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Screening for fetal Down's syndrome has the peculiarity of combining the biochemical assay of 2 or 3 serum markers with the risk associated with maternal age. If the accuracy of measurement of each parameter is known by the biologist, the uncertainty of the ultimate risk to the patient is not. Indeed, the means of risk calculation involve numerous multi-parameter equations which are not practical for daily use. Defining a re-test limit on thresholds of 1/50 and 1/1,000 is therefore impossible. Since the use of an arbitrarily defined threshold is not being satisfactory, we propose, by default, a methodology based on the exploitation of patient files in the laboratory with risks close to the two decision thresholds. Modulations of the concentrations of all the markers according to their uncertainty allow new risks to be obtained, which can be averaged and framed by an interval of several standard deviations. Choosing the level of uncertainty, the number of files to include, the number of standard deviations framing the average risk, as well as the calculation software, are all choices available to the biologist. The proposed methodology is therefore highly empirical but open, and adaptable, to the specific environment and performance capabilities of each and every laboratory involved.

To assess concordance between double human and automated optical reading (AOR) concerning two biological tests for rupture of membranes (ROM) METHODS: We conducted a monocentric, prospective, observational study comparing Actim PromConcerning Actim PromWe demonstrated excellent correlation between biologists and good or excellent correlation between AOR and human reading supporting the use of AOR in clinical practice.

Cirrhosis is a frequent and severe pathology life threatening, due to the occurrence of complications including hepatocellular carcinoma. In order to assess the degree of conformity, of practices, with the French guidelines for the surveillance of patients with compensated cirrhosis and the primary prevention of complications, published in 2007, all general practitioners practicing in three towns from north-east suburb of Paris (Seine-Saint-Denis) representing a population pool of 186 286 inhabitants were asked during the first quarter of 2012 for the face-to-face administration of a standardized questionnaire. Based on the 20 questions, individual practices were analyzed and a compliance score limited to hepatocellular carcinoma screening was established. Of the 106 practitioners surveyed, 40 have agreed to participate (85% of men, mostly based in Aulnay-sous-Bois or Sevran with an average exercice of 22years). The declared practices were heterogeneous and did insuffisantly comply with the recommendations (mean score 1.9/3). The intent of surveillance was not influenced by the origin or severity of cirrhosis. Screening for HCC was based on appropriate tests (abdominal ultrasound, alpha-fœtoprotein serum) but with optimal half-yearly intervals in less than half of the cases. Improved communication to general private practitioners of HAS recommendations for patients with compensated cirrhosis could optimize the prescribing of screening tests for hepatocellular carcinoma.

Premature rupture of membranes (PRM) affects 5 to 15% of pregnancies, leading to prematurity and neonatal infection. PRM can be identified by through various amniotic fluid proteins in vaginal secretions. The aim of this study is to compare two immunochromatographic tests based on the detection of insulin-like growth factor binding protein (IGFBP-1) and alpha-foeto protein (AFP) for one of the two tests in cervico-vaginal secretions. Two tests, Actim(®) Prom and Amnioquick(®) Duo were performed on 80 pregnant women with suspected PRM. Amnioquick(®) Duo allows the simultaneous detection of IGFBP-1 and AFP with an automated incubation and reading. The number of positive results is similar (Khi-deux = 0.173, p = 0.6773) for IGFBP-1 between the two tests and there is a good agreement (K = 0.621), with a proportion of negative results of 86%. The number of positive results for AFP is more important in comparison to IGFBP-1. Results positive/positive (Actim(®) Prom/Amnioquick(®)) for IGFBP-1 seems to be related to the time when tests have been performed, that is to say in the last weeks of pregnancy. In conclusion, both tests have similar performance, but there is a risk of false positive results with AFP, this can be explained by the presence of non-visible blood in samples. An automated incubation and reading allows a good reproducibility. Moreover, the computer data storage improve the post-analytical quality.

Tumour markers are high molecular weight glycoproteins whose interpretation in the presence of chronic renal disease may be disturbed. Apart from the prostate specific antigen, human chorionic gonadotropin and α-fetoprotein, their levels rise with chronic renal failure. For some markers, such as carcinoembryonic antigen, carcinogen antigen (CA) 15-3, cytokeratin fragment 21-1 or calcitonin, a threshold may be defined in a population with chronic renal failure, which may allow their use in cancer surveillance. Others, such as CA125, CA19-9, squamous cell carcinoma and neurospecific enolase, have a high variability and their use in patients with chronic renal disease remains difficult.

Prenatal screening was set up to identify patients at high-risk of chromosome 21 trisomy based on maternal serum markers measurement. However, the risk of trisomy 21 should not be the only result considered by obstetricians. In fact, abnormal marker values can be associated with other fetal diseases and used to improve maternal and fetal follow-up. Our objective was therefore to study other predictive values of maternal serum markers.A search through publications was conducted using the PubMed® or Cochrane® databases.In case of high PAPP-A there is no link with any complications. Second trimester high hCG or first trimester low hCG are associated with an increased vascular risk. High α-fetoprotein level is a marker of neural tube defects or abdominal wall defect. Persistence of high α-fetoprotein with normal echography can suggest other rare fetal diseases. Low maternal serum markers suggests 18 trisomy. Oestriol reflects the fetal hypothalamo-hypophyseal axis and can be used as a diagnosis tool.Serum markers could be interesting tools for the identification of high-risk pregnancy and the prevention of neonatal complications. They also appear as a potential help to diagnose certain congenital malformations.

