Medullary Thyroid Cancer: Epidemiology and Characteristics According to Data From the Marne-Ardennes Register 1975-2018.

Context: Medullary thyroid cancer (MTC) is a rare disease. Objective: The main objective of our study was to analyze the incidence evolution of MTC with a follow-up of more than 40 years. Further, a descriptive and survival analysis was performed according to the Kaplan-Meier analysis. Design Setting and Patients: This is a retrospective epidemiological study using data from the Marne-Ardennes registry from 1975 to 2018. Two hundred sixty patients with MTC were included. Main Outcome Measures: The incidence was calculated in the territory of the register (Marne and Ardennes departments of France) and standardized on the demographic structure of France. Patient and tumor characteristics were described. An analysis in a subgroup comparing hereditary and sporadic forms was performed. An analysis of survival was performed. Results: The standardized incidence shows an increasing trend over time. The incidence increased significantly from 0.41 to 0.57/100 000 person-years between 1986 and 1996 and 2008 and 2018. The MTC was hereditary in 21.2% of cases. The sex ratio (males:females) was 0.73. The average age at diagnosis was 53 years. Ninety-seven patients (37.3%) were N1, 26 (10%) were M1, and 56 (21.5%) developed metastases during the follow-up. Complete remission was obtained in 58.5% of patients. The disease was refractory for 18.1% of patients. The 5-year survival rate was 88.4%. Sporadic cases had a poorer prognosis than hereditary MTC. Conclusion: Our study demonstrates a moderate increase in the incidence of MTC between 1975 and 2018. The prognosis remains worse for sporadic MTC than for hereditary MTC.

Somatostatin vs. Octreotide for Prevention of Postoperative Pancreatic Fistula The PREFIPS Randomized Clinical Trial A FRENCH 007 - ACHBT Study.

OBJECTIVE: Pharmacological prevention of postoperative pancreatic fistula (POPF) after pancreatectomy is open to debate. The present study compares clinically significant POPF rates in patients randomized between somatostatin versus octreotide as prophylactic treatment. PATIENTS AND METHODS: Multicentric randomized controlled open study in patient's candidate for pancreaticoduodenectomy (PD) or distal pancreatectomy (DP) comparing somatostatin continuous intravenous infusion for 7 days versus octreotid 100 µg, every 8 hours subcutaneous injection for 7 days, stratified by procedure (PD vs. DP) and size of the main pancreatic duct (>4 mm) on grade B/C POPF rates at 90 days based on an intention-to-treat analysis. RESULTS: Of 763 eligible patients, 651 were randomized: 327 in the octreotide arm and 324 in the somatostatin arm, with comparable the stratification criteria - type of surgery and main pancreatic duct dilatation. Most patients had PD (n=480; 73.8%), on soft/normal pancreas (n=367; 63.2%) with a non-dilated main pancreatic duct (n=472; 72.5%), most often for pancreatic adenocarcinoma (n=311; 47.8%). Almost all patients had abdominal drainage (n=621; 96.1%) and 121 (19.5%) left the hospital with the drain in place (median length of stay=16 d). A total of 153 patients (23.5%) developed a grade B/C POPF with no difference between both groups: 24.1%: somatostatin arm and 22.9%: octreotide arm (Chi-2 test, P=0.73, ITT analysis). Absence of statistically significant difference persisted after adjustment for stratification variables and in per-protocol analysis. CONCLUSIONS: Continuous intravenous somatostatin is not statistically different from subcutaneous octreotide in the prevention of grade B/C POPF after pancreatectomy.

Association between polyunsaturated fatty acids in adipose tissue and mortality of colorectal cancer patients.

