Paget's Disease

Author: Deborah Allen

Topic updated on 05/25/13 8:10am

Introduction

A condition of abnormal bone remodeling
- original osseous tissue is reconstructed through active interplay between excessive bone resorption and abnormal new bone formation
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
Epidemiology
- peak incidence in the 5th decade of life
- common in Caucasians (northern European descent)
- equally common in males and females
- location
  - may be monostotic or polyostotic
  - common sites include
    - femur
    - pelvis
    - tibia
    - skull
    - spine
Genetics
- ~40% are autosomal dominant
- 4 genes associated with Paget's disease
  - SQSTM1 (also known as p62) on chromosome 5
  - RANK
  - OPG
  - VCP (also known as p97)
Orthopaedic manifestations
- long bone bowing
- fractures
- large joint osteoarthritis
Prognosis & malignancy
- Paget's sacroma
  - less than 1% will develop malignant Paget's sarcoma (secondary sarcoma)
  - osteosarcoma is the most common, followed by fibrosarcoma and chondrosarcoma
  - most common in pelvis, femur, and humerus
  - Paget's sarcoma has a poor prognosis
    - 5-year survival for non-metastatic Paget's sarcoma is less than 5%
    - appropriate treatment for Paget's sarcoma includes chemotherapy and wide surgical resection

Classification

Phases
- active
  - lytic phase
    - intense osteoclastic resorption
  - mixed phase
    - resorption and compensatory bone formation
  - sclerotic phase
    - osteoblastic bone formation predominates
- inactive

Presentation

Symptoms
- asymptomatic
  - frequently asymptomatic and found incidentally
- pain
  - pain may be the presenting symptom due to
    - stress fractures
    - increased vascularity and warmth
  - new onset intense pain and swelling
    - be suspicious for Paget's secondary sarcoma in a patient with a known history of Paget's who complains of new onset intense pain and swelling
- cardiac symptoms
  - can present with high-output cardiac failure

Imaging

Radiographs
- coarsened trabeculae which give the bone a blastic appearance
  - both increased and decreased osteodensity 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 and 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
  - commonly femoral neck
- 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 alkaline phosphatase
- 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
- fibrous vascular tissue interspersed between trabeculae

Treatment

Nonoperative
- medical therapy aimed at osteoclast inhibition
  - bisphosphonates
    - oral agents
      - 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 agents
      - pamidronate, zoledronic acid (Zometa)
        
        newer generation medications that only inhibit osteoclasts
        
        disadvantageous in that they only come in IV form
  - calcitonin
    - 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
- hip and knee arthroplasty
  - indications
    - affected patients with degenerative joint disease
  - technique
    - treat Paget's with pharmacologic agents prior to arthroplasty to reduce excessive bleeding
  - outcomes
    - greater incidence of suboptimal alignment secondary to pagetoid bone
- metaphyseal osteotomy and plate fixation
  - indications
    - fractures through pathologic bowing of long bones
    - impending pathologic fracture of long bone with bowing deformity

Differentials & Groups

	Benign lesion in older patient (40-80)	Multiple lesions in the older patient	Treated is Observation only(assuming no imp. path. fx.)	Benefits from Bisphosphonate therapy
Paget's Disease	•	•	•	•
Enchondroma	•		•
Bone island	•
Bone infarct	•	•
Hyperparathyroidism	•	•
Metastatic bone disease		•
Myeloma		•		•
Lymphoma		•
NOF			•
Osteochondroma			•
Eosinophillic granuloma			•
Fibrous dysplasia				•
Metastatic Disease				•

IBank

	Location	Age	Xray	Xray	CT	B. Scan	MRI	MRI	Histo(1)
Case A	fibula	10 yrs.

(1) - histology does not always correspond to clinical case

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Qbank (8 Questions)

TAG

(SBQ11.39) 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? Topic

Review Topic

FIGURES: A

1. Inhibition of TNF-a
2. Inhibition of IL-1 receptor
3. Estrogen receptor blockade
4. Osteoclast inhibition
5. COX inhibition

PREFERRED RESPONSE ▶

DISCUSSION: The above clinical scenario is most consistent with Paget's disease. The clinical photo shows a bowed leg and mixed lucent/sclerotic deformity pattern on radiograph. Paget's disease is often asymptomatic, but presenting symptoms can include chronic pain and deformity, similar to the above clinical scenario. Once Paget's disease is diagnosed, patients are often treated with bisphosphonates during the active disease process to help control osteoclast lytic activity and reduce pain.

