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Selective internal radiation therapy (SIRT) has emerged as a viable endovascular treatment strategy for hepatocellular carcinoma (HCC). According to the Barcelona Clinic Liver Cancer (BCLC) classification, SIRT is currently recommended for early- and intermediate-stage HCC that is unsuitable for alternative locoregional therapies. Additionally, SIRT remains a recommended treatment for patients with advanced-stage HCC and portal vein thrombosis (PVT) without extrahepatic metastasis. Several studies have shown that SIRT is a versatile and promising treatment with a wide range of applications. Consequently, given its favourable characteristics in various scenarios, SIRT could be an encouraging treatment option for patients with HCC across different BCLC stages. Over the past decade, an increasing number of studies have focused on better understanding the prognostic factors associated with SIRT to identify patients who derive the most benefit from this treatment or to refine the optimal technical procedures of SIRT. Several variables can influence treatment decisions, with a growing emphasis on a personalised approach. This review, based on the literature, will focus on the prognostic factors associated with the effectiveness of radioembolization and related complications. By comprehensively analysing these factors, we aimed to provide a clearer understanding of how to optimise the use of SIRT in managing HCC patients, thereby enhancing outcomes across various clinical scenarios.

Rationale and objectives: A new microcatheter was recently developed claiming to reduce beads reflux in drug-eluting bead transarterial chemoembolization (DEB-TACE). The aim of this study was to compare the reflux control microcatheter ability versus a standard microcatheter for TACE treatment in patients with hepatocellular carcinoma. Material and methods: Patients were prospectively included between November 2017 and February 2022. They received a DEB-TACE treatment with charged radiopaque beads using standard microcatheters or the SeQure reflux control microcatheter (Guerbet, France) and were assigned respectively to a control and a test group. Beads distribution mismatch was evaluated between the targeted territory on treatment planning CBCT and beads’ spontaneous opacities on the post-intervention CBCT and the 1-month CT scanner. Results: Twenty-three patients (21 men, median age 64 years [12.5 years]) with 37 hepatocellular carcinoma nodules were treated. The control group consisted of 13 patients – 19 nodules, while the test group included ten patients - 18 nodules. Non target embolization (NTE) was found in 20 % (2/10) of patients in the test group and 85 % (11/13) in the control group. NTE involved only an adjacent segment in the test group while it affected the adjacent biliary sector or even the contralateral liver lobe in the control group. No complication linked to NTE was found in the test group, while it led to one case of ischemic cholangitis and another case of biloma in the control group. Conclusion: The reflux control microcatheter may be efficient in reducing NTE and thus eventual adverse events in comparison to standard of care end-hole microcatheters.

Abstract Primary liver cancer arises either from hepatocytic or biliary lineage cells, giving rise to hepatocellular carcinoma (HCC) or intrahepatic cholangiocarcinoma (ICCA). Combined hepatocellular- cholangiocarcinomas (cHCC-CCA) exhibit equivocal or mixed features of both, causing diagnostic uncertainty and difficulty in determining proper management. Here, we perform a comprehensive deep learning-based phenotyping of multiple cohorts of patients. We show that deep learning can reproduce the diagnosis of HCC vs. CCA with a high performance. We analyze a series of 405 cHCC-CCA patients and demonstrate that the model can reclassify the tumors as HCC or ICCA, and that the predictions are consistent with clinical outcomes, genetic alterations and in situ spatial gene expression profiling. This type of approach could improve treatment decisions and ultimately clinical outcome for patients with rare and biphenotypic cancers such as cHCC-CCA.

Purpose: The aim of this study was to report the safety and tumor response rate of combined transarterial radioembolization (TARE) through the intrahepatic arteries and transarterial chemoembolization (TACE) through the extrahepatic feeding arteries (EHFA) in patients with hepatocellular carcinoma (HCC). Methods: Patients with HCC, who had both intrahepatic and extrahepatic arterial supply visible on preinterventional multiphase CT and were treated between 2016 and 2021 with a combination of TACE and TARE on the same nodule, were retrospectively included. Epidemiological, clinical, biological, and radiological characteristics were recorded. Safety and tumor response were assessed at 6 months. Results: Nine patients (8 men, median age 62 years [IQR: 54–72 years]) were included. Seven patients had previous treatments on the target nodule (TARE: 5; TACE: 2). The median longest axis (LA) of the lesion was 70 mm (IQR: 60–79 mm). Three patients had portal vein invasion (VP3). The EHFA originated from the right diaphragmatic artery (n = 6), the right adrenal artery (n = 2), and the left gastric artery (n = 1). The LA of the tumor portion treated with TACE was 47 mm (range: 35–64 mm). The ratio between the LA of the entire lesion and the LA treated with TACE was 1.44 (range: 1.27–1.7). One major complication occurred: acute on chronic liver failure. Median follow-up was 23 months (range: 16–29 months). Seven patients underwent further treatment: on the same lesion (n = 2), on newly appeared nodules (n = 2), and systemic treatment (n = 3). At 6-month follow-up, seven patients showed a local objective response. Time-to-progression was 13 (3.5–19) months. Conclusion: The combination of TARE and extrahepatic TACE for HCC with both intrahepatic and extrahepatic arterial supplies seems feasible and safe. Further studies are needed to validate the effectiveness of these preliminary results.

