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1.
D Monnier† C Vidal‡ L Martin§ A Danzon¶ F Pelletier† E Puzenat† MP Algros†† D Blanc† R Laurent† PH Humbert† F Aubin† 《Journal of the European Academy of Dermatology and Venereology》2006,20(10):1237-1242
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. 相似文献
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L. TIMMER-DE MIK M.D. D.M. BROEKHUIJSEN-VAN HENTEN M.D. † J.M. OLDHOFF M.D. PH.D. D.B. DE GEER M.D. ‡ V. SIGURDSSON M.D. PH.D. S.G.M.A. PASMANS M.D. PH.D. 《Pediatric dermatology》2009,26(3):358-360
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. 相似文献
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HUGH F. MOLLOY F.A.C.D. ERIC LAMONT-GREGORY M.SC. CHRIS IDZIKOWSKI PH.D. F.B.PS.S. TERENCE J. RYAN D.M. F.R.C.P. 《International journal of dermatology》1993,32(9):668-672
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. 相似文献
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P H Navsaria P de Bruyn A J Nicol 《European journal of vascular and endovascular surgery》2005,30(5):499-503
OBJECTIVE: The surgical management and outcome of abdominal vena cava (AVC) injuries is presented. STUDY DESIGN, PATIENTS AND METHODS: A retrospective record review of patients with AVC injuries treated in the Trauma Unit at Groote Schuur Hospital between January 1999 and December 2003 was undertaken. Demographic data, mechanism of injury, surgical management, associated injuries, duration of hospital stay, complications and mortality were extracted from patient records. Patients with acute peritonitis and/or shock underwent emergency laparotomy. RESULTS: Forty-eight patients with AVC injuries were identified. Gunshot wounds accounted for 45 (94%) injuries. The mean weighted revised trauma score, injury severity score (ISS) and penetrating abdominal trauma index (PATI) were 6.3, 24 and 42, respectively. The AVC injury was infrarenal and suprarenal in 41 and seven patients, respectively. Thirty injuries were ligated. There were 15 deaths (31%). Significant differences between survivors and non-survivors included ISS, preoperative hypotension and blood transfusion requirements, whereas site of injury, PATI, and surgical management did not. CONCLUSION: Abdominal vena cava injuries are associated with a high mortality. Ligation of the AVC in critically ill patients is a feasible and life-saving option. 相似文献
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Braffman BH; Coleman BG; Ramchandani P; Arger PH; Nodine CF; Dinsmore BJ; Louie A; Betsch SE 《Radiology》1994,190(3):797
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