Les atteintes oto-rhino-laryngologiques (ORL) sont parmi les plus fréquentes des manifestations observées dans les maladies systémiques ou auto-immunes. Certaines d'entre elles ont une expression ORL prédominante, c'est le cas de la polychondrite atrophiante ou de certains vascularites à ANCA : granulomatose avec polyangéite (Wegener) et syndrome de Churg-Strauss. Pour de nombreuses autres maladies, l'atteinte ORL est plus rare mais vient parfois révéler la maladie et poser des problèmes diagnostiques parfois difficiles. Une coopération entre spécialiste ORL, rhumatologues et internistes est indispensable.Ear nose and throat (ENT) manifestations are amongst the most common clinical features of systemic and auto-immune disorders. In some of them including relapsing polychondritis, granulomatosis with polyangeitis (Wegener's) or Churg-Strauss syndrome, ENT involvement is part of the disease. In many others, ENT manifestations are less common but may be the presenting features and represent a diagnostic challenge. Collaboration between ENT specialists, rheumatologists and general internists is essential to avoid diagnostic delay and improve patients' care.

Introduction: Aggressive angiomyxoma (AA) is a rare benign soft tissue tumour usually affecting the pelvis and perineum of young women. Magnetic resonance imaging (MRI) is crucial in the management of AA patients for its diagnostic contribution and for the preoperative assessment of the actual tumour extension. Given the current development of less aggressive therapeutics associated with a higher risk of recurrence, close follow-up with MRI is fundamental after treatment. In this context, diffusion-weighted (DW) imaging has already shown high efficacy in the detection of early small relapses in prostate or rectal cancer. Case Report: We report here a case of pelvic AA in a 51-year-old woman examined with dynamic contrast enhancement and DW-MRI, including apparent diffusion coefficient mapping and calculation. Conclusions: To our knowledge, this is the first description of DW-MRI in AA reported in the literature. Here, knowledge about imaging features of AA will be reviewed and expanded.

Les histiocytoses sont des maladies hétérogènes actuellement séparées en deux grands groupes. Les histiocytoses non langerhansiennes sont souvent des maladies à expression cutanée dont le pronostic est bon. Les histiocytoses langerhansiennes regroupent les maladies de Hand-Shüller-Christian, Letterer-Siwe et le granulome éosinophile. Leur diagnostic repose sur l'aspect histologique (présence d'un infiltrat histiocytaire), sur les marqueurs immunologiques (marquage par l'anticorps monoclonal anti-CD1a [OKT6] et l'anticorps reconnaissant la protéine S-100) et sur l'aspect en microscopie électronique (présence de granules de Birbeck dans le cytoplasme). Leur pronostic dépend de l'âge du patient, de l'extension de la maladie, de la survenue d'une dysfunction d'un organe. L'hypothèse de l'origine néoplasique de certaines histiocytoses est confortée par la démonstration récente d'une prolifération histiocytaire clonale. Leur traitement comprend en général l'association de corticoïdes et d'une chimiothérapie. Différents protocoles de chimiothérapie ont été utilisés ou sont en cours d'évaluation.Histiocytic disorders are a group of heterogeneous diseases. A logical classification can be based on the type of proliferating cell, either monocyte-macrophage or Langerhans/ dendritic cell, and depends whether the proliferating cells are "reactive" or malignant. The classification now mainly depends on the histological examination. Regarding Langerhans cell hisliocytosis (Hand-Schüller-Christian disease, Letterer-Siwe disease and eosinophilic granuloma), the diagnosis, suspected on various clinical signs, is confirmed with histological examination showing infiltration with CDI positive histiocytes disclosing intracytoplasmic Birbeck granules at electron microscopic examination. The prognosis depends on the patient 's age at onset and the extension of the disease. Treatment is based on chemotherapy and corticotherapy.