INTRODUCTION: Nutritional intake and dysregulation of fatty acid metabolism play a role in the progression of various tumors, but the consumption of fatty acids is difficult to assess accurately with dietary questionnaires. Biomarkers can objectively assess intake, storage and bioavailability. OBJECTIVE: We studied the association between the polyunsaturated fatty acid (PUFA) composition of abdominal subcutaneous adipose tissue (good indicator of dietary intake over 2-3 years) and all-cause mortality. METHODS: In the multicenter AGARIC study, samples from 203 patients with colorectal cancer (CRC) undergoing curative surgery, were harvested from subcutaneous adipose tissue, which were then analyzed for PUFA composition. RESULTS: After a median follow-up of 45 months, 76 patients died. These patients were more often men (72.4% versus 57.5%, P = 0.04), diabetic (32.9% versus 13.4%, P = 0.001), old (median: 74.5 versus 66.6 years, P < 0.001) and with high alcohol consumption (47.4% versus 30.7%, P = 0.005). An increased risk of death was observed with higher levels of 20:2 ω-6 (hazard ratiotertile3 vstertile1 (HRT3vsT1) 2.12; 95% confidence interval (CI) 1.01-4.42; p-trend = 0.04), 22:4 ω-6 (HRT3vsT1 = 3.52; 95% CI = 1.51-8.17; p-trend = 0.005), and 22:5 ω-6 (HRT3vsT1 = 3.50; 95% CI = 1.56-7.87; p-trend = 0.002). Conversely, the risk of death seemed lower when higher concentrations of 18:3 ω-6 (HRT3vsT1 = 0.52; 95% CI = 0.27-0.99; p-trend = 0.04) and the essential fatty acid, α-linolenic acid 18:3 ω-3 (HRT3vsT1 = 0.47; 95% CI = 0.24-0.93; p-trend = 0.03) were observed. CONCLUSION: The risk of death was increased in CRC patients with higher concentrations of certain ω-6 PUFAs and lower concentrations of α-linolenic acid in their subcutaneous adipose tissue. These results reflect dietary habits and altered fatty acid metabolism. Our exploratory results warrant confirmation in larger studies with further exploration of the mechanisms involved.

The Need for Centralization for Small Intestinal Neuroendocrine Tumor Surgery: A Cohort Study from the GTE-Endocan-RENATEN Network, the CentralChirSINET Study.

BACKGROUND: The concept of surgical centralization is becoming more and more accepted for specific surgical procedures. OBJECTIVE: The aim of this study was to evaluate the relationship between procedure volume and the outcomes of surgical small intestine (SI) neuroendocrine tumor (NET) resections. METHODS: We conducted a retrospective national study that included patients who underwent SI-NET resection between 2019 and 2021. A high-volume center (hvC) was defined as a center that performed more than five SI-NET resections per year. The quality of the surgical resections was evaluated between hvCs and low-volume centers (lvCs) by comparing the number of resected lymph nodes (LNs) as the primary endpoint. RESULTS: A total of 157 patients underwent surgery in 33 centers: 90 patients in four hvCs and 67 patients in 29 lvCs. Laparotomy was more often performed in hvCs (85.6% vs. 59.7%; p < 0.001), as was right hemicolectomy (64.4% vs. 38.8%; p < 0.001), whereas limited ileocolic resection was performed in 18% of patients in lvCs versus none in hvCs. A bi-digital palpation of the entire SI length (95.6% vs. 34.3%, p < 0.001), a cholecystectomy (93.3% vs. 14.9%; p < 0.001), and a mesenteric mass resection (70% vs. 35.8%; p < 0.001) were more often performed in hvCs. The proportion of patients with ≥8 LNs resected was significantly higher (96.3% vs. 65.1%; p < 0.001) in hvCs compared with lvCs, as was the proportion of patients with ≥12 LNs resected (87.8% vs. 52.4%). Furthermore, the number of patients with multiple SI-NETs was higher in the hvC group compared with the lvC group (43.3% vs. 25.4%), as were the number of tumors in those patients (median of 7 vs. 2; p < 0.001). CONCLUSIONS: Optimal SI-NET resection was significantly more often performed in hvCs. Centralization of surgical care of SI-NETs is recommended.

Prevention and management of post-thyroidectomy cervical haematoma. Recommendations of the AFCE (Association francophone de chirurgie endocrinienne) with the SFE (Société française d'endocrinologie) and the SFMN (Société française de médecine nucléaire).

