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Biomedical subjects

D Satgé

Publications and source records attributed to D Satgé.

At least 19 recordsLinked to original sources
PubMed2003

The epidemiology of neonatal tumours. Report of an international working group.

Neonatal tumours occur every 12,500-27,500 live births and comprise 2% of childhood malignancies, but there is little clarity as to their real prevalence, sites of origin and pathological nature as reported series vary. As an entity, neonatal tumours provide a unique window of opportunity to study tumours in which minimal environmental interference has occurred. The majority of tumours present with a mass at birth (e.g., teratomas, neuroblastomas, mesoblastic nephroma, fibromatosis), which are not infrequently identified on antenatal ultrasound. Histologically, teratoma and neuroblastoma remain the two main tumour types encountered with soft tissue sarcoma, renal tumours, CNS tumours and leukaemia being the next most common tumour types identified. Malignant tumours are uncommon in the neonatal period per se and benign tumours may have malignant potential. A particular problem exists in clinical classification, as histological features of malignancy do not always correlate with clinical behaviour. Benign tumours may also be life threatening because of their size and location. Other tumours may demonstrate local invasiveness, but no metastatic potential, and tumours that are clearly malignant may demonstrate unpredictable or uncertain behaviour. Screening programmes have brought more tumours to light, but do not appear to affect the overall prognosis. They may provide clues to the stage at which tumours develop in foetu. The aetiology of cancer in children is multifactorial and includes both genetic and environmental factors. The association between congenital abnormalities and tumours is well established (15% of neonatal tumours). Genetic defects are highly likely in neonatal tumours and include those with a high risk of malignancy (e.g., retinoblastoma), but also genetically determined syndromes with an increased risk of malignancy and complex genetic rearrangements. Tumours are mostly genetically related at a cellular level and factors influencing cellular maturation or apoptosis within the developing foetus may continue to operate in the neonatal period. Cytogenetics of neonatal neoplasms appear to differ from neoplasms in older children, thus possibly explaining some of the observed differences in clinical behaviour. Certain constitutional chromosome anomalies, however, specifically favour tumours occurring in the foetal and neonatal period. In support of this hypothesis, certain cytogenetic anomalies appear to be specific to neonates, and a number of examples are explored. Other environmental associations include ionizing radiation, drugs taken during pregnancy, infections, tumours in the mother and environmental exposure.

Female↗
PubMed2002

Ulcerative terminal ileitis after BCG therapy for bladder carcinoma.

AIM: We evaluated the aetiology of ulcerative ileitis in a 77-year-old patient receiving BCG therapy for recurring bladder carcinoma. METHODS AND RESULTS: Histological examination, laboratory data and the clinical history allowed us to exclude a classical cause such as Crohn's disease, tuberculosis or ischaemia. The important locoregional reaction suggests a BCG ileitis of the terminal ileum. Intestinal BCG ileitis has been exceptionally described after BCG vaccination. CONCLUSION: Our observation of ulcerative ileitis indicates that a digestive tract location is possible as a reaction to BCG therapy.

Administration, Intravesical↗
PubMed2001

Aspects of intracranial and spinal tumors in patients with Down syndrome and report of a rapidly progressing Grade 2 astrocytoma.

BACKGROUND: Brain tumors in patients with Down syndrome (DS) rarely are reported, and their behavior is not well known. METHODS: The authors report on a male patient age 19 years who had DS with diffuse astrocytoma (World Health Organization Grade 2) that recurred twice despite treatment, leading to a glioblastoma and, finally, to death in just over 2 years. The literature on brain tumors in patients with DS is reviewed. RESULTS: Although brain neoplasms were suspected to be in excess in patients with DS, the authors found only 36 patients with brain neoplasms and 2 spinal tumors. An unusual distribution of histologic tumor types, with an over-representation of germ cell and mesenchymal tumors and a lack of embryonal tumors, was observed, in agreement with what is known currently about the tumor profile of patients with DS. CONCLUSIONS: Cerebral tumors in patients with DS have a specific distribution and may behave differently compared with the general population. These features may be related to the gene dosage effect of oncogenes, antioncogenes, and genes involved in cerebral development due to the supernumerary chromosome 21.

