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Camptocormia, also referred to as bent spine syndrome (BSS) is defined as an abnormal flexion of the trunk, appearing in standing position, increasing during walking and abating in supine position. BSS was initially considered, especially in wartime, as a psychogenic disorder. It is now recognized that in addition to psychiatric syndromes, many cases of reducible BSS have a somatic origin related to a number of musculo-skeletal or neurological disorders. The majority of BSS of muscular origin is related to a primary idiopathic axial myopathy of late onset, appearing progressively in elderly patients. Diagnosis of axial myopathy first described by Laroche et al. is based upon CT/MRI examination demonstrating massive fatty infiltration of paravertebral muscles. The non-specific histological aspect includes an extensive endomysial fibrosis and fat tissue with irregular degenerated fibers. Weakness of the paravertebral muscles can be secondary to a wide variety of diseases generating diffuse pathologic changes in the muscular tissue. BSS can be the predominant and sometimes revealing symptom of a more generalized muscular disorder. Causes of secondary BSS are numerous. They must be carefully assessed and ruled out before considering the diagnosis of primary axial myopathy. The principal etiologies include on the one hand inflammatory myopathies, muscular dystrophies of late onset, myotonic myopathies, endocrine and metabolic myopathies, and on the other hand neurological disorders, principally Parkinson's disease. Camptocormia in Parkinsonism is caused by axial dystonia, which is the hallmark of Parkinson's disease. There is no specific pharmacologic treatment for primary axial myopathy. General activity, walking with a cane, physiotherapy, and exercises should be encouraged. Treatment of secondary forms of BSS is dependent upon the variety of the disorder generating the muscular pathology. Pharmacologic and general management of camptocormia in Parkinson's disease merge with that of Parkinsonism. Levodopa treatment, usually active on tumor rigidity and akinesia, has poor or negative effect on BSS. [ABSTRACT FROM AUTHOR]

STUDY DESIGN: A case report. OBJECTIVE: To illustrate a rare case of oncogenous osteomalacia caused by a spinal thoracic myopericytoma. SUMMARY OF BACKGROUND DATA: Osteomalacia related to a tumor is well known. The cause of the disorder is usually a highly vascularized, benign tumor of mesenchymal origin. Location of the tumor in the spine is very rare. Removal of the tumor is followed by resolution of osteomalacia. METHODS: Diagnosis of oseomalacia was established on the presence of cardinal clinical, biologic, and radiologic features of osteomalacia. Localization of the tumor at T5 and T6 levels was obtained by magnetic resonance imaging. Surgical treatment consisted in a circumferential correction-fusion with hemivertebrectomy of T5 and T6 and tumor removal. RESULTS: Tumor removal was rapidly followed by disappearance of the clinical symptoms of osteomalacia, and by correction of hypophosphatemia. At 2-years follow-up, no recurrence of the tumor was detectable on imaging studies-the correction fusion remained stable. Histologically, the tumor was classified as a myopericytoma. There was no relapse of the clinical features of osteomalacia. However, secondary recurrence of the biologic markers due to an incomplete tumor removal was disclosed. CONCLUSION: Removal of the tumor was followed by healing of the clinical features of osteomalacia, demonstrating the causal connection between the myopericytoma and the osteopathy. [ABSTRACT FROM AUTHOR]

