[ENT benign lesions and pseudo-tumors: Case No. 2].

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Lésions bénignes et pseudo-tumeurs en ORL : cas no 1

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Adénocarcinomes nasosinusiens (étude clinique, histologique et phénotypique de 41 patients)

MONTPELLIER-BU Médecine UPM (341722108) / SudocPARIS-BIUM (751062103) / SudocMONTPELLIER-BU Médecine (341722104) / SudocSudocFranceF

Sarcomes des fosses nasales et des sinus (Une étude clinicopathologique de 48 cas)

Dans le tractus naso-sinusien, une grande variété de sarcomes a été observée, mais chaque entité a été décrite séparément. Nos objectifs étaient d'obtenir des informations épidémiologiques sur les sarcomes des fosses nasales et des sinus de l'adulte, et de définir des critères pronostiques de ces tumeurs. 48 cas de sarcomes des fosses nasales et des sinus, de l'adulte âgé d'au moins 18 ans, ont été inclus. Pour chaque patient le type histologique était déterminé ainsi que le grade de la tumeur. Les survies globales (OS), sans récidive locale (LRFS) et sans métastase (MFS) ont été déterminées. 9 variables ont été étudiées pour leur valeur pronostique. Les types histologiques de sarcomes des fosses nasales et des sinus les plus fi'équemment rencontrés étaient les suivants: rhabdomyosarcomes alvéolaires (RMSA) (33.3%), rhabdomyosarcomes embryonnaires (RMSE) (14.6%), sarcomes inclassés (14.6%), léiomyosarcomes (l2.5%).L'OS, la MSF et la LRFS à 5 ans étaient de 62.3%,73% et 88.8%. L'histotype était le facteur prédictifle plus significatif, avec un pronostique plus péjoratif pour le groupe des rhabdomyosarcomes (RMS).Les facteurs prédictifs d'une réponse incomplète étaient le sous type RMS et l'absence de chirurgie. Il n'existait pas de différence pronostique significative entre RMSA et RMSE. Nos résultats montrent que l'histotype est le facteur pronostique le plus significatif des sarcomes des fosses nasales et des sinus, avec un pronostic péjoratif associé aux RMS, sans différence entre les RMSA et RMSE. La chirurgie quelque soit la qualité des berges d'exérèse est associée à une rémission complète et devrait donc toujours être envisagée devant un sarcome nasosinusien.MONTPELLIER-BU Médecine UPM (341722108) / SudocSudocFranceF

Tumeurs nasosinusiennes à translocation

International audienceIn recent years, several nasal cavity and sinus entities have been described with fusion genes. Salivary gland tumors with fusion genes will not be discussed in this article, but it should be kept in mind that accessory salivary glands are present in the nasal cavity and sinuses and can therefore lead to tumoral lesions. Entities with specific or more frequently described rearrangements in the nasal cavities and sinuses are DEK::AFF2 squamous cell carcinomas,;non-intestinal and non-salivary nasosinusal adenocarcinomas, some of which displaying ETV6 gene rearrangements; biphenotypic nasosinusal sarcomas, most of which displaying PAX3 gene rearrangements; and Ewing's adamantinoma-like sarcomas, which display the same rearrangements as conventional Ewing's sarcomas, mainly the EWSR1::FLI1 rearrangement. Each entity will be described morphologically, immunohistochemically, and prognostically

Cholangiocarcinoma occurring in a patient with type 1 Gaucher disease treated with velaglucerase alfa enzyme replacement therapy: First case report

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A 37 mm Spindle Cell Lipoma on the Floor of the Mouth

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Decompression: a first-intention treatment for “large” non-syndromic odontogenic keratocysts

