Elevated plasma level of lipotropin revealing an occult carcinoid tumor with normal plasma adrenocorticotropin.

The increases in the level of plasma lipotropin (LPH) and in the LPH/ACTH ratio are considered diagnostic tools in ectopic ACTH syndrome. However, plasma ACTH is also elevated in this syndrome. We report a case of a small carcinoid tumor with an increase in both ACTH and LPH in plasma before surgery. Eight months after the tumoral resection, plasma LPH alone was increased again, whereas plasma ACTH and plasma and urinary cortisol remained normal in this apparently cured patient. This repeated abnormality was the only available feature that allowed successful removal of the occult tumoral residue.

Differential gene expression in mesothelioma.

To investigate the molecular events controlling malignant transformation of human pleural cells, we compared constitutive gene expression of mesothelioma cells to that of pleural cells. Using cDNA microarray and high-density filter array, we assessed expression levels of > 6500 genes. Most of the highly expressed transcripts were common to both cell lines and included genes associated with stress response and DNA repair, outcomes consistent with the radio- and chemo-resistance of mesothelioma. Interestingly, of the fewer than 300 genes that differed between cell lines, most functioned in (i) macromolecule stability, (ii) cell adhesion and recognition, (iii) cell migration (invasiveness), and (iv) extended cell division. Expression levels of several of these genes were confirmed by RT-PCR and could be useful as diagnostic markers of human mesothelioma.

Contribution of cervical ultrasound and ultrasound fine-needle aspiration biopsy to the staging of thoracic oesophageal carcinoma.

This study was performed to evaluate the use of cervical ultrasonography and ultrasound-guided fine-needle aspiration for pretherapeutic staging of oesophageal cancer. 50 patients with a thoracic-oesophageal cancer (upper third = 8, middle = 36, lower = 6), previously untreated, underwent cervical ultrasonography to detect supraclavicular lymph node metastases (LN). An ultrasound fine-needle aspiration biopsy was attempted in 12 cases of suspected LN. 26 patients were operated on, of which 13 had surgical exploration of the neck. All patients were followed after treatment with special attention to the supraclavicular area. 14 patients (28%) were ultrasonography positive, 5 of 8 in the upper third, 9 of 42 in the two other thirds. Of the 12 patients where a fine-needle biopsy was attempted, 9 showed neoplastic cells (75%). 5 patients had cervical metastatic LN at surgery, and 5 other patients demonstrated supraclavicular LN metastases during the follow-up. There was one false positive and six false negatives from cervical ultrasonography and two false negatives of UGFAB (ultrasound-guided fine-needle aspiration biopsy). The sensitivity and the specificity of the cervical ultrasonography were 68 and 97%, respectively. The pretherapeutic staging was modified: 7 patients initially stage II-III were regraded to stage IV. Cervical ultrasonography is a reliable method of assessment of supraclavicular LN in thoracic oesophageal carcinoma.

Expression of the chemokine RANTES in pulmonary Wegener's granulomatosis.

Wegener's granulomatosis (WG) is an inflammatory, destructive, angiotropic lesion. The inflammatory process involves accumulation of macrophages, lymphocytes, and polymorphonuclear neutrophils. We studied 6 lung biopsy specimens from patients with WG to characterize the cellular infiltrate and to analyze the mechanism of immune cell recruitment. We show that lymphocytes accumulating in WG lesions are mostly memory CD4(+)CD45RO(+) T lymphocytes and, although less numerous, CD8(+)CD45RO(+) T lymphocytes. Few if any B lymphocytes or natural killer cells are present within lesions. The chemokine RANTES (regulated upon activation in normal T cells, expressed and secreted) has been reported to recruit memory T lymphocytes and macrophages selectively. We used reverse-transcription polymerase chain reaction, in situ hybridization, and immunohistochemistry to study its production in WG. RANTES was expressed at a higher level in WG lungs than in normal controls, especially around microabscesses. As visualized immunohistochemically in serial sections with anti-RANTES monoclonal antibody, RANTES production was produced mainly by macrophages. Expression of the gene coding for interferon-gamma (IFN-gamma), a potent RANTES inducer, was also studied. Its expression was also much stronger in WG than in controls. Our observations are consistent with a cascade of events leading to the recruitment of immune cells in WG, sequentially involving production of IFN-gamma by T lymphocytes and RANTES production by macrophages, leading to the homing of memory T-helper lymphocytes and macrophages. HUM PATHOL 32:320-326.

Recurrent left-sided heart leiomyosarcoma: should heart transplantation be legitimate?

