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.     

Teaching psychiatric ethics

In the last decade, we have witnessed a burgeoning of interest in ethical issues amongst psychiatrists. Teaching of the subject, however, remains at a rudimentary stage. Various approaches to such instruction are available, particularly modelling (students observe their experienced counterpart), the case method (examining specific clinical situations which involve a need for ethical decision-making), and the seminar approach (trainees are exposed to a core body of knowledge, mainly theoretical in nature). Faced with these different teaching models, the University of Oxford Department of Psychiatry has opted for a blend of all three approaches, which incorporates two goals: an increase in the trainees' sensitivity to the many intricate moral dilemmas facing the psychiatric profession; and their familiarity with salient concepts in moral philosophy which constitute a basis for ethical reasoning and which have a bearing on clinical practice. The teaching programme comprises the following: a pair of trainees prepares a presentation on an aspect of psychiatric ethics under the supervision of a senior psychiatrist. A moral philosopher assumes the role of discussant of the ethical problems raised by the trainees; this is followed by a general discussion. Topics have included involuntary hospitalization, dual loyalty, suicide, psychiatric diagnosis, and ethical issues in various spheres of psychiatric practice such as sex therapy, psychotherapy and child psychiatry. The approach has worked effectively and proved rewarding to all participants involved.     

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.     

Comparative toxicity of total lymphoid irradiation and immunosuppressive drug treated patients with intractable rheumatoid arthritis

Outcomes were compared between consecutive patients who had received either total lymphoid irradiation (TLI) or immunosuppressant treatment for intractable rheumatoid arthritis (RA). There were 33 TLI and 32 immunosuppressive recipients; all patients had failed standard therapy. Average followup from the start of therapy was 2.7 years for TLI and 5.9 years for immunosuppressive recipients. Final disability levels were the same in both groups; mortality was equal in both groups as well. There were more hospitalizations for infections in the TLI group and the infecting organisms tended to be staphylococcus or gram negative organisms. Apart from infections, there were more adverse effects reported in the immunosuppressive therapy group.     

High risk factors for rehospitalization within six months

Objective. 1. To identify risk factors associated with psychiatric rehospitalization within six months, using global clinical assessments and demographic information and; 2. To determine if risk factors for a hospital in a rural region are similar to those reported for urban hospitals. Method. The setting was a psychiatric unit within a general hospital. All adult admissions for one year were assigned scores on the North Carolina Functional Assessment Scale (NCFAS) and the Global Assessment of Functioning (GAF) scale. Patients were interviewed six months after discharge to determine if they had been rehospitalized and to assign new NCFAS and GAF scores. Results. Significant risk of rehospitalization was predicted by: 1. NCFAS score >90; 2. history of prior hospitalization; 3. nursing home residence; 4. referral from a small community hospital and; 5. non-compliance with outpatient appointments. Conclusions. Global assessments and demographic information collected during an index admission can generate factors to identify patients at risk for rehospitalization within six months. History of prior admissions and non-compliance with outpatient treatment, reported as risk factors in urban settings, were found also to be risk factors in a rural region.He was formerly Assistant Professor, Center for Health Sciences Statistics, East Carolina University School of Medicine.