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Updated: 6/21/2021

Paget's Disease

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  • summary
    • Paget's Disease is an idiopathic condition of abnormal bone remodeling with normal bone being replaced through an active interplay between excessive bone resorption and abnormal new bone formation. The condition typically present in patients age > 40 with asymptomatic lesions discovered incidentally on radiographs.
    • Diagnosis is made with radiographs showing characteristic findings of lesions with diffuse blastic appearance and labs showing elevated serum ALP and elevated urinary collagen cross-links.
    • Treatment is observation for asymptomatic patients. Medical management with bisphosphonates is indicated in symptomatic patients. 
  • Epidemiology
    • Demographics
      • peak incidence in the 5th decade of life
      • common in Caucasians (northern European / Anglo-Saxon descent)
      • males = females
    • Anatomic location
      • may be monostotic or polyostotic
      • common sites include femur > pelvis > tibia > skull > spine
  • Etiology
    • Pathophysiology
      • increased osteoclastic bone resorption is the primary cellular abnormality
      • cause is thought to be a slow virus infection (intra-nuclear nucleocapsid-like structure)
        • paramyxovirus
        • respiratory syncytial virus
    • Genetics
      • inheritance
        • most cases are spontaneous
        • hereditary
          • familial clusters have been described with ~40% autosomal dominant transmission
      • genetics
        • most important is 5q35 QTER (ubiquitine binding protein sequestosome 1) SQSTM1 (p62/Sequestosome)
          • tend to have severe Paget disease
        • also insertion mutation in TNFRSF11A for gene encoding RANK
    • Orthopaedic manifestations
      • bone pain
      • long bone bowing
      • fractures, due to brittle bone and tend to be transverse
      • large joint osteoarthritis
        • excessive bleeding during THA
        • malalignment during TKA
      • secondary sarcoma
    • Associated conditions
      • high output heart failure
  • Classification
    • Phases
      • lytic phase
        • intense osteoclastic resorption
      • mixed phase
        • resorption and compensatory bone formation
      • sclerotic phase
        • osteoblastic bone formation predominates
      • all three phases may co-exist in the same bone
  • Presentation
    • Symptoms
      • asymptomatic
        • frequently asymptomatic and found incidentally
      • pain
        • pain may be the presenting symptom due to
          • stress fractures
          • increased vascularity and warmth
        • new intense pain and swelling
          • suspicious for Paget's sarcoma in a patient with history of Paget's + new intense pain and swelling
      • cardiac symptoms
        • can present with high-output cardiac failure particularly if large/multiple lesions & pre-existing diminished cardiac function
  • Imaging
    • Radiographs
      • coarsened trabeculae which give the bone a blastic appearance
        • both increased and decreased density may exist depending on phase of disease
          • lytic phase
            • lucent areas with expansion and thinned, intact cortices
            • 'blade of grass' or 'flame-shaped' lucent advancing edge
          • mixed phase
            • combination of lysis + sclerosis with coarsened trabeculae
          • sclerotic phase
            • bone enlargement with cortical thickening, sclerotic and lucent areas
      • remodeled cortices
        • loss of distinction between cortices and medullary cavity
      • long bone bowing
        • bowing of femur or tibia
      • fractures
      • hip and knee osteoarthritis
      • osteitis circumscripta
        • (cotton wool exudates) in skull
      • Paget's secondary sarcoma
        • shows cortical bone destruction
