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Pheochromocytomas and paragangliomas can induce severe cardiovascular manifestations such as Takotsubo-like cardiomyopathy. What the perioperative outcomes are of patients presenting with pheochromocytomas/paragangliomas preceded by Takotsubo-like cardiomyopathy remains an unresolved question.From 2006 to 2019, all patients who underwent surgery for pheochromocytomas/paragangliomas preceded by Takotsubo-like cardiomyopathy were included from 3 high-volume centers, with specific attention to perioperative hemodynamic instability and postoperative outcomes.Overall, 37 patients were included, with a median age of 45 years. Patients were operated on 2 months (1-4) after a Takotsubo-like cardiomyopathy episode; 33 (89%) had a laparoscopic approach. All those who underwent surgery presented in a hemodynamically stable situation. All except 1 of the pheochromocytomas/paragangliomas patients had at least 1 antihypertensive treatment at the time of surgery. The median preoperative systolic blood pressure in the Takotsubo-like cardiomyopathy group was 120 mm Hg (95-132). Overall, 27/34 (79%) of patients required vasoactive drugs during surgery with nicardipine (n = 22), esmolol (n = 12), and/or norepinephrine (n = 8). No patient presented a catecholamine-induced life-threatening complication such as hypertensive crisis, cardiac arrhythmias, pulmonary edema, cardiac ischemia, or Takotsubo-like cardiomyopathy in the perioperative period. Severe morbi-mortality was nil. The systematic review identified 5 studies including 38 pheochromocytomas/paragangliomas patients with at least 1 episode of acute heart failure considered as Takotsubo-like cardiomyopathy before surgery, of which 28 patients had delayed surgery with 1 postoperative death.Hemodynamically stabilized patients with pheochromocytomas/paragangliomas preceded by Takotsubo-like cardiomyopathy can be safely scheduled for an elective pheochromocytomas/paragangliomas surgery, with similar intra and postoperative outcomes as those without Takotsubo-like cardiomyopathy.

Supplementary Tables S1-S2.

Purpose: Activation of the Wnt/β-catenin signaling pathway is frequent in adrenocortical carcinoma (ACC) and might be associated with a more aggressive phenotype. The objective of this study was to assess the prognostic value of β-catenin immunohistochemistry and CTNNB1 (β-catenin gene)/APC (adenomatous polyposis coli gene) mutations in patients with resected primary ACC.Experimental design: In 79 patients with resected primary ACC from a French cohort (Cochin-COMETE), β-catenin expression was assessed on tumor specimens by immunohistochemistry. For patients with available DNA (n = 49), CTNNB1, and APC hotspot (mutation cluster region), were sequenced. Association between these results and the clinicopathologic characteristics of the ACC and overall and disease-free survival were studied. Results were confirmed on a tissue microarray from an independent multicentric cohort of 92 ACC from Germany (German-ENSAT cohort).Results: In the Cochin-COMETE cohort, the presence of a β-catenin nuclear staining was significantly associated with a higher ENSAT tumor stage (i.e., stages III and IV), higher Weiss score, more frequent necrosis, mitoses, and CTNNB1/APC mutations. β-Catenin nuclear staining and the presence of CTNNB1/APC mutations were both associated with decreased overall and disease-free survival, and were independent predictive factors of survival in multivariate analysis. The same results were observed in the German-ENSAT cohort.Conclusions: Wnt/β-catenin activation, confirmed by the presence of β-catenin nuclear staining, is an independent prognostic factor of overall and disease-free survival in patients with resected primary ACC. Clin Cancer Res; 17(2); 206–11. ©2010 AACR. Clin Cancer Res; 17(2); 328–36. ©2010 AACR.

Supplementary Table 3 from Transcriptome Analysis Reveals that p53 and β-Catenin Alterations Occur in a Group of Aggressive Adrenocortical Cancers

Supplementary Table 2 from Transcriptome Analysis Reveals that p53 and β-Catenin Alterations Occur in a Group of Aggressive Adrenocortical Cancers

Supplementary Table 1 from Transcriptome Analysis Reveals that p53 and β-Catenin Alterations Occur in a Group of Aggressive Adrenocortical Cancers

The purpose of this study was to assess the diagnostic capabilities of preoperative conventional imaging (A total of 51 consecutive patients (6 men, 45 women; mean age, 62 ± 11.6 [SD] years; age range: 28-86 years) with biochemically confirmed PHPT who underwent CUS, single-tracer dual phaseAt surgery, 74 lesions were removed (32 parathyroid adenomas, 38 parathyroid hyperplasia and 4 subnormal glands). Thirty-six patients (71%) had single-gland disease and 15 patients (29%) had multiglandular disease at histopathological analysis. On a patient basis, sensitivity and accuracy of FCH PET/CT, CUS andOur results suggest that CUS

