•This Clinical Practice Guideline provides key recommendations on the management of soft tissue and visceral sarcomas.•Recommendations have been agreed following a consensus meeting of ...representatives from ESMO, EURACAN and GENTURIS.•Authorship includes a multidisciplinary group of experts from several European institutions, India and Japan.
Desmoid-type fibromatosis is a rare and locally aggressive monoclonal, fibroblastic proliferation characterized by a variable and often unpredictable clinical course. Currently, there is no ...established or evidence-based treatment approach available for this disease. Therefore, in 2015 the European Desmoid Working Group published a position paper giving recommendations on the treatment of this intriguing disease. Here, we present an update of this consensus approach based on professionals’ AND patients’ expertise following a round table meeting bringing together sarcoma experts from the European Organization for Research and Treatment of Cancer/Soft Tissue and Bone Sarcoma Group with patients and patient advocates from Sarcoma PAtients EuroNet. In this paper, we focus on new findings regarding the prognostic value of mutational analysis in desmoid-type fibromatosis patients and new systemic treatment options.
•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.
The availability of high-resolution global digital elevation data sets has raised a growing interest in the feasibility of obtaining their spherical harmonic representation at matching resolution, ...and from there in the modelling of induced gravity perturbations. We have therefore estimated spherical Bouguer and Airy isostatic anomalies whose spherical harmonic models are derived from the Earth’s topography harmonic expansion. These spherical anomalies differ from the classical planar ones and may be used in the context of new applications. We succeeded in meeting a number of challenges to build spherical harmonic models with no theoretical limitation on the resolution. A specific algorithm was developed to enable the computation of associated Legendre functions to any degree and order. It was successfully tested up to degree 32,400. All analyses and syntheses were performed, in 64 bits arithmetic and with semi-empirical control of the significant terms to prevent from calculus underflows and overflows, according to IEEE limitations, also in preserving the speed of a specific regular grid processing scheme. Finally, the continuation from the reference ellipsoid’s surface to the Earth’s surface was performed by high-order Taylor expansion with all grids of required partial derivatives being computed in parallel. The main application was the production of a 1′ × 1′ equiangular global Bouguer anomaly grid which was computed by spherical harmonic analysis of the Earth’s topography–bathymetry ETOPO1 data set up to degree and order 10,800, taking into account the precise boundaries and densities of major lakes and inner seas, with their own altitude, polar caps with bedrock information, and land areas below sea level. The harmonic coefficients for each entity were derived by analyzing the corresponding ETOPO1 part, and free surface data when required, at one arc minute resolution. The following approximations were made: the land, ocean and ice cap gravity spherical harmonic coefficients were computed up to the third degree of the altitude, and the harmonics of the other, smaller parts up to the second degree. Their sum constitutes what we call ETOPG1, the Earth’s TOPography derived Gravity model at 1′ resolution (half-wavelength). The EGM2008 gravity field model and ETOPG1 were then used to rigorously compute 1′ × 1′ point values of surface gravity anomalies and disturbances, respectively, worldwide, at the real Earth’s surface, i.e. at the lower limit of the atmosphere. The disturbance grid is the most interesting product of this study and can be used in various contexts. The surface gravity anomaly grid is an accurate product associated with EGM2008 and ETOPO1, but its gravity information contents are those of EGM2008. Our method was validated by comparison with a direct numerical integration approach applied to a test area in Morocco–South of Spain (Kuhn, private communication 2011) and the agreement was satisfactory. Finally isostatic corrections according to the Airy model, but in spherical geometry, with harmonic coefficients derived from the sets of the ETOPO1 different parts, were computed with a uniform depth of compensation of 30 km. The new world Bouguer and isostatic gravity maps and grids here produced will be made available through the Commission for the Geological Map of the World. Since gravity values are those of the EGM2008 model, geophysical interpretation from these products should not be done for spatial scales below 5 arc minutes (half-wavelength).
Background
Guidelines recommend that retroperitoneal sarcoma (RPS) be managed in a reference sarcoma center (RSC), but the benefit remains to be demonstrated. This study investigated the impact of ...initial surgery performed within the NetSarc network on overall survival (OS).
Methods
NetSarc is a network of 26 RSCs with specialized multidisciplinary tumor boards (MDTs) that is funded by the French NCI. Since 2010, presentation to an MDT and second pathological review are mandatory for sarcoma patients, and data have been collected in a nationwide database. We extracted data for all patients who received surgery in or outside the network and who presented at a NetSarc center (NSC) for primary nonmetastatic RPS between 2010 and 2017.
Results
A total of 2945 patients were included: 1078 (36.6%) underwent the first surgery in an NSC, and 1867 (63.4%) in an out-of-network center. The median number of operations at an NSC during the study period was 23 (range: 3–209), and the corresponding median was 1 (range: 1–2) at out-of-network centers. The diagnostic procedures followed significantly more clinical practice guidelines within NetSarc, where there were significantly more first R0 resections 452 (41.9%) vs. 230 (12.3%). The OS was significantly superior for patients treated within NetSarc, with a 2-year OS of 87% vs. 70% (
p
< 0.001). In the multivariate analysis, surgery within an NSC was an independent predictor of OS, with a twofold lower odds ratio of death.
