[Thymic and bronchial neuroendocrine tumors in multiple endocrine neoplasia type 1. GENEM1]. / Tumeurs neuro-endocrines thymiques et bronchiques au cours des néoplasies endocrines multiples de type 1. GENEM1.

OBJECTIVES: Multiple endocrine neoplasia type 1 (MEN 1) mainly affects parathyroid glands, pancreatic islets and pituitary gland. The aim of this study in 95 MEN 1 patients was to examine less frequent localizations thymic and bronchic neuroendocrine tumors (NET). PATIENTS AND METHODS: Two cases of bronchic NET were observed, both in women, and 4 cases of thymic NET, all in men, giving a prevalence of 7.3%. These NET were often asymptomatic. A metastatic diffusion was observed in 3 cases. Elevated plasma levels of glycoprotein hormone alpha subunit (SU) and FSH were observed in 3 and 1 cases respectively. Immunohistochemistry indicated the tumor cells to be frequently positively stained for HCG alpha and FSH. DISCUSSION: We conclude that all patients with MEN 1 should undergo screening for thoracic NET, especially in high risk familial subgroups and in case of elevated plasma alpha SU or FSH.

Localized invasive pulmonary aspergillosis in patients with neutropenia. Effectiveness of surgical resection.

BACKGROUND: Invasive pulmonary aspergillosis (IPA) is a major cause of morbidity and mortality in patients with neutropenia. Two severe complications with poor outcome can be observed after apparently successful IPA medical treatment: severe hemoptysis and IPA relapse during subsequent cytotoxic treatments. Early surgical therapy has not been considered routinely in the management of localized IPA. METHODS: Six consecutive patients (four women, two men; median age, 52 years) with localized cavitating IPA diagnosed during chemotherapy-induced aplasia were treated with early surgical resection after hematologic recovery. RESULTS: All patients received a lobectomy. Surgery was uneventful. This procedures allows patients to proceed with further intensive chemotherapy and/or bone marrow transplantation without IPA reactivation. CONCLUSIONS: For selected patients, surgical resection of localized IPA with unique cavitating lesion, which prevents hemoptysis and IPA recurrence and allows for subsequent cytotoxic treatment, may be recommended.

[Pulmonary lesions in Wegener's disease. Report of the French Anatomo-clinical Research Group. Study of 40 pulmonary biopsies]. / Les lésions pulmonaires de la maladie de Wegener. Rapport du groupe français de recherche anatomo-clinique. Etude de 40 biopsies pulmonaires.

We report the results of a morphological analysis of 60 pulmonary biopsies gathered from a multi center study, organised by the clinico-pathological research group on Wegener's Disease under the auspices of the French Language Society of Thoracic Medicine. Forty of the sixty cases analysed were retained after indexing the histological aspects in order to specify their diagnostic value. Two groups of lesions were distinguished, which had different significance. Group A: These include the three major diagnostic criteria, which reinforce one another as they associate: 1) The polymorphoneutrophil microabscesses with limited central necrosis or an extended necrosis like the contours of a relief map. 2) An angiitis (arteries, veins, capillaries) with eccentric focal parietal crescent-shaped microabscesses. 3) Polymorphous granulomas with giant cells. Group B: In this group are the minor morphological observations (table II) of a lesser value and significance. 1) Acute or chronic lesions with alveolar haemorrhage, endogenous lipid pneumonia, xanthomatous granulomas, an organising pneumonia with an alveolitis. 2) Bronchial lesions: Bronchitis and necrotising bronchiolitis, which is more rarely follicular. 3) Sero-fibrinous or infiltrative neutrophil pleural lesions with focal microabscesses, elastolysis and elastophagia with giant cells in the elastic lamina. Thirteen cases presented with misleading lesions, which was a possible source of diagnostic error and led to a discussion of several associated disorders (Goodpasture's syndrome, and collagen disorder syndrome) or there may be systemic angiitis (Giant cell or lymphocytic) or also systemic or tissue eosinophilia (Churg-Strauss syndrome, bronchocentric granulomatosis) or necrotising bronchitis (atrophic polychondritis) or other forms of nodular interstitial fibrosis, such as histiocytosis X. We would like to stress the great polymorphic variation of the lesions and the difficulties which confront pathologists in the diagnosis of Wegener's Disease, above all when it is localised to the lung. There is value in finding at least one major diagnostic criteria which is associated with a minor criteria and with the help of the C.ANCA levels may lead to a narrow clinicopathological correlation and allows for a fairly precise approach to the diagnosis and identification of early or unusual lesions and thus to the early treatment of patients before irreversible renal failure appears.