ContextThe contribution of [18F]F-fluorocholine (FCH)-positron emission tomography (PET)/computed tomography (CT) in normocalcemic primary hyperparathyroidism (nPHPT) remains unknown.ObjectiveTo evaluate the sensitivity and specificity of FCH-PET/CT in a cohort of osteoporotic patients with nPHPT and discordant or negative [99mTc]Tc-sestamibi scintigraphy and ultrasonography who all underwent parathyroidectomy (PTX).DesignLongitudinal retrospective cohort study in patients referred for osteoporosis with mild biological primary hyperparathyroidism.SettingTertiary referral center with expertise in bone metabolism and surgical management of hyperparathyroidism.PatientsAmong 109 patients with PHPT analyzed, 3 groups were individualized according to total serum calcium (tCa) and ionized calcium (iCa): 32 patients with hypercalcemia (HtCa group), 39 patients with normal tCa and elevated iCa (NtCa group), and 38 patients with both normal tCa and iCa (NiCa). All patients had biochemical follow-up confirming or not the success of PTX.Main outcome measuresTo evaluate the performance of FCH-PET/CT in terms of sensitivity and specificity, and to compare with first-line imaging procedures in the setting of nPHPT.ResultsThe sensitivity of FCH-PET/CT was 67% in the hypercalcemic group, 48% in the NtCa group (P = .05 vs HtCa), and 33% in the NiCa group (P = .004 vs HtCa). Specificity ranged from 97% to 99%. FCH-PET/CT was positive in 64.3% of patients with negative conventional imaging, with biochemical resolution after PTX in 77.8% of patients. Triple negative imaging was observed in 20 patients, with PHPT resolution in 85% of these patients.ConclusionThis study highlights the contribution of FCH-PET/CT in a well-phenotyped cohort of normocalcemic patients with discordant or negative findings in [99mTc]Tc-sestamibi scintigraphy and ultrasonography. However, negative imaging in nPHPT does not rule out the possibility of surgical cure by an experienced surgeon.

International audience; Background: Ampullary lesions are rare and can be locally treated either with endoscopic papillectomy or transduodenal surgical ampullectomy. Management of local recurrence after a first-line treatment has been poorly studied.Methods: Patients with a local recurrence of an ampullary lesion initially treated with endoscopic papillectomy or transduodenal surgical ampullectomy were retrospectively included from a multi-institutional database (58 centers) between 2005 and 2018.Results: A total of 103 patients were included, 21 (20.4%) treated with redo endoscopic papillectomy, 14 (13.6%) with transduodenal surgical ampullectomy, and 68 (66%) with pancreaticoduodenectomy. Redo endoscopic papillectomy had low morbidity with 4.8% (n = 1) severe to fatal complications and a R0 rate of 81% (n = 17). Transduodenal surgical ampullectomy and pancreaticoduodenectomy after a first procedure had a higher morbidity with Clavien III and more complications, respectively, 28.6% (n = 4) and 25% (n = 17); R0 resection rates were 85.7% (n = 12) and 92.6% (n = 63), both without statistically significant difference compared to endoscopic papillectomy (P = .1 and 0.2). Pancreaticoduodenectomy had 4.4% (n = 2) mortality. No deaths were registered after transduodenal surgical ampullectomy or endoscopic papillectomy. Recurrences treated with pancreaticoduodenectomy were more likely to be adenocarcinomas (79.4%, n = 54 vs 21.4%, n = 3 for transduodenal surgical ampullectomy and 4.8%, n = 1 for endoscopic papillectomy, P < .0001). Three-year overall survival and disease-free survival were comparable.Conclusion: Endoscopy is appropriate for noninvasive recurrences, with resection rate and survival outcomes comparable to surgery. Surgery applies more to invasive recurrences, with transduodenal surgical ampullectomy rather for carcinoma in situ and early cancers and pancreaticoduodenectomy for more advanced tumors.