Leiomyosarcoma of the heart is an uncommon primary malignant tumor with poor postoperative survival that may be measured in months. A leiomyosarcoma of the left atrium was diagnosed in a 47-year-old man. Initial admission was for acute pulmonary edema requiring emergency surgery. The tumor involved the left atrial cavity, and a radical resection was performed. Six months later an isolated myxomatous recurrence was detected. Heart transplantation was then performed. The patient is in good health 20 months after operation with no evidence of residual disease or recurrence. The literature has been reviewed. Surgical resection is not an adequate treatment for leiomyosarcoma of the left atrium and early heart transplantation probably offers the only hope for these patients.

Severe lupus with infectious thyroiditis and lethal cardiomyopathy.

Clinical cardiomyopathy is an uncommon complication of systemic lupus erythematosus (SLE) and intracavitary thrombosis is rare. We describe a patient with active SLE who developed rapidly progressive cardiomyopathy, the fatal course of which was complicated by an intracavitary thrombus. Repeat cardiac echography studies and the endomyocardial biopsy proved to be helpful in diagnosing the lupus myocarditis and aided the regulation of therapy. Furthermore, the patient presented an acute suppurative thyroiditis never before described, to our knowledge, in SLE.

Necrotizing granulomata of the aortic valve in Wegener's disease.

Wegener's disease is an inflammatory disease of unknown etiology, characterized by a granulomatous-necrotizing general vasculitis. Cardiac involvement in the form of aortic pathology is not frequent. We report a case of Wegener's granulomatosis which required prosthetic aortic valve replacement for aortic valve insufficiency. Microscopic examination of the valve demonstrated histopathology typical of Wegener's disease.

Desmoid fibromatosis of the shoulder and of the upper chest wall following a clavicular fracture.

A desmoid tumor of the shoulder girdle infiltrating the upper chest wall and weighing 1500 g was almost completely removed in an 18-year-old man, 27 months after a bifocal fracture of the clavicule. Thirteen years later, the patient was free of recurrence. The interval time between trauma and diagnosis, as the particular characteristics of aggressive fibromatosis, strongly support a major causal role of the clavicular fracture in the occurrence of this tumor.

Tumour of the juxtaoral organ.

The juxtaoral organ is a normal and constant structure of the oral cavity. It consists of benign epithelial nests. We describe an intraoral tumour of the juxtaoral organ in a child. The tumour was not diagnosed after clinical and radiological examinations because it is extremely rare. A histological examination revealed a tumour of the juxtaoral organ, presumed to be neuroid hamartoma. This is only the second time that a tumour of the juxtaoral organ has been described in a child. We also describe the location, the embryology, the histology and the function of this organ. This is important because this structure can be confused with carcinomas of the oral cavity when examining frozen sections.

An animal model for the evaluation of graft thrombosis in the acute phase on carbon-lined PTFE prosthesis.

Forty-five carbon-lined (CL) and 45 standard (ST) 4 mm internal diameter polytetra-fluoroethylene (PTFE) grafts were implanted as aortic interposition in 90 rabbits. A pilot study of 20 animals: 10 CL and 10 ST grafts were used to develop microsurgical techniques, then 60 grafts were placed in 60 New Zealand rabbits with lower morbidity. The two hours graft patency (Doppler and angiographic studies) showed better patency rate in CL group (93% versus 80%). In 10 animals, platelet accumulation was investigated in vivo using gammacamera imaging after injection of autologous platelets labeled with Indium111. In vitro, radioactivity counting of the explanted midgraft sections at 2 hours revealed 6 times greater activity in ST grafts (6.60 +/- 1.98 x 10(3) platelets/mm2 versus 0.82 +/- 0.25 x 10(3) platelets/mm2; p < 0.05). Light microscopy found platelet and fibrin deposition (PFD) in nearly all ST grafts whereas PFD were found in only 13% of the CL grafts corresponding to those thrombosed (chi 2: 61.117, p < 0.001). Carbon-lining decreases platelet accumulation on PTFE grafts in the acute phase of a new experimental model.

Detection of cardiac myxoma by F-18 FDG PET.

Intracardiac tumors occur infrequently and are difficult to diagnose with CT and MRI. The authors describe the successful imaging of a right atrial myxoma with F-18 FDG PET.

[Initial primary sarcoma of the pulmonary valve and trunk of the pulmonary artery]. / Sarcome intimal primitif de la valve et du tronc de l'artère pulmonaire. Suivi échocardiographique.

Primary sarcomas arising from the trunk or branches of the pulmonary artery are exceedingly rare and usually diagnosed at autopsy. The authors report the case of a 31 year old man referred for investigation of recurrent syncope. Echocardiography, right ventriculography and the thoracic computed tomography led to early diagnosis of a pulmonary artery tumour and surgical resection. Histology revealed a primary sarcoma. The early postoperative course was uncomplicated but one month after surgery a local recurrence was diagnosed at routine echocardiographic examination. The syncopal symptoms recurred seven months after surgery and echocardiography showed tumour recurrence on the interventricular septum and in the pulmonary artery. A second palliative operation was attempted but was unsuccessful because of the size of the tumour.