A recent review of the literature identified more than 150 reported cases of pancreatoblastoma in children. However, the clinical, histological and therapeutic characteristics of this tumour are hardly known by most paediatric surgeons and oncologists. The clinical symptomatology is often discrete, such as abdominal pain and/or intestinal transit disturbances, and the revealing sign is usually the discovery of a voluminous abdominal mass. Pancreatoblastoma is most often located in the head or body of the pancreas but can be seen in any part of the pancreas. It forms a full mass, rather well encapsulated, round and soft in consistency, often large in size and that can develop beyond the limits of the pancreatic gland. The metastases may be present in the lymph nodes, liver, lungs and spleen. It is an embryonic organ tumour that morphologically resembles what the nephroblastoma or the hepatoblastoma are for the kidneys or liver, respectively. The pathological analysis characteristically shows two components in which cell density is often high: an epithelial component and a mesenchymatic component. The lab test evaluation should include an assay of alpha-foetoprotein. Elevated levels of this marker are often present in these tumours. An assay of this marker is therefore interesting, not only at the time of diagnosis, but especially for early diagnosis of relapses. The pancreatoblastoma treatment is above all surgical and only complete exeresis makes recovery possible. However, at the time of diagnosis, many patients are inoperable due to the extension of the tumour. The combination of cisplatin + adriamycin seems to be the most effective neoadjuvant chemotherapy regimen. Patients who have had an incomplete tumour exeresis pose a real problem due to the frequency of local relapses and/or metastases. Local irradiation is indicated in this case, as chemotherapy has not yet provided proven results in this context.

Ovarian yolk sac tumour (OYST) is a very rare malignancy arising most often in young women. Preoperative clinical, biological (alpha-foetoprotein) and radiological findings should help to establish the diagnosis of OYST, in order to propose adequate surgical treatment. The aim of surgery is to remove the primary tumour, to obtain an accurate histological diagnosis and to assess the disease extent. In young women, fertility-sparing surgery should be performed, in order to preserve the possibility of pregnancy later on. Chemotherapy has substantially modified the prognosis of these tumours, and practically all patients will be cured. The overall 5-year survival rate is 94% when patients are treated with BEP chemotherapy. Depending on the clinical situation, two to four cycles of the BEP regimen should be administered after surgery. Identification of prognostic factors may help to propose risk-adapted treatment in order to increase the cure rate in patients with a poor prognosis and to decrease toxicity in patients with a low risk of relapse. Fertility preservation represents a major objective in women treated for OYSTs.

The newly developed, high throughput, Vitros 3600 immunoassay system associates different technologies on board: MicroWell (analytes immunocapture), MicroSensor (visible interference analysis), and Intellicheck (real time process control for analytical error detection). During a three months period, Vitros 3600 system, ease of use, practicability and MicroSensor technology efficiency have been tested using the following assays: betaHCG, troponin, ferritin, aHBcT, HBsAg, aHCV, AFP and CEA. Within-day and within-batch, between-calibration precisions are acceptable. Overall assay correlations versus the methods in use in our laboratory are good, despite small numerical differences that never affect clinical interpretation of the results. We have appreciated the system reliability, reinforced by the ease of use (simple software, on-board VDocs, continuous access to reagent and sample loading, reagent stability, very low noise (60 dB)), the quality of the results, MicroSensor and Intellicheck technologies efficiency, the short maintenance time and its traceability, and the very low liquid waste volume (5 L of liquid waste for 1600 tests). In conclusion and considering its practicability, the Vitros 3600 system is fully validated and well suited for our laboratory.

To describe clinical, biological characteristics and virological aspects of patients with hepatocellular carcinoma (HCC).Sera obtained from consent patients with clinical suspicion of HCC. Routine biochemical tests and serological markers of hepatitis B virus (HBV), hepatitis D virus (HDV) and hepatitis C virus (HCV) were searched. A hepatic ultrasound scan was realized and, when a suspected mass lesion was observed, ultrasonographic-guided fine needle aspiration of the hepatic mass lesion was made to ascertain the diagnosis of HCC.One hundred and seventy-five sera were collected from 99 men and 76 women. Approximately 96.6% (169/175) of them had previous contact with HBV. HBs surface antigen was positive in 41% (69/169) of whom 53.6% (37/69) were co-infected by the HDV. Three patients (1.71%=3/175) were positive for anti-HCV antibodies. The other three patients (1.71%=3/175) did not present any markers for HBV or HCV. At the time of diagnosis, right upper quadrant pain, hepatomegaly, cachexia, were present in all patients. An increase in serum transaminases (70%), high bilirubin concentrations (40%), high AFP levels (40%) and low prothrombin levels (52%) were the most frequent biological abnormalities. Only 17 guided fine needle aspirations of mass lesions were realized. The diagnosis of HCC was confirmed by cytopathological examination for 14 of them.HCC is a frequent pathology in the Central African Republic (CAR). According to the ultrasound imaging data, patients consult at terminal stage of the disease. This study confirms also the association between HBV and HCC in CAR. Moreover, in 26.5% of patients HDV is associated with severe forms of the disease.

