Odile Groussard

Survival benefit of lung transplantation for patients with idiopathic pulmonary fibrosis

OBJECTIVE Although lung transplantation is viewed as an acceptable option for patients with end-stage idiopathic pulmonary fibrosis, the survival benefit of this approach is still debated. This study examined whether there was a survival benefit of lung transplantation in a cohort of patients referred to our transplant center with a diagnosis of idiopathic pulmonary fibrosis according to American Thoracic Society criteria. METHODS Forty-six patients accepted for lung transplantation during a 12-year period with a diagnosis of idiopathic pulmonary fibrosis form the basis of this study. Survival benefit offered by lung transplantation was assessed using Cox proportional-hazards modeling, with patients on a waiting list as the control group. RESULTS Twenty-eight patients underwent lung transplantation (27 single and 1 double), 16 patients died while waiting, and 2 patients remained on the active waiting list. Diagnosis of idiopathic pulmonary fibrosis was made on histologic examination of the explanted lung or lung biopsy before lung transplantation. There was a pattern of usual interstitial pneumonia in 31 cases (67%). The 15 remaining patients fulfilled all American Thoracic Society criteria for idiopathic pulmonary fibrosis. The median waiting time for organs was 51 days. Survival after lung transplantation was 79.4% at 1 year, 63.5% at 2 years, and 39% at 5 years. The multivariable analysis showed that lung transplantation reduced the risk of death by 75% (95% confidence interval, 8%-86%; P =.03) after adjustment on potential confounding variables. CONCLUSIONS Lung transplantation is effective in improving the survival of selected patients affected by idiopathic pulmonary fibrosis.

Primary intrathoracic synovial sarcoma: a clinicopathologic study of 40 t(X;18)-positive cases from the French Sarcoma Group and the Mesopath Group.

Synovial sarcoma (SS), an aggressive neoplasm accounting for up to 14% of soft tissue sarcomas, was recently recognized as a primary tumor in the lung and pleura. SS is characterized by the chromosomal translocation t(X;18)(SYT-SSX) found in more than 95% of the tumors. We report a cooperative study from the French Sarcoma Group and the Mesopath Group on 40 t(X;18)(SYT-SSX)-positive primary intrathoracic SS. There were 22 males and 18 females, whose age ranged from 16 to 79 years (median, 47 years). Neoplasms were mostly circumscribed and of large size (median, 7.5 cm; range, 2-16 cm). Thirty-nine tumors were monophasic SS, including 24 (60%) monophasic fibrous and 15 (37.5%) poorly differentiated cases, and one lesion was a biphasic SS. A larger proportion of poorly differentiated tumors were observed among intrathoracic SS as compared with soft tissue SS. Immunohistochemically, 90% of the cases reacted with at least one epithelial marker. CD34 was focally expressed in 3 cases. SYT-SSX1 fusion transcripts were detected in 22 cases (56.4%) and SYT-SSX2 fusion transcripts in 17 cases. Median and 5-year disease-specific survival in 33 patients was 50 months and 31.6%. Median and 5-year disease-free survival was 24 months and 20.9%. Patient sex, age, tumor size, histologic subtype, grade, and SYS-SSX fusion type had no significant impact on outcome. In conclusion, intrathoracic SS are rare but aggressive tumors with poor prognosis. In this unusual location, the detection of SYT-SSX fusion transcripts is a valuable diagnostic adjunct.

Ectopic hepatocellular carcinoma arising in the left chest wall: a long-term follow-up.

We report the case of a 66-year-old man with chronic hepatitis C and a slowly growing left chest wall mass. Two years after the patient first noticed the mass, it was resected. A diagnosis of hepatocellular carcinoma (HCC) was established. The liver was studied by ultrasound, computed tomography (CT), magnetic resonance imaging (MRI) and angiography, but no mass was found. Blind liver biopsy showed mild chronic hepatitis without cirrhosis or HCC. Three years after the discovery of the chest wall HCC, no liver mass had appeared at CT and MRI. We conclude that solitary extrahepatic HCC (i) may arise in ectopic liver tissue; (ii) should not be considered as a metastasis of an occult HCC; and (iii) can be amenable to cure through resection.

Morbidity and mortality related to the native lung in single lung transplantation for emphysema

It has been advocated that a major drawback of single lung transplantation (SLT) is the risk of serious complications arising from the native lung. The morbidity and mortality related to the native lung in 46 patients who underwent SLT for pulmonary emphysema in Clichy from 1988 to 1997 were reviewed retrospectively. In particular, infectious complications and native lung hyperinflation were searched. Complications arising from the native lung are not unusual after SLT for subjects with emphysema, and it was concluded they are not responsible for a substantial mortality.

Mésothéliome : les dispositifs en place en France « le réseau mésothéliome » 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 patients 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.

Endothelial cell chimerism associated with graft rejection after human lung transplantation.