[Fixation of Indium111-labeled antifibrin monoclonal antibody on the vegetation in endocarditis: evaluation in a rabbit model]. / Fixation d'un anticorps monoclonal anti-fibrine marqué à l'indium111 sur les végétations d'endocardite: évaluation sur le modèle d'endocardite du lapin.

The uptake of a 111In labelled antifibrin antibody was studied in left ventricle endocarditis on a rabbit model. The immunospecificity of the antibody for the cardiac vegetations is favorable, exhibiting an uptake at least 4 times that of blood, or myocardium. A planar scintigraphy of the opened left ventricle showed a radionuclide imaging of vegetations, according to anatomical lesions. The use of antifibrin monoclonal antibodies could prove helpful to improve the specificity of valvular lesions visualized by echocardiography, or to detect the small vegetations at the early stage of acute endocarditis.

[Pulmonary arteriovenous fistula]. / Fistule artério-veineuse pulmonaire.

A case of pulmonary arteriovenous fistula in a 6 1/2 year-old girl is reported. The fistula which was first seen on X-ray films resulted in low arterial oxygen saturation, asthenia and dyspnea during exercise. It was confirmed by right catheterism and angiography. The child was cured after left inferior lobectomy. No other vascular malformations could be demonstrated in the child or in her family. Literature data are reviewed.

[Experimental study of the subacute toxicity of mitoxantrone and doxorubicin in rats (subcutaneous and/or intraperitoneal route)]. / Etude expérimentale de la cardiotoxicité subaiguë de la mitoxantrone et la doxorubicine chez le rat. (voie sous-cutanée et/ou intrapéritonéale).

The aims of this study were to compare experimentally the subacute cardiotoxicity of a new anthracycline, mitoxantrone (MIT) with doxorubicin (ADM), the reference anthracycline drug. Seventy nine male rats were divided into 5 groups receiving one of the drugs or a placebo by subcutaneous (SC) or intraperitoneal (IP) injection, each week for 13 weeks. The surviving rats were sacrificed at the 21st week. Group I comprised 16 rats which received 2 mg/kg AMD-IP. The mortality rate was 50% between the 14th and 18th week. Twelve of the 16 cases had a haemorrhagic ascites. The heart was normal macroscopically and on light microscopy. However, electro microscopy showed moderate myocytic degeneration. Group II comprised 16 rats which received 0.6 mg/kg MIT-IP. Morality was 100% at the 11th week. Groups III and IV comprised 32 rats; half of the animals were given 0.6 mg/kg MIT-SC and the other half 0.4 mg/kg MIT-SC. The results were identical: good clinical tolerance, normal macroscopy. Three cases of lymphocytic myocarditis and in 4 out of 8 cases very mild myocardial degeneration on electron microscopy. Group V comprised 15 rats which were given NaCl 9% SC or IP; there were no complications. These results show that mitoxantrone given IP produced major peritoneal toxicity. On the other hand, it was well tolerated when given SC and only produced mild myocardial degenerative changes.

[Aortic sigmoid cusp anomaly: rare cause of myocardial infarction in children]. / Anomalie des sigmoïdes aortiques: cause rare d'infarctus myocardique de l'enfant.

A three and a half year old child with no previous medical history died, 20 hours after extensive anterior myocardial infarction. Post-mortem examination showed an abnormal aortic valve: the cusps were thick, indurated and white in colour. The free edge of the left antero-lateral cusp was higher than that of the other two cusps and presented a discrete thickening which overlapped the superior border of the left coronary ostium, obstructing the orifice at its thickest part. The other two cusps were tipped by fibrous spurs, one of which partially obstructed the right coronary ostium which was slightly narrowed at its origin. A review of the literature on aortic pathology causing coronary obstruction revealed rare cases of malignant disease and, more commonly, malformations usually associated with supravalvular aortic stenosis. This case might illustrate a partial and atypical form of supravalvular aortic stenosis.

[Comparison of the cardiotoxicity of adriamycin and aclacinomycin A in the rat. Optical and electron microscopic study]. / Comparaison de la cardiotoxicité de l'adriamycine et de l'aclacinomycine A chez le rat. Etude en microscopie optique et électronique.