Tieg provides a review of Paget's disease of bone and treatment indications. In his review, he discusses that most patients with Paget's disease are asymptomatic, but in those that do present, pain is the most common presenting symptom. He recommends medical and surgical treatment of asymptomatic patients who have active disease at sites where complications are likely to develop.

Altman did a review of 290 patients with Paget's disease of bone. His findings showed 83% had one or more rheumatic syndromes. He found the rate of osteoarthritis related to Paget's disease was elevated in the hip and knee. Rheumatoid arthritis, hyperuricemia, and gout did not appear increased in this group.

Mangham et al looked the rate of secondary sarcomas as a result of Paget's disease of bone. They found the rate to be around 0.3%, with male predominance. They also found that widespread skeletal involvement by Paget's disease was not a significant risk factor for malignant transformation.

Incorrect answers:
Answer 1: TNF-a inhibition is used to treat rheumatoid arthritis (Infliximab, Etanercept).
Answer 2: IL-1 receptor inhibition is used to treat rheumatoid arthritis (Anakinra).
Answer 3: Estrogen receptor blockade is used to treat certain types of breast cancer and ovarian cancer (Tamoxifen).
Answer 5: COX inhibition is used to treat inflammation (NSAIDs).

REFERENCES:

1. Tiegs RD. Paget's disease of bone: indications for treatment and goals of therapy. Clin Ther. 1997 Nov-Dec;19(6):1309-29; discussion 1523-4. PMID:9444442 (Link to Abstract)

2. Altman RD. Musculoskeletal manifestations of Paget's disease of bone. Arthritis Rheum. 1980 Oct;23(10):1121-7. PMID:6448603 (Link to Abstract)

3. Mangham DC, Davie MW, Grimer RJ. Sarcoma arising in Paget's disease of bone: declining incidence and increasing age at presentation. Bone. 2009 Mar;44(3):431-6. PMID:19064007 (Link to Abstract)

Question Authors:

David Abbasi MD, Ben Taylor MD, Michael Hughes MD,

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TAG

(SBQ05.100) 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 evaulation 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? Topic

Review Topic

FIGURES: A

1. Figure A
2. Figure B
3. Figure C
4. Figure D
5. Figure E

PREFERRED RESPONSE ▶

DISCUSSION: Elevated N-telopeptides, alpha-C-telopeptides, and urinary hydroxyproline levels are all consistent with a diagnosis of Paget's disease. Radiograph E most closely represents this pathologic condition.

Paget's disease is a disease marked by abnormal decoupling of bone resorption and bone remodeling. The classic radiographic findings include coarsened trabelculae, bowing of long bones, and remodeling of cortices which blurs the distinction between medullary and cortical bone. Paget's disease can progress to malignancy (most often osteosarcoma) in approximately 1% of patients.

Figure E shows a left hip radiograph with bone enlargement, cortical thickening, and mixed sclerotic and lucent areas.

Incorrect Answers:
Answer 1: This is the typical appearance of a bone infarct.
Answer 2: This is the typical appearance of multiple myeloma.
Answer 3: This is the typical appearance of Melorheostosis, a benign disorder characterize by new periosteal bone formation.
Answer 4: The radiograph shows new periosteal bone formation consistent with a stress fracture.

REFERENCES:

1. Menendez LR (ed): Orthopaedic Knowledge Update: Musculoskeletal Tumors. Rosemont, IL, American Academy of Orthopaedic Surgeons, 2002, pp 211-215.

2. Resnick D (ed): Diagnosis of Bone and Joint Disorders. Philadelphia, PA, WB Saunders, 2002, pp 1947-2000.

Question Authors:

Anay Patel MD, Jan Szatkowski MD, Ben Taylor MD,

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TAG

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

Review Topic

1. Chondrosarcoma
2. Osteosarcoma
3. Spindle cell sarcoma of bone
4. All of the above
5. None of the above

PREFERRED RESPONSE ▶

DISCUSSION: Patients with Paget's disease are predisposed to secondary osteosarcoma, chondrosarcoma, and spindle cell sarcoma of bone (e.g. fibrosarcoma) all which can occur through Pagetoid lesions.

Huvos describes their clinical analysis of 117 patient older than 60 years of age with osteoarcoma and found that this population developed sarcomas more frequently related to other preexisting bone conditions, such as Paget's disease or following irradiation. Five-year survival for this patient cohort was related to the development of primary (37%) vs. secondary osteosarcoma associated with other skeletal conditions (7.5%).