International audience; The impacts of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic on cancer care are multiple, entailing a high risk of death from coronavirus disease 2019 (COVID-19) in patients with cancer treated by chemotherapy. SARS-CoV-2 vaccines represent an opportunity to decrease the rate of severe COVID-19 cases in patients with cancer and also to restore normal cancer care. Patients with cancer to be targeted for vaccination are difficult to define owing to the limited contribution of these patients in the phase III trials testing the different vaccines. It seems appropriate to vaccinate not only patients with cancer with ongoing treatment or with a treatment having been completed less than 3 years ago but also household and close contacts. High-risk patients with cancer who are candidates for priority access to vaccination are those treated by chemotherapy. The very high-priority population includes patients with curative treatment and palliative first- or second-line chemotherapy, as well as patients requiring surgery or radiotherapy involving a large volume of lung, lymph node and/or haematopoietic tissue. When possible, vaccination should be carried out before cancer treatment begins. SARS-CoV-2 vaccination can be performed during chemotherapy while avoiding periods of neutropenia and lymphopenia. For organisational reasons, vaccination should be performed in cancer care centres with messenger RNA vaccines (or non-replicating adenoviral vaccines in non-immunocompromised patients). Considering the current state of knowledge, the benefit-risk ratio strongly favours SARS-CoV-2 vaccination of all patients with cancer. To obtain more data concerning the safety and effectiveness of vaccines, it is necessary to implement cohorts of vaccinated patients with cancer.

biBackground:/i/bNuclear protein in testis (NUT) carcinomas (NC) are a rare, highly aggressive, subset of squamous cell carcinomas, characterized by a translocation involving theiNUTM1/igene. Thyroid location of NUT carcinomas has rarely been described.biMethods:/i/bWe report here two cases of thyroid NC withiNSD3::NUTM1/itranslocation.biResults:/i/bThe first case presented as a very aggressive undifferentiated thyroid carcinoma in a 38-year-old man who died 21 months after the diagnosis. The second case was diagnosed after multiple lymphadenopathy recurrences mainly in the neck in a 37-year-old woman 7 years after total thyroidectomy for papillary thyroid carcinoma with a classic and a solid/trabecular component.biConclusions:/i/bOur case reports highlight the challenges in diagnosing these exceptional carcinomas. The therapeutic impact of the administration of pharmacological compounds with epigenetic action, in line with the physiopathology of these carcinomas, is also discussed.

International audience; OBJECTIVE: Defining robust and standardized outcome references for distal pancreatectomy (DP) by using Benchmark analysis. BACKGROUND: Outcomes after DP are recorded in medium or small-sized studies without standardized analysis. Therefore, the best results remain uncertain. METHODS: This multicenter study included all patients undergoing DP for resectable benign or malignant tumors in 21 French expert centers in pancreas surgery from 2014 to 2018. A low-risk cohort defined by no significant comorbidities was analyzed to establish 18 outcome benchmarks for DP. These values were tested in high-risk, minimally invasive and benign tumor cohorts. RESULTS: 1188 patients were identified and 749 low-risk patients were screened to establish Benchmark cut-offs. Therefore, Benchmark rate for mini-invasive approach was ≥q36.8%. Benchmark cut-offs for postoperative mortality, major morbidity grade ≥q3a and clinically significant pancreatic fistula rates were 0%, ≤q27% and ≤q28%, respectively. The benchmark rate for readmission was ≤q 16%. For patients with pancreatic adenocarcinoma, cut-offs were ≥q75%, ≥q69.5% and ≥q66% for free resection margins (R0), 1-year disease free-survival and 3-year overall survival, respectively. The rate of mini-invasive approach in high-risk cohort was lower than the Benchmark cut-off (34.1% vs. ≥q36.8%). All Benchmark cut-offs were respected for benign tumor group. The proportion of benchmark cases was correlated to outcomes of DP. Centers with a majority of low-risk patients had worse results than those operating complex cases. CONCLUSION: This large-scale study is the first benchmark analysis of DP outcomes and provides robust and standardized data. This may allow for comparisons between surgeons, centers, studies, and surgical techniques.

