Paget's Disease of Bone: Histologic Analysis of 754 Patients

Abstract

Although Paget's disease of bone (PDB) is the second most common metabolic bone disease, to our knowledge, there is only one quantitative analysis on the histological and especially on the histomorphometric level. Therefore, the aim of this study was to analyze, on the basis of the Hamburg Bone Register, PBD in terms of incidence, skeletal distribution, malignant transformation, and histological and histomorphometric characteristics. Bone biopsies and patient files of 754 cases with histologically proven PDB were reviewed in a retrospective study. Quantitative static histomorphometry was performed on a representative subgroup of 247 biopsies derived from patients with manifestation of PDB at the iliac crest and compared with an age-and sex-matched control group. The peak incidence of PDB was between 70 and 80 yr of age. The majority of monostotic skeletal manifestation was localized at the os ilium, followed by the spine and femur. Histomorphometric results showed a high bone turnover with a significant increase in bone resorption and bone formation indices leading to an increased bone volume. Paget sarcoma was diagnosed in 6 of 754 patients, indicating a malignant transformation in 0.8% of the affected patients. Taken together, our study characterizes PDB in Germany on the basis of one of the largest cohorts of patients with histologically proven PDB. Moreover, for the first time, a quantitative histomorphometric approach was taken for >200 cases, where we could show local high bone mass lesions as a result of an increase of both osteoclast and osteoblast indices.

The fracture mechanics of human bone: influence of disease and treatment

“…70,71 Paget's disease affects localized skeletal sites, most often the pelvis, spine, femur or tibia. 70,71 The disease is most often diagnosed through X-rays, by the bone's strikingly high increase in bone volume due to high levels of bone turnover. 71 Individuals with Paget's disease commonly suffer from bone enlargement, bowing/deformity and pain.…”

“…70,71 The disease is most often diagnosed through X-rays, by the bone's strikingly high increase in bone volume due to high levels of bone turnover. 71 Individuals with Paget's disease commonly suffer from bone enlargement, bowing/deformity and pain. Incomplete fractures, The fracture mechanics of human bone EA Zimmermann et al termed pseudo or fissure fractures, can be found at sites with severe bowing and deformity.…”

Paget disease of the spine: an evaluation of 101 patients with a histomorphometric analysis of 29 cases

“…The spine has been reported to be the second most commonly affected site in Paget disease of bone (PDB), a chronic metabolically active bone disease [1][2][3][4][5][6][7]. Monostotic pagetic involvement of the spine is rarely described, and polyostotic cases are more common [8].…”

A case of mandible Paget’s disease of the bone treated with intravenous neridronate

“…In this particular case, it was probably performed to exclude the possibility of a chronic osteomyelitis. The typical histology of PDB lesions shows increased osteoclasts, which are often enlarged and multinucleated, and disorganised woven bone (2,3). In our case, the findings of the biopsy were consistent with the these features.…”

“…It is hypothesized that the primary abnormality might be an increased osteoclast activity. Osteoclasts prove to be enlarged in size and high in number (2). The newly formed bone is irregular, lacking the normal lamellar pattern and increased in volume, sclerotic and weaker than normal bone.…”