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.     

Food choice in response to computer-generated nutrition information provided about meal selections in workplace restaurants

Aim : The study was designed to determine the effect of computerized nutrition information on consumer food choice in two workplace restaurants, one in which customers had to pay for their lunch and the other providing a free lunch. Methods : Customers entering the restaurants were asked to make selections from the menu on a computer screen. The energy, saturated fatty acids, non-milk extrinsic sugars and non-starch polysaccharide content of the meal selected was displayed graphically in proportion to the dietary reference values. They were given the opportunity to change their selected meal and the composition of all meals was recorded. Results : The nutritional composition of the first meal provoked 16% of customers to make a second selection. The proportion of energy in the first selection had been 31% higher for saturated fatty acids and 23% higher for non-milk extrinsic sugars than the first selection made by people who were satisfied with their first choice. In their second attempt they succeeded in reducing both nutrients to levels similar to those present in the meals selected by people who had been satisfied with their first selection. Customers>> selections for non-starch polysaccharide and energy did not differ between the groups. The main changes made by customers to achieve improved second choices were to omit dishes (44%), add dishes (19%), make changes within a menu category (46%), and make changes from one menu category to another (26%). Conclusion : It was concluded that provision of graphical nutrition information on a computer screen could be used by a subset of the users of both restaurants to enable them to improve their menu selections to a similar composition to that selected by the other people who used the computer system.     

Alcoholism in patients with sexual disorders

The authors screened for alcoholism 145 consecutive patients who presented with sexual dysfunction or disorders. Using the Michigan Alcoholic Screening Test (MAST), 29% of the patients scored in the probable alcoholic range. Probable alcoholics were more likely to present without a partner and claimed higher sex drive than nonalcoholic patients. Probable alcoholic males reported less joy and vigor than probable alcoholic females, while the reverse held for the nonalcoholic groups. Blind to the MAST results, the staff made six alcohol-related diagnoses and referred one patient for alcohol treatment. The authors discussed the importance of training faculty and resident staff in the relationship of alcohol abuse associated with psychosexual dysfunctions.     

Chronic treatment with scopolamine and physostigmine changes nerve growth factor (NGF) receptor density and NGF content in rat brain

Nerve growth factor (NGF) and NGF receptors were measured in cortex and hippocampus of rats treated with drugs affecting cholinergic neurotransmission. High (K_d= 0.045nM) and low (K_d= 21nM) affinity¹²⁵I-NGF binding sites were present in both cortical and hippocampal membranes with hippocampus containing higher numbers of both sites than cortex. Chronic treatment of rats with the muscarinic receptor antagonist scopolamine (5 mg/kg, twice daily) decreased the density of high- and low-affinity sites by 50–90% in cortical and hippocampal membranes. These changes were seen after 7 days, but not 3 days, of scopolamine treatment. Chronic infusion of physostigmine (1 mg/kg/day) using minipumps increased the number of high- and low-affinity sites in cortex 3- and 6-fold, respectively. The changes in receptor-binding parameters induced by physostigmine were transient as they were evident after 3 days of treatment, but returned to control levels after 7 days. NGF content in cortex and hippocampus was reduced by about 50% following 7, but not 3, days of chronic physostigmine infusion. In contrast, scopolamine treatment failed to change NGF levels in the cholinergic neuronal target regions but it decreased NGF content in the septal area. The content of NGF mRNA in the cortex measured by Northern blot analysis failed to change following either scopolamine or physostigmine treatment. The results suggest that levels of NGF and NGF receptors in the target regions of cholinergic neurons are regulated by the extent of cholinergic neurotransmitter activity.     

Acquired Cutis Laxa in Childhood Sweet's Syndrome

Abstract:  In Sweet's syndrome, the essential features are the characteristic morphology of the lesions, their histologic appearance, the dramatic response to corticosteroids and the absence of scarring. We report an 8-month-old infant in whom Sweet's syndrome was diagnosed and who developed acquired cutis laxa in the skin lesions.