Aim To assess postoperative complications and control of hormone secretions following pancreatoduodenectomy (PD) performed on multiple endocrine neoplasia type 1 (MEN1) patients with duodenopancreatic neuroendocrine tumors (DP-NETs). Background The use of PD to treat MEN1 remains controversial, and evaluating the right place of PD in MEN1 disease makes sense. Methods Thirty-one MEN1 patients from the Groupe d’étude des Tumeurs Endocrines MEN1 cohort who underwent PD for DP-NETs between 1971 and 2013 were included. Early and late postoperative complications, secretory control and overall survival were analyzed. Results Indication for surgery was: Zollinger–Ellison syndrome (n = 18; 58%), nonfunctioning tumor (n = 9; 29%), insulinoma (n = 2; 7%), VIPoma (n = 1; 3%) and glucagonoma (n = 1; 3%). Mean follow-up was 141 months (range 0–433). Pancreatic fistulas occurred in 5 patients (16.1%), distant metastases in 6 (mean onset of 43 months; range 13–110 months), postoperative diabetes mellitus in 7 (22%), and pancreatic exocrine insufficiency in 6 (19%). Five-year overall survival was 93.3% [CI 75.8–98.3] and ten-year overall survival was 89.1% [CI 69.6–96.4]. After a mean follow-up of 151 months (range 0–433), the biochemical cure rate for MEN-1 related gastrinomas was 61%. Conclusion In MEN1 patients, pancreatoduodenectomy can be used to control hormone secretions (gastrin, glucagon, VIP) and to remove large NETs. PD was found to control gastrin secretions in about 60% of cases.

International audience

Purpose Adrenocortical carcinoma (ACC) is a rare endocrine malignancy, whose diagnosis is suspected by conventional radiology and hormonal investigations. 18F-fluorodeoxyglucose positron emission tomography ([18F]-FDG PET) could help predict malignancy, but more consistent data are necessary to support future guidelines.MethodsA cohort of 63 patients with histologically proven ACC (n=55) or metastatic ACC with steroid oversecretion (n=8) was assembled. All patients underwent a [18F]-FDG PET and the maximum Standardized Uptake Value (SUVmax) and the adrenal-to-liver SUVmax ratio were calculated. The [18F]-FDG PET parameters were compared with clinical, pathological and outcome data.ResultsA positive correlation between [18F]-FDG PET parameters (SUVmax and adrenal-to-liver SUVmax ratio) and tumor size, ENSAT (European Network for the Study of Adrenal Tumors) staging, total Weiss score and the proliferation marker Ki67 was found. The strong correlation between SUVmax and Ki67 (r=0.47, p=0.0009) suggests a relationship between FDG uptake levels and tumor proliferation. Associations between outcome parameters (progression free or overall survival) and [18F]-FDG PET parameters were weaker. Fifty-six out of 63 patients (89%) had an adrenal-to-liver SUVmax >1.45, a previously defined cutoff value to predict malignancy. Seven ACC (11%) had a lower uptake, with a Ki67 expression level statistically lower compared to other ACC.ConclusionThis large cohort study shows that most ACC demonstrate high [18F]-FDG uptake. However, the positive correlation observed between SUVmax and Ki67 expression levels seems to explain the possibility of identifying some ACC with a low [18F]-FDG uptake and Ki67 ≤ 10%.

Radiomics is a relatively new approach for image analysis. As a part of radiomics, texture analysis, which consists in extracting a great amount of quantitative data from original images, can be used to identify specific features that can help determining the actual nature of a pancreatic lesion and providing other information such as resectability, tumor grade, tumor response to neoadjuvant therapy or survival after surgery. In this review, the basic of radiomics, recent developments and the results of texture analysis using computed tomography and magnetic resonance imaging in the field of pancreatic tumors are presented. Future applications of radiomics, such as artificial intelligence, are discussed.

Background Adrenal lesions diagnosed during pregnancy remain rare, and their management is challenging because of maternal physiologic modifications, restricted imaging investigations, and contraindications to several treatments. Surgical issues of adrenalectomy during pregnancy and consequences on perinatal outcomes are poorly described. We therefore aimed to report maternal and fetal outcomes after adrenalectomy during pregnancy. Methods All pregnant women who underwent adrenalectomy over a 15-year inclusion period were identified from a prospectively maintained database. Surgical management and maternal and fetal outcomes were reviewed. Results From January 2003 to July 2018, a total of 12 women underwent adrenalectomy at a median gestation of 20 weeks. Of these women, 11 had hyper-secreting lesions, including 8 with cortisol oversecretion, and 11 had benign lesions, including cortisol-secreting adenoma (n = 5), pheochromocytoma (n = 2), primary pigmented, nodular adrenal disease (n = 1), severe Cushing’s disease (n = 2), and hematoma (n = 1). A total of 3 patients with severe Cushing’s disease (n = 2) and primary pigmented, nodular adrenal disease (n = 1) required bilateral adrenalectomy. One patient presented with a malignant adrenal Ewing sarcoma. Adrenalectomy during pregnancy was performed by the lateral laparoscopic transabdominal laparoscopic route in 9 patients. Postoperative morbidity occurred in 3 women. Maternal mortality was nil, but preterm birth occurred in 7 cases and intrauterine growth retardation was observed in 3 cases. Finally, among the 12 women, 10 had a child in good health. Conclusion During pregnancy, a lateral laparoscopic transabdominal approach is a feasible procedure. Maternal outcome is acceptable but fetal outcome is determined by the underlying disease, with a worse outcome when the adrenalectomy is indicated for malignant lesions or Cushing’s syndrome.

