Economic burden and health‑related quality of life in tenosynovial giant‑cell tumour patients in Europe: an observational disease registry

Background: Tenosynovial Giant-Cell Tumour (TGCT) is a benign clonal neoplastic proliferation arising from the syn‑ ovium, causing a variety of symptoms and often requiring repetitive surgery. This study aims to defne the economic burden—from a societal perspective—associated with TGCT patients and their health-related quality of life (HRQOL) in six European countries. Methods: This article analyses data from a multinational, multicentre, prospective observational registry, the TGCT Observational Platform Project (TOPP), involving hospitals and tertiary sarcoma centres from six European countries (Austria, France, Germany, Italy, the Netherlands, and Spain). It includes information on TGCT patients’ health-related quality of life and healthcare and non-healthcare resources used at baseline (the 12-month period prior to the patients entering the registry) and after 12 months of follow-up. Results: 146 TGCT patients enrolled for the study, of which 137 fulflled the inclusion criteria. Their mean age was 44.5 years, and 62% were female. The annual average total costs associated with TGCT were €4866 at baseline and €5160 at the 12-month follow-up visit. The annual average healthcare costs associated with TGCT were €4620 at baseline, of which 67% and 18% corresponded to surgery and medical visits, respectively. At the 12-month follow up, the mean healthcare costs amounted to €5094, with surgery representing 70% of total costs. Loss of productivity represented, on average, 5% of the total cost at baseline and 1.3% at follow-up. The most-afected HRQOL dimensions, measured with the EQ-5D-5L instrument, were pain or discomfort, mobility, and the performance of usual activities, both at baseline and at the follow-up visit. Regarding HRQOL, patients declared a mean index score of 0.75 at baseline and 0.76 at the 12-month follow-up. Conclusion: The results suggest that TGCT places a heavy burden on its suferers, which increases after one year of follow-up, mainly due to the healthcare resources required—in particular, surgical procedures. As a result, this condi‑ tion has a high economic impact on healthcare budgets, while the HRQOL of TGCT patients substantially deteriorates over time

Development and external validation of a dynamic prognostic nomogram for primary extremity soft tissue sarcoma survivors.

Background:Prognostic nomograms for patients with extremity soft tissue sarcoma (eSTS) typically predict survival or the occurrence of local recurrence or distant metastasis at time of surgery. Our aim was to develop and externally validate a dynamic prognostic nomogram for overall survival in eSTS survivors for use during follow-up. Methods:All primary eSTS patients operated with curative intent between 1994 and 2013 at three European and one Canadian sarcoma centers formed the development cohort. Patients with Fédération Française des Centres de Lutte Contre le Cancer (FNCLCC) grade II and grade III eSTS operated between 2000 and 2016 at seven other European reference centers formed the external validation cohort. We used a landmark analysis approach and a multivariable Cox model to create a dynamic nomogram; the prediction window was fixed at five years. A backward procedure based on the Akaike Information Criterion was adopted for variable selection. We tested the nomogram performance in terms of calibration and discrimination. Findings:The development and validation cohorts included 3740 and 893 patients, respectively. The variables selected applying the backward procedure were patient's age, tumor size and its interaction with landmark time, tumor FNCLCC grade and its interaction with landmark time, histology, and both local recurrence and distant metastasis (as first event) indicator variables. The nomogram showed good calibration and discrimination. Harrell C indexes at different landmark times were between 0.776 (0.761-0.790) and 0.845 (0.823-0.862) in the development series and between 0.675 (0.643-0.704) and 0.810 (0.775-0.844) in the validation series. Interpretation:A new dynamic nomogram is available to predict 5-year overall survival at different times during the first three years of follow-up in patients operated for primary eSTS. This nomogram allows physicians to update the individual survival prediction during follow-up on the basis of baseline variables, time elapsed from surgery and first-event history

Gastrointestinal stromal tumours: ESMO-EURACAN-GENTURIS Clinical Practice Guidelines for diagnosis, treatment and follow-up

Gastrointestinal stromal tumours (GISTs) are malignant mesenchymal tumours with a variable clinical behaviour, marked by differentiation towards the interstitial cells of Cajal. GISTs belong to the family of soft tissue sarcomas (STSs) but are treated separately due to their peculiar histogenesis, clinical behaviour and specific therapy. This European Society for Medical Oncology (ESMO)–European Reference Network for Rare Adult Solid Cancers (EURACAN)–European Reference Network for Genetic Tumour Risk Syndromes (GENTURIS) Clinical Practice Guideline (CPG) will cover GISTs while other STSs are covered in the ESMO–EURACAN–European Reference Network for Paediatric Oncology (ERN PaedCan)–GENTURIS STS CPG

Bone sarcomas: ESMO-EURACAN-GENTURIS-ERN PaedCan Clinical Practice Guideline for diagnosis, treatment and follow-up

This Clinical Practice Guideline provides key recommendations on the management of bone sarcomas. // Recommendations have been agreed following a consensus meeting of representatives from ESMO, EURACAN, GENTURIS and ERNPaedCan. // Authorship includes a multidisciplinary group of experts from different institutions and countries worldwide

Age-related differences of oncological outcomes in primary extremity soft tissue sarcoma: a multistate model including 6260 patients.