Introduction and Objectives: The lockdown policy introduced in 2020 to minimize the spread of the COVID-19 pandemic, significantly affected the management and care of patients affected by hepatocellular carcinoma (HCC). The aim of this follow-up study was to determine the 12 months impact of the COVID-19 pandemic on the cohort of patients affected by HCC during the lockdown, within six French academic referral centers in the metropolitan area of Paris. Materials and Methods: We performed a 12 months follow-up of the cross-sectional study cohort included in 2020 on the management of patients affected by HCC during the first six weeks of the COVID-19 pandemic (exposed), compared to the same period in 2019 (unexposed). Overall survival were compared between the groups. Predictors of mortality were analysed with Cox regression. Results: From the initial cohort, 575 patients were included (n = 263 Exposed_COVID, n = 312 Unexposed_COVID). Overall and disease free survival at 12 months were 59.9 ± 3.2% vs. 74.3 ± 2.5% (p

Background & Aims: Assessment of computed tomography (CT)/magnetic resonance imaging Liver Imaging Reporting and Data System (LI-RADS) v2018 major features leads to substantial inter-reader variability and potential decrease in hepatocellular carcinoma diagnostic accuracy. We assessed the performance and added-value of a machine learning (ML)-based algorithm in assessing CT LI-RADS major features and categorisation of liver observations compared with qualitative assessment performed by a panel of radiologists. Methods: High-risk patients as per LI-RADS v2018 with pathologically proven liver lesions who underwent multiphase contrast-enhanced CT at diagnosis between January 2015 and March 2019 in seven centres in five countries were retrospectively included and randomly divided into a training set (n = 84 lesions) and a test set (n = 345 lesions). An ML algorithm was trained to classify non-rim arterial phase hyperenhancement, washout, and enhancing capsule as present, absent, or of uncertain presence. LI-RADS major features and categories were compared with qualitative assessment of two independent readers. The performance of a sequential use of the ML algorithm and independent readers were also evaluated in a triage and an add-on scenario in LR-3/4 lesions. The combined evaluation of three other senior readers was used as reference standard. Results: A total of 318 patients bearing 429 lesions were included. Sensitivity and specificity for LR-5 in the test set were 0.67 (95% CI, 0.62–0.72) and 0.91 (95% CI, 0.87–0.96) respectively, with 242 (70.1%) lesions accurately categorised. Using the ML algorithm in a triage scenario improved the overall performance for LR-5. (0.86 and 0.93 sensitivity, 0.82 and 0.76 specificity, 78% and 82.3% accuracy for the two independent readers). Conclusions: Quantitative assessment of CT LI-RADS v2018 major features is feasible and diagnoses LR-5 observations with high performance especially in combination with the radiologist’s visual analysis in patients at high-risk for HCC. Impact and implications: Assessment of CT/MRI LI-RADS v2018 major features leads to substantial inter-reader variability and potential decrease in hepatocellular carcinoma diagnostic accuracy. Rather than replacing radiologists, our results highlight the potential benefit from the radiologist–artificial intelligence interaction in improving focal liver lesions characterisation by using the developed algorithm as a triage tool to the radiologist’s visual analysis. Such an AI-enriched diagnostic pathway may help standardise and improve the quality of analysis of liver lesions in patients at high risk for HCC, especially in non-expert centres in liver imaging. It may also impact the clinical decision-making and guide the clinician in identifying the lesions to be biopsied, for instance in patients with multiple liver focal lesions.