Primary hyperparathyroidism (HPT1) is the most frequent endocrinopathy in multiple endocrine neoplasia type 1 (MEN1). Its surgical management is challenging. We aimed to describe and compare the imaging findings of parathyroid ultrasound (US), sestaMIBI scintigraphy (sestaMIBI), and 18F-fluorocholine (FCH) PET/CT in a series of MEN1 patients with HPT1. Retrospective analysis of a cohort of MEN1 patients with HPT1 assessed by parathyroid US, sestaMIBI scintigraphy and SPECT/CT, and FCH-PET/CT for potential surgery between 2015 and 2019. Twenty-two patients with a confirmed diagnosis of MEN1 who presented with HPT1 and were assessed by the 3 imaging modalities were included. After imaging workups, 11 patients were operated on for the first time, 4 underwent a redo surgery, and 7 did not undergo an operation. The overall patient-based positivity rate of imaging was 91% (20 of 22) for parathyroid US and 96% (21 of 22) for both sestaMIBI and FCH-PET/CT. The 3 imaging modalities demonstrated negative findings in 1/22 patient who did not undergo surgery. Overall, 3 pathologic glands were not detected by any imaging technique. SestaMIBI and FCH-PET/CT both resulted in the same 3 false-positive results in ectopic areas with a significant uptake on two thymic carcinoid tumors and one inflammatory lymph node. FCH-PET/CT provided more surgically relevant data than sestaMIBI in 4/11 patients with initial surgery and in 1/4 patient who underwent redo surgery. Compared to sestaMIBI scintigraphy, FCH-PET/CT provides additional information regarding the number of pathologic parathyroid glands and their localization in MEN1 patients with HPT1.

Introduction This document is a summary of the French Intergroup guidelines regarding the management of digestive neuroendocrine neoplasms (NEN) published in February 2020 ( www.tncd.org ). Methods All French medical societies involved in the management of NEN took part in this work. Recommendations were graded into four categories (A, B, C or D), according to the level of evidence found in the literature until May 2019. Results The management of NEN is challenging because of their heterogeneity and the increasing complexity of diagnostic and therapeutic procedures. Pathological analysis is required for their diagnostic and prognostic characterization, which mainly relies on differentiation, grade and stage. The two main emergency situations are functioning syndromes and poorly-differentiated carcinoma. Chromogranin A is the main biochemical marker of NET, although of limited clinical interest. Initial characterization relies on morphological and isotopic imaging. The treatment of localized NET relies on watchful follow-up and local or surgical resection depending on its supposed aggressiveness. Treatment options for metastatic disease include surgery, somatostatin analogues, chemotherapy, targeted therapies, organ-driven locoregional therapies and peptide-receptor radionuclide therapy. As specific predictive factors of treatment efficacy are yet to be identified and head-to-head comparisons have not or only rarely been performed, the therapeutic strategy currently depends on prognostic factors. Cumulative toxicity and the impact of treatment on quality of life must be considered since survival is relatively long in most patients with NET. Conclusion These guidelines are proposed to achieve the most beneficial therapeutic strategy in clinical practice as the therapeutic landscape of NEN is becoming ever more complex. These recommendations are permanently being reviewed.

Postoperative pancreatic fistula (POPF) is the most serious complication following pancreaticoduodenectomy (PD). Identifying patients at high or low risk of developing POPF is important in perioperative management. This study aimed to determine a predictive risk score for POPF following PD, and compare it to preexisting scores. All patients who underwent open PD from 2012 to 2017 in two high-volume centers were included. The training dataset was used for the development of the POPF predictive risk score (using the 2016 ISGPS definition), while the testing dataset was used for external validation. The proposed score was compared to the fistula risk score (FRS), the NSQIP-modified FRS (mFRS), and the alternative FRS (aFRS). Overall, 448 and 213 patients were included in the training and testing datasets, respectively. A probabilistic predictive risk score was developed using four independent POPF risk factors (increasing age, no preoperative radiation therapy, soft pancreatic stump, and decreasing main pancreatic duct diameter). The discriminative capacities of the new score, FRS, mFRS, and aFRS were similar (AUC ranging from 0.73 to 0.79 in the training cohort and from 0.73 to 0.76 in the testing cohort). However, the new score identified more specifically patients at low risk of POPF compared with other scores, in both cohorts, with a 6% false-negative rate. Preoperative radiation therapy is an independent protective factor of POPF following PD. It should be included in the risk score of POPF to identify more precisely patients at low risk for this complication.

