unknown how, where, and why adults with achondroplasia access care later in life. Lifelong, multidisciplinary specialist care led by an identified physician should be accessible to all individuals with achondroplasia. It is important to ensure barriers to optimal care are addressed to enable access to appropriate care for all individuals with achondroplasia.

Project description:BackgroundThe clinical significance of GLI1 expression either through canonical Hedgehog signal transduction or through non-canonical mechanisms in rhabdomyosarcoma (RMS) or Ewing sarcoma (EWS) is incompletely understood. We tested a role for Hedgehog (HH) signal transduction and GL11 expression in development of vincristine (VCR) resistance in RMS and EWS.MethodsWe characterized baseline expression and activity of HH pathway components in 5 RMS (RD, Rh18, Ruch-2, Rh30, and Rh41) and 5 EWS (CHLA9, CHLA10, TC32, CHLA258, and TC71) cell lines. We then established VCR-resistant RMS and EWS cell lines by exposing cells to serially increasing concentrations of VCR and determining the IC50. We defined resistance as a ≥ 30-fold increase in IC50 compared with parental cells. We determined changes in gene expression in the VCR-resistant cells compared with parental cells using an 86-gene cancer drug resistance array that included GLI1 and tested the effect of GLI1 inhibition with GANT61 or GLI1 siRNA on VCR resistance.ResultsWe found evidence for HH pathway activity and GLI1 expression in RMS and EWS cell lines at baseline, and evidence that GLI1 contributes to survival and proliferation of these sarcoma cells. We were able to establish 4 VCR-resistant cell lines (Ruch-2VR, Rh30VR, Rh41VR, and TC71VR). GLI1 was significantly up-regulated in the Rh30VR, Rh41VR, and TC71VR cells. The only other gene in the drug resistance panel that was significantly up-regulated in each of these VCR-resistant cell lines compared with their corresponding parental cells was the GLI1 direct target and multidrug resistance gene, ATP-binding cassette sub-family B member 1 (MDR1). We established major vault protein (MVP), which was up-regulated in both vincristine-resistant alveolar RMS cell lines (Rh30VR and Rh41VR), as another direct target of GLI1 during development of drug resistance. Treatment of the VCR-resistant cell lines with the small molecule inhibitor GANT61 or GLI1 siRNA together with VCR significantly decreased cell viability at doses that did not reduce viability individually.ConclusionsThese experiments demonstrate that GLI1 up-regulation contributes to VCR resistance in RMS and EWS cell lines and suggest that targeting GLI1 may benefit patients with RMS or EWS by reducing multidrug resistance.

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Optimal Delivery of Follow-Up Care Following Treatment for Adults Treated for Ewing Sarcoma.

Publications

Optimal Delivery of Follow-Up Care Following Treatment for Adults Treated for Ewing Sarcoma.

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