Paget disease of a single vertebra

Clinical Cases 06.10.2002
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Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 52 years, female
Authors: A. Drevelengas, G. Boulogianni, D. Chourmouzi, I. Sofroniadis
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Details
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AI Report

Clinical History

Intermittent low back pain of two years duration. No history of trauma or other chronic disease.

Imaging Findings

The patient was admitted because of intermittent low back pain of two years duration. No history of trauma or other chronic disease was noted.
On physical examination local tenderness over the lower thoracic spine was found. Neurological and laboratory examinations, the latter including alkaline phosphatase activity, were normal. Conventional radiography, whole body scintigraphy, CT scan, MRI of the thoracoclumbar spine, and bone biopsy were performed.
On conventional radiograph of the thoraco-lumbar spine (AP-view) (fig. 1), the Th 12 vertebra appears hyperdense and moderately enlarged. Bone scintigraphy of the thoraco-lumbar spine (AP-view) (fig. 2) shows highly increased uptake at the vertebral body of Th12.
CT scan at the level of Th12 (fig. 3) demonstrates focal radiodense areas ("cotton wool" aspect) with interspersed lucent areas mainly affecting the vertebral body.
MR of the thoraco-lumbar spine (fig. 4) included a sagittal T1-weighted image (A) showing low signal intensity of the Th12 vertebral body, a sagittal T2-weighted image (B) demonstrating a moderately decreased signal intensity of Th12, and a Gd-enhanced sagittal T1-weighted image (C) displaying a rim of peripheral enhancement ("picture frame") at the level of Th12. Enhancement of some vertical trabeculae within this vertebral body is also noted.
The radiological and clinical findings were diagnostic of Paget disease affecting a single vertebra. Bone biopsy confirmed this diagnosis.

Discussion

Monostotic Paget’s disease is uncommon (10 to 20%). The radiographic appearance during the course of the disease is explained by the underlying pathologic processes. The initial phase of increased osteoclastic activity resulting in bone resorption is not commonly seen radiographically. During the second phase a mosaic pattern is observed, which reflects the increased formation of abnormally coarsened trabeculae, following the first episod of increased bone resorption. The last phase, during which osteoclastic activity declines and osteoblastic activity proceeds, results in disorganized new bone formation of increased density, replacing the previous lytic areas. This phase represents the sclerotic phase. All three types of lesions may be found in the affected vertebra, which appears characteristically enlarged.
The MR appearance in Paget of the spine is variable. Focal or diffuse low signal intensity on short TR/TE and long TR/TE images are due to the presence of dense bone and fibrous tissue. However, in some patients different patterns are encountered, such as low signal intensity on short TR/TE and high signal intensity on long TR/TE images or high signal intensity on both T1 and T2 sequences. Vertebral enlargement is seldom seen in osteoporotic or malignant disease. Pathologic fractures are the commonest complication of Paget’s disease and may cause spinal nerve compression.
Secondary malignant degeneration to osteogenic sarcoma and to a lesser extent fibrosarcoma, chondrosarcoma, and malignant fibrous histiocytoma may occur.

Differential Diagnosis List

Paget disease

Final Diagnosis

Paget disease

Liscense

Figures

Conventional radiograph

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Conventional radiograph

Bone scintigraphy

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Bone scintigraphy

CT scan

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CT scan

MR of the thoraco-lumbar spine

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MR of the thoraco-lumbar spine
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MR of the thoraco-lumbar spine
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MR of the thoraco-lumbar spine