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.     

Stereotaxic needle localization and biopsy of occult breast lesions: first year's experience.

During a 12-month period 115 patients with abnormal mammograms had stereotaxic needle localization and biopsy of nonpalpable breast lesions. The procedure was performed on a Fischer Mammotest II machine (Fischer Imaging; Denver, CO) and the biopsies were taken with a #18 gauge Bard biopsy needle using a Bard biopty gun (distributed by Bard Urological; Covington, GA; manufactured by Radiplast; Uppsala, Sweden). Mammographic lesions were suspicious matrix densities (85), clustered microcalcifications (22), or a combination of both (8). The pathologist recommended open biopsy in 16 per cent (18/115) of the patients. Pathology on the 18 open biopsies revealed that 11 (9 matrix densities and 2 calcifications) were carcinomas and true positives, whereas the other 7 (all matrix densities) were benign mastopathies and false positives. Further analysis of the pathologic data showed that there were three possible diagnoses from the needle biopsies on the patients that later went to open biopsy: cancer (6), very suspicious lesion (9), and slightly suspicious lesion without atypical hyperplasia (3). All 6 cancers were confirmed by open biopsy; about half (5/9) of the very suspicious lesions were cancer and none (0/3) of the slightly suspicious lesions were cancer. More cases, followed by open biopsy, are needed to refine the selection procedure for open biopsy and careful follow-up of the patients who did not have open biopsy will also be needed to determine the false negative rate. Excellent patient acceptance was found and the test was easy to perform in the office without serious complications. Furthermore, the test was cost effective because it avoided open biopsy in 97 patients.