Post-thyroidectomy cervical haematoma (PTCH) requiring reoperation occurs in fewer than 5% of patients but can be fatal or leave severe neurological sequelae if compressive. Risk factors besides anticoagulant treatments are discussed. Preoperative prevention complies with the recommendations of the French Society of Anaesthesia and Resuscitation (SFAR) for the management of antiaggregants and anticoagulants before and after the operation. Intraoperative prevention is centred on careful haemostasis, sometimes aided by coagulation tools and haemostatic agents, although there is no firm evidence of their effectiveness against the occurrence of PTCH. Systematic drainage of the thyroid cavity is no longer standard practice for the prevention of PTCH. Postoperatively, maintenance of normal blood pressure is essential to prevent PTCH, together with control of pain, coughing, nausea and vomiting. To reduce the risk of serious complications, medical and paramedical teams must be trained to recognise a haematoma and manage it so that it can be evacuated as a matter of extreme urgency, if necessary bedside, and then treated for its cause in the operating theatre.

Extent of thyroidectomy: When should hemithyroidectomy be performed? Recommendations from the Francophone Association for Endocrine Surgery, the French Society of Endocrinology and the French Society of Nuclear Medicine.

These recommendations, drawn from current data in the medical literature, incorporate the risks of hemithyroidectomy (HT) and total thyroidectomy (TT) and clarify the place of these two procedures in clinical settings. Discussions leading to a consensus were then assessed by the Francophone Association for Endocrine Surgery (Association francophone de chirurgie endocrinienne [AFCE]), along with the French Society of Endocrinology (Société française d'endocrinologie [SFE]), and the French Society of Nuclear Medicine (Société française de médecine nucléaire [SFMN]). The complication rate was twice as high after TT compared to HT. Total thyroidectomy requires life-long thyroid hormone supplementation, whereas such supplementation is required in only 30% of patients after HT. When surgery is indicated for Bethesda category II nodules, and in the absence of any indication for surgery on the contralateral lobe, HT is recommended. In patients with thyroid cancer (TC)≤1cm requiring surgical management or TC≤2cm, in the absence of risk factors for TC and in the absence of pre- or intraoperative detection of extrathyroidal extension, lymph node metastases (cN0) and/or suspected contra-lateral disease, HT is the preferred technique as long as the patient accepts the possibility of TT which might be required when aggressive forms of cancer are detected on definitive cytohistology (extrathyroidal extension, lymphovascular invasion, high-grade histology). For TC measuring between 2 and 4cm, the debate between HT and TT remains open today, although some surgeons tend to prefer TT. In patients with TC>4cm, macroscopic lymph node involvement (cN1), signs of extrathyroidal extension or predisposing factors for TC, TT is the treatment of choice.

High amylase concentration in drainage liquid can early predict proximal and distal intestinal anastomotic leakages: A prospective observational study.

Background: Anastomotic leak (AL) is a serious complication in digestive surgery. Early diagnosis might allow clinicians to anticipate appropriate management. The aim of this study was to assess the predictive value of amylase concentration in drain fluid for the early diagnosis of digestive tract AL. Materials and Methods: Hundred and fourteen consecutive patients "at risk" of AL, in whom a flexible drainage was placed by surgeon's choice after digestive anastomosis were included. Patients with eso-gastric, bilio-digestive, and pancreatic anastomoses were excluded. Drain amylase measurement (DAM) was routinely performed on postoperative day (POD) 1, 3, 5-7. DAM values were compared between patients with postoperative AL versus patients without AL. A receiver-operating curve (ROC) with calculation of the areas under the ROC curves area under curves was performed and a cutoff value of DAM was calculated. Results: AL occurred in 25 patients (AL group) and 89 patients did not present AL (C group). The mean DAM was significantly higher in AL group versus C Group on POD 1, 3, and 5. A cutoff value of 307 IU/L predicted the occurrence of AL with a sensitivity and specificity of 91% and 100%, respectively. Positive and negative predictive values were 100% and 97.5%, respectively. Patients with AL had an elevated DAM prior to the appearance of any clinical signs of AL. Conclusion: High level DAM could accurately predict AL for proximal and distal digestive tract anastomoses. This simple, noninvasive, and low-cost method can accurately predict early AL and help physicians to perform appropriate imaging and treatment.