Background & Aims: Transcatheter arterial chemoembolisation (TACE) is recommended for patients with hepatocellular carcinoma devoid of macrovascular invasion or extrahepatic spread but not eligible for curative therapies. We compared the efficacy and safety of the combination of a single TACE and external conformal radiotherapy (CRT) vs. classical TACE. Methods: TACERTE was an open-labelled, randomised controlled trial with a 1:1 allocation rate to two or three TACE (arm A) or one TACE + CRT (arm B). Participants had a mean age of 70 years, and 86% were male. The aetiology was alcohol in 85%. The primary endpoint was liver progression-free survival (PFS) in the intention-to-treat population. The typical CRT schedule was 54 Gy in 18 sessions of 3 Gy. Results: Of the 120 participants randomised, 64 were in arm A and 56 in arm B; 100 participants underwent the planned schedule and defined the ‘per-protocol’ group. In intention-to-treat participants, the liver PFS at 12 and 18 months were 59% and 19% in arm A and 61% and 36% in arm B (hazard ratio [HR] 0.69; 95% CI 0.40–1.18; p = 0.17), respectively. In the per-protocol population, treated liver PFS tended to be better in arm B (HR 0.61; 95% CI 0.34–1.06; p = 0.081) than in arm A. Liver-related grade III–IV adverse events were more frequent in arm B than in arm A. Median overall survival reached 30 months (95% CI 23–35) in arm A and 22 months (95% CI 15.7–26.2) in arm B. Conclusions: Although TACE + CRT tended to improve local control, this first Western randomised controlled trial showed that the combined strategy failed to increase PFS or overall survival and led more frequently to liver-related adverse effects. Impact and implications: Hepatocellular carcinoma is frequently treated by arterial embolisation of the tumour and more recently by external radiotherapy. We tried to determine whether combination of the two treatments (irradiation after embolisation) might produce interesting results. Our results in this prospective randomised study were not able to demonstrate a beneficial effect of combining embolisation and irradiation in these patients. On the contrary, we observed more adverse effects with the combined treatment. Clinical Trials Registration: NCT01300143.

Abstract Background Patients with end‐stage renal disease may display both a loss of skeletal muscle mass and an increase in muscle fat deposits. We aimed to analyse the impact of low skeletal muscle mass index (SMI, surrogate marker of sarcopenia) and low muscle density (MD, surrogate marker of myosteatosis) on patient survival after kidney transplantation (KT). Methods In a retrospective cohort of 200 kidney transplant recipients (KTr), we measured on an unenhanced cross‐sectional computed tomography scan taken at the level of the third lumbar vertebra within the previous year or at the time of KT, both SMI (muscle cross‐sectional area normalized for height2, reported in cm2/m2) and MD (mean attenuation of muscle cross‐sectional area, expressed in Hounsfield units). We determined age‐specific and sex‐specific normality thresholds on 130 healthy subjects. The baseline factors associated with low MD were assessed by logistic regression analysis. Cox proportional hazard univariable and multivariable models were constructed to identify predictive factors of patient survival. Results Among the 200 patients of the cohort, 123 were male (62%), and mean age was 54.8 ± 13.8 years. A total of 181 KTr required renal replacement therapy before KT (91%), and 36 KTr (18%) received repeat kidney transplant after previous failed KT. Mean MD was 30.6 ± 9 HU in men and 29.7 ± 8.3 HU in women, whereas SMI was 49.7 ± 8.6 cm2/m2 in men and 42.3 ± 7.3 cm2/m2 in women. MD was below the 2.5th percentile for the healthy population in 49 KTr (25%), defining the myosteatosis group, while SMI was below the 2.5th percentile for the reference population in 10 KTr (5%). Independent risk factors for myosteatosis were two or more KT [adjusted odds ratio (aOR) 5.2, 95% confidence interval (95% CI): 2.22–12.4, P = 0.0001], a history of stroke (aOR 3.7, 95% CI: 1.30–10.7, P = 0.015), and body mass index > 25 kg/m2 (aOR 2.94, 95% CI: 1.4–6.18, P = 0.004). Myosteatosis was independently associated with mortality [adjusted hazard ratio (aHR) 2.12, 95% CI: 1.06–4.24, P = 0.033], as were cardiovascular disease (HR 2.06, 95% CI: 1.02–4.15, P = 0.043) and age (aHR 1.06, 95% CI: 1.03–1.09, P = 0.0003). Low SMI was not associated with mortality. Conclusions Myosteatosis, which was more prevalent than low skeletal muscle mass, might be an important prognostic marker in patients undergoing KT.