PURPOSE: No studies extensively compared the young adults (YA, 18-39 years), middle-aged (40-69 years), and elderly (≥70 years) population with primary high-grade extremity soft tissue sarcoma (eSTS). This study aimed to determine whether the known effect of age on overall survival (OS) and disease progression can be explained by differences in tumour characteristics and treatment protocol among the YA, middle-aged and elderly population in patients with primary high-grade eSTS treated with curative intent. METHODS: In this retrospective multicentre study, inclusion criteria were patients with primary high-grade eSTS of 18 years and older, surgically treated with curative intent between 2000 and 2016. Cox proportional hazard models and a multistate model were used to determine the association of age on OS and disease progression. RESULTS: A total of 6260 patients were included in this study. YA presented more often after 'whoops'-surgery or for reresection due to residual disease, and with more deep-seated tumours. Elderly patients presented more often with grade III and larger (≥10 cm) tumours. After adjustment for the imbalance in tumour and treatment characteristics the hazard ratio for OS of the middle-aged population is 1.47 (95% confidence interval [CI]: 1.23-1.76) and 3.13 (95% CI: 2.59-3.78) in the elderly population, compared with YA. DISCUSSION: The effect of age on OS could only partially be explained by the imbalance in the tumour characteristics and treatment variables. The threefold higher risk of elderly could, at least partially, be explained by a higher other-cause mortality. The results might also be explained by a different tumour behaviour or suboptimal treatment in elderly compared with the younger population

Conventional Primary Central Chondrosarcoma of the Pelvis: Prognostic Factors and Outcome of Surgical Treatment in 162 Patients

BACKGROUND: Studies focusing on the oncological outcome after treatment of conventional primary central chondrosarcoma of pelvic bone are lacking. We conducted this retrospective study at 5 referral centers to gain insight in the outcome of treatment for this tumor type and to identify risk factors for impaired oncological outcome. METHODS: One hundred and sixty-two consecutive patients (118 male patients [73%]) who underwent resection of a conventional primary central chondrosarcoma of pelvic bone from 1985 to 2013 were evaluated. The median age was 51 years (range, 15 to 78 years). The median follow-up was 12.6 years (95% confidence interval [CI], 8.4 to 16.9 years). There were 30 grade-I lesions (19%), 93 grade-II lesions (57%), and 39 grade-III lesions (24%). RESULTS: Sixty-two patients (38%) experienced local recurrence: 9 grade-I lesions (30%), 31 grade-II lesions (33%), and 22 grade-III lesions (56%). Forty-eight patients (30%) developed metastases. The risk of disease-related death was 3% for grade-I tumors (1 of 30; this patient had a grade-II recurrence and died of metastases), 33% (31 of 93) for grade-II tumors, and 54% (21 of 39) for grade-III tumors. Identified risk factors for impaired disease-specific survival were tumor grade (grade II: hazard ratio [HR], 20.18; p = 0.003; and grade III: HR, 58.94; p < 0.001), resection margins (marginal: HR, 3.21; p = 0.001; and intralesional: HR, 3.56; p < 0.001), and maximal tumor size (HR, 1.08 per cm; p = 0.026). Deep infection (19% [n = 31]) was the predominant complication. CONCLUSIONS: This study offers a standard for survival rates for conventional primary central chondrosarcoma of the pelvis. The survival for grade-I tumors was excellent. Wide resection margins were associated with a significant survival advantage for higher-grade tumors. Because of the inability to reliably distinguish low-grade and high-grade tumors preoperatively, we conclude that any central pelvic chondrosarcoma should be treated with aggressive primary resection with the aim of obtaining wide resection margins. There may be aggressive biologic features in some tumors for which a surgical procedure alone may not be adequate to improve outcomes

Extrameningeal solitary fibrous tumors-surgery alone or surgery plus perioperative radiotherapy: A retrospective study from the global solitary fibrous tumor initiative in collaboration with the Sarcoma Patients EuroNet.

BACKGROUND: Solitary fibrous tumor (SFT) is a rare mesenchymal malignancy. Although surgery is potentially curative, the local relapse risk is high after marginal resections. Given the lack of prospective clinical trial data, the objective of the current study was to better define the role of perioperative radiotherapy (RT) in various SFT presentations by location. METHODS: This was retrospective study performed across 7 sarcoma centers. Clinical information was retrieved from all adult patients with extrameningeal, primary, localized SFT who were treated between 1990 and 2018 with surgery alone (S) compared with those who also received perioperative RT (S+RT). Differences in treatment characteristics between subgroups were tested using analysis of variance statistics and propensity score matching. Local control and overall survival rates were calculated from the start of treatment until progression or death from any cause. RESULTS: Of all 549 patients, 428 (78%) underwent S, and 121 (22%) underwent S+RT. The median follow-up was 52 months. After correction for mitotic count and surgical margins, S+RT was significantly associated with a lower risk of local progression (hazard ratio, 0.19: P = .029), an observation further confirmed by propensity score matching (P = .012); however, this association did not translate into an overall survival benefit. CONCLUSIONS: The results from this retrospective study investigating perioperative RT in patients with primary extrameningeal SFT suggest that combining RT with surgery in the management of this patient population is significantly associated with a reduced risk of local failures, especially in patients who have less favorable resection margins and in those who have tumors with a high mitotic count

