Clinical History:
- A 65-year-old caucasian man has noted increasing low back pain, and left proximal leg pain, particularly after sitting for extended periods of time, worsening over the past 5 years. Laboratory studies show Hgb 13.7 g/dL, Hct 42.2%, serum calcium 8.7 mg/dL, phosphorus 3.4 mg/dL, alkaline phosphatase 177 U/L, albumin 4.4 g/dL, and total protein 6.4 g/dL. A CT scan of the pelvis is performed. A bone biopsy is performed.
- What is the diagnosis?
This is Paget disease of bone. The radiograph shows coarsened bony trabeculae of the left intertrochanteric region. There is also prominent sclerosis of the left sacroiliac joint. The CT scan of the left proximal femur shows trabecular and cortical thickening without cortical break through or a soft tissue mass. The microscopic mosaic pattern of the bony trabeculae is diagnostic.
- In whom and at what sites does this lesion occur?
Males are affected more often than females. Paget disease
of bone tends to occur in older caucasian persons after the age of 50, and
approaches a 10% incidence in the very old, but it is asymptomatic in up to
90% of cases. If it involves a single site, it is "monostotic". If several
sites are involved, it is "polyostotic". Pelvis, femur, and skull are often
affected. The classic history is given by the man who says his hat size is
increasing.
- What is the clinical course of this disease?
Early, there is osteolysis. This is followed by the classic
osteoblastic-osteolytic phase (as in this case) with bone deformity.
Finally, there is a sclerotic phase. There is a 1% risk for development of a
sarcoma. In fact, this man also had a sarcoma of the right ilium (the most
common site for a Paget sarcoma).
|
|