Introduction and objectives: Liver resection is the only curative therapeutic option for large hepatocellular carcinoma (> 5 cm), but survival is worse than in smaller tumours, mostly due to the high recurrence rate. There is currently no proper tool for stratifying relapse risk. Herein, we investigated prognostic factors before hepatectomy in patients with a single large hepatocellular carcinoma (HCC). Material and methods: We retrospectively identified 119 patients who underwent liver resection for a single large HCC in 2 tertiary academic French centres and collected pre- and post-operative clinical, biological and radiological features. The primary outcome was overall survival at five years. Secondary outcomes were recurrence-free survival at five years and prognostic factors for recurrence. Results: A total of 84% of the patients were male, and the median age was 66 years old (IQR 58–74). Thirty-nine (33%) had Child–Pugh A cirrhosis, and the mean Model for End-Stage Liver Disease (MELD) score was 6 (6–6). The aetiology of liver disease was predominantly alcohol-related (48%), followed by nonalcoholic steatohepatitis (22%), hepatitis B (18%) and hepatitis C (10%). The mean tumour size was 70 mm (55–110). The median overall survival was 72.5 months (IC 95%: 56.2-88.7), and the five-year overall survival was 55.1 ± 5.5%. The median recurrence-free survival was 26.6 months (95% CI: 16.0-37.1), and the five-year recurrence-free survival rate was 37.8 ± 5%. In multivariate analysis, preoperative prognostic factors for recurrence were baseline alpha-fetoprotein (AFP) > 7 ng/mL (p

Abstract Bile duct injury (BDI) is a dangerous complication of cholecystectomy, with significant postoperative sequelae for the patient in terms of morbidity, mortality, and long-term quality of life. BDIs have an estimated incidence of 0.4–1.5%, but considering the number of cholecystectomies performed worldwide, mostly by laparoscopy, surgeons must be prepared to manage this surgical challenge. Most BDIs are recognized either during the procedure or in the immediate postoperative period. However, some BDIs may be discovered later during the postoperative period, and this may translate to delayed or inappropriate treatments. Providing a specific diagnosis and a precise description of the BDI will expedite the decision-making process and increase the chance of treatment success. Subsequently, the choice and timing of the appropriate reconstructive strategy have a critical role in long-term prognosis. Currently, a wide spectrum of multidisciplinary interventions with different degrees of invasiveness is indicated for BDI management. These World Society of Emergency Surgery (WSES) guidelines have been produced following an exhaustive review of the current literature and an international expert panel discussion with the aim of providing evidence-based recommendations to facilitate and standardize the detection and management of BDIs during cholecystectomy. In particular, the 2020 WSES guidelines cover the following key aspects: (1) strategies to minimize the risk of BDI during cholecystectomy; (2) BDI rates in general surgery units and review of surgical practice; (3) how to classify, stage, and report BDI once detected; (4) how to manage an intraoperatively detected BDI; (5) indications for antibiotic treatment; (6) indications for clinical, biochemical, and imaging investigations for suspected BDI; and (7) how to manage a postoperatively detected BDI.

Abstract Background Ischemic colitis (IC) is a severe emergency in gastrointestinal surgery. The aim of the present study was to identify the predictors of postoperative mortality after emergent open colectomy for IC treatment. Additionally, we compared postoperative outcomes of patients undergoing emergent colectomy due to aortic surgery-related IC (AS-IC group) vs. other IC etiologies (Other-IC group). Methods We analyzed records of consecutive patients who underwent emergency open colectomy for IC between 2008 and 2019. Logistic regression analysis was performed to identify clinical and operative parameters associated with postoperative mortality. The AS-IC and Other-IC groups were compared for mortality, morbidity, ICU stay, hospital stay, and survival. Results During the study period, 94 patients (mean age, 67.4 ± 13.7 years) underwent emergent open colectomy for IC. In the majority of cases, IC involved the entire colon (53.2%) and vasopressor agents were required preoperatively (63.8%) and/or intraoperatively (78.8%). Thirty-four patients underwent surgery due to AS-IC, whereas 60 due to Other-IC causes. In the AS-IC group, 9 patients had undergone endovascular aortic repair and 25 open aortic surgery; 61.8% of patients needed aortic surgery for ruptured abdominal aortic aneurism (AAA). Overall, 66 patients (70.2%) died within 90 days from surgery. The AS-IC and Other-IC groups showed similar operative outcomes and postoperative complication rates. However, the duration of the ICU stay (19 days vs. 11 days; p = 0.003) and of the total hospital stay (22 days vs. 16 days; p = 0.016) was significantly longer for the AS-IC group than for the Other-IC group. The rate of intestinal continuity restoration at 1 year after surgery was higher for the Other-IC group than for the AS-IC group (58.8% vs. 22.2%; p = 0.05). In the multivariate model, preoperative increased lactate levels, a delay between signs/symptoms’ onset and surgery > 12 h, and the occurrence of postoperative acute kidney injury were statistically associated with postoperative mortality. Neither IC etiology (aortic surgery vs. other etiology) nor ruptured AAA was associated with postoperative mortality. Conclusion Emergency open colectomy for IC is associated with high postoperative mortality, which appears to be unrelated to the IC etiology. Preoperative lactate levels, > 12-h delay to surgery, and postoperative acute kidney injury are independent predictors of postoperative mortality.

