[Bronchial presentation of stage IVB Hodgkins disease]. / Localisation bronchique révélatrice d'une maladie de Hodgkin.

INTRODUCTION: Bronchial localisation of Hodgkins disease has been described only rarely and it is exceptional as the presenting feature. More frequently the clinical presentation is extrinsic bronchial compression. OBSERVATION: Mr B.C., 34 years old, had a past history of cured alcoholism and abuse of cannabis and buprenorphine. In February 2001 he presented with generalised pruritus, weight loss of 8 kg in one year, a 1 cm node above the right clavicle, hypereosinophilia, and CT evidence of non-obstructive mediastinal adenopathy and interstitial lesions in the right lower lobe. Five months later he was admitted to hospital with a febrile illness. The right supraclavicular node had disappeared but there was gross mediastinal adenopathy, associated with a right perihilar soft tIssue mass encircling the upper, middle and lower lobe bronchi and multiple peripheral parenchymal nodules. Bronchoscopy revealed complete stenosis of the right upper lobe bronchus and bronchial histology confirmed Hodgkins disease. CONCLUSIONS: Bronchoscopy is an effective investigation for the detection of endobronchial Hodgkins disease, often overlooked because parenchymal abnormalities are attributed to extrinsic compression. In order to determine the disease stage more precisely we advise immediate bronchoscopy when there is suspicion that Hodgkins disease may be the cause of clinical.

Evaluation of cardiac troponin I and T levels as markers of myocardial damage in doxorubicin-induced cardiomyopathy rats, and their relationship with echocardiographic and histological findings.

BACKGROUND: Cardiac troponins I (cTnI) and T (cTnT) have been shown to be highly sensitive and specific markers of myocardial cell injury. We investigated the diagnostic value of cTnI and cTnT for the diagnosis of myocardial damage in a rat model of doxorubicin (DOX)-induced cardiomyopathy, and we examined the relationship between serial cTnI and cTnT with the development of cardiac disorders monitored by echocardiography and histological examinations in this model. METHODS: Thirty-five Wistar rats were given 1.5 mg/kg DOX, i.v., weekly for up to 8 weeks for a total cumulative dose of 12 mg/kg BW. Ten rats received saline as a control group. cTnI was measured with Access(R) (ng/ml) and a research immunoassay (pg/ml), and compared with cTnT, CK-MB mass and CK. By using transthoracic echocardiography, anterior and posterior wall thickness, LV diameters and LV fractional shortening (FS) were measured in all rats before DOX or saline, and at weeks 6 and 9 after treatment in all surviving rats. Histology was performed in DOX-rats at 6 and 9 weeks after the last DOX dose and in all controls. RESULTS: Eighteen of the DOX rats died prematurely of general toxicity during the 9-week period. End-diastolic (ED) and end-systolic (ES) LV diameters/BW significantly increased, whereas LV FS was decreased after 9 weeks in the DOX group (p<0.001). These parameters remained unchanged in controls. Histological evaluation of hearts from all rats given DOX revealed significant slight degrees of perivascular and interstitial fibrosis. In 7 of the 18 rats, degeneration and myocyte vacuolisation were found. Only five of the controls exhibited evidence of very slight perivascular fibrosis. A significant rise in cTnT was found in DOX rats after cumulative doses of 7.5 and 12 mg/kg in comparison with baseline (p<0.05). cTnT found in rats after 12 mg/kg were significantly greater than that found after 7.5 mg/kg DOX. Maximal cTnI (pg/ml) and cTnT levels were significantly increased in DOX rats compared with controls (p=0.006, 0.007). cTnI (ng/ml), CK-MB mass and CK remained unchanged in DOX rats compared with controls. All markers remained stable in controls. Analysis of data revealed a significant correlation between maximal cTnT and ED and ES LV diameters/BW (r=0.81 and 0.65; p<0.0001). A significant relationship was observed between maximal cTnT and the extent of myocardial morphological changes, and between LV diameters/BW and histological findings. CONCLUSIONS: Among markers of ischemic injury after DOX in rats, cTnT showed the greatest ability to detect myocardial damage assessed by echocardiographic detection and histological changes. Although there was a discrepancy between the amount of cTnI and cTnT after DOX, probably due to heterogeneity in cross-reactivities of mAbs to various cTnI and cTnT forms, it is likely that cTnT in rats after DOX indicates cell damage determined by the magnitude of injury induced and that cTnT should be a useful marker for the prediction of experimentally induced cardiotoxicity and possibly for cardioprotective experiments.

[Rapidly progressive dementia disclosing primary angiitis of the central nervous system]. / Démence d'installation subaiguë révélant une angéite primitive du système nerveux central.