Bone metastases in midgut neuroendocrine tumors: imaging characteristics, distribution, and risk factors.

PURPOSE: Bone metastases (BM) affect 10-30% of patients with small intestine neuroendocrine tumors (siNET), but little descriptive data are available regarding their distribution throughout the skeleton or potential risk factors. Aim of the study is to better describe the imaging characteristics, distribution, and risk factors of siNET bone metastases using 18F-FDOPA PET/CT. METHODS: All patients with well-differentiated siNET who underwent an 18F-DOPA PET/CT examination in our institution were retrospectively screened between October 2017 and February 2020. Location, SUVmax and CT density of each BM were collected. Sex, metabolic tumor volume (MTV) excluding bone, and metastatic sites other than bone were studied to determine risk factors of BM. RESULTS: Among the 69 patients included, 11 patients (15.9%) presented BM on 18F-FDOPA (65 metastases). The most frequently involved sites were: thoracic spine, pelvic bones and ribs. About 64% of patients presented multiple BM. On coupled CT scan, 63% of BM were not visible. Using an optimal threshold of 19.2 ml, MTV was an independent predictor of BM (p = 0.004) with a derived sensitivity of 100% [65.0-100.0] and a specificity of 70.9% [57.7-81.2]. Hepatic metastatic involvement was also a significant predictor of BM (p = 0.044). CONCLUSION: The development of BM in siNETs appears to be a late event, occurring in patients with a high tumor burden and hepatic involvement. They are often multiple and predominate in the axial skeleton.

SFE-AFCE-SFMN 2022 consensus on the management of thyroid nodules: Surgical treatment.

The SFE-AFCE-SFMN 2022 consensus deals with the management of thyroid nodules, a condition that is a frequent reason for consultation in endocrinology. In more than 90% of cases, patients are euthyroid, with benign non-progressive nodules that do not warrant specific treatment. The clinician's objective is to detect malignant thyroid nodules at risk of recurrence and death, toxic nodules responsible for hyperthyroidism or compressive nodules warranting treatment. The diagnosis and treatment of thyroid nodules requires close collaboration between endocrinologists, nuclear medicine physicians and surgeons, but also involves other specialists. Therefore, this consensus statement was established jointly by 3 societies: the French Society of Endocrinology (SFE), French-speaking Association of Endocrine Surgery (AFCE) and French Society of Nuclear Medicine (SFMN); the various working groups included experts from other specialties (pathologists, radiologists, pediatricians, biologists, etc.). This section deals with the surgical management of thyroid nodules.

Benefit of a flash dose of corticosteroids in digestive surgical oncology: a multicenter, randomized, double blind, placebo-controlled trial (CORTIFRENCH).

BACKGROUND: The modulation of perioperative inflammation seems crucial to improve postoperative morbidity and cancer-related outcomes in patients undergoing oncological surgery. Data from the literature suggest that perioperative corticosteroids decrease inflammatory markers and might be associated with fewer complications in esophageal, liver, pancreatic and colorectal surgery. Their benefit on cancer-related outcomes has not been assessed. METHODS: The CORTIFRENCH trial is a phase III multicenter randomized double-blind placebo-controlled trial to assess the impact of a flash dose of preoperative corticosteroids versus placebo on postoperative morbidity and cancer-related outcomes after elective curative-intent surgery for digestive cancer. The primary endpoint is the frequency of patients with postoperative major complications occurring within 30 days after surgery (defined as all complications with Clavien-Dindo grade > 2). The secondary endpoints are the overall survival at 3 years, the disease-free survival at 3 years, the frequency of patients with intraabdominal infections and postoperative infections within 30 days after surgery and the hospital length of stay. We hypothesize a reduced risk of major complications and a better disease-survival at 3 years in the experimental group. Allowing for 5% of drop-out, 1 200 patients (600 per arm) should be included. DISCUSSION: This will be the first trial focusing on the impact of perioperative corticosteroids on cancer related outcomes. If significant, it might be a strong improvement on oncological outcomes for patients undergoing surgery for digestive cancers. TRIAL REGISTRATION: ClinicalTrials.gov, NCT03875690, Registered on March 15, 2019, URL: https://clinicaltrials.gov/ct2/show/NCT03875690 .