To review the obstetrical outcomes associated with abnormally elevated or decreased level of one or more of the most frequently measured maternal serum marker analytes used in screening for aneuploidy. To provide guidance to facilitate the management of pregnancies that have abnormal levels of one of more markers and to assess the usefulness of these markers as a screening test.Perinatal outcomes associated with abnormal levels of maternal serum markers analytes are compared with the outcomes of pregnancies with normal levels of the same analytes or the general population.The Cochrane Library and Medline were searched for English-language articles published from 1966 to February 2007, relating to maternal serum markers and perinatal outcomes. Search terms included PAPP-A (pregnancy associated plasma protein A), AFP (alphafetoprotein), hCG (human chorionic gonadotropin), estriol, unconjugated estriol, inhibin, inhibin-A, maternal serum screen, triple marker screen, quadruple screen, integrated prenatal screen, first trimester screen, and combined prenatal screen. All study types were reviewed. Randomized controlled trials were considered evidence of the highest quality, followed by cohort studies. Key individual studies on which the recommendations are based are referenced. Supporting data for each recommendation are summarized with evaluative comments and references. The evidence was evaluated using the guidelines developed by the Canadian Task Force on Preventive Health Care.The evidence collected was reviewed by the Genetics Committee of the Society of Obstetricians and Gynaecologists of Canada.The benefit expected from this guideline is to facilitate early detection of potential adverse pregnancy outcomes when risks are identified at the time of a maternal serum screen. It will help further stratification of risk and provide options for pregnancy management to minimize the impact of pregnancy complications. The potential harms resulting from such practice are associated with the so called false positive (i.e., uncomplicated pregnancies labelled at increased risk for adverse perinatal outcomes), the potential stress associated with such a label, and the investigations performed for surveillance in this situation. No cost-benefit analysis is available to assess costs and savings associated with this guideline. SUMMARY STATEMENTS: 1. An unexplained level of a maternal serum marker analyte is defined as an abnormal level after confirmation of gestational age by ultrasound and exclusion of maternal, fetal, or placental causes for the abnormal level. (III) 2. Abnormally elevated levels of serum markers are associated with adverse pregnancy outcomes in twin pregnancies, after correction for the number of fetuses. Spontaneous or planned mutifetal reductions may result in abnormal elevations of serum markers. (II-2) RECOMMENDATIONS: 1. In the first trimester, an unexplained low PAPP-A (0.4 MoM) and/or a low hCG (0.5 MoM) are associated with an increased frequency of adverse obstetrical outcomes, and, at present, no specific protocol for treatment is available. (II-2A) In the second trimester, an unexplained elevation of maternal serum AFP (2.5 MoM), hCG (3.0 MoM), and/or inhibin-A (or =2.0 MoM) or a decreased level of maternal serum AFP (0.25 MoM) and/or unconjugated estriol (0.5 MoM) are associated with an increased frequency of adverse obstetrical outcomes, and, at present, no specific protocol for treatment is available. (II-2A) 2. Pregnant woman with an unexplained elevated PAPP-A or hCG in the first trimester and an unexplained low hCG or inhibin-A and an unexplained elevated unconjugated estriol in the second trimester should receive normal antenatal care, as this pattern of analytes is not associated with adverse perinatal outcomes. (II-2A) 3. The combination of second or third trimester placenta previa and an unexplained elevated maternal serum AFP should increase the index of suspicion for placenta accreta, increta, or percreta. (II-2B) An assessment (ultrasound, MRI) of the placental-uterine interface should be performed. Abnormal invasion should be strongly suspected, and the planning of delivery location and technique should be done accordingly. (III-C) 4. A prenatal consultation with the medical genetics department is recommended for low unconjugated estriol levels (0.3 MoM), as this analyte pattern can be associated with genetic conditions. (II-2B) 5. The clinical management protocol for identification of potential adverse obstetrical outcomes should be guided by one or more abnormal maternal serum marker analyte value rather than the false positive screening results for the trisomy 21 and/or the trisomy 18 screen. (II-2B) 6. Pregnant woman who are undergoing renal dialysis or who have had a renal transplant should be offered maternal serum screening, but interpretation of the result is difficult as the level of serum hCG is not reliable. (II-2A) 7. Abnormal maternal uterine artery Doppler in association with elevated maternal serum AFP, hCG, or inhibin-A or decreased PAPP-A identifies a group of women at greater risk of IUGR and gestational hypertension with proteinuria. Uterine artery Doppler measurements may be used in the evaluation of an unexplained abnormal level of either of these markers. (II-2B) 8. Further research is recommended to identify the best protocol for pregnancy management and surveillance in women identified at increased risk of adverse pregnancy outcomes based on an abnormality of a maternal serum screening analyte. (III-A) 9. In the absence of evidence supporting any specific surveillance protocol, an obstetrician should be consulted in order to establish a fetal surveillance plan specific to the increased obstetrical risks (maternal and fetal) identified. This plan may include enhanced patient education on signs and symptoms of the most common complications, increased frequency of antenatal visits, increased ultrasound (fetal growth, amniotic fluid levels), and fetal surveillance (biophysical profile, arterial and venous Doppler), and cervical length assessment. (III-A) 10. Limited information suggests that, in women with elevated hCG in the second trimester and/or abnormal uterine artery Doppler (at 22-24 weeks), low-dose aspirin (60-81 mg daily) is associated with higher birthweight and lower incidence of gestational hypertension with proteinuria. This therapy may be used in women who are at risk. (II-2B) 11. Further studies are recommended in order to assess the benefits of low-dose aspirin, low molecular weight heparin, or other therapeutic options in pregnancies determined to be at increased risk on the basis of an abnormal maternal serum screening analyte. (III-A) 12. Multiple maternal serum markers screening should not be used at present as a population-based screening method for adverse pregnancy outcomes (such as preeclampsia, placental abruption, and stillbirth) outside an established research protocol, as sensitivity is low, false positive rates are high, and no management protocol has been shown to clearly improve outcomes. (II-2D) When maternal serum screening is performed for the usual clinical indication (fetal aneuploidy and/or neural tube defect), abnormal analyte results can be utilized for the identification of pregnancies at risk and to direct their clinical management. (II-2B) Further studies are recommended to determine the optimal screening method for poor maternal and/or perinatal outcomes. (III-A).

