[The French mesothelioma network from 1998 to 2013]. / Mésothéliome: les dispositifs en place en France « le réseau mésothéliome ¼ 1998-2013.

Mesothelioma is a rare disease less than 0.3% of cancers in France, very aggressive and resistant to the majority of conventional therapies. Asbestos exposure is nearly the only recognized cause of mesothelioma in men observed in 80% of case. In 1990, the projections based on mortality predicted a raise of incidence in mesothelioma for the next three decades. Nowadays, the diagnosis of this cancer is based on pathology, but the histological presentation frequently heterogeneous, is responsible for numerous pitfalls and major problems of early detection toward effective therapy. Facing such a diagnostic, epidemiological and medico-legal context, a national and international multidisciplinary network has been progressively set up in order to answer to epidemiological survey, translational or academic research questions. Moreover, in response to the action of the French Cancer Program (action 23.1) a network of pathologists was organized for expert pathological second opinion using a standardized procedure of certification for mesothelioma diagnosis. We describe the network organization and show the results during this last 15years period of time from 1998-2013. These results show the major impact on patient's management, and confirm the interest of this second opinion to provide accuracy of epidemiological data, quality of medico-legal acknowledgement and accuracy of clinical diagnostic for the benefit of patients. We also show the impact of these collaborative efforts for creating a high quality clinicobiological, epidemiological and therapeutic data collection for improvement of the knowledge of this dramatic disease.

[Bronchopulmonary aspergillosis: new developments]. / Aspergilloses broncho-pulmonaires: les nouveaux enjeux.

Bronchopulmonary aspergillosis are in the news. Invasive pulmonary aspergillosis raise early diagnostic problems and prevention problems in immunocompromised patients. These infections are no unusual in chronic obstructive pulmonary disease. The diagnosis between aspergilloma and chronic necrotizing pulmonary aspergillosis can be difficult. In allergic bronchopulmonary aspergillosis, epidemiology and therapy are questionable. Real progress has been made due to thoracic computed tomographic scan and mycological methods. Better use of amphotericin B, of amphotericin B lipid formulations and of azole antifungal agents, combined with surgical resection if necessary should improve aspergillosis prognosis.

[Surgical treatment of pulmonary metastases of colorectal cancers. 8-year survival and main prognostic factors]. / Traitement chirurgical des métastases pulmonaires des cancers colorectaux. Survie à 8 ans et principaux facteurs pronostiques.

OBJECTIVE: In order to achieve a better definition of the indications for surgical excision of pulmonary metastases in colorectal cancer (CCR), a retrospective study of the eight year survival of patients who had been operated on was carried out with reference to the principal prognostic factors. METHODS AND RESULTS: Between May 1986 and December 1997, 38 patients had an excision for pulmonary metastases for CCR. The mean delay between diagnosis of the metastases and surgical treatment of the CCR was 39 +/- 24 months (0-98). Thirty two patients (84%) had a single pulmonary metastasis. The mean diameter of the metastasis was 38 +/- 22 mm. Twenty metastases had a diameter < 30 mm. Five patients had a locoregional recurrence of their CCR before pulmonary surgery. Fourteen patients had an abnormally elevated level of carcinoembrionic antigen (ACE-CEA) before the pulmonary excision. Five pneumonectomies, 23 lobectomies, 1 bilobectomy and 11 atypical resections were carried out. A lymph node clearance was performed in 25 cases. Six patients (16%) had an associated excision of an hepatic metastasis. The in-hospital mortality was 2.6%. Chemotherapy was associated with a pulmonary excision in 17 patients (46%). The mean survival was 2.7 years (0.13-8.7 years). The survival at one year was 89 +/- 5.2% and at five years 35.2 +/- 10.1% and at eight years 18.8% +/- 10.3%. Age, sex, histological stage of the primary tumor, the size and the delay in appearance in the pulmonary metastases, the number of metastases, the preoperative CEA, the operative technique and the perioperative chemotherapy did not influence the levels of survival at five years. At the same time associated excision of an hepatic metastasis did not worsen the prognosis at five years. CONCLUSION: Complete excision of pulmonary metastases in a colorectal cancer allows for significantly longer survival. This study associated with a literature review may help in advancing towards better selection of surgical candidates.

[Mucoviscidosis: lung transplantation is always in order]. / Mucoviscidose: la transplantation pulmonaire toujours d'actualité.

