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.     

Quantification of perfusion fMRI using a numerical model of arterial spin labeling that accounts for dynamic transit time effects.

A new approach to modeling the signal observed in arterial spin labeling (ASL) experiments during changing perfusion conditions is presented in this article. The new model uses numerical methods to extend first-order kinetic principles to include the changes in arrival time of the arterial tag that occur during neuronal activation. Estimation of the perfusion function from the ASL signal using this model is also demonstrated. The estimation algorithm uses a roughness penalty as well as prior information. The approach is demonstrated in numerical simulations and human experiments. The approach presented here is particularly suitable for fast ASL acquisition schemes, such as turbo continuous ASL (Turbo-CASL), which allows subtraction pairs to be acquired in less than 3 s but is sensitive to arrival time changes. This modeling approach can also be extended to other acquisition schemes.     

Extent of ictal origin in mesial temporal sclerosis patients monitored with subdural intracranial electrodes predicts outcome.

In patients with mesiotemporal sclerosis, posterior hippocampal involvement at the ictal onset is not associated with an excellent outcome. A study confirmed that ictal onset in the posterior parahippocampal gyrus is associated with a less favorable outcome compared with ictal onset in the anterior parahippocampal gyrus in patients with mesiobasal temporal lobe epilepsy who are undergoing foramen ovale recording. The authors hypothesized that involvement of the two medial contact points of posterior basal temporal subdural (SD) strip at the ictal onset, representing ictal onset in the posterior parahippocampal gyrus, may also adversely influence the surgical outcome. With this objective, the authors assessed the incidence of posterior basal temporal SD strip (the two medial contact points) involvement at the ictal onset in patients with mesiotemporal sclerosis and determined whether presence of this finding influenced surgical outcome. Thirty-six patients with mesiotemporal sclerosis underwent a single SD grid (lateral frontotemporal) and strips (three basal temporal and one orbitosubfrontal) monitoring. Based on the earliest involvement of basal temporal strips (the two medial contact points) during the seizure, patients were classified into (1) anterior and/or middle basal temporal, or (2) posterior basal temporal (with or without involvement of anterior and/or middle basal temporal) ictal onset groups. A temporal lobectomy with adequate resection of the ictal onset zone was performed in all patients. Surgical outcome was based on Engel's classification. Six of 36 (17%) patients were classified into the posterior basal temporal ictal onset group. Only two patients from the posterior basal temporal ictal onset group experienced a good outcome compared with 26 of 30 patients from anterior and/or middle basal temporal ictal onset group (P = 0.01). In patients with mesiotemporal sclerosis who were monitored with SD electrodes, involvement of the two medial contact points of posterior basal temporal strip at the ictal onset (representing ictal onset in the posterior parahippocampal gyrus) occurred in 17% of the patients. These patients might not experience an excellent surgical outcome despite including the ictal onset zone in resection. These findings may be useful in presurgical counseling of patients with mesiotemporal sclerosis who undergo intracranial SD monitoring.     

The fixed and dilated pupils of premature neonates

We examined on a weekly basis the pupils of 30 preterm infants. In relative darkness (less than 10 foot-candles [ft.-c.] of illumination), the pupils measured a mean of 4.7 mm in the youngest infants (26 weeks' postconceptional age) when the corneal diameter was 7.0 mm. The pupils became progressively smaller, reaching 3.4 mm at 29 weeks' postconceptional age (P less than .001). The pupils did not constrict to the stimulating light (600 ft.-c.) until a mean of 30.6 weeks' (+/- 1 week) postconceptional age. Mydriasis should not be considered indicative of a central nervous system disorder, and a pupil unresponsive to light should not be considered suggestive of blindness until a preterm infant reaches at least 32 weeks' postconceptional age.     

Can we understand partnerships in general practice? A pilot study

BACKGROUND: Partnerships have been investigated in different professions,but other than identifying problems, little work has been carriedout on general practice. OBJECTIVE: The aim of this present study was to develop methods for studyingpartnerships in general practice. METHOD: A tripartite methodological approach was used, with questionnairesadapted from other instruments in use in other professions,followed by an individual interview with each partner, and non-participantobservation at a partnership meeting. Results for one case-studypartnership are given. RESULTS: There were no major differences between the partners on alldimensions measured; the minor differences indicated by theresults of the questionnaires were corroborated by the partnerinterviews and observations. CONCLUSIONS: We conclude that the use of such techniques could provide supportto partnerships going through significant periods of change. Keywords. General practice, interview, observation, partnerships, questionnaire.     

Sociomedical aspects of malaria control in Colombia.

A household interview survey combined with a serological survey on the incidence of malaria attacks and prevalence of antibodies has been carried out in rural and urban areas of the pacific coast of Colombia. Additional information on people's knowledge, attitudes and behaviour towards malaria was collected by means of participant observation and informal interviews. The results show that people incorporate modern and traditional elements into their concepts of the disease and treatment strategies. The deficiencies of the official control programmes are shown from the people's point of view. Some human factors which influence malaria transmission are discussed and an estimate of the accuracy of self-diagnosis presented.     

Rapid chemical kinetic techniques for investigations of neurotransmitter receptors expressed in Xenopus oocytes

Xenopus laevis oocytes have been used extensively during the past decade to express and study neurotransmitter receptors of various origins and subunit composition and also to express and study receptors altered by site-specific mutations. Interpretations of the effects of structural differences on receptor mechanisms were, however, hampered by a lack of rapid chemical reaction techniques suitable for use with oocytes. Here we describe flow and photolysis techniques, with 2-ms and 100-μs time resolution, respectively, for studying neurotransmitter receptors in giant (≈20-μm diameter) patches of oocyte membranes, using muscle and neuronal acetylcholine receptors as examples. With these techniques, we find that the muscle receptor in BC₃H1 cells and the same receptor expressed in oocytes have comparable kinetic properties. This finding is in contrast to previous studies and raises questions regarding the interpretations of the many studies of receptors expressed in oocytes in which an insufficient time resolution was available. The results obtained indicate that the rapid reaction techniques described here, in conjunction with the oocyte expression system, will be useful in answering many outstanding questions regarding the structure and function of diverse neurotransmitter receptors.