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Objective:To assess the concordance and discordance between the core Alzheimer’s disease (AD) CSF biomarkers and [18F]FDG-PET patterns evaluated clinically in memory clinic patients who meet appropriate use criteria for AD biomarker investigations.Methods:We retrospectively assessed subjects with atypical and/or early-onset dementia evaluated at a tertiary care memory clinic. All individuals underwent CSF evaluations for Aβ42, P-tau181 and T-tau, and brain [18F]FDG-PET. [18F]FDG-PET data was visually interpreted by two nuclear medicine experts as being consistent with AD or non-AD. CSF biomarker results were similarly grouped into AD biomarker positive/negative. Contingency tables and Kappa coefficients were used to establish the level of agreement and disagreement between CSF and [18F]FDG-PET results in all individuals.Results:136 individuals had both [18F]FDG-PET and lumbar puncture performed as part of the early-onset and/or atypical dementia assessments. [18F]FDG-PET showed a pattern suggestive of AD in 43% of patients, while CSF biomarkers showed results consistent with AD in 57% of subjects. In patients who met criteria for AD biomarker investigations, we found that [18F]FDG-PET was discordant with CSF AD biomarkers in nearly 20% of cases; 12% of individuals with [18F]FDG-PET scans consistent with AD had AD-negative CSF results; and 7% of individuals with [18F]FDG-PET scans not consistent with AD had AD-positive CSF results, potentially suggesting atypical AD variants or less advanced neurodegeneration. [18F]FDG-PET discriminated patients with an AD-positive CSF profile from patients with an AD-negative profile with a sensitivity and specificity higher than 80% (SN: 81%, 95% CI = 71-88%, SP: 81%, 95% CI = 68-89%). Furthermore, [18F]FDG-PET had a positive predictive value of 87% (95% CI=78-93%) and a negative predictive value of 72% (95% CI = 60-82%).Discussion:CSF and [18F]FDG-PET disagreed in nearly 20% of the cases studied in this clinical series. While CSF Aβ42 and P-tau181 biomarkers are specific for AD, the topographical information from [18F]FDG-PET may providecomplementary information.

Adrenocortical carcinoma is a rare and aggressive tumour. The only curative treatment is surgery with negative margins. In most series, the average lesion size ranges from 5.5 to 15 cm.We report the case of a 27-year-old female with hyperandrogenism and Cushing syndrome due to a right adrenocortical carcinoma of 19.7 cm.The tumour abutting on liver and vena cava and the presence two nodules in liver required extensive surgery including a right posterior sectionectomy and an en bloc resection of the adrenal mass together with the right kidney and the gallbladder. The vena cava was also resected with a reconstruction using a pericardial patch since it was invaded on its border. Pathological examination confirmed an adrenocortical carcinoma, with tumour invasion of vessels, tumour capsule, vena cava and two metastases in the liver (pT4N0M1). All margins were negative. Three months after surgery, two lung nodules, cardio-phrenic and internal mammary adenomegalies were noticed on a PET/CT scan, justifying the initiation of chemotherapy, alongside with mitotane. After a 10-month follow-up, CT scan was stable excepted for a lung nodule growing from 4 to 7 mm. Targeted stereotaxic radiotherapy was then administered. Twenty-two months after surgery, the patient has improved considerably and all signs of hyperandrogenism and Cushing syndrome have resolved.This case of adrenocortical carcinoma illustrates one of the largest tumours among those reported. It demonstrates the feasibility and effectiveness of a multimodal approach in its treatment even if it is giant and at high risk.

Portal hyperperfusion and "dearterialization" of the liver remnant are the main pathogenic mechanisms for Small For Size syndrome (SFSS). Associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) induces rapid remnant hypertrophy. We hypothesized a similar increase in portal pressure/flow into the future liver remnant in ALPPS and SFSS-setting hepatectomies. In a rodent model, ALPPS was compared to SFSS-setting hepatectomy. We assessed mortality, remnant hypertrophy, hepatocyte proliferation, portal and hepatic artery flow, hypoxia-induced response, and liver sinusoidal morphology. SFSS-hepatectomy rats were subjected to local (hepatic artery ligation) or systemic (Dimethyloxalylglycine) hypoxia. ALLPS prevented mortality in SFSS-setting hepatectomies. Portal hyperperfusion per liver mass was similar in ALLPS and SFSS. Compared to SFSS, efficient arterial perfusion of the remnant was significantly lower in ALPPS causing pronounced hypoxia confirmed by pimonidazole immunostaining, activation of hypoxia sensors and upregulation of neo-angiogenic genes. Liver sinusoids, larger in ALPPS, collapsed in SFSS. Induction of hypoxia in SFSS reduced mortality. Hypoxia had no impact on hepatocyte proliferation but contributed to the integrity of sinusoidal morphology. ALPPS hemodynamically differ from SFSS by a much lower arterial flow in ALPPS's FLR. We show that the ensuing hypoxic response is essential for the function of the regenerating liver by preserving sinusoidal morphology.