REFERENCES:

1. Campanacci M. High grade osteosarcomas. In: Campanacci M, Enneking WF, eds. Bone and Soft Tissue Tumors: Clinical Features, Imaging, Pathology and Treatment. 2nd ed. New York, NY: Springer-Verlag; 1999:463-515.

2. Huvos AG. Osteogenic sarcoma of bones and soft tissues in older persons. A clinicopathologic analysis of 117 patients older than 60 years. Cancer. 1986 Apr 1;57(7):1442-9. PMID:2418941 (Link to Abstract)

Question Authors:

Patrick O'Donnell MD/PhD, Ben Taylor MD, John Badylak MD,

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TAG

(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 in contraindicated in this patient? Topic

Review Topic

FIGURES: A

1. Teriparatide
2. Alendronate
3. Ergocalciferol
4. Zoledronic acid
5. Calcitonin

PREFERRED RESPONSE ▶

DISCUSSION: The radiographs and histology are consistent with Paget disease. Figure A shows the skull with areas of lysis, areas of sclerosis (‘cotton-wool spots’), and calvarial thickening. Figure B demonstrates coarsened trabeculae, cortical thickening, and enlargement of the left femur. The histology in Figure C shows abnormal bone formation with a mosaic pattern of woven bone and irregular sections of thickened trabecular bone and numerous cement lines.

Teriparatide (Forteo) is a recombinant form of parathyroid hormone, used in the treatment of some forms of osteoporosis. Administration is contraindicated in the patient with Paget disease. Other contraindications to the use of teriparatide include any hypercalcemic disorder, osteosarcoma, metastatic bone disease, pregnancy, and radiation therapy to the skeleton or to soft tissue in which a skeletal port is exposed.

Tastekin and Zateri authored a letter to the editor that discusses the use of teriparatide in patients with Paget disease. They report that the FDA issued a black box warning on teriparatide due to the increased rate of osteosarcoma development in rats that have been given very high doses teriparatide. The FDA limits duration of teraparatide administration in humans to a duration of 18–24 months.

REFERENCES:

1. Morris CD. Orthopaedic pharmacology and therapeutics. In: Einhorn TA, O'Keefe RJ, Buckwalter JA, eds. Orthopaedic Basic Science: Foundations of Clinical Practice. 3rd ed. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2007:315-330.

2. Tastekin N, Zateri C. Probable osteosarcoma risk after prolonged teriparatide treatment: comment on the article by Saag et al. Arthritis Rheum. 2010 Jun;62(6):1837; author reply 1837-8. PMID:20191586 (Link to Abstract)

Question Authors:

Michael Hughes MD, Patrick McCulloch MD, John Badylak MD,

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TAG

(OBQ10.78) Which of the following conditions can be characterized by excessive bone resorption and disordered bone formation? Topic

Review Topic

1. Osteopetrosis
2. Paget's disease of bone
3. Osteogenesis imperfecta
4. Rickets
5. Legg-Calve-Perthes disease

PREFERRED RESPONSE ▶

DISCUSSION: Paget's disease of bone is seen in older patients of Anglo-Saxon descent, and is frequently asymptomatic. It progresses through three radiographic phases: lytic, mixed, sclerotic. During the lytic phase there is excessive osteoclast activity. This is followed by increased bone formation, though the bone is woven and disordered. Treatment is with bisphosphonates to block osteoclast activity. Paget's has a strong autosomal dominant genetic tendency with the candidate gene 5q35-QTER, and may be caused by a slow viral infection (PMV, RSV). Malignant degeneration occurs <1% of the time.

REFERENCES:

1. Zuscik MJ, Drissi MH, Chen D, Rosier RN. Molecular and cell biology in orthopaedics. In: Einhorn TA, O'Keefe RJ, Buckwalter JA, eds. Orthopaedic Basic Science: Foundations of Clinical Practice, 3rd ed. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2007:3-24.

2. Miller JD, McCreadie BR, Alford AI, Hankenson KD, Goldstein SA. Form and function of bone. In: Einhorn TA, O'Keefe RJ, Buckwalter JA, eds. Orthopaedic Basic Science: Foundations of Clinical Practice, 3rd ed. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2007:129-159.