Angiitis of the central nervous system involves a wide spectrum of clinical symptoms. Complementary exams (CSF, neuroradiological exams) are not very specific and sensitive. Diagnostic procedures often require cortical and leptomeningeal biopsy. Treatment, based on corticosteroids and cyclophosphamid, have greatly improved prognosis. We report a rapidly progress dementia syndrome that revealed angiitis of the central nervous system. Corticosteroid treatment did not prevent a fatal outcome. Autopsy findings confirmed the diagnosis of central nervous leucocytoclastic angiitis.

Comparison of the diagnostic value of cardiac troponin I and T determinations for detecting early myocardial damage and the relationship with histological findings after isoprenaline-induced cardiac injury in rats.

Cardiac troponins I (cTnI) and T (cTnT) have been shown to be highly sensitive and specific markers of myocardial cell injury. The purpose of this study was to investigate the diagnostic value of cTnI and cTnT with regard to creatine kinase (CK) and lactate dehydrogenase (LD) and to determine whether they can be used for early diagnosis of myocardial damage in rats, and to examine the relationship between cTnl and cTnT release with histological examinations, using isoprenaline-induced cardiac muscle damage as an experimental model in the rat. Eighteen Wistar rats per group were treated with a single dose of either isoprenaline (iso) or with normal saline as a control group. The anti-cTnI and cTnT monoclonal antibodies (mAbs) employed in the cTnI (Access) and cTnT (Elecsys) assays cross-react with cTnI and cTnT of the rat. A highly significant rise of cTnl or cTnT was found already 2 h after iso. The time-courses of cTnI and cTnT were monophasic in form. The highest cTnI (mean+/-S.D., 1.1+/-2.3 ng/ml) and cTnT (mean+/-S.D. 3.6+/-30 ng/ml) were found 4 h after iso. cTnI and cTnT significantly increased in iso-treated rats in comparison with controls whether the differences between 2-, 4- and 6-h levels and basal levels were considered or not. The areas under cTnl and cTnT curves (AUC) (0-6 h) and the maximal cTnI and cTnT (0-6 h) after iso were significantly different from the controls. For CK and LD, no elevation in comparison with controls could be detected (except a trend for LD whether or not the difference between 6-h levels and basal levels were considered (P=0.08) and for LD AUC (0-6 h) (P=0. 059)). Correlations between maximal cTnI and cTnT and AUC were 0.69 (P=0.0001) and 0.60 (P=0.0066), respectively. Histological examinations of iso-treated rats revealed acute focal or multifocal myofibrillar degeneration of the myocardial tissue in ten out of 14 rats and showed the earliest alterations 4 h after iso in one treated rat. Only four of the controls exhibited evidence of mild changes and slight mononuclear cell infiltration. cTnl and cTnT peak values to at least 0.35 and 1.3 ng/ml, respectively, were necessary to detect histological myocardial cell injury after iso. cTnI and cTnT were found to be early markers for diagnosing iso-induced myocardial damage in comparison with CK and LD. Elevations of cTnI and cTnT appeared to relate to the severity of histologic changes after myocardial injury. Although there was a difference in the absolute concentration of results between cTnI and cTnT assays, due to a lack of standardization and heterogeneity in the cross-reactivities of mAbs to various troponin I and T forms, cTnI and cTnT can be used as easily measurable target parameters for detection of cardiotoxic and/or cardiodegenerative effects in rats.

Dissociated overexpression of cathepsin D and estrogen receptor alpha in preinvasive mammary tumors.

The role of estrogen as a promoter agent of sporadic breast cancer has been considered by assaying, in benign breast disease (BBD) and in situ carcinomas (CIS), 2 markers, the estrogen receptor alpha (ERalpha) and cathepsin D (cath-D) involved in estrogen action on mammary tissue. ERalpha and cath-D were assayed by quantitative immunohistochemistry using an image analyzer in 170 lesions of varying histological risk (94 BBD and 76 CIS), and in "normal" glands close to these lesions. The ERalpha level increased significantly in proliferative BBD with atypia (P < .001), in non-high-grade CIS (P < .001), and in adjacent "normal" glands. ERalpha level was decreased in high-grade ductal CIS (DCIS) and also in adjacent "normal" glands. Cath-D level increased in ductal proliferative BBD (P < or = .01) and in high-grade DCIS (P < or = .003), but not in the other lesions. After menopause, ERalpha level was increased (P = .012) but not cath-D level. According to Mac Neman test, the high-grade DCIS were predominantly ERalpha negative and cath-D positive (P = .0017), and the other CIS were predominantly ERalpha positive and cath-D negative (P = .0002). The 2 markers are overexpressed early in premalignant lesions, but independently. This dissociation suggests a branched model of mammary carcinogenesis involving 1 estrogen-independent pathway with high cath-D and low ERalpha levels (including high-grade DCIS) and 1 estrogen-dependent pathway, with high ERalpha level (including proliferative BBD with atypia and low-grade DCIS). We propose that ERalpha-negative breast cancers may develop directly from high-grade DCIS and that ERalpha assay in preinvasive lesions should be considered in prevention trials with antiestrogens.

