Dermatofibrosarcoma protuberans: a population-based cancer registry descriptive study of 66 consecutive cases diagnosed between 1982 and 2002

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare malignant tumour of the skin, with an estimated incidence of 0.8 to five cases per 1 million people per year. OBJECTIVE: To study epidemiological, immunohistochemical and clinical features, delay in diagnosis, type of treatment and outcome of DFSP from 1982 to 2002. METHODS: Using data from the population-based cancer registry, 66 patients with pathologically proved DFSP were included (fibrosarcomatous DFSP were excluded). Each patient lived in one of the four departments of Franche-Comté (overall population of 1 million people) at the time of diagnosis. The main data sources came from public and private pathology laboratories and medical records. The rules of the International Agency for Research on Cancer were applied. RESULTS: The estimated incidence of DFSP in Franche-Comté was about three new cases per 1 million people per year. Male patients were affected 1.2 times as often as female patients were. The trunk (45%) followed by the proximal extremities (38%) were the most frequent locations. DFSP occurred mainly in young adults between 20 and 39 years of age. Mean age at diagnosis was 43 years, and the mean delay in diagnosis was 10.08 years. Our 66 patients initially underwent a radical local excision. Among them, 27% experienced one or more local recurrences during 9.6 years of follow-up. There was one regional lymph node recurrence without visceral metastases. These recurrences were significantly related to the initial peripheral resection margins. We observed a local recurrence rate of 47% for margins less than 3 cm, vs. only 7% for margins ranging from 3 to 5 cm [P=0.004; OR=0.229 (95%, CI=0.103-0.510)]. The mean time to a first local recurrence was 2.65 years. Nevertheless, there was no death due to the DFSP course at the end of the follow-up, and the final outcome was favourable. CONCLUSION: Our study emphasizes the importance of wide local excision with margins of at least 3 cm in order to prevent local recurrence. However, the recent development of inhibitors of signal transduction by the PDGFB pathway should soon modify the surgical strategy, which is often too mutilating.     

Effect of UVB 311 nm irradiation on normal human skin

Ultraviolet radiation B (UVB) on the skin induces erythema, inflammation and modifications of the immune system. These changes have been reported after excessive short-term or long-term exposure to broad spectrum UVB. In this study, we examined the effects of local repetitive UVB irradiation of 311 nm wavelength on the skin of seven young volunteers. Skin biopsies were taken before and after UVB irradiation, and we immunohistochemically analyzed the expression of CD1a and HLA-DR antigens of Langerhans cells (LC), the possible infiltration of dermis/epidermis by CD11b macrophages, the modifications or the induction of intercellular adhesion molecule-1 (ICAM-1), E-selectin and vascular cell adhesion molecule-1 (VCAM-1) involved in the binding of leukocytes to the endothelial surface and the development of perivascular infiltrates of LFA-1⁺ mononuclear cells. We also determined the expression of substance P receptors (SPR) using biotinylated substance P (SPB). Exposure of UVB 311 nm induced a drastic reduction of CD1a⁺ cells and a moderate increase of HLA-DR⁺ dendritic cells in the epidermis without infiltration by CD11b macrophages. An increase of the binding of SPB to upper layer epidermal cells was noted in five of seven biopsies. In the dermis, vessel-associated ICAM-1 expression increased and an induction of E-selectin occurred on nearly 20 to 40% of endothelial cells, but VCAM-1 expression remained undetectable. The percentage of LFA-1⁺ cells did not change significantly after irradiation. These observations may be compatible with a selective role of UVB 311 nm on the skin immune response.     

Immunohistological identification of cell subsets in human gingival after local treatment for gingivitis or periodontitis

The cellular infiltrate present in human diseased gingiva was analyzed in biopsies from 12 patients with gingivitis or periodontitis. The samples studied had been obtained in the course of surgery at inflammatory sites remaining after institution of periodontal treatment. Histological and immunological techniques were used to identify macrophages, B-cells, plasma-cells, T-cells and T cell subsets, as well as cells expressing class II HLA membrane antigens. T-cells appeared as the predominant population, but plasma-cells were also visualized in nearly all samples. Both OKT4+ and OKT8+ cells were seen in all cases, the latter being more numerous in periodontitis patients. Interdigitating-like cells were observed, positively labelled for class II antigens, as well as macrophages which were more numerous in periodontitis patients. These results suggest the participation of all components of the immune response in gingival disease, in a way resembling chronic recurrent inflammatory diseases.     

Three-dimensional modeling system for unilateral mandibular bone distraction: a clinical case.