L’examen histologique et l’immunohistochimie restent les outils de base du diagnostic et de la classification des sarcomes. Cependant, les outils de biologie moléculaire, FISH et RT-PCR essentiellement, permettent d’enrichir l’analyse du pathologiste: la mise en évidence d’une anomalie moléculaire spécifique permet dans certains cas de confirmer le diagnostic, d’éliminer un diagnostic différentiel et parfois de recueillir des informations pronostiques. Quinze pour cent des sarcomes présentent une translocation spécifique: remaniements des gènes SS18 (SYT) [synovialosarcome], DDIT3 (CHOP) [liposarcome myxoïde], FUS (TLS) [sarcome fibromyxoïde de bas grade, liposarcome myxoïde], FOXO1 (FHKR) [rhabdomyosarcome alvéolaire], PDGFB [dermatofibrosarcome de Darier-Ferrand], ALK [tumeur myofibroblastique inflammatoire] notamment. La recherche d’une amplification de MDM2 est un outil sensible et spécifique pour le diagnostic des tumeurs adipeuses atypiques/liposarcomes bien différenciés et des liposarcomes dédifférenciés. Les anomalies du gène EWSR1 sont moins spécifiques, car présentes dans de nombreuses tumeurs des tissus mous-sarcome d’Ewing, tumeur desmoplastique à cellules rondes, sarcome à cellules claires, liposarcome myxoïde, myoépithéliome. Certaines anomalies moléculaires sont retrouvées dans plusieurs types tumoraux soulignant l’importance d’intégrer les résultats de toute recherche moléculaire au contexte clinique, morphologique et immunohistochimique: translocation ASPSCR1 (ASPL)-TFE3 dans les sarcomes alvéolaires des tissus mous et les carcinomes rénaux juvéniles, translocation ETV6(TEL)-NTRK3 (TRKC) dans les fibrosarcomes congénitaux et les carcinomes sécrétoires du sein, remaniement d’ALK dans les tumeurs myofibroblastiques inflammatoires, certains lymphomes anaplasiques et adénocarcinomes pulmonaires. Dans certains cas, l’immunohistochimie peut mettre en évidence la conséquence protéique d’une anomalie moléculaire: surexpression de MDM2 dans les tumeurs adipeuses atypiques/liposarcomes bien différenciés et dédifférenciés, perte d’expression de SMARCB1 (INI1, hSNF5) dans les tumeurs rhabdoïdes et les sarcomes épithélioïdes, surexpression d’ALK dans les tumeurs myofibroblastiques inflammatoires.

e18556 Background: The prognosis of patients with relapsed/refractory (R/R) T-cell lymphoma (TCL) remains very poor. Novel therapeutic approaches are needed to further improve outcomes. Lenalidomide (LEN) is an immunomodulatory drug that has demonstrated efficacy in several lymphoid malignancies. Recent trial of Lenalidomide in R/R Tcell lymphoma showed some clinical activity with 30% ORR and median PFS of 96 d. Unfortunately no CR was obtained. This limited activity might be improved by combining of LEN with other active drugs. Recently it was confirmed that dexamethasone (DEX) enhances antiproliferative activity of LEN in myeloma and lymphoma cells. At the same time DEX antagonized the immunostimulatory effects of LEN, but in a dose-dependent manner. Methods: We report 2 patients with R/R TCL in whom the combination LEN+steroids permitted to obtain durable responses. Results: First patient was the 32 y.o. male with heavily pre-treated (5 lines) refractory ALK+anaplastic LCL. Patient received 18 cycles of LEN 25 mg/d for 21/28 d with DEX 10 mg/w (first 6 cycles), than with Prednisolone 20-30 mg/d for next 12 cycles with obtaining good clinical and partial radiological response. Because of psycotics episodes steroids were stopped for 2-3 weeks on several occasions. Cessation of steroids was accompanying by reappearance of B-sympthoms and peripheral lymphoadenopathy. Reintroduction of steroids was accompanied by rapid and complete disappearance of symptoms. Second patient was 63 y.o. man with R/R T angioimmunoblastic lymphoma progressing after 2 lines of chemotherapy (8 RCHOP+7 RDHAC in 16 months).Treatment with LEN 15 mg 21/28 days + weekly DEX 20 mg induced PET-negative CR after 4 cycles with remission duration of 9 months. In both patients clinical response was observed after the first month of treatment. Conclusions: It is possible that in R/R TCL the combination LEN + low dose steroids leeds to synergy of cytotoxic and immunomodulatory effects. Considering the rapid onset of response do we have to integrate less toxic and probably more efficacious new strategies at an earlier stade?

