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.     

Effects of a very-low-calorie diet and physical-training regimens on body composition and resting metabolic rate in obese females

Sixty-nine obese females received 90 d of a liquid diet providing 2184 kJ/d in clinical trials. Groups were diet only (C), diet plus endurance exercise (EE), diet plus weight training (WT), or diet plus endurance exercise and weight training (EEWT). Changes in body weight, percent fat, fat weight, and fat-free mass were not different between groups. Declines in resting metabolic rate (RMR) were approximately 7% to approximately 12% of baseline values with no differences among groups. A significant increase in work capacity (approximately 16%) was shown for EEWT. Strength index showed declines of approximately 6% for C and EE and gains of approximately 3% and approximately 10% for EEWT and WT, respectively. These clinical trials did not show advantages of any exercise regimen over diet alone for weight loss, body-composition changes, or declines in RMR. Improvements in work capacity were limited and strength improved in groups that participated in strength training.     

Effect of the calcium entry blocker verapamil on renal ischemia

The ability of the calcium entry blocker verapamil to ameliorate the effects of renal ischemia was studied in ten sheep. Postanesthesia, bilateral cutaneous ureterostomies were placed in each sheep to facilitate urine collection and analysis. Both kidneys were made ischemic for one hour by occluding each renal artery. However, immediately before occlusion of the right renal artery, 0.05 mg/kg of verapamil was injected into the artery. Comparison of urinary creatinine excretion and urine volume for 72 h after reversal of ischemia demonstrated that those kidneys pretreated with verapamil had greater functional preservation (p less than .05). In this study, verapamil appeared to provide protection against renal damage after an ischemic insult.     

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.     

Comparison of three immunoassays for diagnosing sensitization to latex in children with spina bifida

As natural rubber latex (latex) has become more widespread in our environment, physicians have become increasingly aware of the problem of possible allergic reactions. Many fatal and near-fatal incidents have been reported (mainly during surgery) (1—3) and data has been published on groups frequently exposed to latex, such as patients with spina bifida (4—9), healthcare professionals (10—12) and occupationally exposed persons (13). The incidence of latex allergy in children seems to be increasing (14). Tests are therefore needed which can reliably detect sensitization to latex. Our aim was to compare the diagnostic accuracy of three commercial immunoassays for measuring specific IgE in serum to latex.     

Physical symptoms/side effects during breast cancer treatment predict posttreatment distress

Background: Studies suggest that the period following completion of treatment can be distressing for cancer patients. One potentially important predictor of distress is physical symptoms/side effects during treatment.Purpose: A longitudinal, observational design was used to examine whether the number of physical symptoms/side effects experienced during treatment was a correlate of cancer-related distress and general distress 4 months after treatment completion, as measured by the Impact of Events Scale and the Mental Health subscale of the Short Form-36, respectively.Methods: Participants were 151 women who had completed chemotherapy and/or radiotherapy for ductal carcinoma in situ or stage 1 or 2 breast cancer. Hierarchical multiple regression was conducted with relevant sociodemographic, clinical, and psychiatric variables entered as controls.Results: Greater physical symptoms/side effects predicted greater total cancer-related distress, intrusive thoughts, and general distress. Physical symptoms/side effects did not significantly predict avoidance. Follow-up analyses indicated that the relationship between physical symptoms/side effects and general distress was mediated by both total cancer-related distress and intrusive thoughts.Conclusions: These results suggest that patients who experience greater physical symptoms/side effects during treatment are at greater risk for later cancer-related distress and, in turn, general distress. Future research should evaluate whether early intervention with these patients is effective in preventing or reducing distress in the posttreatment period. This work was supported by a grant from the National Cancer Institute (5R01 CA082822).