        • soft tissue mass
    • MRI
      • may show lumbar spinal stenosis
    • Bone scan
      • accurately marks site of disease
      • intensely hot in lytic and mixed phase
      • less hot in sclerotic phase
    • CT scan
      • cortical thickening and coarsened trabeculae
  • Evaluation
    • Laboratory findings
      • elevated serum ALP
      • elevated urinary collagen cross-links
      • elevated urinary hydroxyproline (collagen breakdown marker)
      • increased urinary N-telopeptide, alpha-C-telopeptide, and deoxypyridinoline
      • normal calcium levels
  • Histology
    • Characteristic histology
      • woven bone and irregular broad trabeculae with disorganized cement lines in a mosaic pattern
      • profound bone resorption - numerous large osteoclasts with multiple nuclei per cell
        • virus-like inclusion bodies in osteoclasts
        • Paget's osteoclasts larger, more nuclei than typical osteoclasts
      • fibrous vascular tissue interspersed between trabeculae
  • Differential
      • Differential of Paget's Disease
      • Benign lesion in older patient (40-80)
      • Multiple lesions in the older patient
      • Treatment is Observation only
      • (assuming no imp. path. fx.)
      • Benefits from Bisphosphonate therapy
      • Paget's disease
      • o
      • o
      • o
      • o
      • Enchondroma
      • o
      • o
      • Bone island
      • o
      • Bone infarct
      • o
      • o
      • Hyperparathyroidism
      • o
      • o
      • Metastatic bone disease
      • o
      • o
      • Myeloma
      • o
      • o
      • Lymphoma
      • o
      • NOF
      • o
      • Osteochondroma
      • o
      • Eosinophillic granuloma
      • o
      • Fibrous Dysplasia
      • o
  • Treatment
    • Nonoperative
      • observation and supportive therapy
        • treatment for asymptomatic Paget's disease
          • physiotherapy, NSAIDS, oral analgesics
      • medical therapy aimed at osteoclast inhibition
        • bisphosphonates are 1st line treatment for symptomatic Pagets
          • oral
            • alendronate and risedronate
            • etidronate disodium (Didronel)
              • older generation medication
              • inhibits osteoclasts and osteoblasts
              • cannot be used for more than 6 months at a time
          • intravenous
            • pamidronate, zoledronic acid (Zometa)
              • newer generation medications that only inhibit osteoclasts
              • disadvantageous in that they only come in IV form
        • calcitonin are 2nd line (after bisphosphonates)
          • causes osteoclasts to shrink in size and decreases their bone resorptive activity within minutes
          • administered subcutaneously or intramuscularly
        • teriparatide is contraindicated in Paget's disease due to risk of secondary osteosarcoma
    • Operative
      • THA / TKA
        • indications
          • affected patients with degenerative joint disease
        • technique
          • treat Paget's with pharmacologic agents prior to arthroplasty to reduce bleeding
        • outcomes
          • greater incidence of suboptimal alignment secondary to pagetoid bone
          • the most common complications include
            • malalignment with knee arthroplasty
            • bleeding with hip arthroplasty
      • metaphyseal osteotomy and plate fixation
        • indications
          • fractures through pathologic bowing of long bones
          • impending pathologic fracture of long bone with bowing
  • Prognosis
    • Paget's sarcoma
      • less than 1% will develop malignant Paget's sarcoma (secondary sarcoma)
      • osteosarcoma > fibrosarcoma and chondrosarcoma
      • most common in pelvis, femur, and humerus
      • poor prognosis
        • 5-year survival for metastatic Paget's sarcoma < 10%
        • treatment includes chemotherapy and wide surgical resection

