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Since 2010, the network of rare malignant tumors of the ovary (TMRG) was developed to optimize the management of patients, also allowing a histological second opinion of rare ovarian tumors. The aim of this work was to study the contribution of second opinion to improve histological diagnostic accuracy on ovarian rare malignant tumors included in the TMRG database. Histological data of patients diagnosed with a rare ovarian tumor included in TMRG network over a one-year period (2018) were collected. Initial diagnoses were compared with second opinion from national gynecological pathologist experts. The modalities of histological second opinion requests were studied, as well as the histological characteristics of the tumors. The discordances were classified as minor (if the modification of histological diagnosis did not change patient management) and major (if the patient management can be modified). Of 1185 included patients, 937 matched the inclusion criteria. Full concordance between primary diagnosis and expert second opinion was reached in 611 cases (65,3%), minor discordance was seen in 114 (12,2%) and major discordance in 209 (22,3%) of cases. In systematic review requested by the network, 26% (n = 137) of cases were reported with a change in histological diagnosis, while the change concerned 44% (n = 186) of cases for a second opinion spontaneously requested by the initial pathologist. The discrepancies concerned all categories of ovarian tumors, with a majority of mucinous tumors (43% of major discordances), followed by stromal and sex-cord tumors (13.8% of major discordances) and clear cell tumors (12,4% of major discordances). This analysis confirms the diagnostic difficulty of ovarian tumors, due to their rarity and morphological heterogeneity. French pathologists are aware of these difficulties and spontaneously refer ovarian tumors with unusual histology for a second opinion and collaborate with rare tumor networks for systematic review. • Systematic review of rare ovarian tumors identify minor and major histological discrepancies in all categories of ovarian tumors. • Systematic review of rare ovarian tumors has a direct implication in the management of patients. • Most of the major discrepancies relate to mucinous tumors, in evaluation of the degree of malignancy or in the distinction with a digestive metastasis. • The major discrepancies relating to sex cord tumors tumors mainly concern granulosa cell tumors in the distinction with fibroma or sertoli-leydig cell tumors. [ABSTRACT FROM AUTHOR]

Résumé L’œsophagite à éosinophiles (OE) est une affection récemment décrite, définie par une réponse immunitaire anormale de la muqueuse œsophagienne vis-à-vis d’allergènes exogènes se traduisant par une infiltration chronique de cette muqueuse par des polynucléaires éosinophiles > 15 polynucléaires éosinophiles par champ au fort grossissement (PNEo/CFG). Cette étude rétrospective avait pour but d’évaluer la prise en charge d’enfants atteints d’OE dans 5 centres hospitaliers de l’Ouest de la France, afin d’en montrer la disparité et d’améliorer les pratiques futures. Vingt-huit enfants âgés de 1,5 mois à 17 ans ont été inclus. Le blocage alimentaire avait été le symptôme le plus fréquemment rapporté (46 %). L’endoscopie s’était avérée macroscopiquement normale chez 29 % des patients : le diagnostic avait alors été posé sur les anomalies histologiques des biopsies. Le taux moyen d’éosinophiles dans la muqueuse œsophagienne était de 72,4 PNEo/CFG. Un immunomarquage centralisé avait permis d’identifier la présence de plasmocytes positifs à immunoglobulines G de type 4 (IgG4) chez 76 % des patients et une dégranulation intra-épithéliale d’IgG4 chez 14 % d’entre eux. L’évaluation de la prise en charge thérapeutique a identifié des divergences importantes entre les centres, une réévaluation endoscopique n’étant réalisée que chez 43 % des patients. Cette étude a permis d’objectiver l’hétérogénéité dans le diagnostic et la prise en charge des enfants atteints d’OE. Il paraît indispensable d’uniformiser les pratiques afin de mieux comprendre cette pathologie et d’en améliorer la prise en charge. La place des IgG4 comme nouveau marqueur diagnostique reste à préciser. Summary Eosinophilic esophagitis (EE) is a recent pathology defined by abnormal immune response of the esophageal mucosa to exogenous allergens, leading to chronic mucosa infiltration by 15 eosinophils per High-Power-Field (Eos/HPF). The present retrospective study was designed to assess the hospital care for children suffering from EE in several hospitals in western France in order to highlight discrepancies and improve future care. Twenty-eight children ranging from 1.5 months to 17 years old were included in the study. Episodes of food blockage were the most frequently reported symptoms (46 %). A ratio of 29 % of EE patients reported macroscopically normal endoscopy; diagnosis was then established upon histological anomalies found in biopsies. The mean eosinophil count was 72.4 Eos/HPF. Centralized immunohistochemical staining revealed the presence of IgG4-responding plasma cells in 76.5 % of patients, as well as IgG4 intraepithelial degranulation in 14 % of them. The evaluation of the treatment plan showed important inter-center discrepancies with only 43 % of patients receiving endoscopic reevaluation. This study objectively highlights heterogeneities in diagnosis and care provided to children suffering from EE. Therefore, improving the consistency of practices seems to be crucial to optimize the patients’ outcome. The role of IgG4 as a new diagnosis marker remains to be clarified. [ABSTRACT FROM AUTHOR]

Résumé Les cancers de l’endomètre sont découverts majoritairement à un stade précoce justifiant une retenue thérapeutique. La conduite à tenir thérapeutique est aujourd’hui fondée sur des classifications préopératoires basées sur les éléments histo-pronostiques de la tumeur, et son extension mesurée en imagerie médicale : en particulier le curage ganglionnaire dans les formes à risque bas et intermédiaire n’est plus systématiquement réalisé. Or, un certain nombre de patientes présente in fine une atteinte ganglionnaire justifiant probablement des thérapeutiques adaptées chirurgicales ou adjuvantes. La technique du ganglion sentinelle permettrait de compenser les faiblesses de l’évaluation pronostique préopératoire sous réserve d’un consensus sur sa réalisation technique, la définition de ses critères de qualité et la gestion de ses résultats en particulier en cas de métastases ganglionnaires de faible volume. The endometrial cancers are mainly discovered at an early stage justifying a less aggressive treatment. The therapeutic choices are today based on preoperative classifications themselves based on histo-prognostic factors of the tumor and its extension measured in MRI: consequently, lymph node dissection in case of low and intermediate risk cancer is not systematically achieved. But a number of patients have lymph node involvement finally, probably justifying an adapted surgical and adjuvant treatment. The technique of sentinel node would compensate the weaknesses of preoperative prognostic evaluation. This new operative technique needs to be precised and evaluated: the topics subject to discussion are the technical implementation, the definition of its quality standards and the management of its histological results especially in cases of low volume lymph node metastasis. [ABSTRACT FROM AUTHOR]