[Echocardiographic aspects of multiple myxoma in Carney's syndrome]. / Aspects échocardiographiques des myxomes multiples dans le cas d'un syndrome de Carney.

In Carney's syndrome, the association of cardiac myxomas, spotty pigmentation and endocrine over activity, the myxomas are usually multiple and have atypical locations. The authors report a case in which an accurate diagnosis of these multiple myxomas was made by transoesophageal echocardiography, although transthoracic echocardiography had missed the diagnosis.

[Cardiac sarcoidosis responsible for localized left ventricular ectasia and refractory ventricular tachycardia. Anatomoclinical study]. / Sarcoïdose cardiaque responsable d'une ectasie ventriculaire gauche localisée et de tachycardies ventriculaires réfractaires. Etude anatomo-clinique.

The authors report the case of a 63 year old woman admitted to hospital for recurrent refractory ventricular tachycardia. Echocardiography and cardiac scintigraphy showed global left ventricular function. Ventriculography confirmed the left ventricular dysfunction and also showed a localised aneurysm of the anterior left ventricular wall. Surgical resection of the aneurysm and an encircling endocardial ventriculotomy were performed but the patient died of a low output syndrome. Pathological examination of the excised tissue showed granulomatous lesions associated with fibrosis interrupting the striated myocardial bundles. The granulomata consisted in a large number of epithelioid histiocytes and very large giant cells with many nuclei. The diagnosis made retrospectively was that of cardiac sarcoidosis causing a ventricular aneurysm and global left ventricular dysfunction. The diagnosis of cardiac sarcoidosis is difficult in the absence of systemic extracardiac involvement because the clinical manifestations and complementary investigations are non specific. The diagnosis may be made by endomyocardial biopsy in 25 per cent of cases, thereby leading to specific treatment with steroids which is sometimes effective.

[Right-left shunt caused by sarcoma of the right atrium]. / Shunt droit-gauche secondaire à un sarcome de l'oreillette droite.

A 70 year old woman was admitted for right ventricular failure and cyanosis of recent onset. Echocardiography showed a very large, homogenous, immobile, smooth-contoured mass filling the right atrium. Right atrial pressures were raised but the other intracardiac pressures were normal at catheterisation. Right heart angiography confirmed the voluminous right atrial mass and dilatation of the hepatic veins and showed early opacification of the left heart chambers. A right-to-left shunt was confirmed by oximetry which showed significant desaturation of the blood in the left atrium and ventricle (saturation 78% in the left ventricle). The tumour was also documented by a thoracic CT scan. At surgery, a very large, malignant right atrial tumour was resected which histological examination showed to be an angiosarcoma. The interatrial septum seemed to be intact: there was no true atrial septal defect but a persistent foramen ovale was found. After resection of the tumour the right atrium was reconstructed. The initial postoperative period was uncomplicated but the patient died nine months later of metastatic disease particularly affecting the liver and brain. The clinical presentation of malignant cardiac tumour is very variable but a right-to-left interatrial shunt through a patent foramen ovale has only been reported previously in 2 cases of primary malignant tumours (a rhabdomyosarcoma and an angiosarcoma) and in 1 case of a secondary cardiac metastasis.

[Myocardial dissection in infarction of the right ventricle. Clinical echocardiographic and pathological aspects]. / La dissection myocardique dans l'infarctus du ventricule droit. Aspects cliniques, échocardiographiques et autopsiques.

Dissection of the inferior wall of the right ventricle during the acute phase of myocardial infarction with right ventricular involvement is a mechanical complication which has been recently identified, the diagnosis being almost exclusively post-mortem. The authors report the clinical, echocardiographic and pathological features of myocardial dissection in four patients. Between 1985 and 1988, the diagnosis of myocardial dissection was made by echocardiography in 4 patients aged 77 to 80 years, admitted to hospital for an acute inferior wall myocardial infarction. All 4 patients had signs of acute right ventricular failure indicating right ventricular necrosis and a loud systolic murmur at the left sternal border; 2 patients were in shock. The ECG showed signs of inferior wall infarction with, in 2 patients, electrical changes suggestive of right ventricular involvement. Echocardiography showed dissection of the inferior wall of the right ventricle as a pulsatile, echo-free space in the diaphragmatic wall of the right ventricle which appeared to obstruct right ventricular ejection in end systole to a variable degree. The outcome was fatal in all cases with death resulting from refractory myocardial failure. Pathological analysis confirmed biventricular inferior wall infarction also involving the posterior part of the interventricular system, the site of a small tear on the left side which communicated with a neo-cavity dissecting the RV posterior wall. The right coronary artery was totally occluded in all cases. The anatomical lesions were fully concordant with the echocardiographic data: the dissection filled with blood from the left ventricle at each systole creating a pulsatile space in the diaphragmatic wall of the ventricle obstructing ejection.