Question Authors:

Aaron Bott MD,

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TAG

(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 Topic

Review Topic

FIGURES: A

1. Decreased urinary N-telopeptide and increased urinary alpha-C-telopeptide
2. Low serum vitamin D levels
3. Increased urinary N-telopeptide and decreased urinary alpha-C-telopeptide
4. Increased urinary N-telopeptide and alpha-C-telopeptide
5. Increased urinary N-telopeptide and decreased urinary excretion of pyridinium crosslinks

PREFERRED RESPONSE ▶

DISCUSSION: Figure A and B, show a representative radiograph and biopsy of a patient with Paget's disease. This is a condition characterized by increased bone resorption, bone formation, and remodeling. This results in altered bone quality, thickening, enlargement, and deformity as seen in Figure A. Current treatment is with bisphosphonates to block osteoclast activity. Urinary N-telopeptide, alpha-C-telopeptide, and pyridinium crosslinks are all biochemical markers of bone turnover. Delmas reviews the biochemical markers of bone turnover and its possible treatment implications in Pagets. Delmas found that patients treated with bisphosponates had near normal laboratory findings for bone turnover.

REFERENCES:

1. Murray MA. Bone metabolism and metabolic bone disease. In: Vaccaro AR, ed. Orthopaedic Knowledge Update 8. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2005:187-196.

2. Delmas PD. Biochemical markers of bone turnover in Paget's disease of bone. J Bone Miner Res. 1999 Oct;14 Suppl 2:66-9. Review. PMID:10510216 (Link to Abstract)

Question Authors:

Jan Szatkowski MD,

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TAG

(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 believed to be the etiology for Paget's disease ? Topic

Review Topic

FIGURES: A

1. Figure A
2. Figure B
3. Figure C
4. Figure D
5. Figure E

PREFERRED RESPONSE ▶

DISCUSSION: Paget's disease is a condition characterized by increased bone resorption, bone formation, and remodeling. An intense focal resorption of normal bone by abnormal osteoclasts (Figure C) leads to rapid, unorganized bone formation. This results in altered bone quality, thickening, enlargement, and deformity. Chondroblasts (Figure A), Osteoblasts (Figure B), Synoviocytes (Figure D), Osteocytes (Figure E) are not implicated as a primary etiology for Paget's disease.

The review article by Parvizi focuses on the surgical management of this primarily non-surgical disease process including: long bone osteotomies, fracture fixation, spinal decompression, joint arthroplasty, and tumor resection.

REFERENCES:

1. Parvizi J, Klein GR, Sim FH. Surgical management of Paget's disease of bone. J Bone Miner Res. 2006 Dec;21 Suppl 2:P75-82. PMID:17229013 (Link to Abstract)

Question Authors:

Michael Hughes MD, John Badylak MD, Ben Taylor MD,

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TAG

(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? Topic

Review Topic

FIGURES: A

1. Continued orthotic and brace treatment
2. Corrective osteotomy and plate fixation
3. Corrective osteotomy and intramedullary fixation
4. Amputation
5. Corrective osteotomy, knee arthrodesis, and plate fixation

PREFERRED RESPONSE ▶

DISCUSSION: Radiograph demonstrates tibial bowing in a Pagetoid tibia. Paget's is a metabolic bone disease disrupting the physiologic cycle of bone remodeling. Illustrations provided demonstrate further examples of pagetoid changes within long bones. Given failure to relieve symptoms with conservative measures, surgical management is recommended.

Parvizi et al demonstrates shorter time to union and superior clinical results with metaphyseal osteotomies over diaphyseal osteotomy and plate fixation over intramedullary fixation.

Illustrations: A

REFERENCES:

1. Parvizi J, Frankle MA, Tiegs RD, Sim FH. Corrective osteotomy for deformity in Paget disease. JBJS 2003 PMID:12672847 (Link to Abstract)

Question Authors:

John Badylak MD, Michael Hughes MD, Ben Taylor MD,

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TAG

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

Review Topic

1. The 5-year survival for non-metastatic Paget's sarcoma is less than 5%
2. Less than 1% of patients with Paget's disease develop secondary Paget's sarcoma
3. While osteosarcoma is the most common histologic sub-type of Paget's sarcoma, fibrosarcoma and chondrosarcoma sub-types also occur
4. Paget's sarcoma typically occurs in patients over 50 years of age
5. Treatment of Paget's sarcoma is via surgery alone