Adult↗
PubMed2001

An infant with Down syndrome and retinoblastoma. A possible non-fortuitous association.

AIM: To evaluate the association between Down syndrome and retinoblastoma. METHOD: Presentation of a case report and review of the literature. RESULTS: A retinoblastoma was observed in a 10-month-old boy with Down syndrome. A review of the literature yielded 14 other cases, suggesting a possible excess of retinoblastoma in Down syndrome, as previously proposed by two epidemiological studies. The possible roles of external physical agents and hyperplastic and dysplastic lesions of the retina in subjects with Down syndrome is discussed. CONCLUSION: A positive association between Down syndrome and retinoblastoma is possible. An epidemiological study on this subject is needed to better ascertain this potential link.

Child↗
PubMed2001

[Breast cancer in women with trisomy 21].

The population with Down's syndrome has a different cancer profile compared to the general population, even after taking into account issues of survival and ageing. Several solid tumours are unusually rare, whereas in contrast leukaemias are increased. In addition, few studies are available on this topic. We therefore decided to conduct a mortality study based on the INSERM national mortality statistics in France comparing over a 24 year period deaths from female breast cancer in the general French population with the cancer deaths in women with Down's syndrome. Only 5 deaths with Down's syndrome could be found compared to 68.98 expected based on national statistics. This clear reduction in risk agrees with other studies available in Down's syndrome patients. This observation could be partly explained by over expression of genes linked to gene dosage effects on chromosome 21, playing a role in cell growth, differentiation, survival and death. An additional protective effect could come from the marked and continued decreased exposure to oestrogens, starting in utero for women with trisomy 21 and lasting all over life.

Adolescent↗
PubMed1999

[Fatal mesenteric ischemic accident caused by mesenteric inflammatory veno-occlusive disease].

A 72-year-old woman in good general health presented a massive intestinal and colonic ischemia with rapid post-operative death, due to mesenteric inflammatory veno-occlusive disease. Mesenteric inflammatory veno-occlusive disease, first described in 1994, is a vasculitis of unknown etiology limited to the mesenteric area, affecting exclusively veins and venules and sparing arteries. Histologically, there are acute lesions of lymphocytic and/or necrotizing, sometimes granulomatous phlebitis and chronic lesions of myointimal hyperplasia reducing the lumen of the veins. In this case, polymorphonuclear eosinophils were particularly numerous.

Aged↗
PubMed1998

A tumor profile in Down syndrome.

We conducted a review of cancers in Down syndrome (DS), because solid tumors are poorly understood in DS. Cancers are in excess in this condition because of the 20-fold excess of leukemias, whereas malignant solid tumors seem to be globally underrepresented as compared with those in the general population. However, among these tumors, some tumors are in excess: lymphomas, gonadal and extragonadal germ cell tumors, and possibly retinoblastomas and pancreatic and bone tumors. Neoplasms in excess are seen earlier, sometimes in fetal life (leukemias and testicular germ cell tumors) or neonatally (leukemias and lymphoma) and affect mainly male subjects. There seems to exist an excess of rare karyotypes. Other tumors are underrepresented, particularly neuroblastomas and nephroblastomas, in young children, and perhaps common epithelial tumors in adults. These observations suggest that DS has a particular tumor profile, with some tissues more affected by malignant diseases (hematopoietic tissue and germ cells) and others that seem to be protected (central and peripheral nervous system, renal tissue, and epithelial tissues). The mechanism is mainly genetic, but differences in exposure to exogenous agents compared with the general population must be kept in mind. These findings are of interest for the management of these patients and early detection of cancers. Better knowledge of this tumor profile could help us to understand the mechanisms of carcinogenesis and should be compared to the current knowledge of genes on chromosome 21.