Background: The prognostic value of splenic vessel involvement in distal pancreatic adenocarcinoma remains controversial. The aim of the study was to assess its prognostic relevance in a large multicenter cohort., Methods: Patients who underwent pancreatosplenectomy for distal pancreatic adenocarcinoma were identified from 5 pancreatic surgical centers. A pathology review of the surgical specimens was performed to assess splenic vessel involvement, defined as invasion of the vessel's adventitia or deeper, and confirm the presence of splenic vein tumor thrombosis. Prognostic factors associated with overall and relapse-free survival were evaluated., Results: 149 patients underwent upfront surgery. Splenic vascular involvement was observed in 69 of them (46.3%). A parietal infiltration of the splenic artery or splenic vein was observed in 26 (17.5%) and 49 patients (32.8%), respectively. A pathologic tumor thrombosis of the splenic vein was identified in 22 patients (14.8%) and associated with larger tumors (>20 mm) (P = .023), more perineural (P = .017), and lymphovascular (P = .002) invasion, and more positive lymph node (P = .001). After a median follow-up of 50.8 months (95% confidence interval: 44.3-57.3), the cumulative 5-year overall and relapse-free survival were 46.2% and 33%, respectively. In multivariate analysis, in addition to lymph node metastasis (hazard ratio = 1.8; 95% confidence interval [1.1-3.1]; P = .023) and perineural invasion (hazard ratio = 3.5; 95% confidence interval [1.3-9.7]; P = .016), presence of splenic vein tumor thrombosis was the only splenic vascular involvement that affected independently the overall survival (HR = 2.3; 95% confidence interval [ 1.3-4.3]; P = .006)., Conclusion: In resectable distal pancreatic adenocarcinoma, a pathologic tumor thrombosis of the splenic vein is an independent prognostic factor of overall survival. To define the perioperative oncological strategy, a preoperative evaluation of splenic vessel involvement and thrombosis is needed., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Objective: The aim of this study is to understand the global practice of routine hormonal monitoring (HM) during ovarian stimulation (OS) in the context of assisted reproductive technique (ART) treatment., Methods: An open-access questionnaire was available to 3,845 members of IVF-Worldwide.com from September 8 to October 13, 2021. The survey comprised 25 multiple-choice questions on when and how ultrasound (US) and hormone tests were conducted during ovarian stimulation OS. For most questions, respondents were required to select a single option. Some questions allowed the selection of multiple options., Results: In all, 528 (13.7%) members from 88 countries responded to the questionnaire. Most respondents (98.9%) reported using US to monitor OS cycles. HM was used by 79.5% of respondents during any of the cycle monitoring visits and was most commonly performed on the day of, or a day prior to final oocyte maturation. Overall, 87% of respondents claimed adjusting the dose of gonadotropin during OS, with 61.7% adjusting the dose based on hormonal levels. Oestradiol (E2) was the most frequently monitored hormone during all visits and was used by 74% of respondents for the prediction of ovarian hyperstimulation syndrome (OHSS). On or a day prior to ovulation triggering (OT), the number of respondents who measured progesterone increased from 34.3% in the second/third visit to 67.7%. Approximately one-third of respondents measured luteinizing hormone during all visits., Conclusion: Globally, most ART specialists (~80%) use HM, along with US, for monitoring OS, especially for the prevention of OHSS., Competing Interests: NP has received grants, contracts, payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Merck Serono, Organon, Ferring Pharmaceutical, Roche Diagnostics, Theramex, IBSA, Gedeon Richter, Besins Healthcare over the last 3 years. RH is a shareholder of CHA SMG Australia Holdings. He has received educational sponsorship from MSD, Merck-Serono, Origio, Igenomix, and Ferring Pharmaceuticals. AR has received grant and honoraria for lectures, presentations, or educational events from Merck Serono, Ferring, Organon, Theramex and IBSA. VV has received a grant from Theramex. The remaining author declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision., (Copyright © 2023 Sachs-Guedj, Hart, Requena, Vergara and Polyzos.)

(1) Background: The presence of adenomyosis among pregnant patients has been associated with a higher incidence of miscarriage and pregnancy complications. Although the role of adenomyosis in women undergoing in vitro fertilization (IVF) was investigated in several studies and demonstrated a potentially detrimental effect on live birth rates following IVF, most of them were small studies in which the adenomyosis diagnosis was not confirmed based on solid ultrasonographic criteria. (2) Methods: 3503 patients undergoing their first blastocyst frozen transfer through a hormonal replacement (HRT) FET cycle. Among them, 140 women had a confirmed diagnosis of adenomyosis based on the MUSA criteria. (3) Results: Adenomyosis patients were more likely to proceed with deferred FET compared with no-adenomyosis women ( p = 0.002) and were significantly more likely to be treated with GnRH agonist pre-treatment (2 months) ( p < 0.001). The presence of adenomyosis significantly decreased the clinical pregnancy rates (aOR 0.62, 95% CI: 0.39-0.98, p = 0.040) and live birth rates (aOR 0.46, 95% CI: 0.27-0.75, p = 0.003) and significantly increased the miscarriage rates (aOR 2.13, 95% CI: 0.98-4.37, p = 0.045). Multivariable logistic regression adjusting for age, autologous or donor oocytes, PGT-A, deferred FET, serum progesterone levels the day before FET, GnRH agonist pre-treatment, number of embryos transferred, and adenomyosis demonstrated that the use of the GnRH agonist protocol did not decrease or increase the miscarriage rate, clinical pregnancy rate, or live birth rate. (4) Conclusions: The presence of adenomyosis had a significant negative impact on the clinical outcomes of patients undergoing FET and was associated with higher miscarriage, lower clinical pregnancy, and live birth rates. GnRH agonist pre-treatment does not appear to improve clinical outcomes.