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Flashcards (8)
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Questions (24)
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(OBQ12.81) A 78-year-old female presents with mild right hip pain following a twisting injury. She denies fever, weight-loss, and night sweats. She has a positive family history for breast cancer. Laboratory studies show a WBC of 8.9 k/uL (range 4-11,000) and an ESR of 12 mm/hr (range 0-22). A radiograph is obtained and shown in Figure A. A CT scan of the chest, abdomen, and pelvis is performed and shows no evidence of metastatic lesions. A biopsy is performed and shown in Figures B and C. What is the most appropriate next step in treatment?

QID: 4441
FIGURES:

Wide resection with perioperative chemotherapy

15%

(816/5543)

Wide resection with perioperative radiation

9%

(481/5543)

Wide resection alone

9%

(482/5543)

Radiation alone

5%

(273/5543)

Observation with evaluation for bisphonate therapy

62%

(3443/5543)

L 3 B

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(SBQ11PA.39) A 68-year-old male presents with a history of chronic leg pain and progressive varus deformity of his left leg. A clinical image is seen in Figure A. Radiographs are seen in Figure B. He should be prescribed a medication with which of the following mechanisms of action?

QID: 4064
FIGURES:

Inhibition of TNF-a

5%

(124/2428)

Inhibition of IL-1 receptor

3%

(81/2428)

Estrogen receptor blockade

2%

(46/2428)

Osteoclast inhibition

88%

(2126/2428)

COX inhibition

1%

(36/2428)

L 1 B

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(SBQ11PA.83) A 67-year-old male patient is scheduled for left total hip arthroplasty. A pre-operative examination reveals elevated serum alkaline phosphatase and urine hydroxyproline. A radiograph of his hip/pelvis is seen in Figure A. Taking into context the clinical and radiographic presentation, what would you expect to be the most common complication with this procedure?

QID: 4108
FIGURES:

Implant malalignment

7%

(346/4622)

Increased blood loss

59%

(2728/4622)

Peri-prosthetic fracture

30%

(1367/4622)

Sciatic nerve palsy

2%

(82/4622)

Deep vein thrombosis

1%

(68/4622)

L 4 C

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(OBQ11.194) A 55-year-old male presents with left hip pain and stiffness. Radiographs are shown in Figures A and B. A biopsy of the left femur is performed and shown in Figure C. Which of the following medications is contraindicated in this patient?

QID: 3617
FIGURES:

Teriparatide

67%

(1596/2392)

Alendronate

12%

(278/2392)

Ergocalciferol

4%

(92/2392)

Zoledronic acid

3%

(69/2392)

Calcitonin

14%

(344/2392)

L 3 C

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(OBQ11.11) Paget's disease may predispose a patient to the development of which of the following malignant neoplasms?

QID: 3434

Chondrosarcoma

4%

(123/3319)

Osteosarcoma

52%

(1737/3319)

Spindle cell sarcoma of bone

3%

(89/3319)

All of the above

37%

(1218/3319)

None of the above

4%

(143/3319)

L 5 C

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(OBQ10.78) Which of the following conditions can be characterized by excessive bone resorption and disordered bone formation as characterized by the radiograph in Figure A?

QID: 3166
FIGURES:

Osteopetrosis

4%

(95/2214)

Paget's disease of bone

91%

(2010/2214)

Osteogenesis imperfecta

3%

(57/2214)

Rickets

2%

(37/2214)

Legg-Calve-Perthes disease

1%

(13/2214)

L 1 C

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(OBQ10.104) The disease shown in the radiograph and biopsy specimen, Figures A and B, is best characterized by which of the following laboratory findings

QID: 3198
FIGURES:

Decreased urinary N-telopeptide and increased urinary alpha-C-telopeptide

4%

(120/2960)

Low serum vitamin D levels

9%

(271/2960)

Increased urinary N-telopeptide and decreased urinary alpha-C-telopeptide

18%

(537/2960)

Increased urinary N-telopeptide and alpha-C-telopeptide

60%

(1763/2960)

Increased urinary N-telopeptide and decreased urinary excretion of pyridinium crosslinks

8%

(244/2960)

L 3 C

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(OBQ09.59) A 55-year-old male has a several month history of hip pain. He is diagnosed with Paget's disease based on his radiographs and positive bone scan indicating a lytic phase. What cell demonstrated by the red arrow in the Figures below is most commonly associated with the etiology Paget's disease?

QID: 2872
FIGURES:

Figure A

2%

(45/2687)

Figure B

15%

(398/2687)

Figure C

72%

(1927/2687)

Figure D

5%

(138/2687)

Figure E

6%

(160/2687)

L 2 C

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(OBQ08.129) A 65-year-old man complains of deformity of the right leg. He denies pain at rest but does complain of ankle and lower leg pain when walking more than a half mile. A radiograph is shown in Figure A. Following 6 months of orthotic and brace treatment, he continues to struggle with pain. Which of the following is the best treatment option?

QID: 515
FIGURES:

Continued orthotic and brace treatment

7%

(79/1105)

Corrective osteotomy and plate fixation

50%

(558/1105)

Intramedullary fixation

21%

(236/1105)

Amputation

15%

(165/1105)

Corrective osteotomy, knee arthrodesis, and plate fixation

5%

(58/1105)

L 5 D

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(OBQ06.137) All of the following statements regarding Paget's sarcoma are correct EXCEPT?