THE ONLY SOLUTION: Despite significant progress in the management of patients with multiple sclerosis, lung transplantation remains the only chance for survival in those with severe respiratory failure. WAITING LIST INCLUSION CRITERIA: Lung function tests, the patientís general states and psychological and familial factors all contribute to determining inscription on lung transplantation waiting lists. TECHNICAL ASPECTS: Heart-lung, monoblock two-lung and sequential two-lung transplantations are detailed according to the respective advantages and disadvantages. RESULTS: Hospital mortality is about 5% and 5-year survival about 50%. However, only 10% of the patients on waiting lists due to the lack of organs survive for 2 years. PERSPECTIVES: The number of grafts must be increased by developing lobular grafts from live donors using the bipartition technique. Nevertheless, xenografts remain the most promising perspective for increasing the number of patients who can benefit from this therapy.

[Pulmonary endodermal tumor resembling fetal lung. Low grade adenocarcinoma of the fetal lung type]. / Tumeur pulmonaire endodermique ressemblant au poumon foetal. Adénocarcinome de type poumon foetal de bas grade.

Pulmonary endodermal tumor resembling fetal lung is a rare pulmonary neoplasm, classified either within the pulmonary blastomas spectrum or as a subtype of adenocarcinoma. We report a case revealed by a fever in a 24-year-old woman. The tumor measured 9 cm and extended into the lower right bronchus. The diagnosis was done on a biopsy performed during fiberoptic endoscopy. The patient was treated by lobectomy. She is well without disease 6 years after surgery. This type of predominantly epithelial tumor with neuroendocrine differentiation and a scanty non malignant stromal component should be identified in young women because of its favorable outcome after surgical resection. It must not be confused with ordinary adenocarcinoma nor metastatic adenocarcinoma, especially endometrioid type.

[Bronchial casts associated with pulmonary lymphatic anomalies]. / Moules bronchiques associés à des anomalies lymphatiques pulmonaires.

We report a case of a patient who presented with bronchial moulds. The classic causes, particularly bronchopulmonary aspergillosis could not be found. The pulmonary radiology and CT of thorax revealed an alveolar syndrome. Lymphography and lymphoscintigraphy showed evidence of lymphatic anomalies including the non-visualisation of the thoracic canal. In the absence of evidence for other diseases, we would suggest that these anomalies were responsible for the symptomatology.

Aggressive surgical management in localized pulmonary mycotic and nonmycotic infections for neutropenic patients with acute leukemia: report of eighteen cases.

OBJECTIVE: To prevent hemoptysis and relapse during subsequent chemotherapy-induced neutropenia in patients with localized forms of invasive pulmonary aspergillosis, we adopted an aggressive surgical approach. METHODS: From 1988 to 1996, 18 patients with hematologic diseases were referred with the diagnosis of localized invasive pulmonary aspergillosis. The diagnosis was based on clinical features, failure to respond to antibiotic therapy, an air crescent sign suggestive of aspergillosis on the computed tomographic scan (39%), and retrieval of fungi by bronchoalveolar lavage (44%). RESULTS: The following procedures were done: one pneumonectomy, four bilobectomies, seven lobectomies, six wedge resections, and one lobectomy with wedge resection (one patient had two procedures). No perioperative deaths or complications occurred. The histologic examination confirmed the diagnosis of invasive pulmonary aspergillosis in 12 patients. The six other diagnoses were as follows: one case of classic aspergilloma, one case of pneumonia, and four cases of pulmonary abscess. According to univariate analysis, thoracic pain was less common in the group with noninvasive pulmonary aspergillosis (1/6) than in the group with invasive pulmonary aspergillosis (8/12) (p < 0.05). Sixteen patients required subsequent hematologic treatments. Sixty-six percent of the patients are alive with a mean follow-up of 29.1 +/- 27.8 months (range 2 to 103 months), with no statistically significant difference between the invasive and the noninvasive pulmonary aspergillosis groups. Five patients died of a recurrence of their malignant disease at a mean of 17.2 +/- 12.5 months (range 2 to 30 months), and one had a cerebral recurrence of Aspergillus infection during a bone marrow transplantation 3 months later. CONCLUSION: Aggressive surgical management radically improves the prognosis of invasive pulmonary aspergillosis, even if the surgical indications include some nonmycotic infections because of the difficulty in establishing the clinical diagnosis.

[Heart-lung transplantation and cystic fibrosis. Indications and results]. / Transplantation cardiopulmonaire et mucoviscidose. Indications et résultats.

