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Age / Sex : 69 / M
Chief complaint: Increased skull uptake on bone scan, known rectal cancer
What is your impression?
Two weeks later, you can see the final diagnosis with a brief discussion of this case. (Please submit only one answer)
Courtesy: Yura Kim, Korea University Anam Hospital
Discussion
Findings:
X-ray) Combinations of the osteolytic lesions and osteosclerotic lesions in the skull.
Early phase: “osteoporosis circumscripta” in lytic phase of Paget disease.
Late phase: pronounced sclerosis and calvarial thickening. This has been referred to as the “Tam O’Shanter” sign, with reference to a Scottish hat named after the character in Robert Burn’s poem bearing the same name.
1. CT) Shows similar findings as the radiography. Osteolysis, trabecular coarsening, cortical thickening, and osseous expansion. In addition, CT commonly is helpful in the workup of suspected complications including fractures, spinal stenosis, and secondary neoplasms
Differential Diagnosis: Bony metastasis, Axial osteomalacia, Fibrogenesis imperfecta ossium
Diagnosis: Paget’s disease
Discussion: (=osteitis deformans)
Paget’s disease of the bone is a common, non-inflammatory, metabolic, skeletal disorder of unknown aetiology characterized by an increase in osteoclast-mediated bone resorption and compensatory excessive osteoblast activation. It is usually involves pelvis, spine, skull and proximal long bones and has characteristic radiographic features
Prevalence increases with age, and a pronounced geographical variation is well documented. The disease is often an incidental finding on a radiological examination requested for an unrelated indication. The osteolytic, mixed osteolytic/osteoblastic, and osteosclerotic phases may occur in the same patient and same bone in a synchronous or metachronous fashion. Radiological features in each phase mirror the histopathological appearances, and are distinctive enough to establish a diagnosis with confidence.
The symptoms depend on the bones involved, the most common clinical manifestations being pain at the affected bone and nearby joints. Most patients are entirely asymptomatic. The diagnosis is usually radiological, by means of plain radiography. It may be monostotic or polyostotic and is characterized by excessive bone resorption followed by formation of bone that is structurally abnormal.
References:
1. K. cortis et al. Imaging Paget’s disease of bone from head to toe, Clinical Radiology 66 (2011) 662-672
2. Stuart H. Ralston, Paget's Disease of Bone, N Engl J Med 2013; 368:644-650
3. Daphne J. Theodorou, Imaging of Paget Disease of Bone and Its Musculoskeletal Complications: Review, AJR 2011; 196:S64–S75
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