Conclusions
In this national study, surgery for primary RPS within an NSC was associated with a better OS.
Abstract Although retroperitoneal sarcomas are rare tumors, they can be encountered by a wide variety of clinicians as they can be incidental findings on imaging or present with non specific symptoms ...and signs. Surgical resection can offer hope of cure and patient outcomes are improved when patients are managed in high volume specialist centers. Failure to recognize retroperitoneal sarcomas on imaging can lead to inappropriate management in inexperienced centers. Therefore it is critical that a diagnosis of retroperitoneal sarcoma should be considered in the differential diagnosis of a retroperitoneal mass with prompt referral to a soft tissue sarcoma unit. In particular, the most common retroperitoneal sarcoma subtypes, liposarcoma and leiomyosarcoma, have characteristic imaging appearances which are discussed. This review therefore aims to set the context and guide clinicians through a diagnostic pathway for retroperitoneal masses in adults which arise extrinsic to the solid abdominal viscera.
Aggressive fibromatosis (AF) is a monoclonal proliferative disease but does not metastasize and does not dedifferentiate to a high-grade malignancy in case of recurrence. Biopsy is usually necessary ...to confirm the diagnosis. A hallmark is its apparent unpredictable clinical course producing a large heterogeneity even with an indistinguishable morphology. Additional studies of the molecular determinants of desmoid behavior are needed to guide selection of the various therapeutic modalities. During the last 10 years, the treatment of AF has evolved and the role of routine, aggressive first-line treatment (radiotherapy and surgery) is now debated. If a wait-and-see policy is used at initial presentation, it is observed that >50% of patients will have relatively indolent disease. Aggressive treatments that take their indications from retrospective studies should be re-evaluated in the light of new data. The objective of this article is to propose an algorithm that commences with more conservative approaches before treatments that have associated long-term morbidity, the more aggressive therapies being reserved only for those who really need it.
Background
Extra‐abdominal desmoid‐type fibromatosis (DF) is a rare, locally aggressive neoplasm that is usually managed conservatively. When treatment is indicated, it typically involves surgical ...resection, possibly with adjuvant radiotherapy. The indications for postoperative radiotherapy and its effectiveness are unclear. The objective of this study was to estimate the effect of surgical resection margins and adjuvant radiotherapy on rates of recurrence of DF.
Methods
Literature published between 1999 and 2015 was extracted from MEDLINE, Embase, Cochrane Central Registry of Trials, Web of Science and Google Scholar. Recurrence rate was analysed by meta‐analysis and compared between subgroups.
Results
Sixteen reports were included, consisting of a total of 1295 patients with DF. In patients treated by surgical resection alone, the risk of local recurrence was almost twofold higher for those with microscopically positive resection margins (risk ratio (RR) 1·78, 95 per cent c.i. 1·40 to 2·26). Adjuvant radiotherapy after surgery with negative margins had no detectable benefit on recurrence. In contrast, after incomplete surgical resection, adjuvant radiotherapy improved recurrence rates both in patients with primary tumours (RR 1·54, 1·05 to 2·27) and in those with recurrent DF (RR 1·60, 1·12 to 2·28).
Conclusion
DF resected with microscopically positive margins has a higher risk of recurrence. Adjuvant radiotherapy appears to reduce the risk of recurrence after incomplete surgical resection, particularly in patients with recurrent tumours.
Radiotherapy after surgery for recurrence only
Solitary fibrous tumors (SFT) are rare unusual ubiquitous soft tissue tumors that are presumed to be of fibroblastic differentiation. At present, the challenge is to establish accurate prognostic ...factors.
A total of 214 consecutive patients with SFT diagnosed in 24 participating cancer centers were entered into the European database (www.conticabase.org) to perform univariate and multivariate analysis for overall survival (OS), local recurrence incidence (LRI) and metastatic recurrence incidence (MRI) by taking competing risks into account. A prognostic model was constructed for LRI and MRI. Internal and external validations of the prognostic models were carried out. An individual risk calculator was carried out to quantify the risk of both local and metastatic recurrence.
We restricted our analysis to 162 patients with local disease. Twenty patients (12.3%) were deceased at the time of analysis and the median OS was not reached. The LRI rates at 10 and 20 years were 19.2% and 38.6%, respectively. The MRI rates at 10 and 20 years were 31.4% and 49.8%, respectively. Multivariate analysis retained age and mitotic count tended to significance for predicting OS. The factors influencing LRI were viscera localization, radiotherapy and age. Mitotic count, tumor localization other than limb and age had independent values for MRI. Three prognostic groups for OS were defined based on the number of unfavorable prognostic factors and calculations were carried out to predict the risk of local and metastatic recurrence for individual patients.
LRI and MRI rates increased between 10 and 20 years so relapses were delayed, suggesting that long-term monitoring is useful. This study also shows that different prognostic SFT sub-groups could benefit from different therapeutic strategies and that use of a survival calculator could become standard practice in SFTs to individualize treatment based on the clinical situation.
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