Obesity is a challenging condition for pancreatic surgery, and some authors recommend delaying pancreatic resection for non-malignant pancreatic tumors in obese patients. We present a case of a 45-year-old woman with a body mass index (BMI) of 56 who was surgically treated in our department for a mucinous cystadenoma discovered during preoperative work-up for bariatric surgery. To decrease the risk involved in pancreatic surgery, a glucagon-like peptide-1 receptor agonist was administered for 6 months, which led to a weight loss of 20 kg and a BMI of 48 at the time of surgery. A laparoscopic left splenopancreatectomy was performed within 7 months of the diagnosis. The postoperative length of stay was 19 days. Pathology confirmed that the tumor was mucinous cystadenoma with mild dysplasia. As of 17 months later, the patient is doing well and has lost an additional 10 kg.

Central nervous system (CNS)-type tumors may occur in the ovary, often associated with a mature teratomatous component. Because of their rarity, little is known about the tumor types historically designated within the primitive neuroectodermal tumors (PNET) terminology and whether they share histopathological and molecular features akin to those of their CNS counterparts. Herein, we retrospectively investigated data from 13 ovarian tumors, initially diagnosed as either “PNETs” or CNS-type neoplasms. For each tumor we performed comprehensive histopathologic, genetic and epigenetic analyses and retrieved clinical data when available. Integrated diagnoses were established after a central review of histopathological and molecular data, the following entities were identified: four embryonal tumors with multilayered rosettes (non C19MC-altered), three medulloblastomas, SHH-activated, three ependymomas not elsewhere classified, one Ewing sarcoma, one sarcoma, DICER1-mutant, and one peripheral neuroblastoma. Interestingly, none of the ETMRs harbored a C19MC amplification or DICER1 mutation. The three medulloblastomas, SHH-activated were histopathologically and molecularly similar to their CNS counterparts. Ependymomas did not show any classifying molecular alteration and presented a distinct epigenetic profile when compared with CNS ependymomas. These results indicate that ovarian “PNETs” comprise a heterogeneous spectrum of CNS and extra-CNS embryonal or non-embryonal tumor types, and that brain tumor methylation classifiers may be used to classify these tumors. Moreover, these components are characterized by distinct molecular alterations from primary CNS tumors, without C19MC alterations for ETMRs, with an overrepresented SHH-subgroup for medulloblastomas, and with an epigenetic profile distinct from CNS counterparts in ovarian ependymomas. These data need to be confirmed before they can be incorporated into future patient personalized treatment.

Since the histogenesis of heterologous elements within Sertoli-Leydig cell tumors (SLCTs) is poorly understood, we aimed to study the molecular relationship between Sertoli cells and the heterologous elements in 16 ovarian SLCTs. We performed a comprehensive molecular study on both SLCT and heterologous components, separately. Eleven tumors (68.7%) had one heterologous element and 5/16 (31.3%) had 2. Heterologous elements were epithelial (7/21 [81%]) (benign mucinous epithelium [9/21, 42.9%], borderline mucinous tumor [1/21, 4.8%], infiltrative mucinous adenocarcinoma [3/21, 14.3%], carcinoid tumor [3/21, 14.3%], and hepatocytes [1/21,4.8%]) or mesenchymal (4/21, 19%) (rhabdomyosarcoma [3/21,14.3%] and chondrosarcoma [1/21, 4.8%]). A DICER1 pathogenic variant was shared between SLCT and the heterologous elements in all cases with interpretable results (15/15), and other common likely-pathogenic/pathogenic variants were shared between SLCTs and heterologous components (3/16, 18.75%), favoring a clonal relationship. In contrast, the identification of distinct variants between components favored a different evolution. The molecular profile of heterologous elements differed from that of their ovarian counterparts occurring without SLCT (eg, mucinous heterologous elements were KRAS wild-type). Chromosome 8 gains, TERT and NRAS/KRAS variants, and absence of fusion transcript, were the hallmark of rhabdomyosarcoma components (3/3, 100%). The progression-free survival rate was significantly shorter for patients with TERT pathogenic variant ( P =0.0029). One patient had pleomorphic Sertoli cells associated with TP53 variants and very poor prognosis with early recurrence after complete initial surgery of a stage IA tumor. These data highlight the biological relationship between SLCTs and their heterologous elements, and the clinical usefulness of identifying pathogenic variants (ie, TERT and TP53 ), although this last point needs to be confirmed in a larger series.

