Weekly Case

Title : Case 611

Age / Sex : 43 / F


chief complaint: a bony lesion of L2, found incidentally on medical check-up

What is your impression?

Two weeks later, you can see the final diagonosis with a brief discussion of this case (Please submit only one answer).

Courtesy : So Young Park, Kyung Hee University Hospital at Gangdong



Discussion


Answer:  Paget's disease



Findings:


Radiographs of the lumbar spine show sclerotic change with cortical thickening in the body of L2.


Sagittal reformatted and axial reformatted CT images of the lumbar spine show mixed sclerotic and lytic changes involving vertebral body and posterior osseous element of L2.


MR imaging of the lumbar spine shows heterogeneous signal intensity changes involving body and posterior osseous element of L2 on T2- and T1- weighted images. Enhanced MR images show heterogeneous enhancement in the body and posterior osseous element of L2.


 


Differential Diagnosis: metastasis, lymphoma, hemangioma


 


Discussion:


 



  • A chronic skeletal disorder characterized by abnormal and excessive remodeling of bone

  • Common in United Kingdom, Australia, New Zealand, Western Europe, and the United States

  • Rare in Scandinavia, Asia, the Middle East, and Africa

  • Variable clinical manifestations : asymptomatic, painful, deforming, and various and debilitating musculoskeletal complications

  • Three major phases: the lytic phase (incipient active), the mixed phase (active), and the blastic phase (late inactive)

  • The anatomic distribution

    • usually asymmetric

    • most commonly affected sites: the lumbar spine, pelvis, sacrum, femur, and cranium

    • less commonly affected sites: the shoulder girdle (proximal humerus & scapula)

    • infrequently affected sites: ribs, fibula, and small bones in the hands and feet




  • Polyostotic disease (65-90%)

  • Monostotic Paget disease appears to predominate in the axial skeleton.


  • Radiography and CT

    • The lytic or incipient active phase: osteolysis

    • The mixed phase: osteolysis, coarsening and thickening of bone trabeculation, meshed disorganized trabecular (mosaic) pattern, cortical thickening, and osseous widening

    • The blastic or late inactive phase: osteosclerosis, coarsening of the trabeculae and cortical thickening with marked widening and enlargement of bones, transverse fatigue fractures ("banana fractures"), and multiple fractures and abnormal bone repair associated with the development of progressive bowing deformities in the long bones and pelvis; ivory vertebra, enlarged vertebral body with or without sclerosis, and enlargement of the posterior osseous elements in the spine




  • MR

    • Three major patterns of signal intensity : signal intensity similar to that of fat (the most common), heterogeneous, relatively low T1 signal intensity and high T2 signal intensity (the second most common), and low signal intensity on both T1- and T2-weighted images (the least common pattern)




  • Complications

    • Common complications: osseous deformities, fractures, osteoarthrosis, basilar impression, spinal stenosis, and neurologic abnormalities

    • Other less commonly encountered complications : bone tumor, soft tissue mass, osteomyelitis, extramedullary hematopoiesis, rheumatoid arthritis and its variants, and crystal deposition disease (1)




 


References:


 



  1. Theodorou DJ, Theodorou SJ, Kakitsubata Y. Imaging of paget disease of bone and its musculoskeletal complications: Review. AJR Am J Roentgenol 2011;196:S64-75



Correct Answer
Total applicants 35 Correct answers 17
Name Institution
윤유성 삼성서울병원, 전문의
손상욱 군의관, 전문의
김창현 전문의
한유비 공보의, 전문의
김보람 전문의
이혜란 전문의
최형인 국군의무학교, 전문의
박선영 한림대학교 성심병원, 전문의
김동수 전공의
안준형 공중보건의, 전공의
여현정 전공의
김형민 전문의
이소정 단국대학교병원, 전공의
박준동 뿌리병원, 전문의
이준영 전공의
장현경 분당차병원, 전공의
최수연 전문의


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