QID: 323

The 5-year survival for metastatic Paget's sarcoma is less than 10%

8%

(143/1695)

Less than 1% of patients with Paget's disease develop secondary Paget's sarcoma

7%

(113/1695)

While osteosarcoma is the most common histologic sub-type of Paget's sarcoma, fibrosarcoma and chondrosarcoma sub-types also occur

5%

(83/1695)

Paget's sarcoma typically occurs in patients over 50 years of age

2%

(36/1695)

Treatment of Paget's sarcoma is via surgery alone

77%

(1313/1695)

L 2 D

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(OBQ05.111) A 62-year-old female sustained the injury seen in Figure A after a fall at home. Her past medical history is significant for mild hypertension only. She is not currently taking any medications. Her pre-operative assessment revealed elevated serum alkaline phosphatase. Serum calcium and phosphate were normal. What is the most likely underlying diagnosis?

QID: 997
FIGURES:

Paget's bone disease

86%

(2602/3009)

Osteoporosis

9%

(269/3009)

Osteomyelitis

0%

(9/3009)

Renal osteomalacia

4%

(109/3009)

Aneurysmal bone cyst

0%

(9/3009)

L 1 D

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(SBQ05PA.19) A 70-year-old man presents with intermittent right lower extremity pain over several years. He denies any history of trauma. Physical examination reveals mild tenderness over the proximal third of right tibia with slight tibial bowing. A routine blood test shows an elevated serum alkaline phosphatase. Radiographs of the right tibia are shown in Figures A and B. A total body bone scan is shown in Figure C. What malignant transformation is most commonly associated with this patients underlying condition?

QID: 2004
FIGURES:

Osteosarcoma

77%

(2583/3367)

Fibrosarcoma

9%

(295/3367)

Multiple myeloma

12%

(404/3367)

Giant cell tumor

1%

(39/3367)

Osteoid Osteoma

1%

(30/3367)

L 3 C

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(SBQ05PA.85) A 52-year-old male farmer presents with right hip pain for the past 4 months. On physical examination there is pain with internal rotation of his right hip. Laboratory studies show elevated serum alkaline phosphatase. Serum calcium is normal. Urinary studies show elevated urinary N-telopeptide, alpha-C-telopeptide, and deoxypyridinoline markers. A radiograph of the pelvis is shown in Figures A. What would be the most appropriate treatment for this patient?

QID: 2070
FIGURES:

Antibiotics

1%

(24/4158)

Bisphosphonates

83%

(3435/4158)

Monoclonal-B antibodies

5%

(191/4158)

Chemotherapy

8%

(333/4158)

Wide resection and radiotherapy

4%

(148/4158)

L 2 C

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(SBQ05PA.54) Which of the following would be the most appropriate treatment for a patient with asymptomatic Paget's disease?

QID: 2039

Supportive therapy

48%

(1678/3501)

Calcitonin

4%

(149/3501)

Vitamin D and Calcium

6%

(196/3501)

Bisphosphonates

41%

(1432/3501)

Monoclonal-B antibody

1%

(26/3501)

L 4 D

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(SBQ05PA.100) A 78 year-old male has been having progressive pain in his left thigh and hip when walking. He is otherwise healthy and takes no medications. Laboratory evaluation reveals normal ESR and CRP, but elevated urinary hydroxyproline and increased urinary N- and alpha-C-telopeptides. Which of the following radiographs is most consistent with his clinical picture?

QID: 2085
FIGURES:

Figure A

2%

(22/956)

Figure B

19%

(178/956)

Figure C

8%

(72/956)

Figure D

4%

(38/956)

Figure E

67%

(639/956)

L 3 C

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(OBQ04.169) A 65-year-old male presents with increasing shoulder pain over the past 9 months. He is otherwise healthy and has no other complaints. Radiograph of his shoulder is shown in Figure A. Whole body bone scan and biopsy photograph are shown in Figures B and C. What is the most appropriate treatment for this patient?

QID: 1274
FIGURES:

Referral to endocrinology

19%

(387/2036)

Radiation therapy and chemotherapy

28%

(570/2036)

Wide resection and reconstruction

8%

(169/2036)

Radiation therapy, wide resection, and reconstruction

8%

(170/2036)

Chemotherapy, wide resection, and reconstruction

36%

(729/2036)

L 4 D

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