This study assessed the prognostic significance of an R1 resection after intensive FOLFIRINOX (FX) neoadjuvant chemotherapy in patients with borderline (BL) and locally advanced pancreatic ductal adenocarcinomas. We retrospectively analyzed data collected from January 2010 through December 2024 at a single center. A total of 219 consecutive patients (31 borderline and 188 locally advanced) underwent resection for pancreatic ductal adenocarcinoma after induction FX chemotherapy; the median number of preoperative cycles was 11 (range: 2–26). Venous resection was present in 191 patients (87.7

High-grade appendiceal mucinous neoplasm (HAMN) is used to describe a rare epithelial neoplasm of the appendix characterized by pushing-type invasion and high-grade cytologic atypia. Its implications regarding lymph node spread and the necessity of right colectomy are currently debate. The objective of the present study was to assess the clinicopathologic characteristics, the risk of lymph node and peritoneal metastasis, and long-term outcomes of patients diagnosed as HAMN in comparison to low-grade appendiceal mucinous neoplasm (LAMN) and appendiceal adenocarcinoma, treated by right hemicolectomy. A total of 443 patients diagnosed with LAMN (n=246), HAMN (n=34), or appendiceal adenocarcinoma (n=163) and who underwent right colectomy with lymph node dissection in all cases within 32 institutions of the French Network for Rare Peritoneal Malignancies (RENAPE) were included. The median age was 56.5 years (range: 21 to 91), and the majority were female (n=250, 56.4%) without difference between groups ( P =0.604). Lymph node metastases were identified in 17.8% of appendiceal adenocarcinoma cases (29/163); none were found among LAMN or HAMN cases. A higher number of lymph nodes were analyzed in those treated for appendiceal adenocarcinoma than LAMN ( P <0.001) and HAMN ( P =0.035). Regarding peritoneal metastasis, a higher proportion of cases were classified as high-grade with/without signet cells in patients treated for HAMN ( P <0.001) and appendiceal adenocarcinoma ( P <0.001) than those treated for LAMN. Among patients with perforation of the appendix, those treated for LAMN had longer overall survival (OS; P <0.001) and progression-free survival (PFS; P <0.0001) than those treated for appendiceal adenocarcinoma or those treated for HAMN; among patients without perforation, those treated for LAMN and HAMN had longer OS ( P =0.042) and PFS ( P =0.012) than those treated for appendiceal adenocarcinoma. No lymph node metastases were observed in patients treated for HAMN, and those without appendix perforation had a similar prognosis to LAMN. This study supports staging HAMN using the same system as LAMN and treating it with appendectomy alone in the absence of appendix perforation.

Since the histogenesis of heterologous elements within Sertoli-Leydig cell tumors (SLCTs) is poorly understood, we aimed to study the molecular relationship between Sertoli cells and the heterologous elements in 16 ovarian SLCTs. We performed a comprehensive molecular study on both SLCT and heterologous components, separately. Eleven tumors (68.7%) had one heterologous element and 5/16 (31.3%) had 2. Heterologous elements were epithelial (7/21 [81%]) (benign mucinous epithelium [9/21, 42.9%], borderline mucinous tumor [1/21, 4.8%], infiltrative mucinous adenocarcinoma [3/21, 14.3%], carcinoid tumor [3/21, 14.3%], and hepatocytes [1/21,4.8%]) or mesenchymal (4/21, 19%) (rhabdomyosarcoma [3/21,14.3%] and chondrosarcoma [1/21, 4.8%]). A DICER1 pathogenic variant was shared between SLCT and the heterologous elements in all cases with interpretable results (15/15), and other common likely-pathogenic/pathogenic variants were shared between SLCTs and heterologous components (3/16, 18.75%), favoring a clonal relationship. In contrast, the identification of distinct variants between components favored a different evolution. The molecular profile of heterologous elements differed from that of their ovarian counterparts occurring without SLCT (eg, mucinous heterologous elements were KRAS wild-type). Chromosome 8 gains, TERT and NRAS/KRAS variants, and absence of fusion transcript, were the hallmark of rhabdomyosarcoma components (3/3, 100%). The progression-free survival rate was significantly shorter for patients with TERT pathogenic variant ( P =0.0029). One patient had pleomorphic Sertoli cells associated with TP53 variants and very poor prognosis with early recurrence after complete initial surgery of a stage IA tumor. These data highlight the biological relationship between SLCTs and their heterologous elements, and the clinical usefulness of identifying pathogenic variants (ie, TERT and TP53 ), although this last point needs to be confirmed in a larger series.