Orbital Metastasis: A Rare but Typical Location of Small Intestine Neuroendocrine Tumor on 18F-FDOPA PET/CT.

ABSTRACT: Orbital foci of increased uptake are sometimes visualized on 18F-FDOPA PET/CT, but the literature remains poor as to their nature. The orbit is indeed a rare site of metastatic involvement. Given this low probability of metastatic location, the question of an incidental benign finding may arise. We reviewed all 18F-FDOPA PET/CT examinations performed at our institution between January 2015 and May 2021: 4/149 patients presented at least 1 orbital focus of increased uptake, all of them presented a metastatic small intestine neuroendocrine tumor. Somatostatin receptor expression was confirmed using 68Ga-DOTATOC PET/CT supporting the hypothesis of genuine metastases.

Life expectancy and likelihood of surgery in multiple endocrine neoplasia type 1: AFCE and GTE cohort study.

BACKGROUND: The overall natural history, risk of death and surgical burden of patients with multiple endocrine neoplasia type 1 (MEN1) is not well known. METHODS: Patients with MEN1 from a nationwide cohort were included. The survival of patients with MEN1 was compared with that of the general population using simulated controls. The cumulative probabilities of MEN1-specific operations and postoperative mortality were assessed, and surgical sequences were analysed using sunburst charts and Venn diagrams. RESULTS: A total of 1386 patients with MEN1 were included. Life expectancy was significantly reduced in patients with MEN1 compared with simulated controls from the general population, with a lifetime difference of 15 years. Mutations affecting the JunD interaction domain had a significant negative impact on survival. Survival for patients with MEN1 compared with the general population improved over time. The probability of experiencing at least one specific MEN1 operation was above 95 per cent after 75 years, and most patients had surgery at least twice during their lifetime. Time to a 50 per cent risk of MEN1 surgery was 30.5 years for patients born after 1960, compared with 47.9 years for those born before 1960. Sex and mutations affecting the JunD interacting domain had no impact on time to first surgery. There was considerable heterogeneity in surgical sequences, with no specific clinical pathway. CONCLUSION: Life expectancy was significantly lower among patients with MEN1 compared with the general population, and further decreased in patients with mutations affecting the JunD interacting domain. Almost all patients underwent at least one MEN1-specific operation during their lifetime, but there was no standardized sequence of surgery.

One-year COVID-19 outcomes on the oncology care patient pathway: Results of a French descriptive, cross-sectional comprehensive study (ONCOCARE-COV).

BACKGROUND: The COVID-19 pandemic led to a widely documented disruption in cancer care pathway. Since a resurgence of the pandemic was expected after the first lockdown in France, the global impact on the cancer care pathway over the year 2020 was investigated. AIMS: This study aimed to describe the changes in the oncology care pathway for cancer screening, diagnosis, assessment, diagnosis annoucement procedure and treatment over a one-year period. MATERIALS & METHODS: The ONCOCARE-COV study was a comprehensive, retrospective, descriptive, and cross-sectional study comparing the years 2019 and 2020. All key indicators along the cancer care pathway assessing the oncological activity over four periods were described. This study was set in a high-volume, public, single tertiary care center divided in two complementary sites (Reims University Hospital and Godinot Cancer Institute, Reims, France) which was located in a high COVID-19 incidence area during both peaks of the outbreak. RESULTS: A total of 26,566 patient's files were active during the year 2020. Breast screening (-19.5%), announcement dedicated consultations (-9.2%), Intravenous and Hyperthermic Intraoperative Intraperitoneal Chemotherapy (HIPECs) (-25%), and oncogeriatric evaluations (-14.8%) were heavily disrupted in regard to 2020 activity. We identified a clear second outbreak wave impact on medical announcement procedures (October, -14.4%), radiotherapy sessions (October, -16%), number of new health record discussed in multidisciplinary tumor board meeting (November, -14.6%) and HIPECs (November, -100%). Moreover, 2020 cancer care activity stagnated compared to 2019. DISCUSSION: The oncological care pathway was heavily disrupted during the first and second peaks of the COVID-19 outbreak. Between lockdowns, we observed a remarkable but non-compensatory recovery as well as a lesser impact from the pandemic resurgence. However, in absence of an increase in activity, a backlog persisted. CONCLUSION: Public health efforts are needed to deal with the consequences of the COVID-19 pandemic on the oncology care pathway.

