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.     

The Martin Titanline arterial clamp. A new lightweight alternative

When haemostatic clamps are applied, evidence of injury at the site of clamp application may be seen when the clamp is removed. Rarely, the intima may be disrupted. When a new arterial clamp became available, a study was designed to compare the Martin Titanline arterial clamp (13-143-35, curved arterial clamp) with several other arterial clamps already in use. The Martin clamp is a modified pivot-point, preset-tension, spring-controlled arterial clamp. The closing pressures of several clamps were measured objectively. The injury produced when the clamps were applied to occlude the blood flow on the carotid artery of a dog was assessed by histological study of the excised segments of the arterial wall. Histological cross-sections were prepared from canine carotid artery which had been perfused for 1 h after the clamp had been applied for 1 h. Histological evidence of injury was limited to disruption of the intimal layer and compression of the medial layer. No significant difference between the amount of damage caused by the DeBakey, Satinsky or Martin clamp was identified. When compared to the other varieties of clamp listed above, the Martin clamp had a significantly lower closing pressure (304 g) compared with 580g (Bulldog), 580 g (Satinsky), and 686 g (DeBakey). The Martin clamp was easier to apply, did not obstruct the operative field as readily and had good clamp-retention characteristics throughout the procedure.     

The Association of Different Measures of Insulinaemia with Vascular Risk Factors in Healthy Normoglycaemic Normotensive Non-obese Men and Women

Hyperinsulinaemia is said to be a risk factor for cardiovasculardisease, but the extent to which different insulinaemic measuresare associated with vascular risk factors in ostensibly healthyindividuals, and whether they operate independently in men andwomen, remains uncertain. The association between risk factors and various insulinaemicmeasures was examined in 148 men and 118 women who were normoglycaemic,normotensive, and non-obese (body mass index in men <27,in women <25). A 75 g glucose tolerance test was administeredafter blood sampling for fibrinogen, lipids, lipoproteins andinsulin. Insulin was also measured after 1 and 2 hours. Significantunivariate correlations (p<0.01) were most consistently recordedbetween insulinaemic measures and fasting serum triglyceridesin men and women, whilst systolic blood pressure only correlatedwith insulinaemia in women, and diastolic blood pressure correlatedwith fasting and 2 hour insulinaemic measures in men and women.Inconsistent associations were noted with total serum cholesterolin men and women, with high density lipoprotein cholesterol,body mass index, apoprotein B and A1 in men, and with fibrinogenin women. Age was not correlated with any insulinaemic measurein men or women. Differences in vascular risk factors between quintiles of theinsulinaemic measures were examined, after correction for bodymass index. The dominant association with fasting and post-glucoseload insulinaemic measures was with triglycerides, especiallyin women, with less frequent graded differences between quintilesobserved for total cholesterol, and diastolic and systolic bloodpressures in men and women. The incidence of other risk factors often only differed in thelowest or highest quintile in comparison to other quintiles,suggesting a threshold rather than a graded effect. Furthermore,differences in HDL cholesterol and apoprotein B were only recordedfor top quintiles of post-glucose challenge/integrated insulinaemicmeasures in men, whilst serum fibrinogen concentrations onlydiffered significantly in women in the top insulinaemic areaunder the curve quintile. In the absence of additional risk factors such as diabetes,hypertension and obesity, insulinaemic measures are not consistentlyrelated to blood pressure and measures of lipid metabolism andcoagulation, and are thus a weak predictor of other cardiovascularrisk factors. The vascular risk profile associated with insulinappears somewhat different in apparently healthy men and women.     

Herpetic croup: two case reports and a review of the literature

Croup is an acute infectious illness usually occurring in children; it is characterized by brassy cough and stridor. The main pathogens include mainly parainfluenza and influenza viruses. Recently there have been reports of prolonged croup caused by the herpes simplex viruses. We report two cases of prolonged croup due to herpes simplex types 1 and 2. We also review and summarize the reported pediatric cases of herpetic croup.     

Transferrin receptor expression by human bladder transitional cell carcinomas

Summary The expression of transferrin receptors (TFR) by normal and neoplastic urothelial cells was studied in control patients and in patients with transitional cell carcinoma of the bladder. These tumours were graded independently and consisted of 19 grade I, 30 grade II and 19 grade III lesions. TFRs were identified using a monoclonal antibody specific for TFR (OKT9) in an immunofluorescent or avidin/biotin-immunoperoxidase technique on fresh frozen sections. TFRs were not detected on normal urothelium. However, positive staining was found to increase with increasing pathological grade and stage of the tumours, ranging from 31.6% of grade I to 78.9% of grade III tumours and 51.2% of pTa (mucosa only lesions) to 87.5% of pT2/pT2+ (muscle invasion±deeper) primary urothelial malignancies.     

Fusarium endocarditis involving aortic valve following coronary artery surgery

This article describes a case of infective aortic valve endocarditisdue to Fusarium oxysporum occurring 4 years after coronary arterybypass grafting. It is the first reported case of endocarditiscaused by Fusarium.