AIMS:Leiomyomas (LM) are the most common uterine mesenchymal neoplasms and encompass a variety of histological subtypes. Bizarre nuclei are described in both leiomyomas with bizarre nuclei (LM-BN) and fumarate hydratase-deficient leiomyomas (FH-LM), which raise diagnostic concerns regarding leiomyosarcoma (LMS). Recently, an immunohistochemical algorithm to support the diagnosis of LMS based on the genomic landscape of these neoplasms was proposed. This study aimed to evaluate the algorithm's accuracy in distinguishing LM-BN and FH-LM from LMS. METHODS AND RESULTS:We collected 68 LM (29 LM-BN, 30 FH-LM, and 9 LM) and 9 LMS, along with clinicopathological and molecular data. An immunohistochemical panel comprising p53, Rb, PTEN, ATRX, DAXX, and MDM2 was applied. Nine cases were non-interpretable due to fixation issues. The algorithm demonstrated 100% accuracy for LM without bizarre nuclei (9/9) and for nonmyxoid LMS (5/5). Notably, 28.6% (14/49) of LM-BN and FH-LM exhibited at least two abnormalities, leading to potential misclassification as LMS. However, their clinical course, morphology, and genomic profile supported a benign diagnosis. Frequent alterations included Rb (20/49; 40.8%) and p53 (19/49; 38.8%), particularly in bizarre cells, while no abnormal staining was observed for ATRX, DAXX, or MDM2. CONCLUSION:The proposed algorithm has limitations in differentiating LMS from LM-BN and FH-LM, misclassifying 28.6% of the latter. Accurate interpretation requires proper internal controls, particularly for markers whose loss of expression favours malignancy. Morphology remains central for diagnosis, although integration of molecular data may provide additional insights for a definitive classification in challenging cases.

OBJECTIVE:To evaluate the prognostic implications of the type of arterial resection performed during pancreatectomy with arterial resection (PAR) for adenocarcinoma. SUMMARY OF BACKGROUND DATA:The advent of efficacious neoadjuvant treatment has led to renewed interest in PAR. Debate remains regarding the prognostic impact of the type of artery resected on short and long-term outcomes. METHODS:We retrospectively evaluated all consecutive PARs performed between September 1, 1991, and July 30, 2024. Multivariate logistic and Cox analyses were performed to identify prognostic factors for major morbidity and survival. RESULTS:A total of 278 patients consecutively underwent PAR during the study period. The type of artery resected included the hepatic arteries (HA) (n=74), superior mesenteric artery (SMA) (n=75), celiac trunk (CT) (n=117), and others (n=12). Simultaneously, venous resection was carried out on 247 patients (88.8%). Postoperative mortality and morbidity rates over 90 days stood at 5.39% and 47% respectively. From a multivariate regression analysis for severe morbidity, the following were identified as independent prognostic factors: need for more than six perioperative blood red units (HR: 2.24; CI 95%: 1.41-4.39; P=0.002), and POPF (HR: 3.64; CI 95%: 1.63-8.13; P=0.002). Median overall survival (OS) was 26 months from diagnosis and following surgery was 20 months (95% CI: 15.7-24.2 mo) with 1-, 3-, and 5-year survival rates of 69%, 28%, and 17% respectively. OS was similar among PAR: HA (18.8 mo, 17.8%), SMA (15.8 mo, 21.7%), CT (23.2 mo, 16%). Normal /normalized preoperative CA 19-9 (HR: 0.69; 95% CI: 0.48-0.98; P=0.04) and tumor size (T3, T4) (HR: 3.26; 95% CI: 1.72-6.16; P=0.008) were prognostic factors for OS. CONCLUSIONS:The type of arterial resection does not influence the prognosis for patients with pancreatic adenocarcinomas undergoing PAR. The biological and pathological response to preoperative treatment are better predictors of the prognosis after PAR for adenocarcinoma.

