Aggressive angiomyxoma with diffusion-weighted magnetic resonance imaging and dynamic contrast enhancement: a case report and review of the literature.

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. CONCLUSION: 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.

[Mediastinal lymphomas: histopathology]. / Lymphomes médiastinaux: anatomie pathologique.

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.

[Mediastinal germ cell tumors: anatomopathology, classification, teratomas and malignant tumors]. / Les tumeurs germinales du médiastin: anatomopathologie, classification, tératomes et tumeurs malignes.

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.

[Cutaneous large B-cell leg-type lymphoma occurring on a leg burn]. / Lymphome B cutané a grandes cellules "de type jambe" sur cicatrice de brûlure.

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.

Multicystic peritoneal mesothelioma: report of three cases.

Multicystic peritoneal mesothelioma is a rare lesion occurring mainly in women in a reproductive age. Its pathogenesis is unclear. We report three cases of multicystic peritoneal mesothelioma in patients that were 28, 38 and 47 years of age (one male, two females). A history of abdominal surgery was reported in two cases. Explorative laparotomy was presumptive of a pseudomyxoma peritoni in two cases, and hyperthermic intraperitoneal chemotherapy was performed. Histological examination demonstrated multicystic lesions with mesothelial cells lining confirmed by immunohistochemical analysis. Unusual findings such as hyperplasia, hobnail features, cytoplasmic vacuolisation and papillary pattern were occasionally noted. The clinical presentation, pathogenesis and pathologic features including differential diagnosis of multicystic peritoneal mesothelioma are discussed.

A phase II trial of rituximab as adjuvant to intensive sequential chemotherapy in patients under 60 years with untreated poor-prognosis diffuse large B-cell lymphoma.

The potential benefit of rituximab as adjuvant to high-dose therapy (HDT) has been investigated in patients under 60 years with poor-risk (age-adjusted international prognostic index at 2-3) CD20+ diffuse large B-cell lymphoma (DLBCL). The treatment consisted of four cycles of high-dose CEOP (cyclophosphamide, epirubicin, vincristine, prednisone), plus etoposide and cisplatin during the two last cycles. Peripheral blood stem cells were collected after cycle 1, and reinfused after cycles 3 and 4. Four weekly rituximab infusions were subsequently delivered. Among the 36 patients included, 30 could complete chemotherapy schedule, and 24/36 received rituximab. A complete response occured in 26/36 patients (72%). With a median follow-up of 30 months, the estimated 5-year overall survival (OS) and event-free survival (EFS) rates (mean +/- s.d.) were 65 +/- 16 and 63 +/- 15%, respectively. For the 24 patients who received both chemotherapy and rituximab, the estimated 5-year OS and EFS rates were 86 +/- 14 and 82 +/- 15%. These data suggest that rituximab after HDT is feasible. Both complete remission rate and survival curves compare favorably with the poor outcome usually observed in high-risk DLBCL patients managed with HDT without rituximab.

[Acute respiratory distress syndrome following inhalation of barium sulfate]. / Syndrome de détresse respiratoire aiguë après inhalation de sulfate de baryum.

INTRODUCTION: Barium sulfate (BS) is chosen to explore swallowing disorders because of its reduced osmolality allowing no adverse reaction if aspirated in the bronchial tree. CASE REPORT: A 66-years old man treated for an advanced stage mesothelioma experienced a BS aspiration during an esophagography. He developed 3 days after an acute respiratory distress syndrome (ARDS) and deceased. The post-mortem examination revealed a diffuse alveolar damage (DAD). CONCLUSION: Whereas BS aspiration is generally well tolerated, serious adverse event as a DAD would exceptionally occurs. Thus, a close watch over respiratory symptoms has to be kept after BS administration, especially in debilitated and elderly patients.

Positive thyroid transcription factor 1 staining strongly correlates with survival of patients with adenocarcinoma of the lung.

