[Mohs Micrographic Surgery for the management of basal cell carcinoma: experience at the Nîmes Univeristy Hospital]. / La chirurgie micrographique de Mohs dans la prise en charge des carcinomes basocellulaires. Application au CHU de Nîmes.

AIMS: The goal of this work was to assess the validity of Mohs Micrographic Surgery (MMS) for basal cell carcinoma (BCC) in a routine clinical setting. MATERIAL AND METHODS: Our adaptation of the technique described by Mohs and coll allows intraoperative histological examination of all surgical edges of the resection. Sixteen men and 4 women were selected. RESULTS: Average operative time was 2 hours 30 minutes. No false results were noted. The cosmetetic and functional outcomes were good. CONCLUSION: MMS is a safe and reproducible surgical technique made possible by solid team work. It is adapted for the treatment of BCC with a high risk of recurrence. The cosmetetic and functional results are quite satisfactory. The recurrence rate at 5 years is 10 times less than with other methods of treatment. The additional time required for this surgery be put in balance with the number of tumors for which a second intervention would have been necessary if conventional surgery had been used. All procedures were performed under local anesthesia, and none or the patients required a second intervention.

Sox9 regulates cell proliferation and is required for Paneth cell differentiation in the intestinal epithelium.

The HMG-box transcription factor Sox9 is expressed in the intestinal epithelium, specifically, in stem/progenitor cells and in Paneth cells. Sox9 expression requires an active beta-catenin-Tcf complex, the transcriptional effector of the Wnt pathway. This pathway is critical for numerous aspects of the intestinal epithelium physiopathology, but processes that specify the cell response to such multipotential signals still remain to be identified. We inactivated the Sox9 gene in the intestinal epithelium to analyze its physiological function. Sox9 inactivation affected differentiation throughout the intestinal epithelium, with a disappearance of Paneth cells and a decrease of the goblet cell lineage. Additionally, the morphology of the colon epithelium was severely altered. We detected general hyperplasia and local crypt dysplasia in the intestine, and Wnt pathway target genes were up-regulated. These results highlight the central position of Sox9 as both a transcriptional target and a regulator of the Wnt pathway in the regulation of intestinal epithelium homeostasis.

[Gastrointestinal adenocarcinomas with a choriocarcinomatous component: 2 cases and a review of 120 cases in the literature]. / Adénocarcinome gastro-intestinal à composante choriocarcinomateuse: à propos de 2 cas et revue des 120 cas de la littérature.

Gastrointestinal adenocarcinoma with a choriocarcinomatous component (GACC) is an extremely rare and highly malignant human chorionic gonadotrophin-producing neoplasm. The development of this tumour from dedifferentiation or trans-differentiation of adenocarcinomatous cells is the most likely mechanism. 102 of the 120 cases reviewed in the literature presented enough clinicopathological information to be analysed. This tumour can be purely choriocarcinomatous or associated with an adenocarcinoma as in half of the observations. It is usually found in the stomach although it may be found throughout the digestive tract. The diagnosis of GACC should only be retained after having clearly eliminated a metastasis from a choriocarcinoma in particular in the gonads. The prognosis is very poor in particular in the gastric forms of this disease, with death observed within 12 months following diagnosis in 2/3 of the patients. There is no treatment consensus, and it is decided on a case-by-case basis.

[Primary angiosarcoma of the male breast]. / Angiosarcome primitif du sein chez l'homme.

We report an exceptional case of primary breast angiosarcoma in a 58-year-old man. This is a very rare breast tumor (0.04% of breast tumors) which may be difficult to diagnose. Treatment is now standardized: radical mastectomy associated with adjuvant chemotherapy for grade III or poorly differentiated tumors. Prognosis is variable, depending on tumor size and histological grade. Diagnosis should be established as early as possible because the 10-year overall survival rate is 80% for low grade tumors and only 20% for high grade tumors.

Dominant form of vanishing white matter-like leukoencephalopathy.

Leukoencephalopathy with vanishing white matter syndrome (childhood ataxia with central nervous system hypomyelination/vanishing white matter disease) is an autosomal recessive disorder characterized by the occurrence of acute episodes of deterioration after minor head trauma or infection, and symmetrical demyelination on magnetic resonance with cavitation aspects. Mutations in each of the five subunits of eIF2B have been identified. We report in an affected man and his mother an adult-onset form of childhood ataxia with central nervous system hypomyelination/vanishing white matter disease-like disorder with no mutations in the EIF2B genes and normal guanine nucleotide exchange factor eIF2B activity, suggesting a new dominant inheritance of this syndrome that may involve other genes.

Wall mechanics of the stented rabbit aorta: long-term study and correlation with histological findings.

PURPOSE: To evaluate long-term changes in arterial wall mechanics induced by stenting of the rabbit aorta. METHODS: Eighteen New Zealand white rabbits had initial stent deployment (3x8 mm Multilink) at 110% of the pre-stenting abdominal aortic diameter. Group A (n=10) had no postdeployment stent expansion and group B (n=8) had 30% overdilation of the stent. A noninvasive B-mode ultrasound examination coupled with image processing allowed measurement of diameters at systole and diastole and the calculation of diameter compliance. Measurements were performed before stenting and compared to those recorded immediately after stenting and at 3 months at 3 locations: upstream from the stent, at the stent level, and downstream from the stent. Measurements were also compared among measurement sites. The pathological study included measurement of intimal thickening and calculation of an injury score. RESULTS: At the stent level, diameter compliance was significantly lower after initial stenting and at 3 months than before stenting (group A: p<0.005; group B: p<0.001) and than downstream or upstream from the stent (group A: p<0.0001, group B: p<0.005). No significant difference in diameter compliance was found between groups A and B. In group B, intimal thickening and the injury score were greater than in group A (p<0.05 and p<0.0001, respectively). CONCLUSIONS: Endovascular stenting of the rabbit aorta impairs wall mechanics. Performing 30% overdilation of the stent does not worsen this impairment but induces greater in-stent intimal hyperplasia.