[Ectopic intraspinal extradural anaplastic ependymoma in an infant]. / Ependymome anaplasique ectopique intrarachidien extradural chez un nourrisson.

BACKGROUND: Ependymomas represent about 10% of the spinal tumors in children. Some of them may be unusually located. CASE REPORT: A 10-month-old boy was admitted for an abdominal mass syndrome with dehydration asthenia and acute bladder dysfunction. A few hours later, he developed a flaccid paraplegia. Ultrasonic and magnetic resonance spinal imaging showed a giant intraspinal tumor extending from T9 to IA level, posteriorly located to the dural compartment, widening the spinal cord. Ultrasonography also showed right ureterohydronephrosis due to the neurological bladder dysfunction. A conservative laminotomy-laminoplasty was performed in emergency. Total removal of the tumor that was attached to the right dorsal root was achieved extradurally, requiring resection of the proximal part of the root. Histological features were typical of malignant ependymoma. Chemotherapy was initiated 2 weeks later. The severe renal destruction and the persistent bladder dysfunction led to a heminephrectomy and a cystostomy, 3 weeks later. The neurological recovery was only partial with a follow-up of 18 months. CONCLUSION: Ectopic intraspinal extradural localization of ependymomas is rare and their development from a nerve root is exceptional.

[Neuroendocrine tumor of the bladder apropos of a case. Review of the literature]. / Tumeur neuroendocrine de vessie à propos d'un cas. Revue de la littérature.

Neuroendocrine bladder tumours are exceptional, and the positive diagnosis is only established when they are already large and advanced. We report an original case in view of its small dimensions. We discuss the differential diagnosis (mainly bladder metastases from lung cancer) and pathological specificities, particularly the value of epithelial immunolabelling allowing exclusion of lymphoma. Because of the similarities with bronchial neuroendocrine tumours, the potential value of serum NSE assay should be emphasized. Combined surgery-cisplatin-based adjuvant chemotherapy is recommended.

[Lymph node eosinophilic granuloma. Apropos of 2 cases of Langerhans-cell histiocytosis with isolated lymph node involvement]. / Le granulome éosinophile ganglionnaire. A propos de deux cas d'histiocytose langerhansienne à localisation ganglionnaire isolée.

The authors report two cases of isolated lymph node involvement by Langerhans' cell histiocytosis which affected two young children. The histologic aspect reveals that lymph nodes have been modified by a proliferation of large histiocyte-like cells, associated with eosinophils. An immunohistochemical study on paraffin sections and for one case on frozen sections, reveals the usual phenotype of Langerhans' cells: these cells stain positively with S 100 protein and CD1 and are negative for both lysozyme and al antichymotrypsine. After a period of two years for one child and four years for the other, these children are in total remission, one spontaneously, the other after chemotherapy.

[Renal nephrogenic nephroma: an little known exceptional tumor. Apropos of a case disclosed by xanthogranulomatous pyelonephritis]. / Le néphrome néphronogène rénal: une tumeur exceptionnelle peu connue. A propos d'un cas révélé par une pyélonéphrite xantho-granulomateuse.

The authors report a new case of an exceptional benign renal tumour: nephronogenic nephroma, composed of differentiation of the blastema into primitive nephronic formations. It raises the problem of differential diagnosis with adult Wilms' tumour and renal blastematosis. In this patient, it was associated with segmental xanthogranulomatous pyelonephritis, presenting in the form of psoïtis and an abscess of the thigh. Total nephrectomy was performed.

Postoperative pseudosarcomatous nodule: report of one case and review of the literature.

We present a case of a large postoperative pseudosarcomatous bladder tumour and review the literature for this exceptional benign tumour. Several lesions, especially leiomyosarcoma, must be discussed. Immunohistochemical studies are helpful. The evolution is always benign, even with an incomplete tumour ablation.

Immunohistochemical study of tumor cell-associated plasminogen activators and plasminogen activator inhibitors in lung carcinomas.

STUDY OBJECTIVE: To compare the expression of plasminogen activators (PA) and plasminogen activator inhibitors (PAI) in normal lung mucosa and lung carcinomas. DESIGN: Immunohistochemical localization of urokinase-type PA (uPA), tissue-type PA (tPA), type 1 PAI (PAI-1), and type 2 PAI (PAI-2) in four normal lung biopsy specimens and in four adenocarcinomas (AC), four squamous carcinomas (SC), two large-cell carcinomas (LCC), and ten small-cell carcinomas (SCC) biopsy specimens. Qualitative immunostaining scoring system. RESULTS: tPA and uPA immunostaining scores from all specimens were statistically similar. The cellular staining for uPA and tPA was generally constant (normal epithelial layers, AC cells, SC cells) except for LCC cells (inconstant uPA staining, p < 10(-3). Both PAIs were detected in cells of the normal epithelial layer. The four carcinoma cell types stained for PAI in a statistically different pattern (p < 10(-3)). Cells of the peripheral cords of SCC were inconstantly PAI-1 and PAI-2 positive (p < 10(-3)). LCC were PAI-2 negative and inconstantly stained for PAI-1. SCC cells were PAI-1 and PAI-2 negative. CONCLUSION: Lung carcinomas of worst clinical prognosis no longer express PAI reactivity. The imbalance of the plasminogen activation pathway may favor the spreading of the more invasive histologic types of bronchogenic carcinomas.

