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
QUESTIONS 1 of 24 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 Previous Next Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (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: A B C Type & Select Correct Answer 1 Wide resection with perioperative chemotherapy 15% (816/5543) 2 Wide resection with perioperative radiation 9% (481/5543) 3 Wide resection alone 9% (482/5543) 4 Radiation alone 5% (273/5543) 5 Observation with evaluation for bisphonate therapy 62% (3443/5543) L 3 Question Complexity B Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (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: A B Type & Select Correct Answer 1 Inhibition of TNF-a 5% (124/2428) 2 Inhibition of IL-1 receptor 3% (81/2428) 3 Estrogen receptor blockade 2% (46/2428) 4 Osteoclast inhibition 88% (2126/2428) 5 COX inhibition 1% (36/2428) L 1 Question Complexity B Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept Review Full Topic Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (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: A Type & Select Correct Answer 1 Implant malalignment 7% (346/4622) 2 Increased blood loss 59% (2728/4622) 3 Peri-prosthetic fracture 30% (1367/4622) 4 Sciatic nerve palsy 2% (82/4622) 5 Deep vein thrombosis 1% (68/4622) L 4 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic (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: A B C Type & Select Correct Answer 1 Teriparatide 67% (1596/2392) 2 Alendronate 12% (278/2392) 3 Ergocalciferol 4% (92/2392) 4 Zoledronic acid 3% (69/2392) 5 Calcitonin 14% (344/2392) L 3 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 1 Review Tested Concept Review Full Topic (OBQ11.11) Paget's disease may predispose a patient to the development of which of the following malignant neoplasms? QID: 3434 Type & Select Correct Answer 1 Chondrosarcoma 4% (123/3319) 2 Osteosarcoma 52% (1737/3319) 3 Spindle cell sarcoma of bone 3% (89/3319) 4 All of the above 37% (1218/3319) 5 None of the above 4% (143/3319) L 5 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept Review Full Topic (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: A Type & Select Correct Answer 1 Osteopetrosis 4% (95/2214) 2 Paget's disease of bone 91% (2010/2214) 3 Osteogenesis imperfecta 3% (57/2214) 4 Rickets 2% (37/2214) 5 Legg-Calve-Perthes disease 1% (13/2214) L 1 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic (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: A B Type & Select Correct Answer 1 Decreased urinary N-telopeptide and increased urinary alpha-C-telopeptide 4% (120/2960) 2 Low serum vitamin D levels 9% (271/2960) 3 Increased urinary N-telopeptide and decreased urinary alpha-C-telopeptide 18% (537/2960) 4 Increased urinary N-telopeptide and alpha-C-telopeptide 60% (1763/2960) 5 Increased urinary N-telopeptide and decreased urinary excretion of pyridinium crosslinks 8% (244/2960) L 3 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept Review Full Topic (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: A B C D E Type & Select Correct Answer 1 Figure A 2% (45/2687) 2 Figure B 15% (398/2687) 3 Figure C 72% (1927/2687) 4 Figure D 5% (138/2687) 5 Figure E 6% (160/2687) L 2 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review Tested Concept Review Full Topic (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: A Type & Select Correct Answer 1 Continued orthotic and brace treatment 7% (79/1105) 2 Corrective osteotomy and plate fixation 50% (558/1105) 3 Intramedullary fixation 21% (236/1105) 4 Amputation 15% (165/1105) 5 Corrective osteotomy, knee arthrodesis, and plate fixation 5% (58/1105) L 5 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (OBQ06.137) All of the following statements regarding Paget's sarcoma are correct EXCEPT? QID: 323 Type & Select Correct Answer 1 The 5-year survival for metastatic Paget's sarcoma is less than 10% 8% (143/1695) 2 Less than 1% of patients with Paget's disease develop secondary Paget's sarcoma 7% (113/1695) 3 While osteosarcoma is the most common histologic sub-type of Paget's sarcoma, fibrosarcoma and chondrosarcoma sub-types also occur 5% (83/1695) 4 Paget's sarcoma typically occurs in patients over 50 years of age 2% (36/1695) 5 Treatment of Paget's sarcoma is via surgery alone 77% (1313/1695) L 2 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic (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: A Type & Select Correct Answer 1 Paget's bone disease 86% (2602/3009) 2 Osteoporosis 9% (269/3009) 3 Osteomyelitis 0% (9/3009) 4 Renal osteomalacia 4% (109/3009) 5 Aneurysmal bone cyst 0% (9/3009) L 1 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 1 Review Tested Concept Review Full Topic (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: A B C Type & Select Correct Answer 1 Osteosarcoma 77% (2583/3367) 2 Fibrosarcoma 9% (295/3367) 3 Multiple myeloma 12% (404/3367) 4 Giant cell tumor 1% (39/3367) 5 Osteoid Osteoma 1% (30/3367) L 3 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 1 Review Tested Concept Review Full Topic (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: A Type & Select Correct Answer 1 Antibiotics 1% (24/4158) 2 Bisphosphonates 83% (3435/4158) 3 Monoclonal-B antibodies 5% (191/4158) 4 Chemotherapy 8% (333/4158) 5 Wide resection and radiotherapy 4% (148/4158) L 2 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic (SBQ05PA.54) Which of the following would be the most appropriate treatment for a patient with asymptomatic Paget's disease? QID: 2039 Type & Select Correct Answer 1 Supportive therapy 48% (1678/3501) 2 Calcitonin 4% (149/3501) 3 Vitamin D and Calcium 6% (196/3501) 4 Bisphosphonates 41% (1432/3501) 5 Monoclonal-B antibody 1% (26/3501) L 4 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 1 Review Tested Concept Review Full Topic (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: A B C D E Type & Select Correct Answer 1 Figure A 2% (22/956) 2 Figure B 19% (178/956) 3 Figure C 8% (72/956) 4 Figure D 4% (38/956) 5 Figure E 67% (639/956) L 3 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic (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: A B C Type & Select Correct Answer 1 Referral to endocrinology 19% (387/2036) 2 Radiation therapy and chemotherapy 28% (570/2036) 3 Wide resection and reconstruction 8% (169/2036) 4 Radiation therapy, wide resection, and reconstruction 8% (170/2036) 5 Chemotherapy, wide resection, and reconstruction 36% (729/2036) L 4 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic
All Videos (3) Podcasts (1) Login to View Community Videos Login to View Community Videos Adult Reconstruction Core Webinars - by AAHKS, Hip Society, Knee Society, and AAOS Core Webinar - RECON CLINICAL SCIENCE - by AAHKS Derek W. Moore Pathology - Paget's Disease E 2/4/2017 1313 views 4.0 (1) Login to View Community Videos Login to View Community Videos Histopathology of Paget`s disease Basem Attum (OB Team Editor) General - Paget's Disease E 11/9/2014 385 views 4.0 (1) Login to View Community Videos Login to View Community Videos Histopathology of Bone in Paget's Disease Deborah Allen General - Paget's Disease B 12/19/2012 2429 views 4.7 (32) Pathology⎪Paget's Disease Pathology - Paget's Disease Listen Now 17:43 min 10/16/2019 804 plays 4.8 (5)