Meta-analysis of 10 randomized prospective trials demonstrated a higher risk of postoperative bleeding from pancreaticogastrostomy (PG) compared with pancreatojejunostomy following pancreatoduodenectomy (PD). This study evaluated the incidence, risk factors, and treatment of anastomotic bleeding from invaginated PG. We retrospectively evaluated all consecutive PDs performed between April 1, 2011 and December 31, 2022 using invaginated PG by the double purse-string technique. Multivariate analysis identified risk factors for anastomotic PG bleeding. During the study, 695 consecutive patients with a median age of 66 years underwent PD; the majority was performed for ductal pancreatic adenocarcinomas. Simultaneous vascular resections were performed in 328 patients. Postoperative mortality was 4.1

Le mésothéliome pleural malin (MPM) est une maladie rare et agressive (taux de survie à 1 an de 58 % en France [1]), dont la principale cause est liée à une exposition à l'amiante. L'objectif de cette étude est d'analyser les caractéristiques épidémiologiques, les modalités diagnostiques et thérapeutiques des patients pris en charge au sein du CHU de Strasbourg. Nous avons mené une étude de cohorte rétrospective et monocentrique sur les patients diagnostiqués entre janvier 2013 et décembre 2022. Le recueil des données cliniques, d'imagerie et d'anatomopathologie s'est fait à partir des dossiers médicaux, enregistrés dans le cadre de la prise en charge médicale. L'étude porte sur 84 patients (60 hommes et 24 femmes). L'âge médian au diagnostic est de 71,2 ans. L'exposition à l'amiante est retrouvée chez 88 % des patients, dont 90 % d'origine professionnelle. Il est retrouvé 79,8 % de mésothéliomes épithélioïdes, 10,7 % de mésothéliomes sarcomatoïdes et 9,5 % de mésothéliomes biphasiques. Le délai médian entre les premiers symptômes et le diagnostic reste de 120 jours, notamment dû au délai entre l'apparition des symptômes aspécifiques et la 1re consultation spécialisée (56 jours). La chirurgie à visée curative a concerné 9 patients (5 pleurectomies/décortications et 4 pleuro-pneumonectomies). Les taux de survie à 1 an et à 2 ans sont respectivement de 70 % et de 44 %. La survie globale en population générale est de 21,6 mois, dont 23,5 mois pour les type histologique épithélioïde contre 10,3 mois pour les non épithélioïdes. La principale modalité thérapeutique systémique en première ligne est une combinaison de chimiothérapie à base de sels de platine associé au pémetrexed (n = 59/70, 84 %) suivie par l'immunothérapie (n = 9/70, 13 %). Les taux de contrôle après la première (n = 62), la deuxième (n = 38) et la troisième ligne de traitement (n = 21) sont respectivement de 87 %, 74 % et 67 %. Les médianes de survie sans progression sont de 7,6 mois après la première ligne, de 6,1 mois après une 2e ligne et de 3,5 mois après la 3e ligne. La progression tumorale se fait majoritairement sous la forme d'une récidive locorégionale (75 %). En analyse univariée sur la survie globale, les facteurs de mauvais pronostic sont un âge supérieur à 75 ans (HR = 2,02, p = 0,007), un état général altéré au diagnostic (PS > 2, HR = 4,63, p < 0,001) et une histologie non épithélioïde (HR = 2,25, p = 0,031). Il n'est pas noté de différence statistique de survie globale selon le sexe, le stade TNM au diagnostic ou le statut d'expression de BAP-1. Le MPM reste une maladie rare avec un pronostic défavorable. Les données de survie dans cette étude semblent meilleures comparées aux données épidémiologiques françaises, reflétant le parcours d'un patient dans un centre universitaire avec accès à une prise en charge chirurgicale, à plusieurs lignes de traitement systémiques ou à des essais thérapeutiques.