This study investigated the relation between positive thyroid transcription factor 1 (TTF1) staining and survival of patients affected by primary adenocarcinoma (ADC) of the lung. Pathological tissue from consecutive ADC patients was collected from 2002 to 2004. The anti-TTF1 antibody (8G7G3/1, dilution of 1/200) was used. Thyroid transcription factor 1 staining was assessed for each tumour as positive or negative. Probability of survival was estimated by Kaplan-Meier and difference tested by log-rank test. A Cox's regression multivariate analysis was carried out. In all, 106 patients were studied (66% male, 69% PS0-1, 83% with stage III or IV). Tumours expressed positive TTF1 staining in 66% of cases. Multivariate analysis demonstrated an independent lower risk of death for patients whose tumour expresses positive TTF1 staining (HR = 0.51, 95% CI 0.30-0.85; P = 0.01) and higher grade of differentiation (HR = 0.40, 95% CI 0.24-0.68; P = 0.001). In conclusion, positive TTF1 staining strongly and independently correlates with survival of patients with primary ADC of the lung.

Expression of the beta chemokines CCL3, CCL4, CCL5 and their receptors in idiopathic inflammatory myopathies.

The idiopathic inflammatory myopathies (IIM) are a group of autoimmune diseases characterized by chronic lymphocytic and macrophagic infiltration in muscle. Because the mechanism for recruitment of these cells probably involves chemokines, we focused on the study of the expression pattern of some beta chemokines and receptors because it may provide a basis for selective immunotherapy. The expression of CCL3 (MIP-1alpha), CCL4 (MIP-1beta), CCL5 (RANTES) and their main receptors (CCR1 and CCR5) was studied by semi-quantitative reverse transcriptase polymerase chain reaction (RT-PCR) and immunohistochemistry in a series of 16 IIM and five controls (four normal muscles and one tonsil). Except for CCL5, strong expression was observed by RT-PCR with all molecules in all IIM subtypes in comparison to control muscle. Immunohistochemistry revealed diffuse CCL4 expression in all vessels in dermatomyositis. In both polymyositis and sporadic inclusion body myositis (s-IBM) it was restricted to vessels in the vicinity of inflammatory exudates. CCL5 expression was low, restricted to a few inflammatory cells in all IIM; CCR1 expression was mainly restricted to macrophages and s-IBM endothelial cells, whereas CCR5 was localized in inflammatory cells invading non-necrotic muscle fibres. Expressions of both receptors were also recorded in few muscle fibres. In conclusion, the upregulation of beta chemokines and receptors in IIM and their differential expression by various cells may contribute to chronic inflammation and to the peculiar distribution of inflammatory exudates in these diseases.

[2000 classification of idiopathic interstitial lung diseases]. / "Classification 2002 des pneumopathies interstitielles idiopathiques"

OBJECTIVES: Diagnosis of interstitial lung diseases was recently improved by the use of diagnostic tools, such as high-resolution Computed Tomography, and by new insights in their pathogenesis and histology. This led the American Thoracic Society and the European Respiratory Society to propose a new classification of these diseases, in the aim to facilitate early diagnosis and specific care. CURRENT KNOWLEDGE AND KEY POINTS: Standard radiography gives the first suspicion of chronic diffuse infiltrative lung disease, and anamnesis and physical examination are essential steps of etiological diagnosis. High-Resolution computed tomography confirms the diagnosis of diffuse infiltrative lung disease. Longitudinal lung function tests are essential to assess the consequences of the lung disease. Lung biopsies are often, but not systematically, a useful tool. The 2000 classification consists of seven entities of idiopathic interstitial diseases which are defined on clinical, radiological and pathological criteria: idiopathic pulmonary fibrosis, non-specific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, respiratory bronchiolitis associated interstitial lung disease, desquamative interstitial pneumonia and lymphoid interstitial pneumonia. The most frequent is Idiopathic Pulmonary Fibrosis, which has a poor prognosis. FUTURE PROSPECT AND PROJECTS: This new classification results from a multidisciplinary confrontation with chest physicians, radiologists and pathologists. A better characterization of anatomoclinical entities should lead to a better pronostic evaluation, more informative comparisons of published studies, and therefore to rational therapeutic approach.

[Surgery for oesophageal cancer: current controversies]. / Chirurgie du cancer de l'oesophage : controverses actuelles.