Concept of reintervention with thorough lymphadenectomy after suboptimal resection of small-intestine neuroendocrine neoplasms: A multicentre preliminary study.

Complete surgical resection is the only hope to cure small intestine neuroendocrine neoplasms (SiNENs). However, inadequate lymphadenectomy or entire small bowel palpation for multiple primary tumours renders at least 20% of resections suboptimal. This study was undertaken to investigate reintervention outcomes after initial suboptimal resections (ISORs), and agreement between residual tumour identification on interval imaging and during reintervention. This retrospective, multicentre study included all patients undergoing reintervention within 18 months post ISOR. Disease-free survival (DFS) was defined as the time from reintervention resection date to recurrence or any-cause of death. The kappa coefficient assessed agreement rates between suspected residual tumour on interval imaging and its presence at reintervention. A total of 21 patients underwent reintervention for nonmetastatic SiNENs (median follow-up 2.3 [IQR 0.6-3.75] years). Residual tumour, suspected in 17/21 (81%) patients based on interval imaging, was found in 20/21 (95%) during reintervention. Interval imaging-intraoperative detection agreement was fair for residual primary tumours (kappa = 0.28, 95% CI: 0.05-0.62; p = .09) and residual lymph node metastases (kappa = 0.17, 95% CI: 0.28-0.62; p = .45). Reintervention achieved complete tumour clearance in 16/21 (76%) patients, among whom 5/16 (31%) developed liver metastases during follow-up. Median DFS was 70.6 months (IQR 39.7-not reached). Reintervention post-ISOR can obtain tumour clearance and prolonged remission. It should be systematically discussed after suspected ISOR, even when postoperative imaging does not find any residual tumour. To maximize detection of potentially resectable residual disease, imaging modalities after "curative" surgery should be redefined.

Performances of 18F-FDOPA PET/CT in the Preoperative Evaluation of the Peritoneal Cancer Index in Small Intestine Neuroendocrine Tumors.

PURPOSE: Peritoneal carcinomatosis (PC) concerns up to 30% of patients with a neuroendocrine tumor (NET), especially of the small intestine. Aggressive management of carcinomatosis seems to be justified, especially with regard to possible mechanical complications. 18F-FDOPA PET/CT is known to be the most sensitive imaging modality for the detection of small bowel NET metastases, yet its performance in the detection of PC is not well studied. The main objective of our study is to evaluate the performances of preoperative 18F-FDOPA PET/CT in the prediction of surgical peritoneal cancer index. METHODS: All patients referred to our center for an 18F-FDOPA PET/CT from October 2017 to January 2021 were retrospectively screened. Images were analyzed by a blinded nuclear medicine physician, and peritoneal abnormalities were reported to comply with the surgical peritoneal cancer index standard. Per patient analysis and per region analysis were then conducted. RESULTS: Thirty-three patients were included; 6 patients (35 regions) presented a peritoneal carcinosis. Peritoneal Carcinomatosis Index (PCI) estimated on 18F-FDOPA PET/CT was significantly and strongly correlated to surgical PCI (r = 0.96, P < 0.001). Patient-based sensitivity, specificity, negative predictive value, and positive predictive value for 18F-FDOPA PET/CT were 100%, 93%, 100%, and 75%, respectively. The agreement between 18F-FDOPA and surgery regarding PC was excellent (Cohen κ = 0.82 on per patient analysis, 0.74 on per region analysis). CONCLUSIONS: A preoperative estimation of PCI is achievable based on 18F-FDOPA PET/CT for small intestine NET and could allow to optimize surgical procedures and patient selection.