BACKGROUND: Heart lung transplantation for++ cystic fibrosis is now performed in patients with severe lung disease but the experience is still scarce with the exception of some specialized centers. PATIENTS AND METHODS: Twenty-one patients underwent heart-lung transplantation between September 1989 and November 1994 in our institution, with a high standard of reliability in tracheal anastomosis and with a low incidence of hospital mortality (5%). RESULTS: The actuarial patient survival is 90.2% (95% confidence interval, 70 to 97%) at 1 year and 75.7% (95% confidence interval, 51 to 90%) at 3 and 4 years. The mean forced expiratory volume in 1 second (FEV1) increases from 20.1% predicted preoperatively to 76.1%. CONCLUSION: Despite the presence of airway pathogens, these results confirm that heart-lung transplantation for cystic fibrosis leads to a pronounced improvement in lung function and good rehabilitation after surgery. The two main obstacles are the shortage of donor organs and the possibility of late deterioration in lung function with a progressive airflow obstruction.

[Pulmonary and cardiopulmonary transplantation in mucoviscidosis]. / Transplantation pulmonaire et cardiopulmonaire au cours de la mucoviscidose.

As early as 1987, several teams in France began lung transplantation for patients with cystic fibrosis. Most of these teams propose transplantation when the life expectancy is under 2 years. The major functional criteria are VEMS < 30%, PaC02 > 50 mmHg and PaO2 < 55 mmHg. This contribution focuses on psychologic, nutritional and infectious aspects required in preparing the patients for transplantation and on graft selection. Surgical techniques and patient care after transplantation are also reported. The overall probability of survival after transplantation for cystic fibrosis is 48, 35 and 29% at 1, 2 and 3 years respectively with wide intercentre variation. The lack of sufficient graft supply and the risk of post-transplantation degradation remain the two principal problems for transplantation in cystic fibrosis.

[Epidemiology of malignant pleural mesothelioma in the Nantes-Saint Nazaire region. Course in 1956-1992]. / Epidémiologie du mésothéliome pleural malin dans la région de Nantes-Saint Nazaire. Evolution 1956-1992.

The aim of this study was to estimate the evolution of the incidence of mesothelioma in the Loire-Atlantique department since 1956 and to register all the cases diagnosed between 1985 and 1992 and to complete the data of earlier studies carried out between 1956 and 1984. The cases were indexed from the files of the pathology department and also from demographic and occupational data concerning the patients which had been gathered retrospectively from an inquiry of patients attached either to private physicians or to a hospital service. From 1956 to 1984 there were 125 cases (119 men, 6 women) who had been diagnosed; 92 cases were registered between 1985 and 1992 (79 men and 13 women). An increase in the annual number of cases was significant. The incidence during the period 1985-1992 was 10.9 per million inhabitants (men 19.4, women 3) against 8.7 (men 17.2, women 0.8) for the period 1975 to 1984 and 2.6 (men 5.2, women 0.2) for the period 1956-1974. The mean age of the subjects at the time of diagnosis rose during the period studied (59.2 +/- 9.4 between 1956-1974 to 63.1 +/- 11.9 between 1975-1984 and 67.0 +/- 9.7 between 1985-1992). Occupational exposure to asbestos was certain or probable in 85 per cent of cases with a median duration of exposure of 25 years (range 2 months to 48 years) with a median interval between the first exposure to diagnosis of 44 years (range 10-70 years). The industrial sector most often implicated was naval construction (127 cases).(ABSTRACT TRUNCATED AT 250 WORDS)

[Horton disease of pulmonary localization]. / Maladie de Horton avec localisation pulmonaire.

A case of Horton's disease with interstitial pulmonary involvement is reported. Transbronchial biopsy of the lung and biopsy of the temporal artery provided evidence of giant cell lesions. Outcome was favourable with general corticosteroid therapy with regression of the pulmonary disease.

[Well-differentiated papillary mesothelioma of the peritoneum: an attenuated malignant tumor. Review of the literature apropos of a case]. / Le mésothéliome péritonéal papillaire bien différencié: une tumeur à malignité atténuée. Revue de la littérature à propos d'un cas.

The authors present a new case of a well-differentiated papillary mesothelioma of the peritoneum. This is an uncommon tumor which have a slow evolution like a low malignant potential tumor. But, because of its tendency to recurrence, the designation of Well-Differentiated Tumor is better. The diagnosis with others peritoneal tumors is sometimes difficult, especially with the Peritoneal Serous Tumors. Tumor recurrence must be treated by curative surgery. Adjuvant therapy is discussed for the diffuse form.