Bone sarcomas: ESMO–EURACAN–GENTURIS–ERN PaedCan Clinical Practice Guideline for diagnosis, treatment and follow-up ☆

none81noA. Kawai43, K. Kopeckova44, D. A. Krakorova45, A. Le Cesne46, F. Le Grange1, E. Legius47, A. Leithner48, A. Lopez Pousa49, J. Martin-Broto36, O. Merimsky50, C. Messiou51, A. B. Miah52, O. Mir53, M. Montemurro54, B. Morland55, C. Morosi56, E. Palmerini57, M. A. Pantaleo58, R. Piana59, S. Piperno-Neumann60, P. Reichardt61, P. Rutkowski62, A. A. Safwat63, C. Sangalli64, M. Sbaraglia19, S. Scheipl48, P. Schoffski65, S. Sleijfer66, D. Strauss67, K. Sundby Hall13, A. Trama68, M. Unk69, M. A. J. van de Sande70, W. T. A. van der Graaf66,71, W. J. van Houdt72, T. Frebourg73x, R. Ladenstein41z, P. G. Casali2,74z &noneStrauss S.J.; Frezza A.M.; Abecassis N.; Bajpai J.; Bauer S.; Biagini R.; Bielack S.; Blay J.Y.; Bolle S.; Bonvalot S.; Boukovinas I.; Bovee J.V.M.G.; Boye K.; Brennan B.; Brodowicz T.; Buonadonna A.; de Alava E.; Dei Tos A.P.; Garcia del Muro X.; Dufresne A.; Eriksson M.; Fagioli F.; Fedenko A.; Ferraresi V.; Ferrari A.; Gaspar N.; Gasperoni S.; Gelderblom H.; Gouin F.; Grignani G.; Gronchi A.; Haas R.; Hassan A.B.; Hecker-Nolting S.; Hindi N.; Hohenberger P.; Joensuu H.; Jones R.L.; Jungels C.; Jutte P.; Kager L.; Kasper B.; Kawai A.; Kopeckova K.; Krakorova D.A.; Le Cesne A.; Le Grange F.; Legius E.; Leithner A.; Lopez Pousa A.; Martin-Broto J.; Merimsky O.; Messiou C.; Miah A.B.; Mir O.; Montemurro M.; Morland B.; Morosi C.; Palmerini E.; Pantaleo M.A.; Piana R.; Piperno-Neumann S.; Reichardt P.; Rutkowski P.; Safwat A.A.; Sangalli C.; Sbaraglia M.; Scheipl S.; Schoffski P.; Sleijfer S.; Strauss D.; Sundby Hall K.; Trama A.; Unk M.; van de Sande M.A.J.; van der Graaf W.T.A.; van Houdt W.J.; Frebourg T.; Ladenstein R.; Casali P.G.; Stacchiotti S.Strauss S.J.; Frezza A.M.; Abecassis N.; Bajpai J.; Bauer S.; Biagini R.; Bielack S.; Blay J.Y.; Bolle S.; Bonvalot S.; Boukovinas I.; Bovee J.V.M.G.; Boye K.; Brennan B.; Brodowicz T.; Buonadonna A.; de Alava E.; Dei Tos A.P.; Garcia del Muro X.; Dufresne A.; Eriksson M.; Fagioli F.; Fedenko A.; Ferraresi V.; Ferrari A.; Gaspar N.; Gasperoni S.; Gelderblom H.; Gouin F.; Grignani G.; Gronchi A.; Haas R.; Hassan A.B.; Hecker-Nolting S.; Hindi N.; Hohenberger P.; Joensuu H.; Jones R.L.; Jungels C.; Jutte P.; Kager L.; Kasper B.; Kawai A.; Kopeckova K.; Krakorova D.A.; Le Cesne A.; Le Grange F.; Legius E.; Leithner A.; Lopez Pousa A.; Martin-Broto J.; Merimsky O.; Messiou C.; Miah A.B.; Mir O.; Montemurro M.; Morland B.; Morosi C.; Palmerini E.; Pantaleo M.A.; Piana R.; Piperno-Neumann S.; Reichardt P.; Rutkowski P.; Safwat A.A.; Sangalli C.; Sbaraglia M.; Scheipl S.; Schoffski P.; Sleijfer S.; Strauss D.; Sundby Hall K.; Trama A.; Unk M.; van de Sande M.A.J.; van der Graaf W.T.A.; van Houdt W.J.; Frebourg T.; Ladenstein R.; Casali P.G.; Stacchiotti S