Malignant cutaneous angio-endothelioma are tumors which develop from vascular endothelium. This is a study of one case along with the data compiled from 118 previously published cases. Clinically, this rare tumor is chiefly seen before the age of twenty of after sixty. Its appearance is polymorphic and often misleading. The clinical course of this disease in adults is always fatal, however, in children 20 p. 100 of the cases are reported curable. The histological picture shows a polymorphic pattern associated with vascular channels anastomosing in a plexiform network with large lacunae surrounded by papillary vegetations and plaques of fusiform, sarcomatoid cells as well as areas of angioblastic secretory cells. The ultrastructural study confirms that the tumor originates from endothelial cells which have lost their capacity to cover the capillary walls.

Aged↗

PubMedDate not supplied

[Trisomy 13 and lesions of the great vessels of the base of the heart].

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Abnormalities, Multiple↗

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[Unusual cause of intestinal invagination: congenital polypoid-form gastric heterotopia. Study of a case and review of the literature].

The authors report a case of polypoid heterotopic gastric mucosa in the ileum presenting as intussusception with hypochromic anemia in a 6 year-old boy. Tumorous heterotopic gastric mucosa in the small bowel is very uncommon. So far 22 similar cases have been published in the literature. This congenital intestinal anomaly is discovered in one third of the cases during the first decade. In 9 of 23 cases gastric heterotopia was diagnosed after an ileal resection for intussusception induced by the heterotopia presenting as a pedunculated or sessile polyp. In our case the great number of the polypoid formations is exceptional and has not been previously described.

Child, Preschool↗

PubMedDate not supplied

[Treatment and prognosis of gastric carcinoma. Study of the population in the Côte d'Or Department].

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Adenocarcinoma↗

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[Dysembryoplastic neuroepithelial tumors. A benign tumor cause of partial epilepsy in young adults].

Dysembryoplastic neuroepithelial tumor (DNT) is a newly recognized brain lesion first reported in 1988 by Daumas-Duport et al. The authors described five cases of DNT, that occurred in young people and were characterized by partial seizures. Seizures could become intractable and secondary generalised. Usually, the interictal neurological examination was normal. In most cases, computed tomography showed a supratentorial, "pseudocystic" low density appearance associated in some cases with calcific hyperdensity or focal contrast enhancement. Magnetic resonance imaging demonstrated a predominantly intracortical lesion. Common features included low signal intensity on T1-weighted images and high signal on T2-weighted images. Temporal and frontal lobes were mainly involved. Dysembryoplastic origin explained the clinical and radiological stability and the benign evolution of this tumor. Tumor resection was required only when epilepsy was intractable or when there was intracranial hypertension. Earlier intervention can prevent the physical and psychosocial damage resulting from chronic seizures and can improve the prognosis for these young patient. Histologically, DNT have been incorporated amond the category of neuronal and mixed neuronoglial tumors. Three patterns are described: a simple form with a unique glioneuronal element, a complex form with specific glioneuronal element, nodules being made of multiple variants looking like astrocytomas, oligodendrogliomas or oligo-astrocytomas, foci of dysplastic cortical disorganisation, and a non specific form. When specific glioneuronal composant is absent (50% of cases), the identification of DNT has therapeutic and prognostic implications because aggressive therapy may be avoided, sparing these young patients the long term effects of radio-or chemotherapy.

Adult↗

PubMedDate not supplied

Prevalence of ANCA in a hospitalized elderly French population.

OBJECTIVE: The prevalence of some autoantibodies in the elderly population has been reported to be greater than in younger controls. The prevalence of ANCA has been shown to be low in a generally healthy population, but has not been established in the elderly. Thus, the presence of ANCA in elderly patients might not have the same clinical significance as in younger people. The aim of this study was to evaluate the prevalence of ANCA in elderly subjects. PATIENTS AND METHODS: Serum samples from 137 subjects (96 females, 41 males; mean age = 82.2 years +/- 6.97 SD) were evaluated. Criteria for exclusion included suspected or established systemic vasculitis, connective tissue or neoplastic diseases, acute infection, HIV infection, current therapy with corticosteroids or cytotoxic drugs, and recent blood transfusion. ANCA were detected by indirect immunofluorescence on ethanol-fixed normal human neutrophils. Fluorescence patterns were classified as c-ANCA, p-ANCA or nuclear. Sera exhibiting p-ANCA or nuclear fluorescence were further tested by IIF on formalin-acetone fixed normal human neutrophils. Sera whose reaction pattern was cytoplasmic were considered as positive for "true" pANCA. Additionally, sera were tested for the presence of antinuclear antibodies (IIF), anti-double-stranded DNA (enzyme immunoassay) and IgM rheumatoid factors (enzyme immunoassay). RESULTS: The prevalence of c-ANCA was 0% (95% CI = 0-2.66), the prevalence of p-ANCA was 2.2% (95% CI = 0.45-6.3), and the prevalence of "true" p-ANCA was 0.73% (95% CI = 0.02-4). The prevalence of ANA, anti ds-DNA and RF were respectively 38%, 3.6%, and 11.7%. CONCLUSION: The prevalence of ANCA is not increased in elderly people. Thus, the presence of ANCA in elderly subjects may have the same clinical significance as in younger people.

Aged↗