Maternal age per se is not an indication for chorionic villous sampling or amniocentesis anymore. Although the risk for fetal Down syndrome increases with maternal age, it must be adjusted after other screening tests are performed. Current recommendations include ultrasound measurement of the fetal nuchal transluciency, and of maternal PAPP-A and free-b-hCG in addition to maternal age. This strategy has a sensitivity of 80 to 90%, with false positive rate of 5%. The thickness of fetal nuchal transluciency is strongly associated with the risk of Down syndrome, and measurement must be performed by an experie ced sonographer. For women with an intermediate risk (1/380 to 1/1000), a genetic son gram allows a reassessement of the risk and may help couples to make a decision of invasive sampling.

The analysis of longitudinal series of tumour markers during chemotherapy in patients with germ cells tumors is not easy. The purpose of this work is to present various characteristic profiles of the evolution of serum alpha-fetoprotein during chemotherapy and review the modalities of the dynamic interpretation of this marker.

The surveillance of cirrhotic patients for early detection of hepatocellular carcinoma is recommended but its efficacy is now discussed. The aim of our study was to present the results of a screening program in 110 patients.it is a retrospective study that included 110 patients with cirrhosis in a screening program of hepatocellular carcinoma, based on the realization of abdominal ultrasound exam and the determination of alpha-fetoprotein amount every 6 months in 95 patients and every 3 months in 15 patients.the mean duration of the surveillance was 36 months. A hepatocellular carcinoma was diagnosed in 13 patients. Curative treatment was done in only 3 cases and consisted in a hepatic resection in 1 patient and an alcoolisation in 2 cases. Ten patients had new treatment : for 8 patients the discussed was very agressive and 2 patients had a server hepotic failure. The cost of this study was 37.500 Tunisian dinars.systematic screening for hepatocellular carcinoma offer a limited cost effectiveness ratio.

The authors report a case of stage N3 pure testicular seminoma associated with paradoxical elevation of alphafoetoprotein (AFP). Despite the absence of histological arguments after review of the slides, this lesion was considered to be a stage pT1 N3 M0 S3 non seminomatous germ cell tumour with a poor prognosis. Simple surveillance was proposed following normalization of tumour markers, regression of retroperitoneal masses and negative PET scan. Laboratory and then clinical relapse at three months was treated by salvage chemotherapy followed by retroperitoneal lymph node dissection. The presence of embryonic carcinoma in one of the residual masses led to a revision of the initial histological diagnosis. The rare data of the literature indicate a variable approach according to alphafoetoprotein levels.

Immunohistochemistry (IHC) has become an indispensable tool in pathology. For proper interpretation, results must be read with knowledge of the diagnostic, clinical, and morphological circumstances. We detail here the contribution of IHC to the classification of lung cancer: small-cell lung cancer and other neuroendocrine tumors, basaloid carcinoma, large-cell carcinoma. Using IHC techniques, pathologists can now determine with certainty that an intrathoracic adenocarcinoma is primary or secondary. The distinction is less clear for large-cell carcinoma or squamous-cell carcinoma, or for tumors with a pleural or mediastinal presentation. IHC is also useful as a diagnostic aid for rare entities: carcinomas with an unusual morphology (alpha-fetoproetin secretors or beta-HCG secretors), melanomas, lymphomas, sarcomas. By demonstrating the presence of carcinomatous cells within the neighboring structures (pleura) or lymph nodes, IHC contributes to lung cancer staging, particularly when there are few of these elements morphologically difficult to distinguish. Finally, IHC contributes to prognosis (proliferation markers, differentiation markers) or prediction of therapeutic response (chemotherapy or targeted therapies). IHC studies may also be requested in a forensic setting, for example to demonstrate that the lung cancer observed in a patient exposed to asbestosis is primary. In light of these different situations, a wide panel of antibodies is required. Other morphological techniques such as hybridization in situ or molecular biology techniques will further complete the histological diagnosis in the future.