Though odontogenic keratocysts (OKCs) are benign lesions, they have a high recurrence rate. Because of their aggressive behavior, they have been classified as tumors by the WHO until 2017. Main differential diagnoses are amelobastoma and dentigerous cyst. Anatomopathological examination can reach a final diagnosis. Several treatments have been proposed: curettage, resection, enucleation (alone or together with peripheral ostectomy) and decompression. Decompression aims to decrease the volume of the lesion of “large” OKCs, in order to prevent surgery-related fractures and to preserve the surrounding important anatomical structures such as the inferior alveolar nerve. It could lead to a complete regression. If not, secondary enucleation can be performed in better conditions: a reduced volume to remove, a thicker epithelium to detach, a lower risk to damage neighboring anatomic structures and a lower recurrence rate. Long-term follow-up however remains necessary. Nowadays, minimally invasive surgery prevails. And since OKC was returned into the odontogenic cysts group in the WHO classification, decompression should be considered as the first intention treatment. The purpose of this paper is to provide an update about OKC features and biological mechanisms, to review the different treatment options and to provide a step-by-step protocol for decompression

The immune contexture of primary central nervous system diffuse large B cell lymphoma associates with patient survival and specific cell signaling

International audienceRationale: Primary central nervous system diffuse large B-cell lymphoma (PCNSL) is a rare and aggressive entity that resides in an immune-privileged site. The tumor microenvironment (TME) and the disruption of the immune surveillance influence lymphoma pathogenesis and immunotherapy resistance. Despite growing knowledge on heterogeneous therapeutic responses, no comprehensive description of the PCNSL TME is available. We hence investigated the immune subtypes of PCNSL and their association with molecular signaling and survival. Methods: Analysis of PCNSL transcriptomes (sequencing, n = 20; microarrays, n = 34). Integrated correlation analysis and signaling pathway topology enabled us to infer intercellular interactions. Immunohistopathology and digital imaging were used to validate bioinformatic results. Results: Transcriptomics revealed three immune subtypes: immune-rich, poor, and intermediate. The immune-rich subtype was associated to better survival and characterized by hyper-activation of STAT3 signaling and inflammatory signaling, e.g., IFNγ and TNF-α, resembling the hot subtype described in primary testicular lymphoma and solid cancer. WNT/β-catenin, HIPPO, and NOTCH signaling were hyper-activated in the immune-poor subtype. HLA down-modulation was clearly associated with a low or intermediate immune infiltration and the absence of T-cell activation. Moreover, HLA class I down-regulation was also correlated with worse survival with implications on immune-intermediate PCNSL that frequently feature reduced HLA expression. A ligand-receptor intercellular network revealed high expression of two immune checkpoints, i.e., CTLA-4/CD86 and TIM-3/LAGLS9. TIM-3 and galectin-9 proteins were clearly upregulated in PCNSL. Conclusion: Altogether, our study reveals that patient stratification according to immune subtypes, HLA status, and immune checkpoint molecule quantification should be considered prior to immune checkpoint inhibitor therapy. Moreover, TIM-3 protein should be considered an axis for future therapeutic development

Composite cutaneous lymphoma of diffuse large B-cell lymphoma-leg type and subcutaneous panniculitis-like T-cell lymphoma

International audienceComposite lymphoma (CL) is a rare disease defined by the occurrence of two distinct lymphomas within a single tissue at the same time. We present the case of an 89-year-old male with a clinical history of immunoglobulin M monoclonal gammopathy of undetermined significance. The patient presented cutaneous eruption of nodules on the right bottom and arm. An excisional biopsy revealed cutaneous infiltration composed of two components. The first one consisted of large B-cells with CD20+/MUM1+/BCL2+ phenotype whereas the second one involved the subcutaneous fat in a panniculitic manner, and was CD3+/CD8+/granzyme B+/TCRβF1+. The final diagnosis was CL of primary cutaneous large B-cell lymphoma-leg type (PCLBCL-leg type) and subcutaneous panniculitis-like T-cell lymphoma (SPTCL). We report and characterize for the first time coexistent PCLBCL-leg type and SPTCL in a patient