PREFERRED RESPONSE ▶

DISCUSSION: Paget's sarcoma is a rare secondary sarcoma which occurs in patients with Paget's disease (Illustration A). Less than 1% of patients with Paget's disease ever develop Paget's sarcoma. It typically presents with progressive pain, soft tissue swelling, or pathological fracture. Typically patients with Paget's sarcoma are older at diagnosis (average 55-80 years). Paget's sarcoma is a very aggressive secondary sarcoma which is histologically similar to osteosarcoma, however fibrosarcoma and chondrosarcoma variants do exist. Treatment consists of surgery with chemotherapy vs. palliative radiotherapy in patients too infirm to withstand surgery or chemotherapy. The 5-year survival for non-metastatic Paget's sarcoma is less than 5%. Mankin and Hornicek review the Mass General experience and report on 43 patients with Paget's sarcoma. Their results are a compilation of two cohorts, the first treated from 1942 to 1967, and the second from 1972 to 2001. As such, they conclude that despite multiple different treatment protocols, the overall survival of Paget's sarcoma is poor.

Illustrations: A

REFERENCES:

1. Mankin HJ, Hornicek FJ. Paget's sarcoma: a historical and outcome review. Clin Orthop Relat Res. 2005 Sep;438:97-102. Review. PMID:16131876 (Link to Abstract)

Question Authors:

Patrick O'Donnell MD/PhD,

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TAG

(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? Topic

Review Topic

FIGURES: A

1. Referral to endocrinology
2. Radiation therapy and chemotherapy
3. Wide resection and reconstruction
4. Radiation therapy, wide resection, and reconstruction
5. Chemotherapy, wide resection, and reconstruction

PREFERRED RESPONSE ▶

DISCUSSION: The radiograph, bone scan, and histology slide are consistent with Paget's sarcoma of the proximal humerus, correctly treated with chemotherapy, wide resection, and reconstruction. The bone scan shows this as a polyostotic disease where the proximal humerus sarcoma has developed from pagetoid bone. Pagetoid bone shows up very "hot" on bone scan, even without sarcomatous change. The histology slide shows a pleomorphic spindle cell sarcoma with many giant cells, large amount of cellular atypia, and high nuclear to cytoplasmic ratio, typical of Paget's sarcoma.

Harrington reviews the diagnosis, treatment, and outcome of patients with Paget's sarcoma. While this review emphasizes the use of amputation for Paget's sarcoma, limb salvage, when appropriate, is still the best choice to decrease patient morbidity. Histologically, Harrington discusses the difficulty in differentiation of Paget's sarcoma, metastatic carcinoma in pagetoid bone, and osteosarcoma and suggests that appropriate pathological training in sarcoma is necessary to make this diagnosis.

Moore et al studied 22 patients with Paget disease in order to better define which patients with Paget's disease are likely to develop these tumors and what radiologic findings confirm the diagnosis. They found that Paget's sarcomas can develop in any bone affected by the disease but are more likely to occur in patients with advanced disease. Radiographically, Paget's sarcoma typically presents as a destructive lesion without periosteal reaction.

REFERENCES:

1. Harrington KD. Surgical management of neoplastic complications of Paget's disease. J Bone Miner Res. 1999 Oct;14 Suppl 2:45-8. Review. PMID:10510213 (Link to Abstract)

2. Moore TE, King AR, Kathol MH, el-Khoury GY, Palmer R, Downey PR. Sarcoma in Paget disease of bone: clinical, radiologic, and pathologic features in 22 cases. AJR Am J Roentgenol. 1991 Jun;156(6):1199-203. PMID:2028867 (Link to Abstract)

Question Authors:

Patrick O'Donnell MD/PhD, Michael Hughes MD, John Badylak MD,

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Videos

Histopathology of Bone in Paget's Disease

Pathology - Educational Presentation - Paget's Disease

The histopathology of abnormal bone in Paget's disease is compared with the norm...

12/19/2012

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Tested Article: Paget's sarcoma: A Historical and Outcome Review.

Mankin HJ, Hornicek FJ. Clin Orthop Relat Res. 2005 Sep;438:97-102. Review...