This article translates and synthesizes a reference guide containing iconographic material of the pre- and post-pubertal female external genitals with and without genital mutilation/cutting (FGM/C). The literature focuses on adults, whereas FGM/C are usually performed before the age of 15. Signs of FGM/C can be subtle and depend on the type of mutilation as well as the experience of the examiner. Published in 2022 and produced with the collaboration of 23 professionals, this illustrated guide "Female Genital Mutilation/Cutting in Children and Adolescents: Illustrated Guide to Diagnose, Assess, Inform and Report, https://link.springer.com/book/10.1007/978-3-030-81736-7 " is currently available in open access. It is aimed at facilitating the training of health professionals in making diagnosis, clinical management, and reporting to child safeguarding/law enforcement agencies, where required., Competing Interests: Les auteur-es n’ont déclaré aucun conflit d’intérêts en relation avec cet article.

Eosinophilic inflammation of the digestive tract is an inflammatory disease characterized by extensive infiltration of eosinophils into the gastrointestinal tract. It can be either a primary disorder of the digestive tract or be secondary to another cause of tissue eosinophilia. Primary disorders include eosinophilic esophagitis (OE) and eosinophilic gastroenteritis (GEEo). These are 2 rare pathologies considered to be diseases related to a Th2-mediated food allergy. The role of the pathologist is twofold: (1) he must make the diagnosis of tissue esosinophilia and propose the various causes, knowing that a secondary cause is the most frequently observed; (2) identify the abnormal number of polymorphonuclear eosinophils, which implies knowing the normal distribution of eosinophils in the different digestive segments. To carry the diagnosis of EO, the threshold of polymorphonuclear eosinophils must be ≥ 15/fields × 400. There is no predefined threshold concerning the other segments of the digestive tract to carry the diagnosis of GEE O . In addition, to make the diagnosis of primary digestive tissue eosinophilia, the patient must be symptomatic with histological evidence of eosinophilia and have ruled out all secondary causes. The main differential diagnosis of OE is gastroesophageal reflux disease. The differential diagnoses of GEEo are multiple, including primarily drugs and parasitic infections., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

