The clinical course, pathological features and response to treatment in a consecutive 16-years series of 19 cases of dermatofibrosarcoma protuberans were studied. A review of the world literature, comparing the results to ours too was performed. The tumour, occurring at every age and equally among females and males, most commonly involves the trunk without any particular pattern. The disease usually runs an indolent course and is too often regarded lightly by patients as well as physicians leading to delayed and often too conservative treatment. The tumour has definite invasive and metastatic potential and is considered a low grade soft tissue sarcoma. To avoid mutilating surgery (and maybe metastases) later in the course, the initial treatment must be wide surgical excision including a surrounding margin of at least 1 inch normal tissue. The removal of underlying deep fascia is essential and adequate resection will require a skin graft replacement in nearly every instance. Follow up periods should be no less than 3 years at the surgical and oncological service and should be continued at the general practitioners because tumours may recur after considerable periods of time, even in apparently adequately treated patients.

A multivariate regression analysis of survival data, using the Cox proportional hazards model (PHM), was performed on the retrospective material of 184 osteosarcoma patients treated at the Aarhus and Copenhagen oncology centers, Denmark, from 1963 to 1984. All patients were previously untreated. Radical surgery, in general ablative when possible, was the primary treatment goal throughout this period. A number of clinical and pathologic variables were tested in the model to elucidate their prognostic importance. Tumors localized to the trunk, pelvis, or femur, and symptom duration of less than 6 months were poor prognostic signs. Tumors dominated by fibroblastic cells and a patient age of approximately 25 to 30 years were associated with an especially good prognosis. The prognosis worsened with advancing age. Children, adolescents, and adults ages 5 to 25 years had significantly poorer prognosis than young adults 25 to 30 years of age. Sex, radiologic appearance, and year of referral had no significant prognostic value in this series. Based on the regression model, a prognostic index is derived and survival is calculated for a good and a poor prognostic case. The overall 10-year survival with one standard deviation was 28.6 +/- 3.5%. Cancer deaths continue to occur 10 years after initial treatment, and the estimated hazard rate is still four times greater than that of a sex- and age-matched group of healthy individuals.

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