Abstract
Between 1944 to 2003, ninety cases of Paget’s sarcoma from the Scottish Bone and Soft Tissue Tumour Registry were reviewed. The mean age of patients was 72.3 years (range, 30 to 85 years). There were 59 males and 30 females. The most frequent sites were the femur (26), pelvis (19), humerus (13), tibia (11), and thoraco-lumbosacral spine (9). Biopsy was done in 69 cases. In the remainder 20 cases, the histological diagnosis was confirmed either from examining amputated limb or at autopsy. The most common type was osteosarcoma. Local excision was performed in seven cases. Resection in two cases and prosthetic replacement in two cases was carried out. Twenty nine patients underwent amputation surgery. Chemotherapy was administered to 15 patients (including 2 preop chemotherapy). Fifty one patients received radiotherapy (preoperatively in eight patients). All the patients died within one year from the date of biopsy with an average survival time of 7.5 months. Patients with Paget’s sarcoma tend to have a very poor prognosis inspite of improvements in therapy strategies including surgery, radiation therapy, and chemotherapy. We emphasise the need for more research by a combined oncosurgical, oncological, radiological and histological approach in the management of Paget sarcoma to improve the prognosis.
Correspondence should be addressed to BOOS at the Royal College of Surgeons, 35 - 43 Lincoln’s Inn Fields, London WC2A 3PN
