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.     

The impact of poverty on educational outcomes for children

Over the past decade, the unfortunate reality is that the income gap has widened between Canadian families. Educational outcomes are one of the key areas influenced by family incomes. Children from low-income families often start school already behind their peers who come from more affluent families, as shown in measures of school readiness. The incidence, depth, duration and timing of poverty all influence a child’s educational attainment, along with community characteristics and social networks. However, both Canadian and international interventions have shown that the effects of poverty can be reduced using sustainable interventions. Paediatricians and family doctors have many opportunities to influence readiness for school and educational success in primary care settings.     

Naphthalimido derivatives as antifolate thymidylate synthase inhibitors

A new series of N-(substituted)benzyl-1,8-naphthalimides 4, structurally related to the previously reported thymidylate synthase (TS) inhibitor naphthaleins 3, were synthesized and compounds tested for their inhibition of several species of TS. Moreover, their in vitro cytotoxicity together with antimycotic and antibacterial properties were assayed. While no activity was detected in the antibacterial tests, the m-nitro (4ae) and the p-nitro (4af) derivatives were found able to partially inhibit TS at low micromolar concentrations. Introduction of nitro or (substituted)-amino groups in position 4 of the naphthalic ring always led to less active compounds.     

The effect of elbow flexion and time of assessment on the measurement of grip strength in rheumatoid arthritis.

Grip strength is one of the traditional outcome measures commonly used in rheumatoid arthritis trials. This study evaluated the degree of morning variation and the effect of elbow flexion in the assessment of grip strength in patients with rheumatoid arthritis. Grip strength was measured in 37 patients (1987 American Rheumatism Association criteria) at 8, 10, and 12 AM with a sphygmomanometer cuff folded on itself, taped permanently, and inflated to 20 mm Hg, with the elbow flexed at 90 degrees. At 10 AM grip strength was also measured with the elbow flexed at 30 and 130 degrees. Mean values for grip strength at 8, 10, and 12 AM were, respectively, 69, 79, and 83 mm Hg. Mean grip strength values with the elbow flexed at 30, 90, and 130 degrees were, respectively, 78, 79, and 75 mm Hg. There was a statistically significant correlation between grip strength and morning stiffness, grip strength and hand function, and grip strength and number of active joints. In rheumatoid arthritis trials, grip strength should be assessed at the same time of the day. Elbow flexion does not play a role in grip strength measurement.     

Stressors in ICU: patients’ evaluation

Objective: To study the physical and psychological stressors in the intensive care unit (ICU) and to correlate stressors with different demographic variables. Design: Cross-sectional analytical survey. Setting: Intensive care unit of a private hospital. Patients and participants: 50 randomly selected ICU patients during the first week of their ICU stay. Measurements and results: The Intensive Care Unit Environmental Stressor Scale was administered to 50 patients. Pain and the impossibility of sleeping due to noise and having tubes in the nose and mouth were considered the most important physical stressors. Loss of self control and lack of understanding about the attitudes and procedures were the main psychological stressors. Conclusions: Interventions should be aimed at relieving the patient's pain and at controlling the level of noise to make sleep possible. From the psychological standpoint, the independence of the patient should be encouraged, thus stimulating the recovery of self-control. The team should also inform the patient about the procedures which will be carried out. Received: 1 April 1997 Accepted: 24 September 1997