Summary Malpractice claims are a regularly increasing concern in gastrointestinal surgery. The goal of this study was to compare the current status of claims in two different French-speaking communities by a retrospective descriptive study of surgeons’ experiences, from the beginning of their practice up until December 31 2014. Data included the number, the reasons, and the results of medicolegal claims and their jurisdictions. Forty-three surgeons participated in this study. Two hundred medicolegal claims were analyzed. The mean number was 5.8 per surgeon. Bariatric surgery, colorectal surgery and parietal surgery were the most exposed. Forty-six (23%) faults were noted, while no fault was pronounced in 139 (69.5%) cases. The main reasons for lodging complaints were nosocomial infections, anastomotic leaks, poor postoperative care, hollow organ perforation, peripheral neurologic complication, and insufficient preoperative information. Forty-four percent of the complaints were analyzed by the conciliation and compensation commissions and 43.5% by the High Court. In the French-speaking group, there were 13 complaints, two of which gave rise to compensation. French surgeons are highly exposed to complaints: in French law, clumsiness or technical maladdress is considered as a fault. The patient should be informed preoperatively of all possible severe risks of a medical procedure. In Belgium, complications are exceptional and are considered random therapeutic events. Adhering to the recommendations emanating from the French High Authority of Health and Learned Societies as well as accreditation issued by the same High Authority should allow to decrease the number of undesirable events related to care and malpractice.

Associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) allows extended hepatectomy in patients with an extremely small future liver remnant (FLR). Current rodent models of ALPPS do not include resection resulting in insufficient-for-survival FLR, or they do incorporate liver mass reduction prior to ALPPS. Differences in FLR volume and surgical procedures could bias our understanding of physiological and hemodynamic mechanisms. We aimed to establish a rat ALPPS model with minimal FLR without prior parenchymal resection. In rodents, the left median lobe (LML) represents 10% of total liver. Partial hepatectomy (PHx) sparing LML and pericaval parenchyma represents our reference 87% resection. The first step in the procedure is either portal vein ligation (PVL) corresponding to ligation of all but the LML portal branches, or PVL with transection between the left and right median lobe segments (PVLT), and is defined as ALPPS stage-1. Second, ligated lobes were removed: PVL-PHx represents a conventional 2-stage hepatectomy, while PVLT followed by PHx is a strict reproduction of human ALPPS. In Group A, liver hypertrophy was analyzed after PVL (n = 38), PVLT (n = 47), T (n = 10), and sham (n = 10); In group B, mortality and FLR hypertrophy was assessed after PHx (n = 42), Sham-PHx (n = 6), PVL-PHx (n = 37), and PVLT-PHx (n = 45). In group A, PVLT induced rapid FLR hypertrophy compared to PVL (p

BACKGROUND Plexiform neurofibromas (PN) are observed in up to 50% of patients with neurofibromatosis type 1 (NF1). Trametinib has been used widely to treat PN but limited data has been reported on its efficacy within a clinical trial. METHODS This ongoing multicenter phase II trial includes patients with pediatric low-grade glioma and PN. Patients received daily oral trametinib (MEK inhibitor) for eighteen 28-day cycles. The volumes of PN were centrally quantified using a new semi-automatic 3D segmentation method. RESULTS As of May 15, 2022, 46 patients with PN were enrolled in the study and the recruitment was completed for this study arm. Thirty-four completed treatment and were available for analysis. For these patients, the median age was 10.5 years (range 0.7-19.8). The median volume of PN at baseline was 51cm3 (range 2.6 to 487.6). Among the 34 patients, 28 (82.4%) completed 18 cycles as planned. Two patients discontinued due to adverse reaction, three patients refused to continue treatment and one patient discontinued treatment based on physician decision. Median duration of treatment was 16.8 months (range 2.8 to 16.8). Median duration of follow-up was 30.4 months (range 8.2 to 42). A total of 38 PN were available for volumetric analysis. Using RECIST evaluation, the overall response rate was 13.1%. Volumetric assessment demonstrated an overall response rate of 64.7% (22/34 patients), and 65.8% (25/38 PN) of PN showed a decrease of more than 20% in volume. Median volume change was -30% (range -93.5 to 14.3). Thirty-one patients (91.1%) had durable response without progression (lasting ≥ 1 year). After discontinuation of treatment, one patient underwent surgery and three patients resumed MEK inhibitor. CONCLUSION We report outcome and volumetric quantification of PN treated with trametinib within a large clinical trial. Based on the current results, trametinib is effective and offers durable response.