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.     

Is herniography useful?

Herniography has been used for 25 years in the diagnosis of occult herniation but has not gained widespread acceptance in the UK, despite studies confirming its high sensitivity and specificity for occult hernias and an excellent record of safety and patient acceptability. The traditional approach in the UK to suspected occult groin herniation has been surgical exploration. This study examined the use of herniography in a single district general hospital to assess its impact in limiting unnecessary groin explorations and allowing discharge of patients without hernias. The case notes of 90 successive patients referred for herniography by the department of general surgery in a single UK district general hospital over an 18-month period were reviewed. Eighty-seven completed examinations were analysed in which 23 hernias were diagnosed in 20 patients. Thirteen patients have undergone hernia repair with resolution of symptoms. There were no false positive examinations, although two inguinal hernias were incorrectly diagnosed radiologically as femoral hernias; there were two false negative examinations where additional hernias were found at laparoscopic repair. There were no reported complications. Twenty-four patients were discharged directly from the surgical clinic after a negative herniogram. Thirty patients were referred to other specialities. No patient had undergone groin exploration after a negative herniogram. Herniography is a useful tool in assessing obscure groin pain and potential occult herniation. It can reliably rule out the presence of a hernia and avoid the need for surgical exploration. Many patients with a negative herniogram can be reassured and discharged, whilst others may be referred on to other specialities safe in the knowledge that an occult hernia has been excluded.     

The 5-Step family intervention in primary care: II. The views of primary healthcare professionals

Background: This is the second of two papers using qualitative methods from a study of an intervention for family members affected by close relatives' substance misuse problems.

Participants: 168 primary healthcare professionals (PHCPs: GPs, practice nurses and health visitors) working in general practices in two areas of England, and who took part in the study.

Data sources: Recruitment and post-session forms completed by PHCPs; telephone interviews with each PHCP 12 weeks after recruitment of a family member; interviews with PHCPs at the end of the study.

Results: At the end of the project PHCPs were overwhelmingly positive about the family member intervention and about primary care as the appropriate site. Difficulties were encountered, however, in identifying and engaging affected family members, who were often excluded on grounds of the complexity of their problems or the level of their distress. Shortage of PHCP time and other practice-related factors added to the difficulty. Active work by a PHCP was often necessary in order to make the link between presenting symptoms of physical or mental ill-health and the existence of a family substance misuse problem. When family members were identified and recruited, PHCPs were usually positive about what was achieved. Nearly all were in favour of an approach that combined giving a self-help manual with some follow-up contact with a family member as needed.

Conclusions: Taken in conjunction with statistical outcome findings of significant reductions in symptoms and changes in ways of coping, plus qualitative analysis of the views of family members, the present results encourage the view that a flexible form of this intervention should be developed for use in primary healthcare, and that further work should build on existing strengths and attempt to overcome weaknesses identified.     

Addition of bevacizumab to bolus fluorouracil and leucovorin in first-line metastatic colorectal cancer: results of a randomized phase II trial.

PURPOSE: Bevacizumab, a monoclonal antibody against vascular endothelial growth factor, increases survival when combined with irinotecan-based chemotherapy in first-line treatment of metastatic colorectal cancer (CRC). This randomized, phase II trial compared bevacizumab plus fluorouracil and leucovorin (FU/LV) versus placebo plus FU/LV as first-line therapy in patients considered nonoptimal candidates for first-line irinotecan. PATIENTS AND METHODS: Patients had metastatic CRC and one of the following characteristics: age > or = 65 years, Eastern Cooperative Oncology Group performance status 1 or 2, serum albumin < or = 3.5 g/dL, or prior abdominal/pelvic radiotherapy. Patients were randomly assigned to FU/LV/placebo (n = 105) or FU/LV/bevacizumab (n = 104). The primary end point was overall survival. Secondary end points were progression-free survival, response rate, response duration, and quality of life. Safety was also assessed. RESULTS: Median survival was 16.6 months for the FU/LV/bevacizumab group and 12.9 months for the FU/LV/placebo group (hazard ratio, 0.79; P = .16). Median progression-free survival was 9.2 months (FU/LV/bevacizumab) and 5.5 months (FU/LV/placebo); hazard ratio was 0.50; P = .0002. Response rates were 26.0% (FU/LV/bevacizumab) and 15.2% (FU/LV/placebo) (P = .055); duration of response was 9.2 months (FU/LV/bevacizumab) and 6.8 months (FU/LV/placebo); hazard ratio was 0.42; P = .088. Grade 3 hypertension was more common with bevacizumab treatment (16% v 3%) but was controlled with oral medication and did not cause study drug discontinuation. CONCLUSION: Addition of bevacizumab to FU/LV as first-line therapy in CRC patients who were not considered optimal candidates for first-line irinotecan treatment provided clinically significant patient benefit, including statistically significant improvement in progression-free survival.     

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.     

Primary mesenteric venous thrombosis: a study from western India.

INTRODUCTION: The prevalence and clinical spectrum of mesenteric venous thrombosis (MVT) in India is largely unknown. METHODS: We retrospectively re-viewed the case records of patients with primary mesenteric venous thrombosis seen over a 10-year period and retrieved information on clinical picture, underlying hypercoagulable states and outcome. RESULTS: The 28 cases (mean age 41.2 [SD 10.2] years; 19 male) included 13 with acute MVT, 10 with subacute MVT and 5 with chronic MVT. Ten patients had past thromboembolic events (multiple events in five); four patients had isolated superior mesenteric vein involvement and 14 had multiple vessel involvement. Hypercoagulable state was identified in 17 patients, with multiple etiologies in 7 patients. Pre-operative diagnosis was made in all patients. Ten patients needed surgical management; the rest were managed medically initially, but 2 required surgery on follow up. Seven patients died during a follow up of up to 10 years, with in-hospital mortality during index admission in six. CONCLUSIONS: Most of the patients with MVT have multiple intra-abdominal vessel involvement and underlying hypercoagulable state. The policy of early treatment with anticoagulation in all and surgical treatment as per need, achieves low mortality.