[Cystic mucinous pulmonary tumor of borderline malignancy. Report of a case]. / Tumeur kystique mucineuse pulmonaire de malignité frontière. A propos d'un cas.

A cystic mucinous borderline tumor was discovered in a sixty two year old man, presenting as a cystic mass in the right lower lobe. A first diagnosis of bronchiolo-alveolar carcinoma was made on bronchial biopsies. Surgical specimen examination revealed a lung mucinous cystic borderline proliferation similar to ovary borderline mucinous tumors. Such an unusual localisation has been already reported. The diagnostic difficulties are reviewed.

[Multiple and recurrent cardiac myxomas. Is it a familial disease?]. / Les myxomes cardiaques multiples et récidivants. S'agit-il d'une maladie familiale?

From 1964 to 1992, 56 patients with cardiac myxoma have been operated on. Sixty seven operations have been made during which 85 myxomas have been excised. Five patients had been reoperated from recurrent myxoma: 4 patients 2 times, 1 patient one time. In this population we observe 7 cases of multiple myxomas implanted 7 times in many cavities. The study of this series displays a lot of risk factors: implantation outside the left atrium, multiple myxomas in one or several cavities, true recurrence of myxoma, young age of the patient, associated lesions as part of the Carney's complex. In this series, 13 patients, representing 5 families, have one or more of these risk factors. in two of them, a familial character has been demonstrated. Analysis of these special kinds of cardiac myxomas displays very closed analogy between familial myxomas and high risk group. These findings suggest that recurrent and/or multiple and/or familial myxoma represent the same entity.

[Cardiovascular complications in chronic atrophic polychondritis. Apropos of an anatomo-clinical case. Review of the literature]. / Les complications cardiovasculaires de la polychondrite chronique atrophiante. A propos d'un cas anatomo-clinique. Revue de la littérature.

The case of a man, 25 years of age and presenting with chronic atrophic polychondritis (CAP), complicated by a complete atrioventricular block, double mitral valve incompetence and aneurysm of the ascending aorta, offered the possibility of investigating the various clinical manifestations and cardiovascular complications of this common disorder. CAP is a connectivitis of unknown etiology, it corresponds to ubiquitous and recurrent cartilage inflammation, leading to characteristic chondritis of the ears and nose, joint disease and laryngo-trachco-bronchial disorders. Other systemic impact is seen at sites containing high levels of proteoglycans, such as the eye, inner ear and cardiovascular system. Respiratory problems are the main cause of death, but cardiovascular effects occur in 25% of cases and constitute the second most frequent cause of mortality. These effects consist mainly of aortic and/or mitral valve incompetence. Annular dilatation, which is often associated with ectasia of the ascending aorta, is the main cause of aortic incompetence. Several cases of isolated AVB or AVB secondary to Al have been reported. Aneurysms develop along the aorta and the large and medium caliber arteries (sub-clavicular, coronary, mesenteric arteries). These are characterized by destruction of the elastic fibers and a reduction in the proteoglycan content of the walls, which is also observed when dystrophy of the cartilage occurs. Other vascular disorders reported include arteritis of the legs, superficial migratory varices and vascularitis, which in some cases gave rise to skin, renal or neurological reactions.

[Cardiac transplantation at the University Hospital Center of Nantes. 2 years' experience]. / Transplantation cardiaque au CHU de Nantes. Deux ans d'expérience.

Between March, 1985 and April, 1987, 25 orthotopic heart transplantations were performed in 20 men and 5 women aged from 17 to 58 years (mean 42 years) on account of cardiomyopathy (n = 15), ischaemic heart disease (n = 6) or miscellaneous lesions (n = 4). The immunosuppressive treatment consisted of antilymphocyte serum and corticosteroids during 10 days; cyclosporine was introduced on the 7th day and continued thereafter in association with low-dose corticosteroid therapy. Endomyocardial biopsies were performed. Acute rejection, responsible for 2 deaths (one on the 10th day, the other in the 10th week), usually occurred within the first 3 months. Infections were frequent and often serious, resulting in one death in the 7th week. One out of patients had to be treated for arterial hypertension, and 3 patients presented with renal impairment (blood creatinine over 200 mumoles/l). The actuarial survival rate at 2 years is 84 p. 100. More than one-half of the patients have resumed social and occupational activities.