[Clear cell dermatofibroma: a case report with cytogenetic study]. / Le dermatofibrome à cellules claires: à propos d'un cas avec étude cytogénétique.

We report an observation concerning a 48-year-old male who presented with a 5 cm tumour on his left leg, first noticed 18 months ago. The remainder of clinical examination was normal. Histological assessment revealed a tumoral infiltration of entire dermis and superficial hypodermis. This tumour consisted of monomorphous, ovoid or spindle cells, with clear cytoplasm and PAS+ granulations. There was strong immunoreactivity by tumoral cells only for vimentin. Ultrastructural studies revealed fibrohistiocytic-like tumoral cells, without epithelial, muscular, vascular or melanocytic differentiation. These results were consistent with the diagnosis of clear cell dermatofibroma. Cytogenetic evaluation and FISH analysis showed a deletion of p12. Clear cell dermatofibroma is a rare and recent variant of dermatofibroma, with a difficult histological evaluation and which must be differentiated from clear-cell sarcoma. This observation is the first case-report of this entity, to our knowledge, showing a cytogenetic abnormality.

Prognostic value of TP53 transcriptional activity on p21 and bax in patients with esophageal squamous cell carcinomas treated by definitive chemoradiotherapy.

PURPOSE: The aim of this study was to evaluate biologic factors on survival and clinical response after definitive concomitant chemoradiotherapy (CRT) in patients with esophageal squamous cell carcinoma (ESCC). METHODS AND MATERIALS: TP53 protein hyperexpression (immunochemistry [IHC]) and functional assay (FA) of TP53, measuring the ability of TP53 to transactivate p21 and bax reporter systems, were performed in patients with ESCC treated by CRT. The impact of parameters studied on survival and clinical response to CRT was assessed. RESULTS: Thirty-eight patients with ESCC were included. TP53 alterations were detected in 84.2% of cases with FA. All TP53 mutations abolished the transactivation of p21 and bax reporter systems. After CRT, complete response rate was 55.3%. The median survival of the population was 17.5 months. Serum albumin (p = 0.002), weight loss <10% (p = 0.005), and response to treatment (p < 0.001) were significantly linked with survival. TP53 alteration in FA was not significantly predictive of response to CRT (p = 0.132) nor survival (p = 0.154). CONCLUSIONS: Our results suggest that wild-type TP53 in ESCC could be associated with good response to definitive CRT. However, the small rate of ESCC with wild-type TP53 suggests that systematic determination of TP53 status is not appropriate for the management of the ESCC population.

Glucocorticoid treatment in an ischaemic-like excitotoxic model of periventricular leucomalacia in mice.

OBJECTIVE: To determine the effects of fluorinated glucocorticoids on the occurrence and extent of ischaemic-like excitotoxic grey and white matter cerebral injuries in an animal model. DESIGN: A study of the influence of single or repeated doses of glucocorticoids when creating excitotoxic lesions in mice pups, mimicking some aspects of periventricular leucomalacia and cortical-subcortical stroke as observed in human neonates. SAMPLE: Four hundred and sixty-seven mouse pups out of more than 30 litters. METHODS: An excitotoxic lesion was created by intracerebral injection of ibotenate, a glutamatergic agonist, in day five postnatal mice pups. A single dose of betamethasone or dexamethasone was administered, in a dose of 0.006-6 and 0.001-1 mg/kg, respectively, 24 hours before or 15 minutes after each ibotenate injection. Repeated doses of dexamethasone (0.01 mg/kg per day) or betamethasone (0.006 mg/kg) were given for five days before or after ibotenate injections. The measurement of white matter and grey matter lesions and the occurrence of cysts were assessed under light microscope on cresyl violet-stained brain sections. MAIN OUTCOME MEASURES: Size of white matter cystic lesions. RESULTS: A single injection of betamethasone or dexamethasone had a significant neuroprotective effect when administered after the excitotoxin. Betamethasone injected once prior to ibotenate also had a protective effect. Repeated administration of each steroid before or after excitotoxin injection provided more protection than a single injection. CONCLUSION: Fluorinated glucocorticoids reduced neonatal brain lesions observed in a mouse model treated by excitotoxin injection.