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Paper 2
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
Fibrous dysplasia typically causes a ‘ground glass’ appearance within the bones rather than lysis. With myeloma there would usually be more diffuse skull and skeletal lytic lesions causing a ‘raindrop’ appearance when affecting the skull. Metastatic disease is possible, although again, more diffuse lesions may be expected; the distal long bones are also not typically affected initially. When there is malignant vertebral body marrow infiltration the uptake on nuclear medicine bone scan would usually be focal rather than the diffuse uptake described. Similarly, the peripheral uptake in the skull lesions is typical for osteoporosis circumscripta. Langerhans cell histiocytosis can cause lytic skull lesions but is not typical in this age group and lesions also tend to have bevelled, sclerotic margins.
Case 45
Published in Simon Lloyd, Manohar Bance, Jayesh Doshi, ENT Medicine and Surgery, 2018
Simon Lloyd, Manohar Bance, Jayesh Doshi
With the mixed hearing loss seen on the audiogram and the diffuse bilateral involvement of the calvarium seen on the CT scan, the most likely diagnosis is Paget's disease. This diagnosis can be strongly suspected from the CT appearance, with the washed out and fluffy appearance of the bone and with an apparent increase in calvarial bone volume. The CT scan may also show osteoporosis circumscripta, which are round, punched-out lesions, seen primarily in the frontal bones and occur in 10%–15% of patients. Other helpful tests are Technetium99 bone scans, which may show widespread skeletal involvement, and the biochemical markers alkaline phosphatase and urinary hydroxyproline, which are often elevated. Serum calcium is normal. Acid phosphatase may be elevated in about 20% of patients.
Metabolic and Hematological Bone Disease
Published in Harry Griffiths, Musculoskeletal Radiology, 2008
However, this is nearly always associated with blastic changes in the base of the skull and occasionally with platybasia and basilar invagination. Hence, osteoporosis circumscripta is fairly characteristic of early Paget’s disease. In the later osteoblastic stage, the metabolic madness has taken over and the calvarium becomes thickened with a mixed and disorganized blastic and lytic pattern. Usually the base of the skull is also involved in these patients, so that the differential diagnosis between Paget’s disease and metastatic disease from breast carcinoma, for example, is easy, since metastases will not usually involve the base of the skull (Fig. 73).
Paget’s disease of bone: an update on epidemiology, pathogenesis and pharmacotherapy
Published in Expert Opinion on Orphan Drugs, 2018
Luigi Gennari, Domenico Rendina, Tommaso Picchioni, Simone Bianciardi, Maria Materozzi, Ranuccio Nuti, Daniela Merlotti
The characteristic feature of the disease is an increased resorption followed by an increase in bone formation. Excessive bone breakdown and formation can cause the bone to weaken. As a result, bone pain, arthritis, noticeable deformities and fractures can occur. Generally the evolution of PDB follows three major phases. In the early phase, termed ‘osteolytic phase’ bone resorption predominates together with an increased vascularity of involved bones. In this phase the typical radiological picture is represented by an advancing lytic wedge or ‘blade of grass’ lesion in a long bone (i.e. femur or tibia) or by osteoporosis circumscripta, as seen in the skull. In the second phase, the excessive resorption of pagetic bone is followed closely by formation of new bone that however, is structurally abnormal, presumably because of the accelerated nature of the remodeling process. In fact, newly deposed collagen fibers are laid down in a disorganized rather than a linear fashion, creating the so called ‘woven bone’. Such a woven-pattern is not specific for PDB but it just reflects a high rate of bone turnover. With the time, the hypercellularity at the affected skeletal sites may diminish leading to development of a sclerotic, less vascular pagetic mosaic without evidence of active bone turnover. This is the so-called ‘sclerotic’ or ‘burned-out’ phase of PDB. All these three phases of the disease can be seen at the same time at different affected sites in a single pagetic patient.