The aim of our study is to expose a practical screening and diagnosis strategy of HCC occuring in cirrhosis liver, which is nowadays a health public matter in Tunisia.HCC routine screening in cirrhosis liver has been conducted every 6 months using a combination of abdominal sonography and serum alpha-foetoprotein levels. PCUS, CT scan or MRI have been performed each time a focal liver lesion was discovered in US, or in case of elevated serum alpha-foetoprotein levels. 30 cases of HCC were identified.US has shown a unique nodule in 46% of the cases, multiple nodules in 50% of them. No lesion has been discovered in 4% of the cases. Nodule size ranged from 8 to 140 mm. Heterogenous hyperechoic pattern was the most frequent one (41%). Hypervascular doppler feature was authentified in 20% of the cases. Serum alpha-foetoprotein levels had respectively a sensitivity and a specificity of 14% and 44% in lesions inferior to 3 cm, 62% and 80% in lesions superior to 3 cm. Typical arterial enhancement of HCC was depicted in 1 case for PCUS, in 76% for CT scan and 60% for MRI.Combination of abdominal sonography and serum alpha-foetoprotein levels practiced every 6 months represent a good compromise between cost and effectiveness. Second intention imaging is based on CT scan in Tunisia because of its availability compared to MRI. PCUS is becoming in the near future the best modality for lesion caracterization thanks to its safety and relative inexpensiveness.

Amniotic fluid embolism is a rare, unpredictable and often lethal complication of pregnancy and childbirth. Because of its variable presentation, an early biologic test would help to establish the diagnosis. We investigated in maternal serum 4 components of amniotic fluid, i.e., alpha-fetoprotein (AFP), l'insuline like growth factor binding protein-1 (IGFBP-1), fetal fibronectin (fFN) and placental alpha1-microglobulin (PAMG-1). On the 6 cesareans controls involved, none of the makers increased after membranes section. PAMG-1 is unsuitable because its detection is always positive or doubtful even in the baseline. On the 7 cases suspected of amniotic fluid embolism, no detectable increase in any of those markers was noted in 3 cases, which is not in favour of this diagnosis. In the remaining cases, IGFBP-1 and fFN became detectable, confirming histological evidences of amniotic fluid embolism in 2 cases. The follow up of those markers in maternal blood confirmed the suspicion of amniotic fluid embolism at 21 wg in one case of ongoing pregnancy. These preliminary results point out the potential interest to assay maternal serum AFP, IGFBP-1 and fFN to confirm amniotic fluid embolism using rapid laboratory tests.

Juvenile granulosa cell tumors of the ovary (JGCTs) are a rare form of neoplasm that makes up less than 5% of ovarians tumors in childhood and adolescence. About 90% are diagnosed in stage I (FIGO) with a favorable prognosis, whereas those at higher stages have a less favorable outecome. The authors describe a juvenile granulosa cell tumor expressed by an early pseudopuberty occurring in a 6 year old child. Clinically, an endocrine syndrome was associated with a pelvic mass. Hyperoestrogenia and serum alphafoetoprotein level were biologically detected. Tumor was localized strictly to the ovary, so conservative surgery was applied and proved sufficient to remove all tumor tissue. Histological examination showed typical microscopic aspect of a juvenile granulosa cell tumor. The patient is well, 14 years after surgery with a normal growth and mental developpment.

Although chemoembolization is known to be an effective palliative treatment in hepatocellular carcinoma, it has a limited effect in large tumors. We report the case of a patient with a large hepatocellular carcinoma of the left liver who had a significant and sustained clinical response after six sessions of chemoembolization with a pirarubicin/amiodarone/lipiodol emulsion. Pirarubicin is an anthracycline which penetrates faster than doxorubicin into cancer cells. Amiodarone is a multidrug resistance inhibitor. Polysorbate 80, an excipient of injectable amiodarone stabilizes the anthracycline/lipiodol emulsion. The clinical efficacy of this new formulation could be evaluated in a phase II clinical trial.

Hepatocellular carcinoma is a rapidly fatal tumor. The only hope for a cure lies in early diagnosis. It follows that an effective screening strategy should be used in high-risk populations. Screening currently relies on tumor markers such as alfa fetoprotein (AFP) and imaging modalities. Because the AFP assay lacks sensitivity and specificity in patient with small tumors, other serum markers are being evaluated.

We report the case of a 42-Year-old man with a poorly differentiated gastric cancer revealed by a very high level of serum alpha-fetoprotein-protein, associated with liver metastasis, and treated by chemotherapy. We discuss the possible diagnosis of hepatoid carcinoma.

A COMPLEX SITUATION: Carcinomas of unknown primary site (CUP) represent at least 3% of cancers in adults. This group is heterogenic and difficult to treat, the central element of which is permanent discussions between the physician and the anatomopathologist. SPECIFIC CLINICAL FORMS: The physician must rapidly identify certain entities of good prognosis or particular treatment: cervical lymph node metastases, axillary adenopathies from occult breast cancer, primary papillary peritoneal carcinomatosis, and median line syndrome. OTHER THAN IN THESE SITUATIONS: Present recommended diagnosis strategy is limited and non-invasive (histological review with immunohistochemistry, measurement of alphaFP, beta-HCG, PSA, chest X ray, mammography, tomography of abdomen and pelvis). Positron emission scintigraphy would merit further evaluation.

Clear cell carcinoma (CCC) of the ovary is uncommon. In young patients, this tumor may simulate a yolk sac tumor. In this case, the morphologic distinction between these tumors is often difficult but the immunohistochemical staining for CA125 and alpha foeto protein (AFP) and the response to chemotherapy are particularly helpful to resolve this problem of differential diagnosis. We report a case of a 17 year old patient who was operated for a tumor of the right ovary. The diagnosis of a yolk sac tumor was first suggested. However, because of the non response to chemotherapy, a second laparotomy was performed; the definitive pathologic examination concluded to the diagnosis of a CCC of the ovary. The young age and the immunohistochemical staining for AFP are unusual and misleading features for a CCC. Our objective about this particular case is to discuss the anatomoclinical aspects of the CCC of the ovary and to prove the role of immunohistochemistry in the differential diagnosis.