Facial hemiatrophies are anomalies of the first branchial arch and affect one in 4000-5000 newborns. Bone distraction is the technique of choice for the treatment of these dysmorphoses. Mandibular osteodistraction requires prior determination of the characteristics of the distraction vector whose three components will serve to activate the distractor. The patient, aged 5 years, presented with a right facial hemiatrophy, Grade IB according to the classification of Pruzansky. Tomodensitometric acquisition was obtained with a CT scanner. Software specifically designed for this application allows segmentation of the anatomical elements by a region-growing algorithm. The 3D representation of each element is added to a 3D scene, in which are placed the built-up landmarks necessary for the surgical simulation after 3D cephalometric analysis. The surgical cleavage plane is oriented according to the surgeon's requirements while preserving the predominant anatomical elements. The software allows performance of rotations and translations of the bone segments rendered independently from the cleavage plane. The distances and angles covered during the virtual movement are measured at its conclusion. The aim of moving the bone segments is to render the mandibular occlusion plane parallel to the reference occlusion plane. The vertical growth of the maxilla is realized by secondary recuperation. The distractor used was of an external multidirectional type allowing elongation of the mandibular ramus and mandibular corpus, closure of the goniac angle, and lateralization or medialization of the ramus. On the 15th day, the mandibular angle was reduced by 10 degrees, which allowed closure of the anterior gap and recentering of the incisive areas by a half-cuspid. The patient presented with a complex bone deficit in the three spatial directions, which allowed the development of software for modeling the distraction. Other clinical cases will be necessary to validate this 3D imaging-based technique.     

Diagnostic value of gastric pHmetry in duodenogastric reflux

Several studies have been performed to examine the problem of diagnosing gastroduodenal reflux (GDR). No single method is widely accepted. The aim of this work was to evaluate the diagnostic value of gastric pHmetry in this regard. A gastric aspiration probe attached to a combined glass electrode was placed in the stomach of 24 patients, with its distal tip located between 9 and 12 cm below the cardia. One ml samples of gastric juice were taken from 8 of the patients every 30 min for 15 h and as well as, every time a spontaneous alkalinization (SA) (defined by a pH greater than or equal to 4 for at least 1 min) was observed. The pH of each sample was measured by colorimetry whereas the concentration of total biliary acids (CTBA) was evaluated by the fluorimetric method (Kit Sterognost 3 alpha Flu); pH value measured via the intragastric electrode during aspiration was also recorded (protocol A). Continuous gastric aspiration was carried out in the remaining 16 patients for the entire duration of the test (6 h) which was divided into periods of 20 min. Apart from the parameters evaluated during protocol A, the percentage of time during which the stomach had a pH greater than or equal to 4 was recorded, as well as the quantity of total biliary acids collected over the 20 min periods (protocol B). Correlation studies were carried out using the Kendall tau and Spearman tests. Percentages were compared using the chi 2 test.(ABSTRACT TRUNCATED AT 250 WORDS)     

The impact of poverty on educational outcomes for children

Over the past decade, the unfortunate reality is that the income gap has widened between Canadian families. Educational outcomes are one of the key areas influenced by family incomes. Children from low-income families often start school already behind their peers who come from more affluent families, as shown in measures of school readiness. The incidence, depth, duration and timing of poverty all influence a child’s educational attainment, along with community characteristics and social networks. However, both Canadian and international interventions have shown that the effects of poverty can be reduced using sustainable interventions. Paediatricians and family doctors have many opportunities to influence readiness for school and educational success in primary care settings.     

Failure to thrive and intestinal diseases in congenital disorders of glycosylation]

Congenital disorders of glycosylation type I (GDG-I) is a class of genetic multisystem disorders characterised by defective glycosylation of glycoproteins. The characteristics and mechanisms of failure to thrive and intestinal diseases present in CDG-I are anectodal. PATIENTS AND METHODS: The aim of this study was to analyse 7 CDG-I (4 CDG-Ia, 2 CDG-Ib and 1 CDG-Ix) with important digestive symptoms and failure to thrive in order to characterise the mechanisms implied. RESULTS: Four children had no skin abnormality or dysmorphia (1 CDG-Ia, 2 CDG-Ib, 1 CDG-Ix). An encephalopathy with cerebellar hypoplasia was present only in the 4 CDG-Ia. Failure to thrive and diarrhea were present during the first month of life in 6 and appeared at 5 years in one CDG-Ia associated to mild or severe hepatopathy in all patients. One CDG-Ia, 1 CDG-Ib, 1 CDG-Ix had an exsudative enteropathy. A positive steatorrhea was present in 3 patients. Five patients had an abnormal small bowel biopsy. Abnormalities were variable: moderate inflammation of the chorion without villous atrophy in 2, intra-enterocyte fat accumulation without villous atrophy in 2, and partial villous atrophy with lymphangectasia in 1. In 2 CDG-Ia the intestinal biopsy was normal. Enteral nutrition in 4 and parenteral nutrition in 2 were effective in 4 patients and 1 patient with an exsudative enteropathy respond to a free fat diet (CDG-Ix). CONCLUSION: The digestive symptoms with failure to thrive is a common feature of CDG-I and could be the first symptoms. The diagnostic should be suspected if no other cause is found. Mechanisms of the intestinal symptoms appear to be multiple such as inflammation, abnormal enterocyte lipid transport or intestinal permeability related to the abnormal glycosylation of intestinal mucosa glycoproteins.