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All of the following statements regarding Paget's sarcoma are correct EXCEPT?
The 5-year survival for metastatic Paget's sarcoma is less than 10%
Less than 1% of patients with Paget's disease develop secondary Paget's sarcoma
While osteosarcoma is the most common histologic sub-type of Paget's sarcoma, fibrosarcoma and chondrosarcoma sub-types also occur
Paget's sarcoma typically occurs in patients over 50 years of age
Treatment of Paget's sarcoma is via surgery alone
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Which of the following conditions can be characterized by excessive bone resorption and disordered bone formation as characterized by the radiograph in Figure A?
Paget's disease of bone
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?
Wide resection with perioperative chemotherapy
Wide resection with perioperative radiation
Wide resection alone
Observation with evaluation for bisphonate therapy
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?
Paget's bone disease
Aneurysmal bone cyst
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?
Giant cell tumor
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?
Increased blood loss
Sciatic nerve palsy
Deep vein thrombosis
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?
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?
Wide resection and radiotherapy
Which of the following would be the most appropriate treatment for a patient with asymptomatic Paget's disease?
Vitamin D and Calcium
The disease shown in the radiograph and biopsy specimen, Figures A and B, is best characterized by which of the following laboratory findings
Decreased urinary N-telopeptide and increased urinary alpha-C-telopeptide
Low serum vitamin D levels
Increased urinary N-telopeptide and decreased urinary alpha-C-telopeptide
Increased urinary N-telopeptide and alpha-C-telopeptide
Increased urinary N-telopeptide and decreased urinary excretion of pyridinium crosslinks
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?
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?
Referral to endocrinology
Radiation therapy and chemotherapy
Wide resection and reconstruction
Radiation therapy, wide resection, and reconstruction
Chemotherapy, wide resection, and reconstruction
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?
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?
Continued orthotic and brace treatment
Corrective osteotomy and plate fixation
Corrective osteotomy, knee arthrodesis, and plate fixation
Paget's disease may predispose a patient to the development of which of the following malignant neoplasms?
Spindle cell sarcoma of bone
All of the above
None of the above