Association of HLA-A in autoimmune myasthenia gravis with thymoma.

We investigated an association of the HLA-A locus in 78 French Caucasian patients with autoimmune myasthenia gravis (MG) and thymic epithelial tumours. The largest effect was a protection associated with HLA-A02 in MG patients with a B2 type thymoma (OR=0.323, 95% CI: 0.

Lymph node pathology in pulmonary veno-occlusive disease and pulmonary capillary heamangiomatosis.

To assess the histological bases of lymphadenomegaly, which has been reported as a frequent radiological finding in pulmonary veno-occlusive disease (PVOD), we have reviewed pulmonary and mediastinal lymph nodes resected during lung transplantations in 19 patients suffering from PVOD and related pulmonary capillary haemangiomatosis (PCH). Lymphatic congestion was common and was often obvious in subsegmental and segmental lymph nodes. Vascular transformation of the sinuses, intra-sinusal haemorrhage with erythrophagocytosis and lymphoid follicular hyperplasia were frequent especially in lobar, hilar and mediastinal lymph nodes.

Cysts of the posterior mediastinum showing müllerian differentiation (Hattori's cysts).

Cysts of probable müllerian origin have recently been recognized in the mediastinum by Hattori (Virchows Arch. 2005;446:82-84; Chest. 2005;128:3388-3390).

[Pulmonary fibrosis in Hermansky-Pudlak syndrome is not fully usual].

A 55-year-old female patient with Hermansky-Pudlak syndrome (albinism, thrombopathia and ceroid accumulation) underwent a single lung transplantation for pulmonary fibrosis. Examination of explanted lung showed usual interstitial pneumonia pattern associated with two unusual lesions: presence of numerous macrophages containing ceroid pigments within fibrosis and characteristic foamy swelling of pneumocytes. This later lesion, which has only been recently described, seems related to the mechanism of the disease by defect of surfactant secretion.

Heart tumors in children and adults: clinicopathological study of 59 patients from a surgical center.

Background: Heart tumors are rare lesions with variegated histological types. Their clinicopathological features could be more comprehensively categorized.

Methods: This is a 19-year retrospective study of 17 infants/toddlers (<2 years of age) and 42 patients aged between 14 and 79 years (mean = 51.

Paraganglioma with ganglioneuromatous component located in the posterior mediastinum.

A 4-cm paravertebral mediastinal tumor was resected in a 70-year-old male patient treated for hypertension. The tumor displayed both paraganglioma and ganglioneuroma areas that were in equal proportion and often merged one into the other. Paraganglioma areas contained synaptophysin and chromogranin-positive chief cells and PS100-positive sustentacular cells.

Cholinesterase activity in human pulmonary arteries and veins: correlation with mRNA levels.

Isolated intact human pulmonary arteries and veins were used to determine the acetylcholinesterase (AChE) or butyrylcholinesterase (BChE) activities in the absence or presence of two selective cholinesterase (ChE) inhibitors, iso-OMPA or BW284c51, respectively. These results were compared with the mRNA levels for each enzyme in human pulmonary vessels. Total ChE activities measured in presence of acetylthiocholine (ACTI, 1 mM) in intact vascular preparations were 45+/-04 and 114+/-07 mU/g tissue in human pulmonary arteries (n=14) and veins (n=14), respectively.

Chest wall resection for invasive lung carcinoma, soft tissue sarcoma, and other types of malignancy. Pathologic aspects in a series of 107 patients.

With improvements in surgical techniques for resection and reconstruction of the chest wall, pathologists are confronted with complicated surgical specimens. There are no currently available guidelines specifically dedicated to the handling of these specimens. Extended resections of lung carcinoma chest wall invasions may change the clinical value of some TNM subsets.

Endocardial location of familial myofibromatosis revealed by cerebral embolization: cardiac counterpart of the frequent intravascular growth of the disease?

Myofibromatosis is a rare infantile benign neoplasia, which may involve the heart in the rare and usually fatal generalized form of the disease. Diagnosis of endocardial myofibromas was made on two surgically excised lesions of the mitral valve that were reveled by a cerebral embolization in a 12-month-old female infant. Surprisingly, the patient had no other obvious lesion of myofibromatosis.

Induction of pulmonary angiogenesis by adenoviral-mediated gene transfer of vascular endothelial growth factor.

Background: We hypothesized that gene transfer of vascular endothelial growth factor (VEGF) mediated by an adenovirus vector might induce pulmonary artery angiogenesis in a lamb model of pulmonary artery hypoplasia.

Methods: Thirteen fetal lambs had left pulmonary artery banding at 106 days of gestation. Following birth, 3 groups were divided: VEGF group (n = 5) and beta-GAL group (n = 4) received an adenoviral vector encoding respectively for human VEGF165 and for galactosidase A.

Identification of genes associated with the corticotroph phenotype in bronchial carcinoid tumors.

The proopiomelanocortin (POMC) gene is occasionally expressed in nonpituitary tumors leading to Cushing's syndrome. Bronchial carcinoid tumors, one of the most frequent source for ectopic ACTH secretion, often display numerous features of the corticotroph phenotype. To identify new markers of corticotroph differentiation in these tumors, we compared the pattern of gene expression in ACTH-secreting (ACTH+) and nonsecreting (ACTH-) bronchial carcinoids by differential display/RT-PCR.

[Thymoma with epithelial micronodules and lymphoid hyperplasia: six cases of a rare and equivocal subtype].

Thymomas with the characteristic pattern of small epithelial nodules separated by an abundant lymphoid tissue have been recently described with divergent interpretations. These thymomas are not specified in currently used classification systems. We present six such thymomas, including three that represented 1.

Prevention of gram-negative translocation reduces the severity of hepatopulmonary syndrome.

Hepatopulmonary syndrome (HPS) is characterized by intrapulmonary vascular dilatations and an increased alveoloarterial oxygen difference (AaPO(2)). These abnormalities are related to augmented pulmonary nitric oxide (NO) production, dependent primarily on increases in the expression and activity of inducible NO-synthase (iNOS) within pulmonary intravascular macrophages and, to a lesser extent, of endothelial NOS (eNOS). Production of iNOS by pulmonary intravascular macrophages might be related to translocated gut bacteria present in the pulmonary circulation.

Solitary fibrous tumors of the pleura: clinical characteristics, surgical treatment and outcome.

Objective: The aim of this paper is to study clinical characteristics, surgical treatment and outcome of patients with solitary fibrous tumor of the pleura operated in our institutions in a 20-year period.

Methods: Clinical records of all patients operated for solitary fibrous tumors of the pleura between 1981 and 2000 were reviewed retrospectively. Tumors were classified as malignant in the presence of at least one of the following criteria: (1) high mitotic activity; (2) high cellularity with crowding and overlapping of nuclei; (3) presence of necrosis; (4) pleomorphism; otherwise they were considered as benign.