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.     

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.     

Is infrainguinal bypass grafting successful following failed angioplasty?

OBJECTIVES: Angioplasty is often used in the management of lower limb ischaemia and can reduce the need for infrainguinal bypass in some patients. There is an associated failure rate with this technique and bypass surgery is often used in this situation as a secondary limb salvage procedure. We aimed to evaluate the outcome of infrainguinal bypass grafting following failed attempt at angioplasty. METHODS: All cases of infrainguinal bypass at a single centre over a seven year period were identified and notes reviewed. Cases were divided into four groups according to their indication for surgery; acute ischaemia, chronic critical ischaemia, failed angioplasty and an 'other' group including aneurysmal disease and claudicants. The failed angioplasty group was compared with the other three groups. Survival analysis was performed using Kaplan Meier curves and groups compared in terms of long term patency and survival. RESULTS: Primary patency was 61.2% in the failed angioplasty group at 12 months compared with 60.6% in the other groups (P=1.11). There was also no significant difference in primary patency at 60 months (50% vs 40.6%, P=0.26). Survival at 12 months was also comparable between the groups (failed angioplasty group 74.2% compared with 77.3% in the other groups, P=0.662) as was 60 months survival (33.3% and 35.4% respectively, P=0.166). DISCUSSION: In this study, outcome of infrainguinal bypass following failed angioplasty was comparable to outcome of surgery performed for another indication. This paper supports the use of distal bypass surgery for limb salvage in cases where minimal access techniques have failed.     

OVERHEATING IN BED AS AN IMPORTANT FACTOR IN MANY COMMON DERMATOSES

Background. Extensive questioning of patients with a wide variety of skin disorders led to the impression that nocturnal overheating was probably an important factor in the initiation and the perpetuation of many skin disorders. Methods. In order to test the hypothesis, 12 “clean-skinned” subjects (6M/6F) aged 18 to 45 years were monitored electronically every 30 seconds during an 8 hour sleep period (2300 to 0700 hours), sleeping under a standard 10 tog duvet. Results. All the subjects were too hot by 3 to 4°C. All showed changes in their EEG patterns with reduced REM sleep, increased awakenings, and all showed changes in their sleep stage patterns. In addition, they all showed evidence of increased sweating in the “heat-sink” area. Conclusions. The mechanisms where by such changes could be implicated in the precipitation and perpetuation of skin disease are discussed. “Lifestyle” modification as a very effective, noninvasive, therapeutic regime is recommended. Further research along these lines would probably be very valuable and instructive.     

Frozen algae as food for Daphnia magna Straus in toxicity testing.

As EC validity criteria for the production of Daphnia magna neonates in standard toxicity tests set fecundity minima which must be attained, it is important that culture conditions provide an adequate ration of high quality food. Maintaining a steady supply of fresh algae can be problematical so the possibility of using frozen food was investigated. The performance of three generations of D. magna fed on diets of fresh or frozen Chlorella was compared. There were no significant effects on fecundity due to food type or generation. Cumulative fecundity (over 21 days) on fresh or frozen food exceeded 120 neonates per female and satisfied that EC validity criterion (60 neonates per female). In addition criteria relating to survival and variability were met. We conclude that frozen Chlorella are an acceptable substitute for fresh algae.