To assess the feasibility and efficiency of the screening for hepatocarcinoma in a cohort of cirrhoseis mainly of alcoholic origin.293 patients with cirrhosis, among them 186 (63.5%) from alcoholic origin, were included in a surveillance programme for hepatocarcinoma by carrying out liver ultrasonography and alpha-foetoprotein dosage every 6 months. Results were analyzed with a mean follow-up of 60 months. Seventeen hepatocarcinoma discovered through the surveillance programme ("screened HCC") were compared with 40 hepatocarcinoma discovered outside the surveillance programme during the same period ("incidental HCC").The alcoholic origin of the cirrhosis was a predictive factor of poor compliance to the surveillance programme. Among the 186 patients with alcoholic cirrhosis, 129 (69%) were lost during the surveillance programme due to lack of compliance (97 cases) or death (32 cases). By comparison, among the 65 patients with hepatitis C-related cirrhosis, 18 were lost by lack of compliance (11 cases) or death (7 cases) (P0.001). Moreover, sustained or relapsing alcohol abuse after inclusion in the surveillance programme were also related to the quality of the compliance. Seventeen hepatocarcinoma were discovered through the surveillance giving an annual incidence of 2% for the emergence of hepatocarcinoma. The comparison between screened (n=17) and incidental (n=40) hepatocarcinoma showed that screened HCC were more often asymptomatic (P0.01), were more often a solitary nodule less than 5 cms (P0.001) and underwent more often curative treatment (P=0.02). However, the survival between screened and incidental hepatocarcinoma was not different.Screening for hepatocarcinoma in patients with alcoholic cirrhosis is a difficult task due to poor compliance and early death. According to our results, a surveillance every 6 months is sufficient to detect early lesions accessible to curative treatment by surgical resection or transcutaneous ablation.

The authors report the case of a one-year-old infant in whom routine examination by the paediatrician demonstrated left testicular swelling. Subsequent assessment (scrotal ultrasound and laboratory tests) revealed only a moderate increase of alpha-foetoprotein levels. Orchidectomy was performed due to a suspicion of vitelline tumour. Histological examination revealed splenogonadal fusion. In the light of this case, the authors discuss the modalities of early diagnosis of this embryonic anomaly for which orchidectomy appears to be excessive, even when the testicular parenchyma has an immature appearance.

Juvenile granulosa cell tumors of the ovary are a rare form of neoplasm that makes up less than 5% of ovarian tumors in childhood and adolescence. About 90% are diagnosed in stage I (FIGO) with a favorable prognosis, whereas those at higher stages have a less favorable outcome. The authors describe a juvenile granulosa cell tumor expressed by an early pseudopuberty occurring in a 6-year old child. Clinically, an endocrine syndrome was associated with a pelvic mass. Hyperoestrogenia and serum alphafoetoprotein level were biologically detected. Tumor was localized strictly to the ovary, so conservative surgery was applied and proved sufficient to remove all tumor tissue. Histological examination showed typical microscopic aspect of a juvenile granulosa cell tumor. The patient is well 14 years after surgery with normal growth and mental development.

Hepatocellular carcinoma is a malignant tumor with a poor prognosis, particularly when it is unresectable. The results of tamoxifen treatment in hepatocellular carcinoma have been disappointing and randomized studies have shown conflicting results. We report the case of a 72-yr-old-man with histologically confirmed hepatocellular carcinoma on a normal liver, that regressed with tamoxifen treatment. Regression was confirmed by normalization of serum alpha-fetoprotein levels and a significant decrease in radiological lesions by 66%. The patient was alive in excellent clinical condition more than 30 months after the initial diagnosis.

The aim of the study was to determine whether simple routine parameters evaluating the first session of transarterial chemoembolization (variation in alfa-fetoprotein concentration, tumor lipiodol uptake, and post-embolization syndrome) can predict survival of patients treated for hepatocellular carcinoma.Seventy-two patients treated with transarterial chemoembolization and evaluated one month after the first sessions with CT scan were included. Transarterial chemoembolization session included hepatic arteriography, lipiodol and doxorubicin (50 mg) emulsion injection, followed by gelatin sponge embolization. The following variables were studied in univariate and multivariate analysis: 6 recorded at the first session (age, cirrhosis etiology, Child-Pugh class, tumor number, largest lesion size, and alpha-fetoprotein concentration), and 5 recorded after the first session (variation in alfa-fetoprotein concentration, tumor lipiodol uptake, post-embolization syndrome, mean interval between each session, and associated treatment).Mean follow-up was 22.7 months (4-106). Mean survival was 30.4 months (95% CI: 23. 3-37.5). Actuarial survival at 1, 2, 3 and 5 years was respectively 65.5%, 44%, 29.5%, and 18%. The only independent prognostic factors in multivariate analysis were the Child Pugh class and the mean interval between sessions (P0.001 and0.01 respectively). None of our criteria evaluating the first TACE session significantly influenced survival.The 3 parameters (variation in alpha-fetoprotein concentration, tumor lipiodol uptake and post-embolization syndrome) after the first transarterial chemoembolization did not predict survival. They could not be used to determine which patient could benefit from repeated transarterial chemoembolization sessions.

