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.     

Shouldice hernia repair: results at a teaching institution

Authors with wide experience report that the Shouldice technique for repair of inguinal hernia is very effective. The technique has not gained widespread acceptance or notoriety. For example, the 13th edition of Textbook of Surgery, edited by Sabiston, devotes only one paragraph to this type of herniorrhaphy. Because of the excellent results reported by the Shouldice Clinic, this technique was adopted at the Augusta Veterans Administration Hospital, a teaching hospital of the Medical College of Georgia. Since 1976, a total of 604 Shouldice repairs have been performed by supervised house staff; 468 patients have been followed for up to 8 years and a recurrence rate of 1.3 per cent is reported. Although the follow-up is brief, the Shouldice hernia repair is widely applicable and good results are not dependent on wide experience alone.     

Incidence of headache,nausea and vomiting following spinal anesthesia

A report of 500 cases of lumbar anesthesia is submitted with special reference to the incidence of postanesthetic headache, nausea, and vomiting in patients who are ambulatory within a short period after surgery.

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1. Postanesthetic headache occurred in 30.6 per cent of the cases; this is a much higher incidence than one finds in most published reports and may be explained upon the basis of the following considerations: cases reported in the literature are not selected cases, but those usually requiring seven to ten days of postoperative bed rest; the cases analyzed in the present study were largely those on whom anorectal operations had been performed, these patients requiring only a short period of recumbency.     

Marked hypodiploidy in blast phase chronic myelogenous leukemia: report of a case and review of the literature

The emergence of a near-haploid clone of cells in blast phase chronic myelogenous leukemia is an unusual event. We report such a case and review eight other cases described in the English literature. The significance of the substantial loss of genetic material is discussed as is the phenotypic and genotypic heterogeneity observed in this group of patients.     

FEASIBILITY OF CONDUCTING CARDIOVASCULAR OUTCOME RESEARCH IN AUSTRALIAN GENERAL PRACTICE: RESULTS FROM THE ANBP2 PILOT STUDY

1. The present study aimed to determine the feasibility of conducting a 5 year cardiovascular outcome trial of the treatment of 6000 elderly hypertensive patients in Australian general practices. 2. General practitioners (GPs) were invited to participate by mail and personal follow-up. Patient records were reviewed to identify subjects for a blood pressure (BP) screening programme. Blood pressure was measured on three occasions and eligible subjects were included if the average BP was 160 mmHg systolic or 90 mmHg diastolic if systolic BP was 140 mmHg. 3. Seven hundred and forty-one GPs were approached and 89 were enrolled in the study (12% of mail invites and 75% of those receiving a personal contact). In 16 practices where screening was completed, 82 000 records were reviewed to identify 4% patients eligible for screening. Twenty-two per cent of eligible subjects attended screening. Of 1938 subjects screened, 180 (9%) had BP 5=160/90 mmHg. Forty-seven percent of subjects (n = 916) were receiving antihypertensive therapy and 184 (20%) were withdrawn from therapy. One hundred and sixteen (63%) of these subjects had BP return to study entry levels within 6 weeks. Fifty-seven newly diagnosed and 81 previously treated subjects were randomized (7% of the screened population). 4. Based on the high participation rate of GPs, the response rate of patients to attend a BP screening programme and the 7% randomization to screening ratio for entry into the study, the ANBP2 pilot study has demonstrated that it is feasible to recruit subjects from Australian general practices to a cardiovascular outcome trial.     

Twenty cases of sympathetic ophthalmia.

We reviewed the charts of 20 patients with sympathetic ophthalmia who were seen in the uveitis clinic at the Eye and Ear Infirmary within an 11-year period. Of these 20 patients 14 maintained 20/50 or better visual acuity in at least one eye. We found early enucleation to be associated with a better visual prognosis, possibly due to earlier diagnosis and faster, more aggressive therapy rather than a reduction in antigenic load. The clinical appearance of Dalen-Fuchs nodules appears to indicate a more severe stage of disease. Chlorambucil was useful in patients with severe disease. To be effective and to lessen its side effects chlorambucil was given in daily dosages that were increased weekly over a short period to achieve bone marrow suppression. After a course of chlorambucil therapy intraocular inflammation could be controlled with topical steroids alone.