Introduction: Au cours des dix dernières années, la ponction sous écho-endoscopie (PSEE) a démontré son efficacité dans le diagnostic des masses péri-digestives. Cette technique, peu invasive, est souvent contributive au diagnostic grâce à la complémentarité des informations apportées par le matériel micro biopsique et le matériel cytologique. Il existe plusieurs aiguilles de calibres différents, dont le choix est dicté par la localisation et la taille de la tumeur. Il s'agit ici d'évaluer une nouvelle aiguille de 19 gauges (G) „Core„ dans le cadre de 29 biopsies.

Le blastome pulmonaire est une tumeur biphasique rare du poumon à haut grade de malignité et associe 3 sous-types: le blastome pulmonaire biphasique (BPB), l’adénocarcinome fœtal et le blastome pleuropulmonaire. Le BPB est le plus fréquent.Nous rapportons le cas d’un patient âgé de 48 ans, tabagique sevré, qui consulte pour douleurs thoraciques gauches et hémoptysie. L’examen physique est normal. La radiographie du thorax montre une opacité bilobée, homogène, mal limitée du lobe supérieur gauche. La fibroscopie bronchique montre des traces de saignement au niveau d’une segmentaire de la bronche culminale. Le scanner thoraco-abdomino-pelvien montre une masse tissulaire culminale de 7 cm de grand axe associée à des nodules pulmonaires bilatéraux. Par ailleurs, le bilan d’extension ne montre pas de localisations secondaires. Le patient a eu une lobectomie supérieure gauche avec curage ganglionnaire. L’étude anatomopathologique de la pièce opératoire met en évidence une prolifération tumorale maligne biphasique nécrosée, comportant une double composante épithéliale glandulaire et mésenchymateuse immatures de type blastomateuse. À l’étude immuno-histochimique, les cellules épithéliales expriment la cytokératine et le TTF1 et les cellules mésenchymateuses expriment la vimentine et l’actine muscle lisse. La tumeur infiltre la plèvre viscérale mais sans atteinte de la plèvre pariétale, ni des ganglions. Il s’agit d’un blastome pulmonaire biphasique (BPB) classé pT3N0M0. Aucun traitement adjuvant n’a été associé. Le contrôle tomodensitométrique montre une stabilité des nodules sous-pleuraux avec un recul de 5 ans.Notre observation clinique souligne l’apport des études immuno-histochimiques dans le diagnostic positif du blastome pulmonaire.Pulmonary blastoma, a rare primary lung malignancy is subdivided in 3 categories: well-differentiated fetal adenocarcinoma (WDFA), classic biphasic pulmonary blastoma (CBPB) and pleuropulmonary blastoma (PPB). Classic pulmonary blastoma is composed of a mixture of immature epithelial and mesenchymal tissue resembling fetal lung tissue.We described a case of a 48-year-old male, cigarette smoker, who presented with left thoracic pain and hemoptysis for 2 months. Chest radiography showed a well-delimited, homogeneous 4 cm mass in the left lung periphery. Bronchoscopic examination revealed left endobronchial bleeding. Computed tomography of the chest revealed a tumor shadow measuring 7 cm in the left upper lobe and bilateral nodules with no lymphadenopathy. A systemic evaluation demonstrated no metastatic lesion. Patient underwent a left upper lobectomy. The diagnosis of CBPB was affirmed on anatomopathology of the tumor resection. Immunohistochemical studies showed that tumor cells were positive for vimentin, desmin, actin, Pan Cytokeratin and TTF-1. The final diagnosis was BPB classified as pathological T3N0M0 and no adjuvant treatment was associated. The patient showed good objective response with no evidence of disease recurrence still in 5 years surgery resection.This case reiterates the importance of pathomorphological or immunohistochemical features in diagnosis of BPB.