Purpose: To evaluate and compare the prevalence and type of abdominal involvements identified on CT scans in COVID-19 critically ill patients to those observed in critically ill patients with non-SARS-CoV-2 viral pneumonia. Methods: Monocentric IRB approved retrospective study comparing all abdominal CT scans performed for patients admitted in the ICU with COVID-19 and those performed in a historical cohort of ICU patients with non-SARS-CoV-2 viral pneumonia. For each patient, gallbladder abnormality, acute pancreatitis signs, acute adrenal infarction, renal infarcts, bowel wall thickening and CT scan signs of bowel ischemia were assessed. Results were then compared between critically ill COVID-19 and non-COVID-19 patients (Chi-2 or Fisher exact tests for categorical data and Student t-test or Mann-Whitney U test for continuous data as appropriate). Results: Ninety-nine COVID-19 patients and 45 non-COVID-19 patients were included.No difference was found between the rate of abnormal findings comparing COVID-19 patients and patients with other viral pneumonia (63/99 [64%] vs 27/45 [61%], p=0.94). Acute pancreatitis signs were more commonly seen in COVID-19 patients but without statistically difference between groups (14/99 [14%] vs 3/45 [6.7%], p=0.31). Bowel wall thickening was slightly more commonly seen in patients with other viral pneumonia (18/99 [18%] vs 11/45 [24%], p=0.52), however ischemic features were observed in higher rate in the COVID-19 group, although without reaching statistically significant differences (7/99 [7.1%] vs 2/45 [4.4%], p=0.75). Conclusion: The rate and severity of abdominal involvement demonstrated by CT in ICU patients hospitalized for COVID-19 although high were not different to that observed in patients with other severe viral pneumoniae

Background & Aims: Patients affected by hepatocellular carcinoma (HCC) represent a vulnerable population during the COVID-19 pandemic and may suffer from altered allocation of healthcare resources. The aim of this study was to determine the impact of the COVID-19 pandemic on the management of patients with HCC within 6 referral centres in the metropolitan area of Paris, France. Methods: We performed a multicentre, retrospective, cross-sectional study on the management of patients with HCC during the first 6 weeks of the COVID-19 pandemic (exposed group), compared with the same period in 2019 (unexposed group). We included all patients discussed in multidisciplinary tumour board (MTB) meetings and/or patients undergoing a radiological or surgical programmed procedure during the study period, with curative or palliative intent. Endpoints were the number of patients with a modification in the treatment strategy, or a delay in decision-to-treat. Results: After screening, n = 670 patients were included (n = 293 exposed to COVID, n = 377 unexposed to COVID). Fewer patients with HCC presented to the MTB in 2020 (p = 0.034) and fewer had a first diagnosis of HCC (n = 104 exposed to COVID, n = 143 unexposed to COVID, p = 0.083). Treatment strategy was modified in 13.1% of patients, with no differences between the 2 periods. Nevertheless, 21.5% vs. 9.5% of patients experienced a treatment delay longer than 1 month in 2020 compared with 2019 (p

[This corrects the article DOI: 10.1371/journal.pone.0230914.].