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Evidence & References Show References

Level of Evidence 3 (Retrospective Cohort Study or Case-Control Study)

Huvos AG. Osteogenic sarcoma of bones and soft tissues in older persons. A clinicopathologic analysis of 117 patients older than 60 years. Cancer. 1986 Apr 1;57(7):1442-9. PMID:2418941 (Link to Abstract)

Level of Evidence 4 (Case Series)

Altman RD. Musculoskeletal manifestations of Paget's disease of bone. Arthritis Rheum. 1980 Oct;23(10):1121-7. PMID:6448603 (Link to Abstract)
Mangham DC, Davie MW, Grimer RJ. Sarcoma arising in Paget's disease of bone: declining incidence and increasing age at presentation. Bone. 2009 Mar;44(3):431-6. PMID:19064007 (Link to Abstract)
Parvizi J, Frankle MA, Tiegs RD, Sim FH. Corrective osteotomy for deformity in Paget disease. JBJS 2003 PMID:12672847 (Link to Abstract)
Mankin HJ, Hornicek FJ. Paget's sarcoma: a historical and outcome review. Clin Orthop Relat Res. 2005 Sep;438:97-102. Review. PMID:16131876 (Link to Abstract)
Moore TE, King AR, Kathol MH, el-Khoury GY, Palmer R, Downey PR. Sarcoma in Paget disease of bone: clinical, radiologic, and pathologic features in 22 cases. AJR Am J Roentgenol. 1991 Jun;156(6):1199-203. PMID:2028867 (Link to Abstract)

Level of Evidence 5 and Other Journal Articles (includes Case Reports, Expert Opinions, Personal Observations, and Biomechanic Studies)

Tiegs RD. Paget's disease of bone: indications for treatment and goals of therapy. Clin Ther. 1997 Nov-Dec;19(6):1309-29; discussion 1523-4. PMID:9444442 (Link to Abstract)
Tastekin N, Zateri C. Probable osteosarcoma risk after prolonged teriparatide treatment: comment on the article by Saag et al. Arthritis Rheum. 2010 Jun;62(6):1837; author reply 1837-8. PMID:20191586 (Link to Abstract)
Delmas PD. Biochemical markers of bone turnover in Paget's disease of bone. J Bone Miner Res. 1999 Oct;14 Suppl 2:66-9. Review. PMID:10510216 (Link to Abstract)
Parvizi J, Klein GR, Sim FH. Surgical management of Paget's disease of bone. J Bone Miner Res. 2006 Dec;21 Suppl 2:P75-82. PMID:17229013 (Link to Abstract)
Harrington KD. Surgical management of neoplastic complications of Paget's disease. J Bone Miner Res. 1999 Oct;14 Suppl 2:45-8. Review. PMID:10510213 (Link to Abstract)

Textbooks

Review of Orthopaedics, 6th Edition, Mark D. Miller MD, Stephen R. Thompson MBBS MEd FRCSC, Jennifer Hart MPAS PA-C ATC, an imprint of Elsevier, Philadelphia, Copyright 2012
AAOS Comprehensive Orthopaedic Review, Jay R. Leiberman. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2009
Orthopaedic Knowledge Update 10, John M Flyn. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2011
Hoppenfeld SP. Surgical Exposures in Orthopaedics: The Anatomic Approach. Lipponcott, Williams, and Wilkins, Philadelphia, PA, Copyright 2009
Orthopaedic In-training Examination (OITE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
Self-Assessment Examination (SAE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
Campanacci M. High grade osteosarcomas. In: Campanacci M, Enneking WF, eds. Bone and Soft Tissue Tumors: Clinical Features, Imaging, Pathology and Treatment. 2nd ed. New York, NY: Springer-Verlag; 1999:463-515.
Morris CD. Orthopaedic pharmacology and therapeutics. In: Einhorn TA, O'Keefe RJ, Buckwalter JA, eds. Orthopaedic Basic Science: Foundations of Clinical Practice. 3rd ed. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2007:315-330.

Undefined

Menendez LR (ed): Orthopaedic Knowledge Update: Musculoskeletal Tumors. Rosemont, IL, American Academy of Orthopaedic Surgeons, 2002, pp 211-215.
Resnick D (ed): Diagnosis of Bone and Joint Disorders. Philadelphia, PA, WB Saunders, 2002, pp 1947-2000.
Zuscik MJ, Drissi MH, Chen D, Rosier RN. Molecular and cell biology in orthopaedics. In: Einhorn TA, O'Keefe RJ, Buckwalter JA, eds. Orthopaedic Basic Science: Foundations of Clinical Practice, 3rd ed. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2007:3-24.
Miller JD, McCreadie BR, Alford AI, Hankenson KD, Goldstein SA. Form and function of bone. In: Einhorn TA, O'Keefe RJ, Buckwalter JA, eds. Orthopaedic Basic Science: Foundations of Clinical Practice, 3rd ed. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2007:129-159.
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