[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.

Prognostic factors in diffuse malignant pleural mesothelioma. A study of 167 patients.

The existence of large shipyards accounts for the particular frequency of pleural mesothelioma in the Nantes-St. Nazaire region of France. From 1955 to 1985, 167 cases were diagnosed. Occupational exposure to asbestos was involved in 131 cases (88 percent). There was a great predominance of epithelial types (135) over mixed (25) and fibrosarcomatous (7) types. Survival, estimated by the actuarial method, was 54 percent at one year from first symptoms and 39 percent from histologic diagnosis. No subject was alive four years after diagnosis. Histologic type and asbestos exposure had no predictive value in our series. Survival was longer in patients under 60 years of age and when mesothelioma originated on the left side. Overall, treated patients had significantly longer survival than untreated patients. However, there was no significant difference in survival with respect to the type of treatment given: surgery, chemotherapy, talc poudrage or their combination.

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.

[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.

[Secondary coronary fistulas]. / Les fistules coronaires secondaires.

These fistulae are very rare: 8 to 11 p. 100 of all coronary fistulae. Three types are recognised: right coronaro-ventricular, left coronaro-ventricular, and coronao-pulmonary. The two first types are found most frequently in association with a sigmoid atresia on the orifice, and with ventricular hypoplasia in the presence of a functional atrio-ventricular valve. The fistula then serves as a means of ventricular ejection. Of the secondary fistulae, the right coronaro-ventricular type is the most common (73 p. 100). The authors have found 30 cases in the published literature, and add 2 of their own. They have also reviewed the clinical features and the findings on coronary arteriography and post mortem studies on such fistulae. They discuss their etiology, pathogenesis, and physiopathology. Surgical correction involves repairing the fistula in one stage. Fistulae of the left coronaro-ventricular type are exceptions to this rule (9 p. 100), as they are always associated with a rapidly fatal hypoplasia of the left side of the heart. Coronaro-pulmonary fistulae (18 p. 100) are usually found in association with extreme forms of Fallot's tetralogy, and a relatively simple surgical correction can form part of the total correction of the tetralogy.

[Endomyocardial biopsy. Innocuousness and clinical value. A 5-year experience]. / La biopsie endomyocardique innocuité et intérêt clinique. Une expérience de cinq ans

The authors present their experience of the endomyocardaial biopsy procedure by Konno's technique; they have carried out 180 biopsies in 94 subjects. When the person taking the biopsy is experienced, the procedure is trouble-free, and yields tissue fragments of good quality which are suitable both for histological study and for study of ultrastructure. Biopsies from 47 cases of primary congestive cardiomyopathy have formed the basis of a precise histological study, and have been compared with the findings of a control group comprising various cardiac defects and three normal hearts. The diagnostic and prognostic value of the method are discussed.

[Myocardial biopsy in congestive myocardiopathies of apparently primary origin]. / La biopsie myocardique dans les myocardiopathies congestives en apparence primitives

38 patients with congestive cardiomyopathy of apparantly primary origin had a myocardial biopsy. The histology of the fragment of myocardium was studied both by light microscopy and electron microscopy. The results were compared with those from 3 "control" cases and with 16 cases of congestive asystole secondary to a known cause. The non-specific patternss which were observed were of 3 types: patterns of degeneration, pattern of hypertrophy, and interstitial fibrosis. Chronic alcholism had no modifying effect on the ultrastructure. Finally, the group in which the morphology was altered had a higher mortality, but the prognostic significance of the degree of severity of the morphological change must be treated with caution in each individual case.

[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.

[Intimal coronary fibrous dysplasia and Prinzmetal's angina]. / Dysplasie fibreuse intimale coronaire et angor de Prinzmetal.

It is currently considered as exceptional for there to be arterial pathology other than atheroma in Prinzmetal angina. The authors have found five cases of coronary fibrous dysplasia in the literature. They add a case, in a woman, of intimal fibrous dysplasia diffusely involving the three main coronary vessels. Coronary arteriography showed that spasm of the right coronary artery was the cause of attacks with the clinical and electrocardiographic features of Prinzmetal angina. The whole coronary tree was otherwise normal angiographically and also macroscopically at autopsy. The dysplasia was only discovered by histological study of serial sections of the three coronary vessels. Histological examination showed no evidence of an atheromatous lesion. The authors place these coronary artery lesions within the general classification of arterial fibrous dysplasias. It remains hypothetical whether there is a relationship between the occurrence of spasm and this very special histological feature of the coronary artery wall.

[Fibromuscular dysplasia of the arteries of the leg. Apropos of 1 case]. / Dysplasie fibro-musculaire des artères de jambe. A propos d'un cas.

A 40 year old man presented fibromuscular hyperplasia of posterior tibial artery media, a rare lesion, and only exceptionally reported in the calves since a total of 3 cases were found in the literature.

[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.

[Carney's complex (myxomas, pigmented spots and endocrine hyperactivity). Apropos of a partial form in monozygotic twins]. / Le complexe de Carney (myxomes, taches pigmentaires et hyperactivité endocrinienne). A propos d'une forme partielle chez des jumeaux monozygotes.

The authors describe monozygotic twins with right ventricular myxoma, one of whom had cutaneous myxomas, large cell calcifying Sertoli cell tumor of the testis (a lesion which was certainly latent in his brother) and recurrent cardiac myxoma. These elements are manifestations of "Carney's complex". This recently reported inherited syndrome should be recognized by pathologists because of major risk of cardiac myxoma. Cutaneous lesions, specially cutaneous myxomas, are heralds of this syndrome.

[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.

[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.

[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.

[Disseminated invasive aspergillosis associated with Mycoplasma pneumoniae infection]. / Aspergillose invasive disséminée associée à une infection à Mycoplasma pneumoniae.

Disseminated aspergillosis is rare in patients not suffering from haematological disorders and myelo-suppression. We report a case of pulmonary cardiac and renal aspergillosis diagnosed at autopsy in a patient with chronic respiratory failure dying in the intensive care unit during an acute exacerbation. The role of risk factors associated with invasive aspergillosis in patients not suffering from myelosuppression is discussed (steroid therapy, chronic respiratory disease, concomitant viral or bacterial infection). The discovery in this patient of a pulmonary infection associated with mycoplasma pneumonia (the micro-organisms were found in necropsy specimens of pulmonary tissue) does not seem to have been reported before in the literature.

[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.

[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.