Osteoblastic osteosarcoma in monostotic Paget’s disease

Abstract

Paget's disease is a disorder of bone remodelling affecting 1-2% of the general population, most frequently men over 50 years of age. Sarcomatous degeneration occurs in 0.1-0.95% of patients with Paget's disease. It can be observed in monostotic and polyostotic disease. Osteosarcomas account for more than 80% of pagetic sarcomas; osteoblastic pagetic osteosarcomas are rare. In this article, we present the case of a 75-year-old man with secondary osteosarcoma arising in monostotic Paget's disease, initially misdiagnosed as rotator cuff tendinopathy. Imaging studies and biopsy were diagnostic.

Premalignant Conditions of Bone

“… 49 Paget osteosarcoma, often referred to as Paget sarcoma, is a devastating complication of Paget disease of bone with poor outcomes. 50 51 52 53 54 Although osteosarcoma is the most common subtype of Paget-associated tumor, chondrosarcoma and fibrosarcoma are also documented. 55 This malignant transformation thankfully occurs in only approximately 1% to 3% of cases.…”