Endotheliitis is a major sign of graft rejection. Recipient-derived endothelial cells found in two series of liver and kidney transplants were related to graft rejection. Here, we assessed the presence and the number of chimeric endothelial cells in lung transplants, and their relation with graft rejection. In six males grafted with female lungs out of 193 lung transplantations, endothelial chimerism was studied by combined XY-fluorescent in situ hybridization with CD31 and CD45 immunostainings and blood group antigens. On samples graded according to the revised working formulation for lung allograft rejection, we found chimeric macrophages (73.1 to 87.2%) in all cases and chimeric endothelial cells (1.3 to 2.1%) in four patients. Another method using ABO blood group also showed endothelial cells positive for recipient-type blood group antigens in three patients. By both methods, presence of chimeric endothelial cells was related to pathological signs of acute rejection (P<0.05).

Transplantation pulmonaire chez les patients atteints de fibrose pulmonaire

Resume La transplantation pulmonaire s’est developpee au cours des 15 dernieres annees pour traiter differentes formes de maladies respiratoires evoluees. Parmi elles figure en bonne place la fibrose pulmonaire idiopathique. Dans cette indication, le type de transplantation effectuee est principalement la greffe mono-pulmonaire, et dans une moindre mesure la greffe bi-pulmonaire. Ces deux interventions donnent des resultats voisins en termes de survie. La survie post-transplantation pour les fibroses se situe approximativement a 65-70 % a 1 an et a 40 % a 5 ans, c’est-a-dire a un niveau un peu inferieur a ce qui est note dans la BPCO ou la mucoviscidose. En l’absence de complications, l’intervention permet le retour a une activite quasi normale. Parmi les complications menacant le patient en post-operatoire, il faut citer particulierement l’œdeme de reimplantation, l’infection, le rejet et les complications bronchiques. A distance, une forme de rejet chronique appelee « syndrome de bronchiolite obliterante » va se developper chez pres d’un patient sur deux. Ces complications expliquent les resultats de la transplantation pulmonaire qui sont encore largement perfectibles. Malgre cela, la survie apres greffe pour fibrose sont superieurs a ceux de la survie spontanee en liste d’attente, la fibrose idiopathique etant la pathologie associee a la mortalite la plus elevee sur liste. Les patients doivent etre referes tres tot pour leur bilan pre-transplantation car leur pronostic individuel est tres difficile a predire.

Non-traumatic pulmonary haematoma complicating oral anticoagulation therapy

Abstract:  Several cases of non‐traumatic pulmonary haematoma have been reported in the literature. However, very few of them are related to anticoagulation therapy. The authors report two cases of pulmonary haematoma caused by oral anticoagulant therapy without any underlying pathological lesion. The evolution was fatal in the first case, whereas a slow spontaneous resolution of the haematoma was noted in the second. Non‐traumatic pulmonary haematoma can be a complication of oral anticoagulation and should be considered in the differential diagnosis of pulmonary densities in this setting.

Expérience de la télépathologie pour la certification diagnostique du mésothéliome

Resume La telepathologie se definit en pratique comme la capacite d’examiner des lames histologiques a partir de lames virtuelles analogues des lames physiques, d’aboutir a un diagnostic histologique sans deroger aux criteres de qualite et de securite et de rapidite que ceux obtenus lors d’un examen conventionnel au microscope. Nous rapportons dans cet article l’experience du reseau Mesotheliome pour la certification standardisee du diagnostic de mesotheliome par le Centre national referent [CNR] MESOPATH, les enjeux et les avantages a utiliser cette nouvelle technologie, les contraintes et le futur de cet outil et des solutions informatiques necessaires a leur mise en place.

Analyse des différentes lésions histologiques observées sur biopsie transbronchique pour suspiscion de rejet aigu. Corrélations anatomo-cliniques au cours des 6 premiers mois suivant la transplantation pulmonaire

Le rejet aigu est une complication tres frequente de la transplantation pulmonaire. Le but de ce travail etait (1) d’estimer la variation interobservateurs pour l’analyse des differentes lesions histologiques, et (2) de determiner la performance des biopsies transbronchiques (BTB) pour le diagnostic de rejet aigu et des pathologies associees. Cette etude incluait 53 patients consecutifs ayant beneficie d’au moins une BTB cliniquement justifiee, au cours des 6 premiers mois suivants la transplantation pulmonaire. Ainsi, 94 BTB etaient obtenues. Les criteres histologiques suivants : infiltrat mononuclee perivasculaire, bronchiolite lymphocytaire et lesions alveolaires etaient reproduits de facon fiable entre 2 pathologistes du meme centre (k : 0,79 a 0,82). Pour l’identification de l’infiltrat mononuclee perivasculaire, la discordance entre les 2 pathologistes etait significativement associe a la presence de lesions alveolaires moderees/severes (p