Adult↗
PubMed1998

A lack of neuroblastoma in Down syndrome: a study from 11 European countries.

An epidemiological investigation in 11 European countries comprising a total childhood population of 54.1 million children and using 8 separate data sources was conducted to evaluate the occurrence of neuroblastoma in Down syndrome (DS). No cases of DS were detected among 6724 infants and children with neuroblastoma, although more than five were expected. This highly significant result (P = 0.0045 according to the Poisson test) is consistent with data in the literature, which contains only two poorly detailed cases in epidemiological studies and one ganglioneuroma in a DS mosaic patient. Like other tumors, such as leukemias, testicular germ cell tumors and lymphomas are in excess in DS patients; the lack of neuroblastomas does not reflect a general decreased incidence of cancer but rather a specific underrepresentation of this precise tumor. S-100 b protein, the gene for which maps to the long arm of chromosome 21, (a) is overproduced in DS patients, (b) produces growth inhibition and differentiation of neural cells in vitro, (c) is abundant in good-prognosis neuroblastomas, and (d) has been shown to induce growth inhibition and differentiation and cell death in several human and murine neuroblastoma cell lines and could be responsible for this variation. Additional epidemiological and experimental studies are warranted to confirm our interpretation of these data.

Adolescent↗
PubMed1998

Antenatal therapeutic drug exposure and fetal/neonatal tumours: review of 89 cases.

Although case reports on the association between neonatal tumours and maternal therapeutic drug use during pregnancy are cited regularly, no systematic review has been published. In a systematic search of the literature on fetal and neonatal tumours, we identified 36 malignant and 54 benign tumours in 89 fetuses and neonates prenatally exposed to various drugs. Six associations with in utero exposure are noteworthy: (1) phenytoin and neuroblastoma, as already suspected; (2) antibiotics and leukaemias; (3) hormonal treatment and vascular tumours; (4) acetaminophen (paracetamol) and fusocellular sarcoma; (5) acetaminophen in combination with petroleum products and hepatoblastoma; and (6) dicyclomine hydrochloride and teratomas. In view of the high level of consumption of drugs by pregnant women and the rarity of neonatal tumours, a possible carcinogenic effect is difficult to establish. However, some associations already observed in older infants, children and adults after antenatal and postnatal exposure to the same drugs are of note. We suggest that these drugs may act as carcinogens or as co-carcinogens in association with other physical and chemical agents and/or a particular genetic background. We therefore feel it is important to encourage the systematic collection, registration and publication of case reports on this subject. The hypotheses generated thereby could then be tested by formal epidemiological studies.

Carcinogens↗
PubMed1997

An excess of testicular germ cell tumors in Down's syndrome: three case reports and a review of the literature.

BACKGROUND: The incidence of specific solid tumors in Down's syndrome (DS) is not well established. Testicular germ cell tumors (TGCT) might be increased in this population. METHODS: The presence of TGCT among male subjects from the French department of Corrèze was recorded and literature on the subject reviewed. RESULTS: A total of 120 living children and adults with DS and 17 pregnancies (12 births and 5 therapeutic abortions) were examined over an 8-year period (1987-1994). Three TGCT were diagnosed. A seminoma and an embryonal carcinoma were observed in two young adults and an intratubular germ cell neoplasm in a 22-week-old fetus. Because testicular tumors occur at an incidence rate of 4 cases per 100,000 person-years in the general population, these observations suggest a clearly increased risk of developing TGCT in the DS population. In addition, a review of the literature also shows an excess of TGCT in this population. Cryptorchidism alone, which is prevalent in individuals with DS, cannot explain this significantly increased incidence of TCGT. The authors hypothesize that an excess of luteinizing hormone and follicle-stimulating hormone gonadotropins and overexpression of the Ets-2 gene through gene dosage effect could predispose patients with DS to the development of TGCT. CONCLUSIONS: Surveillance of the gonads of male patients with DS is recommended. A better understanding of the factors involved could also help to identify risk factors in the general population.

