PAGETS SARCOMA OF THE SPINE: SCOTTISH BONE TUMOUR REGISTRY EXPERIENCE

Abstract

Between 1944 to 2003, eighty nine cases were registered with a diagnosis of Paget’s sarcoma in the Scottish Bone and Soft Tissue Tumour Registry. We found thirteen cases of sarcomatous degeneration of the spine (0.26% of the total bone tumour registry case) which were analysed in this study elaborating clinical, radiological and histopathological features. The mean age was 66.9 years (range 56 to 79 years). There were ten males and three females. There were seven cases involving sacral spine (63.6%), three cases involving lumbar vertebrae and two affecting dorsal spine. One case had diffuse dorso-lumbar involvement from D11 to L3 vertebrae. The mode of presentation was increasing low back pain (in all 13), unilateral sciatica (6, left sided-5, right sided-1), bilateral sciatica (2), lower limb weakness (8) and autonomic dysfunction (4, presented as chronic cauda equina syndrome). The majority of the cases (69.23%) were osteosarcomas. Out of these osteosarcomas, two showed giant cell rich matrix and one revealed predominant telengiectatic areas. Rest of the histological types was shared by chondrosarcoma, fibrosarcoma and malignant fibrous histiocytoma. Decompression laminectomy was performed in three cases. Eight patients had received radiotherapy. The mean survival was 3.93 months (range, 1 week to 7 months), nearly half to the whole Scottish Paget’s sarcoma series with a mean survival of 7.5 months. We found a constellation of symptomatology due to radiculo-medullary compression with a fatal evolution, predominantly lumbosacral involvement, predominantly osteosarcomatous histopathology with a poorest prognosis of all Paget’s sarcoma. Although, decompression laminectomy and adjuvant radiotherapy provided reasonable pain relief and palliation; however, there was no significant influence on the overall prognosis of the patients with Paget’s sarcoma of spine in the last six decades.