Lymphomes malins non-hodgkiniens primitifs du système nerveux central (utilité pronostique des classifications anatomo-pathologiques)

Les LPSNC constituent une forme rare de LMNH de localisation extra-ganglionnaire dont l'atteinte est confinée au système nerveux central, aux méninges et à l'œil, sans localisation systémique associée. Leur incidence a fortement augmentée au cours des trois dernières décennies, en particulier chez les sujets immunocompétents. Morphologiquement, la grande majorité des LPSNC sont des LMNH diffus à grandes cellules B (DLBCL). Cependant, ils diffèrent des DLBCL ganglionnaires par un pronostic plus péjoratif. Les DLBCL sont une entité hétérogène et des classifications en sous-groupes pronostiques ont été proposées à partir de nouvelles découvertes génomiques. Deux algorithmes pronostiques ont ensuite été élaborés à l'aide de marqueurs immunohistochimiques (CD10, Bc16, MUM1, Bc12) classant les DLBCL en sous-groupes GCB ou ABC et en G1 ou G2. A partir d'une étude rétrospective de 41 patients immunocompétents atteints d'un LPSNC diagnostiqués au C.H.U. De Saint-Etienne entre 1995 et 2007, nous avons réalisé une analyse descriptive et statistique de la survie en appliquant conjointement ces deux classifications, pour en apprécier l'utilité et la pertinence pronostique. 70.8% de nos LPSNC étaient de phénotype ABC, correspondant à une origine post-germinative activée. Ils se répartissent de façon homogène en G1 et G2. Le taux de survie globale était de 34.8% à 2 ans et de 19.6% à 5 ans. L'âge <65 ans et l'association thérapeutique CT/RT améliorait significativement la survie de nos patients. Les autres marqueurs cliniques et biologiques testés n'avaient aucun impact pronostique. Les deux classifications n'ont pas montré de différence significative sur la survie. La découverte récente d'une signature transcriptionnelle propre aux LPSNC les différenciant des DLBCL expliquerait que ces classifications pronostiques ne s'appliquent pas aux LPSNC.ST ETIENNE-BU Médecine (422182102) / SudocSudocFranceF

Primary epithelioid sarcoma of bone: report of a unique case, with immunohistochemical and fluorescent in situ hybridization confirmation of INI1 deletion.

International audienceWe report the clinical and pathologic features of, what is to the best of our knowledge, the first case of epithelioid sarcoma of bone. A 31-year-old woman with an unremarkable past medical history presented with pelvic pain and was found by computed tomography scan to have a destructive 5 cm, partially calcified intraosseous lesion of the iliac bone. Histologically, the tumor consisted of relatively uniform but clearly malignant-appearing epithelioid cells, with scattered rhabdoid-appearing cells. A hyalinized to partially calcified matrix was present between the tumor cells, with a "chickenwire" pattern of calcification. By immunohistochemistry, the neoplastic cells expressed cytokeratins, vimentin, epithelial membrane antigen and CD34, and showed complete loss of INI1 protein expression. Fluorescence in situ hybridization showed homozygous deletion of the INI1 gene. An extensive clinical and radiographic workup did not show evidence of a soft tissue tumor, and the diagnosis of a primary epithelioid sarcoma of bone was made. After this, the patient underwent a complete resection of her tumor, and is currently disease free, 6 months after surgery. These extremely rare tumors must be rigorously distinguished from other more common tumors of bone, in particular, chondroblastoma and osteosarcoma. Awareness that epithelioid sarcoma may occur in bone, careful histologic evaluation and ancillary immunohistochemistry for epithelial markers, CD34 and INI1 protein should allow for recognition of such tumors. Study of additional cases of primary epithelioid sarcoma of bone will be necessary to better understand its clinical behavior

Aortic Event Rate in the Marfan Population: A Cohort Study

International audienceOptimal management, including timing of surgery, remains debated in Marfan syndrome because of a lack of data on aortic risk associated with this disease