Resume L’adenocarcinome gastrique (AG) est, au niveau mondial, le 5e cancer le plus frequent, la decroissance de son incidence semble se stabiliser en France. Vingt-cinq experts reunis sous l’egide de l’Association francaise de chirurgie ont collabore a la redaction de ce referentiel dedie a la prise en charge chirurgicale de l’AG. Concernant le bilan pre-therapeutique, la realisation d’une echo-endoscopie chez tous les patients candidats a une exerese chirurgicale (afin de preciser le statut ganglionnaire) et d’une cœlioscopie exploratrice en cas d’AG cT3/T4 et/ou N+ a la recherche d’une carcinose peritoneale a ete retenue. Cependant, le TEP-scanner ne devrait pas etre realise systematiquement mais uniquement lorsque les autres modalites diagnostiques sont insuffisantes. La laparotomie reste la voie d’abord de choix pour la realisation d’une gastrectomie totale ou partielle avec curage ganglionnaire D2 pour les lesions > T2N0. Il est recommande de faire un reservoir jejunal sur anse-en-Y pour le retablissement de continuite apres gastrectomie totale afin de limiter le risque de dumping syndrome et de reflux œsophagien. En cas de carcinose peritoneale sur AG avec PCI limite (≤ 7) chez un patient en bon etat general et dont la maladie est controlee par chimiotherapie, il faut probablement realiser une cytoreduction macroscopiquement complete avec chimiotherapie hyperthermique intraperitoneale en centre expert. Une gastrectomie avec curage D2 peut etre envisagee en cas de cirrhose uniquement si Child A. La realisation d’une gastrectomie palliative a visee symptomatique ou une derivation chirurgicale en cas d’obstruction distale de l’estomac chez un patient en bon etat general peuvent etre envisagees.

This European expert consensus statement provides recommendations for the diagnosis and management of primary hyperparathyroidism (PHPT), chronic hypoparathyroidism in adults (HypoPT), and parathyroid disorders in relation to pregnancy and lactation. Specified areas of interest and unmet needs identified by experts at the second ESE Educational Program of Parathyroid Disorders in 2019 were discussed during two virtual workshops in 2021 and subsequently developed by working groups with interest in the specified areas. PHPT is a common endocrine disease. However, its differential diagnosis of familial hypocalciuric hypercalcemia (FHH), the definition and clinical course of normocalcemic PHPT, and the optimal management of its recurrence after surgery represents areas of uncertainty requiring clarifications. HypoPT is an orphan disease characterized by low calcium concentrations due to insufficient PTH secretion, most often secondary to neck surgery. Prevention and prediction of surgical injury to the parathyroid glands are essential to limit the disease-related burden. Long-term treatment modalities including the place for PTH replacement therapy and the optimal biochemical monitoring and imaging surveillance for complications to treatment in chronic HypoPT need to be refined. The physiological changes in calcium metabolism occurring during pregnancy and lactation modify the clinical presentation and management of parathyroid disorders in these periods of life. Modern interdisciplinary approaches to PHPT and HypoPT in pregnant and lactating women and their newborn children are proposed. The recommendations on clinical management presented here will serve as background for further educational material aimed at a broader clinical audience and were developed with the focus on endocrinologists in training.

Background In search of an ideal cosmesis, transoral endoscopic thyroidectomy via vestibular approach (TOETVA) has recently been introduced to avoid a visible scar. Although ambulatory thyroid surgery is considered safe in carefully selected patients, this remains unclear for TOETVA. Methods All consecutive adult patients who underwent ambulatory TOETVA or open thyroid surgery at a French university hospital were prospectively enrolled from 12/2020 until 11/2021. The primary outcome was postoperative morbidity (recurrent laryngeal nerve (RLN) palsy, re-intervention for bleeding, wound morbidity, or hospital readmission). The secondary outcome was quality of life (QoL), measured by a survey including a validated questionnaire (SF-12) and a modified thyroid surgery questionnaire six weeks after surgery. Results Throughout the study period, 374 patients underwent a unilateral lobectomy or isthmectomy in ambulatory setting, of which 34 (9%) as TOETVA (including 21 (62%) for a possible malignancy). In the TOETVA group, younger age (median 40 (IQR 35-50) vs. 51 (40-60) years, P < 0.001) and lower BMI (median 23.1 (20.9-25.4) vs. 24.9 (22.1-28.9) kg/m(2), P = 0.001) were noted. No cases were converted to open cervicotomy. TOETVA was at least as good as open cervicotomy with nil versus four (1%) re-interventions for bleeding, one temporary (5%) versus 13 (4%) (temporary) RLN palsies, and one (

