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.     

Acquired Cutis Laxa in Childhood Sweet's Syndrome

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.     

Acute leriche syndrome due to paradoxical embolism.

Paradoxical embolism, describing the passage of venous or right-sided cardiac thrombus into the arterial or systemic circulation through patent foramen ovale, is an uncommon cause of acute arterial occlusion. Here, we report acute Leriche syndrome in a young woman attributable to paradoxical embolism. Ischaemia, patent foramen ovale, and venous thrombosis were the triad of evidence for paradoxical embolism.     

OVERHEATING IN BED AS AN IMPORTANT FACTOR IN MANY COMMON DERMATOSES

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.     

Results of surgery in 144 cases for aneurysms of the infra-renal abdominal aorta in patients over 75 years of age

114 patients aged over 75 underwent surgery for aneurysm of the infra-renal abdominal aorta in the University Teaching Hospitals in Nantes and Angers between 1979 and 1988. A retrospective study of these patients was performed to evaluate the immediate and long-term results. The mean age of the patients was 79 (+/- 4) years, the oldest being 94. 70% were men. Half of the patients underwent emergency or semi-emergency surgery (52 cases). Cardiovascular factors (in particular coronary insufficiency in 17% of cases) were the most common risk factors. In all cases grafting after laying open the aneurysm was performed, with an aorto-aortic graft in 32% of patients, an aorto-iliac graft in 37% or an aorto-bifemoral graft in 27% of patients. Combined intestinal revascularisation was performed in 10% of case either involving the inferior mesenteric artery or at least one internal iliac artery; renal revascularisation was performed in 3.5% of cases. 75% of patients underwent simple grafting. The mean duration of hospitalisation was 14 days (+/- 6), including a mean period of 7 days in ICU. 36 patients (31%) died in the first post-operative month. The mortality rate in patients who underwent emergency surgery for a complication of the aneurysm (essentially rupture) was 61% versus 6' for elective surgery. 96% of the patients who survived the first post-operative month were independent at the end of the study or at the time of their death.(ABSTRACT TRUNCATED AT 250 WORDS)     

One stage surgical management of hydatid cyst of lung &; liver—by right thoracotomy &; phrenotomy

Introduction Hydatid cysts of the lung are quite frequent in our country. Some patients have additional cysts in the liver. Though most of the liver cysts remain asymptomatic for long time, but may be symptomatic with increasing size. Surgical removal is the treatment of choice for both lung & liver cysts. Aim of the study was to establish suitability of one stage surgery for pulmonary & hepatic hydatid cysts. Methods From 1996 through 2003 we operated on 216 pulmonary hydatid cysts, out of which 42 patients had hydatid cysts in the right lung as well as in the right lobe of liver. Right thoracotomy was done to remove the lung hydatids followed by phrenotomy to remove the liver cysts. Results Right thoracotomy was done in 42 patients having hydatid cysts of lung & liver. In 36 patients, cysts were removed, bronchial leaks were sutured & residual cavities were obliterated. Out of rest 6 patients, having dense adhesions or destruction of pulmonary parenchyma, 4 had segmentectomy & 2 had lobectomy. Right phrenotomy was then done with radial incision above the palpated liver cysts. Hydatid cyst was removed from liver. Cavity and remaining pericystic liver tissue was inverted with sutures. Water seal chest drain & subdiaphragmatic drain were placed. Post operative albendazole was continued for 3 months in the dose of 10–20 mg/kg with a gap of 2 weeks after each month. Post operative recovery was uneventful in most of the cases. However, air leak continued for almost 3 weeks in 4 patients & 3 months in one patient. There was no death. Conclusion Surgical management of pulmonary and hepatic hydatids with one stage right thoracotomy & phrenotomy is a suitable option. It avoids additional laparotomy and thereby additional cost & hospital stay. Results are quite satisfactory.