Differentiation between heterogeneous adrenal adenoma and non-adenoma adrenal lesion with CT and MRI.

PURPOSE: To assess whether heterogeneous adrenal adenomas can be distinguished from heterogeneous non-adenomas with Computed Tomography (CT) and/or Magnetic Resonance Imaging (MRI). METHOD: From 2009 to 2019, 980 consecutive adrenalectomies were retrospectively identified. Patients without adequate CT/MRI, with homogeneous and/or < 1 cm lesions were excluded. Differences between adenomas and non-adenomas were analyzed using Chi-square, Student t or Fischer tests, and interobserver agreement using weighted kappa test or intraclass correlation coefficient. Independent variables associated with adenomas were searched for using multivariable analysis. Area under the receiver operating characteristic curve (AUC) of the final model and its diagnostic performances were calculated. RESULTS: Final population comprised 183 patients (106 women, 77 men, mean age 53.2 ± 14.4 years) with 124 non-adenomas and 59 heterogeneous adenomas. Macroscopic or microscopic fat on CT/MRI allowed diagnosis of adenoma with 98% specificity and 63% sensitivity. Interobserver agreement was almost perfect for macroscopic fat (k = 0.82; 95% CI 0.66; 0.94) and substantial for microscopic fat (k = 0.75; 95% CI 0.62; 0.86). A multivariable model including micro- or macroscopic fat [Odds ratio (OR) 81.19; 95% CI 20.17; 572.27], diameter < 5.5 cm (OR 7.32; 95% CI 2.17; 31.28), calcifications (OR 5.68; 95% CI 2.08; 16.18), and hemorrhage (OR 3.10; 95% CI 0.70; 15.35) had an AUC of 0.91 (95% CI 0.86; 0.96), 71% (42/59, 95% CI 58; 82) sensitivity, 93% (115/124; 95% CI 87; 97) specificity, and 86% (157/183; 95% CI 79; 90) accuracy for the diagnosis of adenoma. CONCLUSION: A multivariable model enables CT/MR diagnosis of heterogeneous adenomas. Presence of microscopic fat, even if partial, in a heterogeneous mass is highly specific of adenoma.

Small Intestinal Neuroendocrine Neoplasm: Factors Associated with the Development of Local Tumor-Related Symptoms.

INTRODUCTION: Clinical presentations of small intestinal neuroendocrine neoplasms (SiNENs) can range from asymptomatic to life-threatening complications. Other than primary tumor(s), mesenteric mass (MM) can provide local tumor-related (LTR) symptoms. Although some expert centers propose routine primary resection to avoid complications in stage IV patients, some guidelines suggest avoiding primary tumor resection unless in the presence of symptoms. This study was aimed to identify factors associated with the presence or development of LTR symptoms. METHODS: From 2012 to 2019, SiNEN patients with appropriate initial morphological imaging were included. All initial imaging was reviewed. Associations between factors and LTR symptoms were assessed by logistic regression. RESULTS: Among 144 SiNEN patients, 66 met the inclusion criteria. Multivariate analysis identified on initial morphological imaging (i) any visible primary tumor (p < 0.01) and (ii) MM contact ≥180° with the superior mesenteric vessels (p ≤ 0.02), as independent factors associated with LTR symptoms in the whole study population as well as in the subgroup of primary resected patients. Among the 14 (21%) patients with both factors on initial cross-sectional conventional imaging, 12 (18%) were straightaway symptomatic at diagnosis and the remaining became symptomatic during the follow-up. All asymptomatic patients, without upfront surgery and without any predictive factor 16/18 (89%), stayed asymptomatic during the 2.7-year median follow-up. The absence of association between these 2 factors yielded a sensitivity of 100%, a specificity of 62%, and a negative predictive value of 100% for the occurrence of LTR symptoms. CONCLUSION: The presence of any visible primary tumor and/or MM superior mesenteric vessels contact ≥180° at initial cross-sectional imaging are 2 easily identifiable factors, which can help physicians for the decision-making regarding timing and type of surgery for SiNENs. Larger multicenter studies should endorse these results.