Any attempt to define the present role of surgery in the treatment of oesophageal cancer should integrate the dramatic changes that occurred within this disease over the last 2 decades: major shift in the histologic type of tumours, improved staging methods, spectacular reduction of operative risks, standardization of oncologic principles focusing on the completeness of resection, and development of multimodality therapeutic strategies. Surgery has still a pivotal role. Esophagectomy should be performed by trained surgeons in high-volume institutions. Radical surgery with en-bloc resection and 2 fields lymphadenectomy, should be encouraged in low-risk patients with subcarinal tumors. Although multimodality treatment strategy is commonly applied for locally advanced disease, few data support its superiority over surgical resection alone, followed by adjuvant therapy when appropriate. One may thus hypothesize that the risk/benefit ratio of such strategies is probably optimal in case of early stage tumors, and future studies may further clarify this issue. Conversely, locally advanced tumors, particularly those located in the upper mediastinum and the neck, may be managed alternatively without surgery. However, surgery remains an important tool to ensure optimal palliation of dysphagia, to achieve local control, and finally to improve quality of life. In that way, video-assisted techniques and/or trans hiatal approaches aiming to minimize the surgical insult may have a place in the treatment of patients who have substantially responded to induction therapy. Tumors located close to the pharyngo-oesophageal junction are best managed with chemotherapy and radiotherapy. Finally, salvage surgery may be considered in highly selected patients in case of non-response or local relapse without distant metastases.

[Dermatomyositis and polymyositis]. / La dermatomyosite et la polymyosite.

Dermatomyositis (DM) and polymyositis (PM) are the two main forms of idiopathic inflammatory myopathies. They have in common a proximal muscle weakness, but skin manifestations, juvenile forms and increased incidence of malignancies are clinical characteristics of DM. The follow up of creatine-kinases is the best biological test in spite of their possible normality. The significance of antibodies titers is uncertain, except the association Jo-1 interstitial and lung disease indicating a poor prognosis. The association with HLA haplotypes expresses a genetic predisposition of a dysimmunity to develop DM or PM. Pathological changes are well known with a humoral immune effector mechanism in DM, and a muscle fibre aggression by CD8 + T cells in PM. Non inflammatory forms of DM and PM and rhabdomyolytic forms of PM are not very rare, they are recognized by the HLA class 1 immunoreactivity. Pathophysiological processes involve muscle fibers, inflammatory cells and endothelial cells of capillaries, with a complex intervention of cytokines, adhesion molecules, MHC classe 1, membrane attack complex, anti endothelial cells antibodies, perforin secretion and sometimes apoptotic Fas-mediated mechanisms. Despite these recent advances, causal antigens and activator processes of endothelial cell lysis and autoinvasive cytotoxicity of muscle fibers remain to be identified.

True histiocytic lymphoma following B-acute lymphoblastic leukaemia: case report with evidence for a common clonal origin in both neoplasms.

True histiocytic lymphoma (THL) is a very rare type of non-Hodgkin's lymphoma (NHL) in which neoplastic cells exhibit markers of histiocytic differentiation. Some cases of THL have been reported in patients with previous acute lymphoblastic leukaemia (ALL), especially in children and young adults, in whom the acute leukaemia was of T-cell origin. The relationship between the initial lymphoid tumour and the secondary THL remains unclear, as a common monoclonal origin shared by both neoplasms has never been definitively demonstrated. We report a patient with B-ALL who developed a nodal and extranodal tumour with histological and immunohistochemical features of THL 4 years after the initial diagnosis. Genotypic study showed that both neoplasms contained the same immunoglobulin heavy gene rearrangement, which has not been reported previously.

[Bronchial mucoepidermoid carcinoma: apropos of 3 cases]. / Carcinome muco-épidermoïde bronchique: à propos de 3 observations.

We report three cases of bronchial mucoepidermoid carcinoma (BMEC) of low-grade malignancy with a relafase-free follow up. BMEC are rare tumors. The microscopic findings distinguish low-grade tumors which occur in children and young adults and high-grade tumors concerning older patients; this grading is based on the study of the epidermoid component. If possible, conservative therapy is appropriate in low-grade tumors. The prognosis of high-grade tumors is poor.