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

Abstract

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.

Paget’s sarcoma of the patella

“…PDB, documented in 1877 [34], is characterized by disordered architecture predominating in the lumbar spine, pelvis, skull, femur, and tibia [3]. It is usually asymptomatic with few bones affected and multifocal generalized forms are well recognized but rare.…”

Antibody Response to Paramyxoviruses in Paget’s Disease of Bone

“…Many patients are asymptomatic but those that do come to clinical attention can develop various complications including bone pain, deformity, pathological fractures, osteoarthritis, deafness and rarely osteosarcoma [2, 3]. Paget’s disease is a complex disorder.…”

“…Paget’s disease of bone is a common skeletal disorder characterised by focal abnormalities of increased and disorganised bone turnover at one or more skeletal sites [ 1 ]. Many patients are asymptomatic but those that do come to clinical attention can develop various complications including bone pain, deformity, pathological fractures, osteoarthritis, deafness and rarely osteosarcoma [ 2 , 3 ]. Paget’s disease is a complex disorder.…”