A large extended family with hyperparathyroidism-jaw tumor syndrome due to deletion of the third exon of CDC73: clinical and molecular features.

PURPOSE: We described the phenotype of a large 4-generation family with Hyperparathyrodism-Jaw Tumor syndrome (HPT-JT) associated with a rare deletion of exon 3 of the CDC73 gene. METHODS: We collected medical, genetic data on 24 family members descended from a common ancestor carrying a heterozygous deletion of exon 3. RESULTS: Thirteen carried the deletion, the penetrance was estimated at 50% at 40 years. Seven patients (39 ± 14.5 years) presented with HPT which could start at 13. Median plasmatic calcium and PTH levels were 3.13 ± 0.7 mmol/L and 115 ± 406 pg/ml, respectively. Kidney disease related to hypercalcemia were present in 57.1% of patients. All seven patients underwent surgery to remove a single parathyroid adenoma. One recurrence occurred 7 years post-surgery. No parathyroid carcinoma has been found to date. We found two atypical parathyroid adenomas. We described an additional somatic variant in exon 1 of gene CDC73 in two tumors. Jaw tumors were not necessarily associated with hyperparathyroidism, as shown in one case. Two kidney cysts were also reported. Variable phenotype expressivity was emphasized by clinical presentations in 2 monozygotic twins: acute hypercalcemia, kidney failure and ossifying fibroma in one twin, versus normocalcemic parathyroid adenoma in the other one. CONCLUSION: We report a family carrier of a deletion of exon 3 of the CDC73 gene. This is characterized by a high level of hypercalcemia, deleterious kidney effects and atypical parathyroid adenomas without carcinomas. Onset and intensity of HPT remain unpredictable. The additional somatic mutation found in the parathyroid tumor could lead to these phenotypical variations.

Assessing abdominal aortic calcifications before performing colocolic or colorectal anastomoses: A case-control study.

BACKGROUND: Preoperative evaluation needs objective measurement of the risk of anastomotic leakage (AL). This study aimed to determine if cardiovascular disease, evaluated by abdominal aortic calcification (AAC), was associated with AL after colorectal anastomoses. We conducted a retrospective case-control study on patients who underwent colorectal anastomosis between 2012 and 2016 at Reims University Hospital (France). Abdominal aortic calcification was the main variable of measurement. MATERIALS AND METHODS: We reviewed all patients who had a left-sided colocolic or a colorectal anastomosis, all patients with AL were cases; 2 controls, or 3 when possible, without AL were randomly selected and matched by operation type, pathology, and age. For multivariate analysis, 2 logistic regression models were tested, the first one used the calcification rate as a continuous variable and the second one used the calcification rate ≥ 5% as a qualitative variable. RESULTS: Forty-five cases and 116 controls were included. In univariate analysis, the calcification rate and the percentage of patients with a calcification rate ≥5% were significantly higher in cases than in control groups (4.4 ± 5.5% vs. 2.5 ± 5.2%, odds ratio [OR] =1.6 95% CI: 1.1-2.5; n = 22, 49% and n = 34.3 3%, OR = 2.8 95% CI: 1.2-6.2). In multivariate models, calcification rate as a continuous variable and calcification rate ≥5% as qualitative variable were independent significant risk factors for AL (respectively, aOR = 1.8; 95% CI: 1.1-3, P = 0.01; aOR = 3.2; 95% CI: 1.4-7.55, P < 0.01). CONCLUSION: AAC ≥5% should alert on a higher risk of AL and should lead to discussion about the decision of performing an anastomosis.