Background Factors associated with rectal NENs prognosis are poorly investigated. Aim To evaluate the prognostic role of the ENETs staging and grading systems in rectal NENs. Methods Tertiary referral, multicenter, retrospective study. Factors associated with OS and PFS were investigated by Cox-regression analysis, with best size cut-offs calculated by ROC analysis. Results Of 100 patients (mean age 55, 45% male, mean size 16.2 mm) 62, 5, 10 and 23 were TNM stage 1 to 4, and 63, 15 and 22 were G1, G2 and G3. Primary treatment was endoscopic snare resection in 62%, endoscopic mucosal resection/endoscopic submucosal dissection in 10%, surgery in 20% and medical treatment in 8%. The best size cut-offs to predict OS and PFS were 10 and 12 mm. During a mean follow-up of 40.7 months 12% died and 26% progressed. The 5-year OS and PFS were 79.5% and 65.2%. Stage IV and G3 were associated with worse OS (HR 8.16; p = 0.002; HR 15.57; p = 0.0004) and PFS (HR 14.26 p Conclusion Both staging and grading accurately predict rectal NENs prognosis. Size alone has limited accuracy as 26% of patients with stage IV and 16% with G3 have a primary tumour≤10 mm.

Design Molecular classification is important for the diagnosis and prognosis of adrenocortical tumors (ACT). Transcriptome profiles separate adrenocortical adenomas ‘C2’ from carcinomas, and identify two groups of carcinomas ‘C1A’ and ‘C1B’, of poor and better prognosis respectively. However, many ACT cannot be profiled because of improper or absent freezing procedures, a mandatory requirement so far. The main aim was to determine transcriptome profiles on formalin-fixed paraffin-embedded (FFPE) samples, using the new 3’-end RNA-sequencing technology. A secondary aim was to demonstrate the ability of this technique to explore large FFPE archives, by focusing on the rare oncocytic ACT variants. Methods We included 131 ACT: a training cohort from Cochin hospital and an independent validation cohort from Wuerzburg hospital. The 3’ transcriptome was generated from FFPE samples using QuantSeq (Lexogen, Vienna, Austria) and NextSeq500 (Illumina, San Diego, CA, USA). Results In the training cohort, unsupervised clustering identified three groups: ‘C1A’ aggressive carcinomas (n = 28, 29%), ‘C1B’ more indolent carcinomas (n = 28, 29%), and ‘C2’ adenomas (n = 39, 41%). The prognostic value of FFPE transcriptome was confirmed in the validation cohort (5-year OS: 26% in ‘C1A’ (n = 26) and 100% in ‘C1B’ (n = 10), P = 0.003). FFPE transcriptome was an independent prognostic factor in a multivariable model including tumor stage and Ki-67 (OS HR: 7.5, P = 0.01). Oncocytic ACT (n = 19) did not form any specific cluster. Oncocytic carcinomas (n = 6) and oncocytic ACT of uncertain malignant potential (n = 4) were all in ‘C1B’. Conclusions The 3’ RNA-sequencing represents a convenient solution for determining ACT molecular class from FFPE samples. This technique should facilitate routine use and large retrospective studies.

Simple Summary In this narrative review, we update the surgical management of pancreatic neuroendocrine tumours (pNETs) and highlight key elements in view of the recent literature. These tumours are rare and suffer from a lack of data and randomized controlled trials. The pNETs management is difficult due to their heterogeneity and the risks associated with pancreatic surgery. Innovative managements such as “watch and wait” strategies, parenchymal sparing surgery and minimally invasive approach are emerging. The correct use of all these therapeutic options requires a good selection of patients but also a constant update of knowledge. Abstract Pancreatic neuroendocrine tumours (pNETs) represent 1 to 2% of all pancreatic neoplasm with an increasing incidence. They have a varied clinical, biological and radiological presentation, depending on whether they are sporadic or genetic in origin, whether they are functional or non-functional, and whether there is a single or multiple lesions. These pNETs are often diagnosed at an advanced stage with locoregional lymph nodes invasion or distant metastases. In most cases, the gold standard curative treatment is surgical resection of the pancreatic tumour, but the postoperative complications and functional consequences are not negligible. Thus, these patients should be managed in specialised high-volume centres with multidisciplinary discussion involving surgeons, oncologists, radiologists and pathologists. Innovative managements such as “watch and wait” strategies, parenchymal sparing surgery and minimally invasive approach are emerging. The correct use of all these therapeutic options requires a good selection of patients but also a constant update of knowledge. The aim of this work is to update the surgical management of pNETs and to highlight key elements in view of the recent literature.