[Contribution of liver biopsy and serology of hepatitis C virus to the diagnosis of a moderate and prolonged elevation of aminotransferases]. / Apports de la biopsie hépatique et de la sérologie du virus de l'hépatite C au diagnostic d'une augmentation modérée et prolongée des aminotransférases.

The aim of this study was to evaluate the diagnostic usefulness of percutaneous liver biopsy and screening for hepatitis C virus antibodies with 1st and 2nd generation ELISA in asymptomatic blood donors with persistent (> 1 year) and moderate elevation (> 1.5 times the upper limit of normal) of serum alanine aminotransferase. The diagnosis was established from clinical, biological and ultrasound data before biopsies were obtained, then compared to the histological diagnosis. Thirty one of 56 blood donors who satisfied the preceding criteria accepted liver biopsy and were subsequently included in the study. An accurate diagnosis was proposed before biopsy in 20 cases. This was in agreement with the histological results in 19 cases but in 2 of these, unexpected lobular hepatitis was associated with the expected steatosis. Positive hepatitis C virus tests corresponded to chronic hepatitis in all cases (n = 5). No accurate diagnosis could be proposed in the 11 remaining cases owing to the lack of evidence of any etiology (n = 4) or because several potential etiologies were possible for the same subject (n = 7). Histological diagnoses were: isolated steatosis (n = 12), steatosis associated with lobular hepatitis (n = 7) or with chronic persistent hepatitis (n = 1), chronic active (n = 2) or chronic persistent hepatitis (n = 3), alcoholic hepatitis (n = 2), hemochromatosis (n = 1), and normal liver (n = 3). Liver biopsy is essential to the accurate etiological diagnosis of persistent and moderate elevation of aminotransferases despite hepatitis virus C tests which are associated with the correct diagnosis of chronic hepatitis in 16% of cases.(ABSTRACT TRUNCATED AT 250 WORDS)

[Etiology and diagnostic of viral bronchopneumonias]. / Etiologie et diagnostic des bronchopneumopathies virales.

Community viral bronchopneumonias are frequent, mainly in children, and can be associated to all respiratory viruses: influenza- and parainfluenzavirus, respiratory syncytial virus, adenovirus, rhinovirus. The diagnostic method which proves viral infection of the respiratory tissues is selected as the direct detection by an immunofluorescence assay of viral infected cells in respiratory samples. In them, viral isolation or nucleic acid detection by PCR provide an amplification of the viruses. By using PCR-hybridation techniques viral detection is overall increased of 1.5 times for respiratory syncytial virus, 1.9 for parainfluenzavirus 3, 4 for rhinovirus and 10 times for adenovirus. This increased sensitivity raises questions about the meaning of the detection of viral sequences in nasal aspirates, with or without clinical signs. Cytomegalovirus (CMV) is a major agent of pneumonia in immunocompromised patients. All virological markers of CMV infection have to be sought (antigenemia, viremia...), but specific inclusions in pulmonary cells are the single diagnosis criteria. As pulmonary biopsies are rarely available and CMV inclusions rarely found in BAL, it has been reported useful to look for high viral loads or late m-RMA transcripts in these samples. Adenovirus pneumonia are unfrequent in these patients and mostly associated to rare or atypical strains. Such PCR-hybridization systems deserves also to be used in these cases.

[Role of bronchial resection-anastomosis in conservative surgery of lung cancer. Apropos of 34 cases]. / Place des résections-anastomoses bronchiques dans la chirurgie conservatrice du cancer bronchique. A propos de trente-quatre cas.

We report our 20 year experience with bronchial sleeve resection for endobronchial neoplasms. Among 832 patients who underwent resection procedures, thirty-four underwent a bronchial sleeve resection (4.1%): 27 bronchogenic carcinomas out of 780 (3.5%) and 7 carcinoids tumors out of 52 (13.4%). Bronchial sleeve resection is an appropriate treatment for neoplasms with low grade malignant potential and selected cases of bronchogenic carcinoma (tumor invading the adjacent main-stem bronchus without large hilar extension), especially for patients with compromised pulmonary function. There were no operative deaths; but minor complications occurred in 20% of patients without any major complications. Sleeve resection is a safe and adequate resection therapy for tumors with low-grade malignant potential and for selected cases of carcinoma.

[Costal chondroma and chondrosarcoma]. / Chondromes et chondrosarcomes costaux.

The aim of this study was to report two cases of chondrosarcoma located on the chest wall, in order to emphasize the difficulty encountered by the pathologist to differentiate a chondrosarcoma from a chondroma and the importance, in our opinion, of performing a large resection with wide margins in all cases.