Peripheral pulmonary lesions: ultrasonic features and ultrasonically guided fine needle aspiration biopsy.

When transthoracic biopsy is required for diagnosing lung diseases, radiographic procedures are the methods of choice, sonographic application being still limited. Sixty-four consecutive patients with pulmonary lesions adjacent to the chest wall underwent sonography. Findings showed hypoechoic homogeneous lesions (56 cases) with posterior regular margin (57 cases). Increasing echoes deep to the mass and sonographic interruption of hyperechoic surface were seen in all patients. In two cases, chest wall invasion had been diagnosed. Histologic diagnosis was made in 55 of the 64 patients (85.9%). Two pneumothoraces occurred. Sonography is a useful, accurate, and safe technique for diagnosing selected pulmonary lesions.

Ultrasonically guided aspiration biopsy in osteolytic bone lesions of the chest wall.

Sixteen consecutive patients with one or more osteolytic bone lesions of the chest wall radiologically confirmed underwent ultrasonically guided aspiration biopsy. Nine patients (56.2 percent) had bronchogenic carcinoma with a direct extension. Other diagnostic techniques had failed to diagnose disease in these patients. The lesion showed heterogeneous echogenicity (n = 16) caused by the hyperechoic signals of bone fragments. The interruption of the cortex bone was detected in all cases and extraosseous tumor portion in 14 of 16 patients (87.5 percent). No respiratory motions of the lesion could be demonstrated (n = 16). Definitive histologic diagnosis was made in 14 of the 16 patients (87.5 percent). In malignancy, diagnosis was established in 13 of 14 patients (92.8 percent). Of two confirmed benign lesions, one diagnosis of tuberculosis was obtained. No complication occurred. Sonography and consequently ultrasonically guided aspiration biopsy are a useful, accurate, safe, and low-cost technique for osteolytic lesions in thoracic diseases.

Portal hypertension in lymphoproliferative and myeloproliferative disorders: hemodynamic and histological correlations.

The pathogenesis of portal hypertension in patients with lymphoproliferative and myeloproliferative disorders is not fully understood. We investigated 20 patients with myeloproliferative disease and 47 patients with lymphoproliferative disease. Transvenous liver biopsies and hepatic vein pressure gradient measurements were performed in all patients, and portal vein blood flow was measured by pulsed Doppler sonography in 31 of these patients and in 22 normal volunteers. The hepatic vein pressure gradient was significantly higher in patients with hepatic infiltrates, fibrosis or both than in patients without hepatic lesions (8.3 +/- 5.0 mmHg vs. 4.1 +/- 2.3 mmHg; p < 0.01). Portal vein blood flow was significantly higher in patients with hematological disease than in normal volunteers (31.2 +/- 15.5 ml/min.kg vs. 14.2 +/- 4.6 ml/min.kg;p < 0.01). In 81.8% of patients with hepatic infiltrates, fibrosis or both and increased portal vein blood flow, the hepatic vein pressure gradient was greater than 6 mm Hg. Although we saw a significant correlation between splenic vein blood flow and portal vein blood flow (n = 20; p < 0.01), we found no significant correlation between splenic vein blood flow and hepatic vein pressure gradient or spleen size. Hepatic infiltration and fibrosis appear to be major determinants of increased hepatic vein pressure gradient, probably because they increase intrahepatic vascular resistance. The role of increased splenic blood flow is probably not determinant. However, because portal pressure was not measured directly in this study, the incidence of portal hypertension may have been underestimated.

[Epstein-Barr virus infection and syndrome of inappropriate macrophage activation]. / Infection à virus Epstein-Barr et syndrome d'activation macrophagique inappropriée.

An 11-year-old girl presented with a typical serologically proven infectious mononucleosis with persistent fever, jaundice and hepatosplenomegaly in spite of steroid therapy. Laboratory tests showed pancytopenia, fibrinopenia and hypertriglyceridemia. The liver biopsy revealed an infiltration with hyperbasophilic cells. One month later, a slight improvement was noted and fever disappeared after 4 days on acyclovir therapy. The authors recall the spectrum of the macrophagic activation syndrome.