The available literature is controversial regarding the association between the number of oocytes retrieved and the cumulative live birth rate (CLBR). Although some authors report a continuous increase in the CLBR with the number of oocytes retrieved, others have found a plateau. A systematic review was conducted, including all eligible studies published until June 2022, to determine the optimal number of oocytes retrieved to maximize the CLBR. We found a positive association between the number of oocytes and the CLBR. However, this association varies according to patients' age. While in patients younger than 35 years, little benefit is derived from increasing the number of oocytes above 25-30, in patients older than 35 years, the number of oocytes seems to improve the CLBR until the extreme of reproductive age is reached. In women aged 44 years or older, the CLBR will be consistently low, independent of the number of oocytes retrieved., Competing Interests: Conflict of interest N.P.P. reports grants and/or personal fees from MSD, Merck Serono, Roche Diagnostics, Ferring International, Besins Healthcare, Gedeon Richter, Theramex, and Institut Biochimique SA (IBSA). A.R.N, P.M.B and N.S.G report no conflict of interests., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Background & Aims: Although appendectomy may reduce colorectal inflammation in patients with ulcerative colitis (UC), this surgical procedure has been suggested to be associated with an increased risk of colitis-associated cancer (CAC). Our aim was to explore the mechanism underlying the appendectomy-associated increased risk of CAC., Methods: Five-week-old male BALB/c mice underwent appendectomy, appendicitis induction, or sham laparotomy. They were then exposed to azoxymethane/dextran sodium sulfate (AOM/DSS) to induce CAC. Mice were killed 12 weeks later, and colons were taken for pathological analysis and immunohistochemistry (CD3 and CD8 staining). Human colonic tumors from 21 patients with UC who underwent surgical resection for CAC were immunophenotyped and stratified according to appendectomy status., Results: Whereas appendectomy significantly reduced colitis severity and increased CAC number, appendicitis induction without appendectomy led to opposite results. Intratumor CD3+ and CD8+ T-cell densities were lower after appendectomy and higher after appendicitis induction compared with the sham laparotomy group. Blocking lymphocyte trafficking to the colon with the anti-α4β7 integrin antibody or a sphingosine-1-phosphate receptor agonist suppressed the inducing effect of the appendectomy on tumors' number and on CD3+/CD8+ intratumoral density. CD8+ or CD3+ T cells isolated from inflammatory neo-appendix and intravenously injected into AOM/DSS-treated recipient mice increased CD3+/CD8+ T-cell tumor infiltration and decreased tumor number. In UC patients with a history of appendectomy, intratumor CD3+ and CD8+ T-cell densities were decreased compared with UC patients without history of appendectomy., Conclusions: In UC, appendectomy could suppress a major site of T-cell priming, resulting in a less efficient CAC immunosurveillance., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Cholangiocarcinomas (CCA) are heterogeneous tumors that arise from epithelial cells of the biliary tract. They represent the second primary liver malignancy, after hepatocellular carcinoma. Recent epidemiological data show an increased incidence of intrahepatic CCA without any identified causes. According to their location on the biliary tract, intrahepatic, perihilar (p) and distal (d) CCA can be individualized. Intrahepatic CCA (iCCA) are subdivided into small duct type iCCA and large duct type iCCA, according to the level or size of the biliary duct affected. These two subgroups are characterized by distinct risk factors, gross aspect, histopathological and molecular features, and therapeutic management. The role of biopsy in iCCA is to confirm the diagnosis and to eliminate various differential diagnostics, in particular, metastases. In p/d CCA, biopsy requires more invasive approaches, and tissue samples are difficult to obtain, leading to a high rate of false negatives. In this review, we will discuss the different classifications of CCA (anatomical and macroscopic). We will describe the various microscopic and phenotypic subtypes of CCA. Finally, we will deal with their mode of extension, the role of biopsy and pre-neoplastic lesions.

Background and Aims: Zinc finger E-box binding homeobox 1 (ZEB1) is a transcription factor that promotes metastatic and stem cell features, which has been associated with poor prognosis in cholangiocarcinoma (CCA), a desmoplastic cancer enriched in cancer-associated fibroblasts (CAFs). We aimed to define ZEB1 regulatory functions in malignant and stromal compartments of CCA., Approach and Results: Bioinformatic and immunohistochemical analyses were performed to determine correlations between ZEB1 and markers of progressiveness in human intrahepatic CCA (iCCA). Gain-of-function and loss-of-function models were generated in CCA cells and liver myofibroblasts as a model of CAFs. Conditioned media (CM) was used to unravel tumor-stroma interplay. In vivo experiments were performed using a xenograft CCA model. ZEB1 expression in tumor cells of human iCCA was associated with undifferentiated tumor and vascular invasion. In vitro, ZEB1 promoted epithelial-mesenchymal transition and stemness in tumor cells, leading to cell migration and spheroid formation. In vivo, ZEB1-overexpressing CCA cells formed larger tumors with more abundant stroma. Expression of cellular communication network factor 2 (CCN2, encoding connective tissue growth factor [CTGF]) was increased in tumor cells from ZEB1-overexpressing xenografts and correlated with ZEB1 expression in human tumors. In vitro, CM from ZEB1-overexpressing tumor cells or recombinant CTGF induced myofibroblast proliferation. ZEB1 was also expressed by CAFs in human CCA, and its expression correlated with CCN2 in myofibroblasts and CCA stroma. In mice, cotransplantation of CCA cells with ZEB1-depleted myofibroblasts reduced CCA progressiveness compared to CCA cells/ZEB1-expressing myofibroblasts. Furthermore, ZEB1 controls the expression of paracrine signals (i.e., HGF and IL6) in tumor cells and myofibroblasts., Conclusions: ZEB1 plays a key role in CCA progression by regulating tumor cell-CAF crosstalk, leading to tumor dedifferentiation and CAF activation., (© 2021 by the American Association for the Study of Liver Diseases.)