Scapulo-humeral Paget's sarcoma: Scottish Bone Tumour Registry experience.

This study aimed to analyse the clinical, radiological and histological features of 16 scapulo-humeral Paget's sarcoma cases accrued from Scottish Bone Tumour Registry between January 1950 and December 2000. The mean age was 61.5 (range, 44-77) years with 12 men and 4 women. There were three scapular cases and 13 humeral (1 whole length, 5 upper humeral, 5 lower humeral and 2 mid-humeral). These patients presented with progressively increasing pain in the shoulder, arm or elbow (n = 5), a painful mass associated with a pathological fracture (n = 4), a pathological fracture with progressively worsening pain (n = 3), a painful mass (n = 3) and a painless mass associated with wrist drop (n = 1). Histology showed predominantly osteosarcoma (n = 12), followed by pleomorphic sarcoma (n = 2), malignant fibrous histiocytoma (n = 1) and fibrosarcoma (n = 1). Overall, the median survival period was 4.5 months. In summary, Paget's sarcoma of the scapulo-humeral area presents with progressively worsening pain, a painful mass with or without a pathological fracture, predominantly lytic in humeral and purely sclerotic in scapular lesions, osteosarcoma-dominant histology, primarily treated with an early limb ablative surgery and associated with a poor 1-year survival (12.5%) and 5-year survival (6.2%) rate.