ObjectiveThe present study explored the existing literature to describe the outcomes of surgical and regional treatments for colorectal cancer metastases (mCRC) in older patients.MethodsA literature search was conducted in PubMed, EMBASE, Cochrane and ClinicalTrials.gov for studies published since 2000 that investigated the short- and long-term outcomes of regional treatments (surgical or non-surgical) for mCRC in patients aged ≥65 years. Pooled data analyses were conducted by calculating the risk ratio (RR), mean differences (MD) and hazard ratio (HR) between older and younger patients or between two different approaches in older patients.ResultsAfter screening 266 articles, 29 were included in this review. These studies reported the outcomes of surgery (n = 19) and non-surgical local ablation treatments (n = 3) for CRC metastases in older vs. younger patients or compared the outcomes of different interventions in older patients (n = 7). When comparing older vs. younger patients undergoing liver surgery for mCRC, pooled data analysis showed higher postoperative mortality [RR = 2.53 (95%CI: 2.00-3.21)] and shorter overall survival [HR = 1.17 (95%CI: 1.07-1.18)] in older patients, whereas no differences in operative outcomes, postoperative complications and disease-free survival were found. When comparing laparoscopy vs. open surgery for liver resection in older mCRC patients, laparoscopy was associated with fewer postoperative complications [RR = 0.27 (95%CI: 0.10-0.73)].ConclusionLiver resection for mCRC should not be disregarded a priori in older patients, who show similar operative and postoperative outcomes as younger patients. However, clinicians should consider that they are at increased risk of postoperative mortality and have a worse overall survival, which may reflect comorbidities and frailty.

Abstract Several bacterial species have been implicated in the development of colorectal carcinoma (CRC), but CRC‐associated changes of fecal microbiota and their potential for cancer screening remain to be explored. Here, we used metagenomic sequencing of fecal samples to identify taxonomic markers that distinguished CRC patients from tumor‐free controls in a study population of 156 participants. Accuracy of metagenomic CRC detection was similar to the standard fecal occult blood test (FOBT) and when both approaches were combined, sensitivity improved > 45% relative to the FOBT, while maintaining its specificity. Accuracy of metagenomic CRC detection did not differ significantly between early‐ and late‐stage cancer and could be validated in independent patient and control populations (N = 335) from different countries. CRC‐associated changes in the fecal microbiome at least partially reflected microbial community composition at the tumor itself, indicating that observed gene pool differences may reveal tumor‐related host–microbe interactions. Indeed, we deduced a metabolic shift from fiber degradation in controls to utilization of host carbohydrates and amino acids in CRC patients, accompanied by an increase of lipopolysaccharide metabolism.

We report a rare case of emphysematous pyelonephritis complicated by septic shock and multiple gas emboli in the pulmonary artery (with transient cor pulmonale), coronary artery (with transient acute coronary syndrome). Keywords: Emphysematous pyelonephritis, Gas embolism

BACKGROUND:To investigate the diagnostic and prognostic performances of 18F-FDG PET/CT measures of metabolic tumour burden in patients with neurofibromatosis type-1 (NF1), suspect of malignant transformation. METHODS:This retrospective study included 49 patients (15-60 years old, 30 women) with a diagnosis of NF1, followed in our Reference Centre for Rare Neuromuscular Diseases, who presented clinical signs of tumour progression (pain, neurological deficit, tumour growth). Quantitative metabolic parameters were measured on 149 tumoral targets, using semi-automatic software and the best cut off values to predict transformation was assessed by Receiver Operating Characteristics (ROC) analysis. Prognostic value of PET/CT metabolic parameters was assessed by Kaplan-Meier estimates of overall survival. RESULTS:Lesions were histologically documented in 40 patients: a sarcomatous transformation was found in 16, a dysplastic neurofibroma (NF) in 7, and a benign NF in 17; in the remaining 9 patients, a minimal follow-up of 12 mo (median 59 mo) confirmed the absence of transformation. The optimal cut off values for detection of malignant transformation were, in decreasing order of area under the ROC curves, a tumour-to-liver (T/L) ratio >2.5, SUVmax > 4.5, total lesion glycolysis (TLG) > 377, total metabolic tumour volume (TMTV) > 88 cm3, and heterogeneity index (HIsuv) > 1.69. The best prognostic marker was the TLG: the 4-y estimates of survival were 97% [95% CI, 90% - 100%] in patients with TLG ≤ 377 vs. 27% [95% CI, 5% - 49%] in patients with TLG > 377 (P < 0.0001; χ2 27.85; hazard ratio 13.27 [95% CI, 3.72-47.35]). T/L ratio, SUVmax and TMTV demonstrated slightly lower performance to predict survival, with χ2 ranging 14.41-19.12. The HIsuv index was not predictive of survival. CONCLUSION:Our study demonstrates that TLG and TMTV, as PET/CT measures of metabolic tumour burden, may be used clinically to identify sarcomatous transformation in patients with NF1 and predict overall survival, with a higher specificity for the TLG. Conventional measures such as the SUVmax, and T/L ratio also demonstrate high prognostic value.