Adriamycin (ADM) is a very effective antimitotic agent but its use is limited by its cardiotoxicity. New anthracycline drugs such as aclacinomycin A (ACMA) have been developed and have to be compared with ADM after chronic experimental intoxication. Three groups of randomised rats were compared: the ADM group receiving 2 mg/kg/week X 13 by intraperitoneal injection; the ACMA group receiving 4 mg/kg/week X 13 and a control group: 7 rats. The rats were autopsied at the 20th week. The heart was stopped in diastole and fixed by aortic retroinfusion of glutaraldehide for electronic microscopy (EM). In the ADM group, mean weight fell from the 4th week and mortality was 11/16 at 20 weeks. Voluminous haemorrhagic ascites was associated with peritoneal fibrosis in 12/16. Cardiac failure was observed in 4 cases but on light microscopy (LM) myofibril degeneration was constant and focal without sarcoplasmic reticulum or mitochondrial changes on EM. In the ACMA group the loss of weight occurred at 10 weeks and mortality due to toxicity was nil. There was no cardiac failure; myocytolysis was absent on LM and slight in 4/13 cases on EM with a moderate dilatation of the sarcoplasmic recticulum and presence of numerous residual bodies in the striated skeletal fibres in 5/15 cases. In this study, the ACMA had very little cardio and general toxicity in comparison with ADM. The technique of fixing the heart by retrograde infusion prevents, as far as possible, artefacts on EM affecting mainly the mitochondria.

[Endomyocardial biopsy as a method of surveilling adriamycin cardiac toxicity. 28 biopsies in 26 patients]. / Place de la biopsie endomyocardique comme méthode de surveillance de la toxicité cardiaque de l'adriamycine. 28 biopsies chez 26 patients.

The aim of this study was to evaluate the role of endomyocardial biopsy in the surveillance of toxic, latent cardiomyopathy secondary to adriamycin (ADM) therapy, and to assess its value by comparison with non-invasive investigations such as echocardiography. Twenty six patients underwent endomyocardial biopsy and echocardiographic measurement of the fractional shortening of the left ventricular internal diameter after a total dose of 150 to 520 mg/m2 of ADM. Interstitial fibrosis was observed in 8 of the 20 interpretable cases on light microscopy (6 moderate, 2 severe). There were no cellular changes. Slight focal myofibril degeneration was observed in 8 cases, and vacuolisation corresponding to mild dilatation of the sarcoplasmic reticulum was observed in 9 cases out of the 22 technically interpretable cases on electron microscopy. In contrast to the findings of interstitial fibrosis, cellular degeneration correlated significantly with the total dose of ADM (p less than 0,01). We conclude that this histological study confirms the fact that myocardial changes are rare and slight with doses of less than 520 mg/m2 of ADM and that non-invasive techniques are adequate for follow-up until this dose is reached.

[Collective acute poisoning by nitrous gases]. / Intoxication aiguë collective par les vapeurs nitreuses.

A collective nitrous fumes poisoning (five cases) is reported. Two patients (case 3 and case 4) were comatose, in severe respiratory distress. Shock and slate blue cyanosis were noted. Physical examination and chest X ray revealed acute pulmonary edema-Methemoglobin levels were 71,3% (case 3) and 58% (case 4). Despite treatment both of them died from severe hypoxia resulting in cardiorespiratory arrest. Post-mortem examination was performed upon these four men. On admission the last one (case 5) was conscious, and in good hemodynamic condition. Acute pulmonary edema and cyanosis were present. Methemoglobin level was 37,3%. This patient recovered appropriate therapy. For case 1 and 2 acute anoxia due to methemoglobinemia seems to be cause of death. For cases 3 and 4 death is due to hypoxemia associated with pulmonary edema.

[Encapsulating perihepatitis. Report of four cases (author's transl)]. / Le foie glacé de Curschmann. A propos de quatre observations.

The authors report 4 cases of encapsulating perihepatitis, the macroscopic appearance of which corresponded to the morphological definition of the condition. The circumstances surrounding the diagnosis were very variable: association of ascites and porial hypertension, sub-obstructive syndrome due to associated encapsulating peritonitis, chance discovery at laparotomy or an unexpected finding at autopsy. The diagnosis was made pre-operatively in 2 cases by peritoneoscopy. All the patients had portal hypertension with, in particular, evidence of oesophageal varices. Three patients were treated surgically: hepatic decortication in all cases, combined in one another with umbilico-caval anastomosis. In all cases, the underlying perihepatic matrix produced epiglissonian fibrosis. However the inderlying hepatic lesions varied, ranging from definite cirrhosis to mild fibrosis. In one case, cirrhosis was associated with a hepato-cholangioma with nodular metastatic spread. The prognosis depended essentially upon the effects of general condition and the severity of hepatic lesions. In the light of cases reported over the past twenty years, the authors review the various clinical, pathogenic and therapeutic aspects of encapsulating perihepatitis.