Testicular cancer is the most common malignancy in young men and its incidence is increasing. The overall rate of cure can exceed 90% when management is optimal. Ultrasonography for diagnosis and thoraco-abdominal CT for staging are the optimal imaging modalities. In this paper we analyze technical parameters as well as findings and the strategy of examinations in testicular neoplasm.

To describe French practices for screening hepatocellular carcinoma.A standardized questionnaire was mailed to all French hospital hepato-gastroenterologists in June 1999.411 out of 623 practitioners responded (66%). 394 (96%) routinely screen hepatocellular carcinoma, mainly with ultrasound (98%) and mainly at 6-month intervals (77%). Screening was performed in cirrhosis (100%) or extensive fibrosis (54%), independent of the etiology (21%) or the Child-Pugh score of the chronic liver disease (41%), but based on age and treatment feasibility. If of a small hypoechogenic nodule was detected in a young patient with compensated HCV-cirrhosis, 59% of practitioners performed a histological examination. In case of non biopsy-proven hepatocellular carcinoma, a second biopsy (49%), treatment (either percutaneous alcohol injection, resection or transplantation) (24%) or an ultrasonographic follow-up (23%) was proposed. In case of biopsy-proven hepatocellular carcinoma, resection (49%), transplantation (30%) or percutaneous alcohol injection (16%) was proposed.Almost all French specialists routinely screen cirrhotic patients for hepatocellular carcinoma, but use somewhat different modalities. In case of small HCC without contraindications to curative treatment, surgical resection is performed in half the patients.

Screening of Down syndrome using maternal serum markers requires specific quality management. We report here a user's club experience (Down's Club Abbott) in surveying performances of AxSYM total beta hCG and AFP reagents combined to Maciel software. Regular analysis was carried out during three years (1998 to 2000). Values from five laboratories were collected to achieve calculation of medians for each parameter, multiple of the medians and initial positive rate (cut-off of 1/250 established in France). The large size of the observed population (3,1020 women during the studied period) increased the performances of statistical evaluations. In July 1999, five months after a change in reagents leading to new medians, these latter were recalculated in agreement with the manufacturer. Our experience exhibits that only the concomitant analysis of all parameters (medians, multiple of medians and initial positive rate) can allow the monitoring of a potential drift (bound or not to reagents). Moreover, such user's clubs enhance the individual quality management of the laboratories and allow a good follow-up of the performances of the testing in time.

A 45-year-old man presented with headaches and extraocular muscle palsy due to a sellar mass extending into the right cavernous sinus. Hormonal determinations revealed a gonadotrophic insufficiency. A transsphenoidal surgical removal revealed a lymphocytic hypophysitis with fibrosis and necrosis. Rapid growth of the pseudotumor was noted despite a high dose steroid therapy (1 mg/kg/d) for a month. Further biological and histopathological investigations were performed. They showed a high cerebrospinal fluid (CSF) B-human chorionic gonadotropin (ss-HCG) level of 12 UI/L (normal5 UI/L), normal plasma BHCG level, and undetectable CSF and plasma alpha-fetoprotein levels. The tumors cells showed a positive reactivity for placental alkaline phosphatase and for vimentin. These findings were consistent with an inflammatory lymphocytic process caused by an intrasellar germinoma. Chemotherapy was ill-tolerated and external radiotherapy was ineffective.

Hereditary persistence of alpha-fetoprotein is a rare disorder which exists with no simultaneous disease. The tenth case in the world (two brothers with seminoma and their father) is documented and a complete literature review was done.It is transmitted as an autosomal dominant; a disease point mutation has been identified. The failure to recognize hereditary persistence of alpha-fetoprotein sometimes involves unjustified treatments.The occurrence of this situation is probably underestimated and it could explain residual levels of alpha-fetoprotein.

Ensuring the quality is one of the first preoccupations of every biologist. However, measuring the circulating tumor markers could be more vital especially that the result is decisive for the diagnostic and the therapeutic attitude. In this study, we report methods used for an internal and externalor = quality controlor = program applied in the immunology laboratory of Sfax university hospital for the dosage of tumor markers AFP, CEA and CA15-3. The results obtained are analysed comparatively with those reported in literature. Factors causing discrepancies in the results of tumor marker measurements are recalled.

To compare alternative methods of antenatal screening for Down's syndrome (nuchal translucency measurement and second trimester maternal serum screening) in a low-risk population and to evaluate the consequences of a sequential estimation of risk.In a consecutive series of 4308 women aged less than 38 with a singleton pregnancy, we examined the detection rate of nuchal translucency (NT) measurement at 10-14 weeks and maternal serum screening (MSS) by human chorionic gonadotropin and alpha-feto-protein at 14-18 weeks. Women with a NT measurement = 3 mm and women with a MSS derived risk = 1/250 were recommended to have an amniocentesis. A second trimester detailed ultrasound scan was also performed in all women. The outcome of all pregnancies was entered in a computerized database and the detection rate and false-positive rate of different screening strategies were analysed.Of the 4308 pregnancies that were followed (mean maternal age 30.1 years), there were 12 cases of Down's syndrome (0.28%), all detected prenatally. Seven of twelve cases had a NT measurement above 3 mm (58%), and 6 out of ten cases with available MSS had a calculated risk = 1/250 (60%). Four of the five cases with NT measurement below 3 mm were detected by subsequent MSS. At a threshold giving 5% of positive tests, the sensitivity of NT screening and MSS were 75% and 60%, respectively.Sequential screening for Down's syndrome by nuchal translucency and second trimester biochemistry is effective and appears to increase the detection rate compared to the use of any single test. However, this strategy is likely to raise the false-positive rate and the interpretation of MSS derived risk should be combined to the first trimester NT measurement.