At diagnosis, about 15% of patients with pancreatic cancer present with a resectable tumour, 50% have a metastatic tumour, and 35% a locally advanced tumour, non-metastatic but unresectable due to vascular invasion, or borderline resectable. Despite the technical progress made in the field of radiation therapy and the improvement of the efficacy of chemotherapy, the prognosis of these patients remains very poor. Recently, the role of radiation therapy in the management of pancreatic cancer has been much debated. This review aims to evaluate the role of radiation therapy for patients with locally advanced tumours.

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.

Objective Pituitary adenoma (PA) is one of the three major components of multiple endocrine neoplasia type 1 (MEN1). Recent studies have suggested that MEN1-associated PAs are less aggressive than initially estimated. We propose an analysis of the outcome of PAs with a standard of care treatment in a nationwide cohort of MEN1 patients. Design Retrospective observational nationwide cohort study using the MEN1 patient registry from the French Group of Endocrine Tumours (GTE). Methods The GTE database population consists of 1435 patients with MEN1. This analysis focused on 551 patients recruited after 2000 with at least 3 years of follow-up. The study outcome was tumour progression of PA defined by an increase in Hardy classification (HC) during follow-up according to referring physician regular reports. Results Among 551 MEN1 patients (index and related), 202 (36.7%) had PA, with 114 (56.4%) diagnosed by MEN1-related screening. PAs were defined according to HC as microadenoma (grade I) in 117 cases (57.9%), macroadenoma in 59 (29.2%) with 20 HC grade II and 39 HC grades III–IV and unspecified in 26 (12.8%). They were prolactinomas in 92 cases (45.5%) and non-secreting in 73 (36.1%). After a median follow-up of 3 years among the 137 patients with HC grades I–II, 4 patients (2.9%) presented tumour progression. Conclusion PAs in patients with MEN1 are less aggressive than previously thought. Tumour progression is rare with a standard of care monitoring and treatment, especially in related patients who mostly present non-secreting microadenoma. MRI monitoring for asymptomatic MEN1 patients should be reduced accordingly.

La pandémie COVID-19 a un impact majeur à toutes les étapes de prise en charge des cancers. Le risque de décès de la COVID-19 chez les patients traités pour un cancer est élevé. La vaccination contre la COVID-19 représente une opportunité majeure pour réduire les risques de formes graves de la maladie et maintenir une filière de soins oncologiques normale. La définition de la population à vacciner est complexe, avec des données scientifiques très limitées et un contexte de pénurie en vaccin. Il apparaît théoriquement important de vacciner les patients atteints de cancers en cours de traitement ou avec un traitement datant de moins de trois ans, ainsi que leur entourage. Les patients actuellement prioritaires dits « à haut risque » sont ceux atteints de cancers en cours de chimiothérapie. Un groupe d’experts a préconisé une population « ultra-prioritaire » différente, incluant les patients sous chimiothérapie à visée curative ou palliative de première ou deuxième ligne, mais aussi ceux traités par chirurgie ou radiothérapie avec un grand volume pulmonaire, ganglionnaires et/ou de tissus hématopoïétiques. La vaccination doit idéalement être réalisée avant le début des soins. Malgré l’absence de données publiées, elle peut aussi s’effectuer pendant une chimiothérapie en évitant les périodes d’aplasie, avec possibilité de vacciner dans les établissements de soins. La mise en place de cohortes avec suivi immunologique et clinique des patients vaccinés atteints de cancer est nécessaire. Dans l’état actuel des connaissances, le rapport bénéfice/risque est très en faveur de la vaccination de tous les patients atteints de cancers.

International audience; With the advent of FOLFIRINOX and neoadjuvant radiotherapy, the surgical indications for adenocarcinoma have expanded. Locally advanced pancreatic adenocarcinomas of the body/tail with infiltration of the celiac trunk which have a good clinical, biological, and radiological response to neoadjuvant treatment may therefore be eligible for caudal pancreatectomy with resection of the celiac trunk (modified Appleby procedure). In addition to rigorous patient selection and surgical expertise, this procedure also requires experience in interventional radiology.