Mediastinal lymphomas are mainly aggressive tumors affecting young patients. Three main entities summarize this pathology: T lymphoblastic lymphoma, mediastinal (thymic) diffuse large B cell lymphoma, and classical Hodgkin lymphoma. Their diagnosis is usually performed on tissue collected by mediastinoscopy and requires the implementation of techniques such as classical histopathology, immunohistochemistry, and sometimes molecular biology. (C) 2010 Published by Elsevier Masson

Mediastinal germ cell tumors are rare tumors. It is classic to divide those tumors into two categories, seminomas and nonseminomatous germ cell tumors: teratomas (mature or immature), embryonal carcinomas, yolk sac tumors, and choriocarcinomas. Each histological sub-type can be associated to another sub-type that realise a so-called mixed germ cell tumor. Diagnosis strategy is currently well codified for malignant mediastinal germ cell tumors. It greatly benefits from tumoral markers (alpha-fetoprotein and beta human chorionic gonadotrophin). For instance, the treatment strategy still raises some specific problems to each histological type. The treatment of seminomatous tumors is standardised--chemotherapy/surgery on residual tumor greater than 3 cm/radiotherapy on viable persistent residual tumors--and provides very satisfying results. As for the nonseminomatous germ cell tumors, the situation is dramatically different. The treatment strategy is less standardised--association of chemotherapy and surgery--and the prognosis is very severe.

BACKGROUND:Primary cutaneous B-cell lymphomas form a heterogeneous group of lymphoid proliferations found on the skin. We report a case of primary leg-type cutaneous large B-cell lymphoma occurring on the site a previous leg burn. A few rare cases of cutaneous lymphoma forming on burn scars have been described, but these concern primary cutaneous lymphomas of the T-cell phenotype. CASE REPORT:An 85-year-old man with a history of a burn to the left leg 17 years ago, previously treated with several skin grafts, presented numerous ulcerative budding lesions on the scar area. Histological examination of the skin biopsy revealed the existence in the skin ulcers of atypical large lymphoid cells having an immunoblastic or centroblastic morphology and shown by immunohistochemistry to be of the B-cell phenotype, thereby evoking a diagnosis of large B-cell lymphoma. The lymphoma cells were positive for MUM1/IRF4 and BCL2, and more weakly for BCL6, but negative for CD10. The staging examination revealed only cortical lysis of the left tibia. Temporary initial regression was achieved by polychemotherapy comprising cyclophosphamide, vincristine and prednisone in combination with rituximab. DISCUSSION:This case is novel in that it involves primary large B-cell lymphoma, leg type, occurring on burn scar tissue. Venous insufficiency and lymphatic stasis have already been incriminated in the genesis of this type of lymphoma; the prior injury and resulting immune dysregulation at the burn site may have also contributed to the development of this neoplasia.

Primary cutaneous B-cell lymphomas form a heterogeneous group of lymphoid proliferations found on the skin. We report a case of primary leg-type cutaneous large B-cell lymphoma occurring on the site a previous leg burn. A few rare cases of cutaneous lymphoma forming on burn scars have been described, but these concern primary cutaneous lymphomas of the T-cell phenotype.An 85-year-old man with a history of a burn to the left leg 17 years ago, previously treated with several skin grafts, presented numerous ulcerative budding lesions on the scar area. Histological examination of the skin biopsy revealed the existence in the skin ulcers of atypical large lymphoid cells having an immunoblastic or centroblastic morphology and shown by immunohistochemistry to be of the B-cell phenotype, thereby evoking a diagnosis of large B-cell lymphoma. The lymphoma cells were positive for MUM1/IRF4 and BCL2, and more weakly for BCL6, but negative for CD10. The staging examination revealed only cortical lysis of the left tibia. Temporary initial regression was achieved by polychemotherapy comprising cyclophosphamide, vincristine and prednisone in combination with rituximab.This case is novel in that it involves primary large B-cell lymphoma, leg type, occurring on burn scar tissue. Venous insufficiency and lymphatic stasis have already been incriminated in the genesis of this type of lymphoma; the prior injury and resulting immune dysregulation at the burn site may have also contributed to the development of this neoplasia.