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.     

Health insurance and AIDS: the status of state regulatory activity.

Information collected by the National Gay Rights Advocates in 1986 and by the authors in the spring of 1987 was used to determine the extent to which the states currently regulate the practices of the health insurance industry specific to acquired immunodeficiency syndrome (AIDS). Of the 10 states reporting the greatest number of AIDS cases, six prohibit insurers form denying coverage to group policy applicants because of human immunodeficiency virus (HIV) infection. These findings refer only to the status of state regulatory activity specific to AIDS.     

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.     

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.     

Structure-activity relationship of toxic-shock-syndrome toxin-1: derivation and characterization of immunologically and biologically active fragments

Toxic-shock-syndrome toxin-1 (TSST-1), a 22-kilodalton (kDa) polypeptide, was proteolyzed by papain, generating three distinct fragments, identified as 16, 12, and 10 kDa (based on molecular masses estimated from the predicted amino acid sequence). The NH2-terminal sequence analysis of the fragments indicated that the peptide bonds between Tyr-52 and Ser-53 and between Gly-87 and Val-88 were cleaved. Functional activity, evaluated through enzyme-linked immunosorbent and inhibition assays, was demonstrated only with the 16- and 12-kDa fragments. The presence of homologous and heterologous antigenic determinants on the fragments was demonstrated by immunoblotting. In in vitro stimulation of human peripheral blood mononuclear cells, the 12-kDa fragment was significantly (P = .003) more active than the 16-kDa fragment. The former composed 75% of the latter and occupied the COOH-terminal portion of the holotoxin. The functional domains were located on two-thirds of the TSST-1 molecule, toward the COOH-terminal end, and mitogenicity apparently was separable from serological activity.     

Evaluation of contractile state by maximal ventricular power divided by the square of end-diastolic volume

BACKGROUND. Maximal ventricular power (PWRmax) reflects contractile state and has the potential to be noninvasively determined. However, its sensitivities to preload, afterload resistance, and inotropic state are incompletely defined. The present study determines these dependencies and proposes a novel power-based contractile index that is little altered by load. METHODS AND RESULTS. Seven open-chest, autonomically blocked dogs were instrumented with a proximal aortic flow probe, central aortic and ventricular micromanometers, and a conductance catheter for ventricular chamber volume. Preload was transiently reduced by left atrial hemorrhage, and afterload was increased by intra-aortic balloon inflation. Inotropic state was pharmacologically altered by lidocaine, dobutamine, propranolol, or verapamil. PWRmax was highly preload sensitive, altering 1.7 +/- 0.1-fold a given percent change in end-diastolic volume (EDV). This preload dependence was reduced by dividing PWRmax by EDV but was virtually eliminated when PWRmax was divided by EDV2. This latter index also displayed little change in response to as much as 60% increases in afterload resistance. PWRmax/EDV2 varied directly with inotropic state, correlating to both the slope (Ees) of the end-systolic pressure-volume relation (PWRmax x 1,000/EDV2 = 0.31 x Ees - 0.04, r = 0.82, p less than 0.001) and the slope (A) of the dP/dtmax-EDV relation (PWRmax x 1,000/EDV2 = 0.025 x A + 0.02, r = 0.86, p less than 0.001). PWRmax values determined from the product of ventricular pressure and flow versus central aortic pressure and flow were nearly identical over a broad loading range, indicating that PWRmax may be noninvasively assessed (i.e., without requiring left ventricular chamber pressure). CONCLUSIONS. PWRmax divided by EDV2 provides a measure of contractile function that is little influenced by loading conditions and has potential for noninvasive clinical use.