HIGH INCIDENCE: Because of the high incidence of hepatocellular carcinoma (HCC) in patients with cirrhosis (3 to 5% per year) and the fact that curative treatment is currently available only for small sized tumors careful screening is warranted in this high risk population. Earlier screening attempts produced disappointing results in terms of cure and survival, particularly in Europe. Progress in ultrasonography, a better understanding of the risk of developing HCC, and most importantly the advent of local percutaneous treatments have greatly affected the data which should be reexamined. SCREENING METHODS: Patients with cirrhosis, particularly alcoholic or viral cirrhosis, should undergo regular ultrasound examinations, every six months for most screening protocols although the best timing remains unknown. Assay of serum alpha-fetoprotein is of limited use due to its low sensitivity and specificity. Diagnosis of HCC is basically based on helicoidal computed tomography and/or magnetic resonance imaging findings, with or without pathological proof (ultrasound-guided biopsy) that may be difficult to obtain. A probabilistic diagnosis is therefore retained if necessary, based on the presence of risk factors and arterial hypervascularization of a liver nodule. EARLY TREATMENT: With ultrasound screening, the diagnosis of HCC can generally be established early, when curative transplantation, resection or local percutaneous destruction are still feasible. The percutaneous methods use chemical or physical agents to destroy the tumor. There are few contraindications so curative treatment can be proposed for large number of patients. Large-scale prospective studies will be completed in the upcoming years and are expected to provide evidence validating the principle of screening and early treatment.

Alphafetoprotein assay contributes considerably to the diagnosis of hepatocarcinoma in patients with hepatic cirrhosis. We report the case of a cirrhotic patient whose elevated alphafetoprotein level was not associated with liver disease.Alphafetoprotein level was followed in a 64-year-old man with hepatic cirrhosis. A rise from 415 to 7690 ng/ml between June and November 1997 led to the discovery of adenocarcinoma of the cardia with liver metastasis. This extrahepatic adenocarcinoma was probably the cause of inappropriate secretion of alphafetoprotein.Primary liver tumors are obviously not the only source of elevated alphafetoprotein levels. High levels can also be observed in certain, notably digestive tract and embryonary, cancers. Gastric hepatoid adenocarcinoma is a recently described histological entity first described in 1970. Typically, there is an inappropriate secretion of alphafetoprotein due to a secondary liver tumor.

We describe a juvenile granulosa cell tumor expressed by an early pseudopuberty occurring in a 3-year-old black child. Clinically, an endocrine syndrome was associated with a pelvic mass and ascites. Hyperoestrogenia and serum alphafetoprotein level were biologically detected. Histological examination showed typical microscopic features of a granulosa cell tumor. The patient is well four years after surgery. Growth and mental development are normal.

Pleuropulmonary metastasis occurs in 30 to 50% of all patients with cancer. Certain metastases occur specifically in females: breast and ovary cancer. There are six different clinical presentations of bronchopulmonary metastases: unique or multiple nodular images, mediastinal nodes, carcinomatous lymphangitis, bronchial metastasis, tumoral emboli, and metastatic bronchiolo-alveolar metastatic cancer. When the primary cancer is not known, a minimum number of investigations are needed: thyroid and pelvic ultrasound, mammography, colonscopy for certain cases, alfa-fetoprotein assay, neuron-specific enolase and beta HCG. Metastatic pleurisy accounts for 45% of all cases of pleurisy. In women, neoplastic pleural effusions result from breast cancer (37%), genitourinary tract cancer (20%), and lung cancer (15%).

To present epidemiologic profile of this supposedly rare condition, its diagnostic management and the results of surgical treatment in a population of the mediterranean border.Between 1987 and 1999, the records of patients operated on for hepatocellular carcinoma and in whom cirrhosis was not present on pathological examination of liver parenchyma were collected. Data were collected prospectively.Mean age was 57.6 years. There were 61 men (79%) and 16 women but sex ratio was 1/1 for patients under 50. Hepatitis B and/or C serology was positive in 22 patients (29%). At presentation, 56 patients (73%) suffered of symptoms and there was evidence of tumor at clinical examination in 30 (39%). Sensitivity of alpha-fetoprotein measurement was 55%, and 58% for preoperative percutaneous biopsy. Twelve had exploratory laparotomy only, 61 underwent partial liver resection and 4 were transplanted. Resection was presumed curative in 56 cases (73%). There was one single tumor mass in 41 cases (53%) and its mean size was 10.3 cm. Liver fibrosis was present in 24 cases (29%). After curative resection, 5-year survival rate was 46%, and favorable prognostic factors were: alpha-fetoprotein within normal range, single tumor mass or tumor size less than 10 cm.Hepatocellular carcinoma arising on noncirrhotic liver is not a rare occurrence. It is usually encountered at an advanced stage of the disease. Nonetheless, it remains extirpable in many cases with low postoperative mortality and long term results are better than those of resections for hepatocellular carcinoma on cirrhotic liver.