Background: High acinar pancreatic contents are associated with a higher rate of postpancreatectomy acute pancreatitis and pancreatic fistula formation (POPF). Predicting acinar contents preoperatively might identify those at high risk of developing postoperative complications. Methods: A multivariable analysis was performed to identify radiological factors associated with high pancreatic acinar content at histology in patients undergoing pancreaticoduodenectomy. Clinical and radiological variables identified were used to build a composite score predicting low, moderate, and high acinar pancreatic contents. Results: Pancreatic density, wirsung caliber, and pancreatic thickness on preoperative CT-scan predicted acinar contents. These three variables predicted low, moderate, and high acinar content in 94 (26%), 122 (33.6%), and 147 (40.5%) patients, respectively. Patients with high radiological acinar scores compared with patients with intermediate-low risk scores were more frequently male (73.4% vs. 54.1%; p = 0.0003), obese (14% vs. 6%; p = 0.01), and had a statistically significant higher rate of pancreatic-specific complications (23.8% vs. 8.33%; p = 0.01), POPF (12.9% vs. 4.63%; p = 0.005) and pancreaticogastrostomy bleeding (10.8% vs. 4.17%; p = 0.01). Conclusion: A simple radiological score combining pancreatic thickness, density, and wirsung caliber at CT scan preoperatively predicts patients with pancreatic parenchyma that are at higher risk of postoperative pancreatic-specific complications.

Background: The advent of Deep Learning initiated a new era in which neural networks relying solely on Whole-Slide Images can estimate the survival time of cancer patients. Remarkably, despite deep learning’s potential in this domain, no prior research has been conducted on image-based survival analysis specifically for peritoneal mesothelioma. Prior studies performed statistical analysis to identify disease factors impacting patients’ survival time. Methods: Therefore, we introduce MPeMSupervisedSurv, a Convolutional Neural Network designed to predict the survival time of patients diagnosed with this disease. We subsequently perform patient stratification based on factors such as their Peritoneal Cancer Index and on whether patients received chemotherapy treatment. Results: MPeMSupervisedSurv demonstrates improvements over comparable methods. Using our proposed model, we performed patient stratification to assess the impact of clinical variables on survival time. Notably, the inclusion of information regarding adjuvant chemotherapy significantly enhances the model’s predictive prowess. Conversely, repeating the process for other factors did not yield significant performance improvements. Conclusions: Overall, MPeMSupervisedSurv is an effective neural network which can predict the survival time of peritoneal mesothelioma patients. Our findings also indicate that treatment by adjuvant chemotherapy could be a factor affecting survival time.

The landscape of uterine sarcomas is becoming more complex with the description of new entities associated with recurrent driver molecular alterations. Uterine sarcomas, in analogy with soft tissue sarcomas, are distinguished into complex genomic and simple genomic sarcomas. Leiomyosarcomas and undifferentiated uterine sarcomas belong to complex genomic sarcomas group. Low-grade and high-grade endometrial stromal sarcomas, other rare tumors associated with fusion transcripts (such as NTRK, PDGFB, ALK, RET ROS1) and SMARCA4-deficient uterine sarcoma are considered simple genomic sarcomas. The most common uterine sarcoma are first leiomyosarcoma and secondly endometrial stromal sarcomas. Three different histological subtypes of leiomyosarcoma (fusiform, myxoid, epithelioid) are identified, myxoid and epithelioid leiomyosarcoma being more aggressive than fusiform leiomyosarcoma. The distinction between low-grade and high-grade endometrial stromal sarcoma is primarily morphological and immunohistochemical and the detection of fusion transcripts can help the diagnosis. Uterine PEComa is a rare tumor, which is distinguished into borderline and malignant, according to a risk assessment algorithm. Embryonal rhabdomyosarcoma of the uterine cervix is more common in children but can also occur in adult women. Embryonal rhabdomyosarcoma of the uterine cervix is almost always DICER1 mutated, unlike that of the vagina which is wild-type DICER1, and adenosarcoma which can be DICER1 mutated but with less frequency. Among the emerging entities, sarcomas associated with fusion transcripts involving the NTRK, ALK, PDGFB genes benefit from targeted therapy. The integration of molecular data with histology and clinical data allows better identification of uterine sarcomas in order to better treat them.