Surgical management of patients with Marfan syndrome: Evolution throughout the years

International audienceTo evaluate the evolution of surgical management in a large population of patients with Marfan syndrome

An Overview of Marine Non-Indigenous Species Found in Three Contrasting Biogeographic Metropolitan French Regions: Insights on Distribution, Origins and Pathways of Introduction

Biological invasions are one of the main global threats to biodiversity in terrestrial, freshwater and marine ecosystems worldwide, requiring effective inventorying and monitoring programs. Here, we present an updated list of non-indigenous species in French marine and transitional waters. Focused on eukaryote pluricellular species found throughout the three metropolitan French marine regions (Western Mediterranean Sea, Bay of Biscay and the Northern Seas), a total of 342 non-indigenous, including 42 cryptogenic, species are listed as having been introduced since the 13th century. The majority of the species originated from the temperate Northern Pacific. They mainly arrived through both ballast and hull fouling and also are associated with shellfish farming activities. Most of them have been introduced since the 1970s, a time when maritime and aquaculture trade intensified. Despite important human-aided opportunities for species transfer between the three marine regions (for instance, via recreational boating or aquaculture transfers), only a third of these NIS are common to all regions, as expected due to their environmental specificities

An overview of marine non-indigenous species found in three contrasting biogeographic metropolitan French regions: Insights on distribution, origins and pathways of introduction

International audienceBiological invasions are one of the main global threats to biodiversity in terrestrial, freshwater and marine ecosystems worldwide, requiring effective inventorying and monitoring programs. Here, we present an updated list of non-indigenous species in French marine and transitional waters. Focused on eukaryote pluricellular species found throughout the three metropolitan French marine regions (Western Mediterranean Sea, Bay of Biscay and the Northern Seas), a total of 342 non-indigenous, including 42 cryptogenic, species are listed as having been introduced since the 13th century. The majority of the species originated from the temperate Northern Pacific. They mainly arrived through both ballast and hull fouling and also are associated with shellfish farming activities. Most of them have been introduced since the 1970s, a time when maritime and aquaculture trade intensified. Despite important human-aided opportunities for species transfer between the three marine regions (for instance, via recreational boating or aquaculture transfers), only a third of these NIS are common to all regions, as expected due to their environmental specificities

Scedosporiosis/lomentosporiosis observational study (SOS): Clinical significance of Scedosporium species identification

International audienceScedosporiosis/lomentosporiosis is a devastating emerging fungal infection. Our objective was to describe the clinical pattern and to analyze whether taxonomic grouping of the species involved was supported by differences in terms of clinical presentations or outcomes. We retrospectively studied cases of invasive scedosporiosis in France from 2005 through 2017 based on isolates characterized by polyphasic approach. We recorded 90 cases, mainly related to Scedosporium apiospermum (n = 48), S. boydii/S. ellipsoideum (n = 20), and Lomentospora prolificans (n = 14). One-third of infections were disseminated, with unexpectedly high rates of cerebral (41%) and cardiovascular (31%) involvement. In light of recent Scedosporium taxonomic revisions, we aimed to study the clinical significance of Scedosporium species identification and report for the first time contrasting clinical presentations between infections caused S. apiospermum, which were associated with malignancies and cutaneous involvement in disseminated infections, and infections caused by S. boydii, which were associated with solid organ transplantation, cerebral infections, fungemia, and early death. The clinical presentation of L. prolificans also differed from that of other species, involving more neutropenic patients, breakthrough infections, fungemia, and disseminated infections. Neutropenia, dissemination, and lack of antifungal prescription were all associated with 3-month mortality. Our data support the distinction between S. apiospermum and S. boydii and between L. prolificans and Scedosporium sp. Our results also underline the importance of the workup to assess dissemination, including cardiovascular system and brain. Lay Summary Scedosporiosis/lomentosporiosis is a devastating emerging fungal infection. Our objective was to describe the clinical pattern and to analyze whether taxonomic grouping of the species involved was supported by differences in terms of clinical presentations or outcomes