Abstract
BACKGROUND AND PURPOSE: Angiosarcoma (AS), a rare and highly malignant tumour, can manifest spontaneously (primary AS, pAS) or secondary to previous radiation, exposure to chemical agents or Stewart-Treves syndrome (secondary AS, sAS). The aim of this study was to characterise the clinical presentation, management, treatment and outcome-including local recurrence, metastasis and overall survival-among patients diagnosed with AS and treated at Sahlgrenska University Hospital, Gothenburg, Sweden. STUDY METHODS AND DESIGN: This is a retrospective single-centre study analysing patients treated for AS at Sahlgrenska University Hospital. Patients were identified over a 21-year period (1 January 2000 to 31 December 2020) using Systematised Nomenclature of Medicine (SNOMED) code M91203. Medical records for all patients were reviewed by the authors. RESULTS: The study identified 64 patients with AS. The mean age among all patients was 67 (SD 16.6 range 18-96) years. The cohort comprised 37 patients diagnosed with pAS and 27 patients with sAS. Of the patients diagnosed with pAS, 18 (48.6%) were women while 25 (92.6%) women were diagnosed with sAS. Fifty-four (84%) patients were treated with surgery and 34 (63%) with adjuvant chemotherapy, radiation, or both. Local recurrence after primary surgery was 65.4% in pAS and 63% for sAS patients. Upon study completion, 15 (23%) patients were alive. Thirty-nine (60.9%) patients died of AS, while 10 (15.6%) died of other diseases or undetermined causes. Five-year overall survival rates were 10.2% and 43.5% in the pAS and sAS groups, respectively. CONCLUSION: pASs are observed in both men and women, presenting at different locations in the body. In contrast, the majority of sASs arise in the thoracic region in women previously treated for breast cancer. The study shows a 60% local recurrence rate across both pAS and sAS groups, yielding 5-year overall survival rates of 10.2% and 43.5% in the pAS and sAS groups, respectively.