We analyzed whether preoperative 18F-FDG PET/CT adds to conventional primary staging in patients with presumed non-metastatic colonic cancer (CC). The prognostic role of 18F-FDG uptake in the primary tumor was evaluated after a mean follow-up of 15 years. Patients with a new diagnosis of presumed localized CC were prospectively enrolled and underwent presurgical 18F-FDG PET/CT. For each colon lesion, SUVmax, SUVpeak, TLG, and MTV were assessed and tested as prognostic factors. Forty-eight patients were included. Post-surgery pathology identified a total of 103 colon lesions, including 58 invasive adenocarcinomas, 4 in situ adenocarcinomas, 3 adenomas with high-grade dysplasia, and 38 adenomas with low-grade dysplasia. Per lesion sensitivity, specificity, positive (PPVs) and negative predictive values (NPVs) for colonic primary tumor detection were 78%, 97%, 98%, and 73% for conventional workup, and 94%, 87%, 92%, and 89% for 18F-FDG PET/CT. Only sensitivity was significantly different between 18F-FDG PET/CT and conventional workup. PET detected an additional ten pathological colonic lesions in seven patients. SUVmax, SUVpeak, and TLG showed significant differences between invasive adenocarcinomas, in situ adenocarcinomas, and high-grade dysplasia compared to low-grade dysplasia. There was a statistically significant difference between pT1-pT2 and pT3-pT4 adenocarcinomas. On patient-based analysis, sensitivity, specificity, PPV, and NPV for nodal staging were 22%, 84%, 44%, and 65% for CECT, and 33%, 90%, 67%, and 70% for 18F-FDG PET/CT, without a statistically significant difference. PET/CT also identified unknown metastatic spread and one synchronous lung cancer in four patients. Overall, 18F-FDG PETCT had an additional diagnostic value in 11 out of 48 patients (23%). 18F-FDG uptake of the primary tumor did not predict nodal or distant metastases. The difference in disease-free survival categorized by median SUVmax, SUVpeak, TLG, and MTV was not significant. Finally, preoperative 18F-FDG PET/CT is valuable in detecting potential colon lesions not visualized by conventional workups, especially in cases of incomplete colonoscopy. It effectively highlights distant metastases but exhibits limitations for N staging. Mainly due to the relatively small sample size, the quantitative analysis of 18F-FDG uptake in the primary tumor did not reveal any association with recurrence or disease-free survival, adding no significant prognostic information.

Le panorama des sarcomes utérins se complexifie de plus en plus avec la description de nouvelles entités associées à des altérations moléculaires récurrentes. Les léiomyosarcomes et les sarcomes utérins indifférenciés sont des sarcomes à génomique complexe. Les sarcomes du stroma endométrial de bas grade et de haut grade, d’autres sarcomes associés aux transcrits de fusion (tels que NTRK, PDGFB, ALK, RET ROS1) et le sarcome utérin déficient en SMARCA4 sont des sarcomes à génomique simple. Le léiomyosarcome est le sarcome utérin le plus fréquent suivi par les sarcomes du stroma endométrial. Il existe trois différents sous-types histologiques de léiomyosarcome (fusiforme, myxoïde, épithélioïde), les léiomyosarcomes myxoïde et épithélioïde étant plus agressifs que le léiomyosarcome fusiforme. La distinction entre un sarcome du stroma endométrial de bas grade et de haut grade est tout d’abord morphologique et immunohistochimique. La mise en évidence des transcrits de fusions aide au diagnostic. Définitivement reconnu comme entité distincte parmi les tumeurs mésenchymateuses gynécologiques, le PECome utérin fait partie des tumeurs rares. Grâce à des algorithmes d’évaluation du risque, les PEComes utérins sont différentiés en borderline et en malins. Le rhabdomyosarcome embryonnaire du col utérin survient typiquement chez l’enfant, mais peut s’observer également chez la femme adulte. Le rhabdomyosarcome embryonnaire du col utérin est presque toujours DICER1 muté, à la différence de celui du vagin qui est DICER1 sauvage, et de l’adénosarcome qui peut être DICER1 muté mais en moindre fréquence. Parmi les entités émergentes, les sarcomes associés aux transcrits de fusion impliquant les gènes NTRK, ALK, PDGFB bénéficient d’une thérapeutique ciblée. L’intégration des données moléculaires avec l’histologie et la clinique permet de mieux identifier les sarcomes utérins afin de mieux les traiter.