Question 3661
Topic: 10. Pathology and OncologyWhich of the following anesthetic complications is most highly associated with patients who have Osteogenesis Imperfecta?
Correct Answer & Explanation
. Malignant hyperthermia-like hypermetabolic state
Practice Set 184 of 351
This practice set contains high-yield board review questions covering key concepts in 10. Pathology and Oncology. Each clinical scenario is designed to test your diagnostic and management skills relevant to this subspecialty.
Which of the following anesthetic complications is most highly associated with patients who have Osteogenesis Imperfecta?
. Malignant hyperthermia-like hypermetabolic state
A 4-year-old boy with SMA Type II is undergoing surgical release of severe bilateral knee flexion contractures. The anesthesiologist asks about muscle relaxant precautions. Why is succinylcholine absolutely contraindicated in this patient?
. Risk of profound, fatal hyperkalemia
In patients with untreated SMA Type I and II, what is the most common cause of early mortality, dictating the need for aggressive proactive management?
. Respiratory failure due to intercostal muscle weakness
A 10-year-old boy with known mutations in the EXT1 gene is followed for multiple bone lesions. He presents with a rapidly enlarging, painful mass on his proximal tibia. He is at highest risk for developing which of the following malignancies?
. Secondary chondrosarcoma
A 22-year-old male has an osteoid osteoma of the proximal femur treated with radiofrequency ablation.
What is the most critical anatomical structure to protect during ablation if the lesion is located in the posterior aspect of the femoral neck?
. Medial circumflex femoral artery
A 14-year-old boy presents with fever, weight loss, and a diaphysial lesion of the femur with a 'periosteal onion-skin' reaction. Biopsy shows small round blue cells. The t(11;22) translocation associated with this tumor results in which fusion protein?
. EWSR1-FLI1
A 60-year-old man presents with dull, aching pain in his right pelvis. Radiographs reveal a large lytic lesion in the ilium with intralesional calcifications demonstrating a 'ring and arc' pattern. Biopsy confirms conventional high-grade chondrosarcoma. What is the mainstay of treatment?
. Wide surgical excision alone
A 30-year-old female complains of chronic knee swelling and locking. MRI demonstrates a large, nodular, intra-articular soft tissue mass with low signal intensity on both T1 and T2 weighted images (blooming artifact on gradient echo). Which medical therapy is FDA-approved for severe, unresectable cases of this condition?
. Pexidartinib
A 14-year-old boy presents with progressive right thigh pain that is significantly worse at night and reliably relieved by ibuprofen. Radiographs reveal a cortical lesion with a central radiolucent nidus and surrounding reactive sclerosis.
What is the primary biochemical mediator responsible for this classic pain pattern?
. Prostaglandin E2
A 19-year-old male is undergoing definitive, minimally invasive treatment for a functionally limiting osteoid osteoma in the proximal femur.
During CT-guided radiofrequency ablation (RFA), what is the optimal target temperature and duration to ensure destruction of the nidus?
. 90°C for 5 to 6 minutes
A 12-year-old boy presents with a painful, enlarging mass in the mid-diaphysis of his femur. Radiographs show a permeative, 'onion-skin' periosteal reaction. Biopsy reveals uniform, small round blue cells that are CD99 positive. Cytogenetic analysis most commonly demonstrates which chromosomal translocation?
. t(11;22)(q24;q12)
A 60-year-old male is diagnosed with a Grade 2 conventional chondrosarcoma of the proximal humerus following an incisional biopsy. Staging reveals no metastatic disease. What is the standard definitive treatment?
. Wide surgical resection alone
. The degree of tumor necrosis (>90%)
A 18-year-old male presents with chronic right thigh pain that is classically worse at night and dramatically relieved by NSAIDs. Radiographs show dense cortical thickening in the proximal femoral diaphysis. Advanced imaging confirms the diagnosis.
What is the gold standard minimally invasive treatment for this condition if medical management fails?
. Radiofrequency ablation
A 14-year-old boy presents with a painful left-sided thoracic scoliosis. The pain is severe at night and awakes him from sleep. A spinal osteoid osteoma is suspected. Where is the lesion most likely located in relation to his scoliotic curve?
. Concave side of the curve
A 55-year-old man presents with worsening deep pelvic pain. Radiographs demonstrate a large lytic lesion in the ilium with distinct "ring and arc" calcifications. Core needle biopsy confirms a low-to-intermediate grade primary malignant bone tumor. What is the standard of care for definitive management?
. Wide surgical resection
A 12-year-old boy presents with a 3-week history of severe right thigh pain, low-grade fever, and diaphoresis. Radiographs reveal a permeative diaphyseal lesion with an "onion skin" periosteal reaction. Which chromosomal translocation is most strongly associated with this diagnosis?
. t(11;22)
A 30-year-old female presents with progressive aching pain in her left knee. Radiographs reveal an eccentric, expansile, lytic lesion in the distal femur that extends directly to the subchondral bone without a sclerotic rim. If surgical curettage is chosen, what is the best recommended local adjuvant to reduce the recurrence rate?
. High-speed burring and phenol or PMMA
A 15-year-old male presents with severe pain and swelling of the distal femur. Radiographs reveal a mixed lytic and blastic metaphyseal lesion breaching the cortex, with a classic "sunburst" periosteal reaction and Codman's triangle. Where is the most common site of metastasis for this tumor?
. Lung
A 14-year-old female presents with an enlarging, painful bump on her proximal humerus. Radiographs show an eccentric, expansile, lytic lesion. MRI demonstrates prominent multiple fluid-fluid levels within the lesion. Which specific genetic rearrangement is most strongly linked to the primary form of this lesion?
. USP6