Adult↗
PubMed1995

Endometrial müllerian carcinosarcoma after cessation of tamoxifen therapy for breast cancer.

The aim of this report is to draw the attention of clinicians to the possible occurrence of endometrial cancers of rare histological type among women currently undergoing or having in the past undergone tamoxifen therapy, in particular for breast cancer. We report a case of heterologous mixed malignant müller tumor occurring in an 80-year-old woman. At 69, she had been diagnosed with breast cancer and received tamoxifen for a total of 55 months over a 6-year period. In the 5th year after cessation of tamoxifen therapy, an endometrial carcinosarcoma was diagnosed. Although the association between tamoxifen use and endometrial cancer is recognized, only a few reports of occurrence long after cessation of therapy exist. We believe ours is the second for this particular histological type. Tamoxifen may have played a role in the occurrence of this tumor although it is also known that this type of tumor may arise de novo in elderly women. The etiologic hypothesis obtained from this case description will now be tested in a formal epidemiological investigation which hopefully will provide more definitive evidence.

Adenocarcinoma↗
PubMed1993

[Retroperitoneal teratomas in the perinatal period. Review of the literature concerning a neonatal, immature, aggressive teratoma].

A neonate with an immature, poorly demarcated retroperitoneal teratoma invading the aorta-vena cava space died immediately after surgery. Among 34 cases of retroperitoneal teratoma discovered during the first postnatal month, including one renal case and ours, eight can be considered malignant on the basis of histology in two cases and clinical course in six. Five of these tumors exhibited a significant immature component. In neonates, the proportion of malignant teratomas is greater for retroperitoneal tumors than for other sites: sacrum and coccyx, heart, neck, mediastinum, and abdomen. Evaluation of chemotherapy in teratomas with significant immature components is difficult because few of the patients of this small sample received such treatment. As in other sites, regardless of the degree of maturity, exeresis must be as complete as possible.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1988

[Histologic study of 100 pregnant fallopian tubes].

The 100 fallopian tubes had been obtained from women whose mean age was 30 and whose mean period of amenorrhea was 38 days. Each fallopian tube gave rise to at least sixteen samples and was sectioned if necessary several times. There had been tubal rupture in 25 cases, and in at least 7 cases the rupture involved the ovum and its implantation site. The location of the implantation site could be determined in only 30 cases: 10 in the mesosalpin; x 10 in the antimesosalpinx; 8 lateral and 2 circumferential. The anatomic site of the implantation was isthmic in 7 cases; ampullary in 85 cases, of which 6 were near to the isthmus; infundibular in 7 cases and fimbrial in 1 case. Tubal lesions were found in 22% of the cases, but associated were also found frequently: tubal adenomyosis (17 cases); adherent tubal fimbriae (14 cases); peritoneal adhesions (11 cases) and decidual changes in 12 cases. The 74 remaining fallopian tubes were considered to have been previously normal and to have been affected only by changes linked to the pregnant state (edema, congestion, lymphocytic infiltration) or to complications or this state (a smoothing out of the tubal mucosa; a peritoneal granuloma surrounding blood products; a localized infarct of the fimbriae or wall; and haemorrhage involved in thromboses, ovum detachment or necrosis). The ovum and implantation appeared to be normal in 26 cases and to have had development prematurely stopped because of extensive haemorrhage in 41 cases. In 22 cases there was hypoplasia, and difficulty in determining the cause in 11 cases. From a morphological point of view preserving at least one third of gravid fallopian tubes is justifiable, because the chronic lesions are often discrete and the changes of pregnancy generally appear to be reversible. The persisting pathogenic factors are not necessarily tubal, as can be seen by the role of delays in transport of the ova.

Adolescent↗