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.     

A note on the synapses between vesicle containing profiles in the pontine nuclei of the cat

In the cat synapses between vesicle containing profiles were observed in ventral and dorsolateral pontine nuclei. The presynaptic elements consisted of two types of axon terminals: axon terminals characterized by a population of small (38-40 nm) round synaptic vesicles (SSV) and axon terminals containing pleomorphic synaptic vesicles (PSV). The postsynaptic pale elements (PP) had pleomorphic vesicles and some features attributed to dendrites. In the dorsolateral pontine nucleus most of PP profiles took part in serial synapses, usually as an intermediate component, they were rarely observed in triads. On the basis of their electron microscopical appearance and synaptic relations they might be considered to represent a dendritic part of putative interneurons.     

Positive sign of the stenocardia syndrome as a criterion of myocardial ischemia during the treadmill test

Diagnostic validity of the criteria of myocardial ischemia in coronary patients exposed to the treadmill test is assessed. The risk of a false diagnosis of coronary insufficiency on the basis of the pain syndrome was 5 times as high as that of diagnosis based on ischemic electrocardiographic ST depression. In subjective assessment of walking tolerance, patients with severe angina were particularly prone to mistakes. Limitations of angina classifications by severity, based on subjective sensations of coronary patients, are considered.     

Surgical tactics in clotted hemothorax in patients with combined chest injuries

Based on the experience with treatment of 26 patients the authors recommend to use mainly conservative treatment with the intrapleural administration of fibrinolytic drugs. The method was used in 20 patients. All of them recovered.     

Use of endoscopic papillosphincterotomy in the treatment of acute pancreatitis

The authors describe results of using endoscopic papillosphincterotomy in 100 patients with acute pancreatitis. Methods of endoscopic papillosphincterotomy and endoscopic papillosphincterowirsungotomy are presented. The effectiveness of the treatment was 96%. Operative procedures on emergency made up 1%, lethality-1%, complications-3% of the cases.     

Diagnosis and evaluation of the severity of arterial insufficiency of the legs using ultrasonic Doppler flowmetry in the treadmill test

A method of diagnostics and assessment of the severity of arterial insufficiency of lower extremities was developed on the basis of multistep treadmill-test and measuring time of reestablishment of continuous bloodflow in the popliteal artery by the method of ultrasonic doppler flowmetry. An examination of 40 patients with angiographically detected alterations of the vessels and 18 healthy persons was performed. It was found that the diagnostic and assessment criterion of arterial insufficiency in treadmill-test with high parameters of sensitivity and specificity is longer duration of the period of reestablishment of continuous bloodflow over 180 sec. The arterial insufficiency severity is estimated by the value of exercise by the moment of the appearance of the criterion.