This practice set contains high-yield board review questions covering key concepts in Bone Tumors. Each clinical scenario is designed to test your diagnostic and management skills relevant to this subspecialty.
Question 101
Topic: Bone Tumors
A 14-year-old boy complains of progressive distal thigh pain that awakens him at night. Radiographs reveal a metaphyseal, poorly defined lytic lesion with a 'sunburst' periosteal reaction and Codman's triangle. What is the most likely diagnosis?
Correct Answer & Explanation
. Osteosarcoma
Explanation
Osteosarcoma typically presents in the metaphysis of long bones (most commonly the distal femur) in adolescents. The 'sunburst' periosteal reaction and Codman's triangle are classic radiographic findings.
Question 102
Topic: Bone Tumors
A 9-year-old girl presents with fever, weight loss, and severe mid-shaft thigh pain. Radiographs show a permeative diaphyseal lesion of the femur with an "onion skin" periosteal reaction. What specific chromosomal translocation is diagnostic for this malignancy?
Correct Answer & Explanation
. t(11;22)(q24;q12)
Explanation
Ewing sarcoma is classically associated with the t(11;22)(q24;q12) chromosomal translocation, resulting in the EWS-FLI1 fusion protein. It typically presents in the diaphysis of long bones with an "onion skin" appearance.
Question 103
Topic: Bone Tumors
A 70-year-old man presents with generalized fatigue, severe back pain, and hypercalcemia. Radiographs of his skull reveal multiple 'punched-out' lytic lesions. Serum protein electrophoresis shows an M-spike. Which of the following is the most definitive primary treatment for his skeletal disease?
Correct Answer & Explanation
. Systemic chemotherapy and bisphosphonates
Explanation
The clinical presentation is classic for multiple myeloma (CRAB criteria: hyperCalcemia, Renal involvement, Anemia, Bone lytic lesions). Treatment is primarily medical with chemotherapy and bisphosphonates, reserving surgery for impending or actual pathologic fractures.
Question 104
Topic: Bone Tumors
A 16-year-old boy presents with severe night pain in his right tibia that is dramatically relieved by NSAIDs. Radiographs show a 7 mm radiolucent nidus surrounded by dense sclerotic bone. Which of the following is the most appropriate definitive management?
Correct Answer & Explanation
. Radiofrequency ablation
Explanation
The clinical presentation is classic for an osteoid osteoma. Radiofrequency ablation (RFA) is currently the gold standard and most appropriate definitive, minimally invasive treatment.
Question 105
Topic: Bone Tumors
A 60-year-old man presents with back pain, anemia, and elevated creatinine. Radiographs reveal multiple punched-out lytic lesions in the skull and spine. Which laboratory finding is essential to confirm the diagnosis?
Correct Answer & Explanation
. Monoclonal spike on serum protein electrophoresis
Explanation
Multiple myeloma is diagnosed by the presence of a monoclonal spike (M-protein, usually IgG or IgA) on serum protein electrophoresis (SPEP). It is a plasma cell dyscrasia leading to lytic bone lesions, renal failure, anemia, and hypercalcemia.
Question 106
Topic: Bone Tumors
A 65-year-old male with a 40-pack-year smoking history presents with bilateral knee and ankle pain. Radiographs reveal symmetric, solid periosteal new bone formation along the diaphyses and metaphyses of the tibiae and fibulae. Which of the following is the most likely underlying diagnosis?
Correct Answer & Explanation
. Bronchogenic carcinoma
Explanation
Hypertrophic osteoarthropathy (HOA) is characterized by clubbing, polyarthritis, and symmetric periostitis of tubular bones. It is a paraneoplastic syndrome strongly associated with bronchogenic carcinoma.
Question 107
Topic: Bone Tumors
A 15-year-old male is undergoing chemotherapy for conventional osteosarcoma of the proximal tibia. He presents to the emergency department with sudden onset pleuritic chest pain and shortness of breath. Chest radiograph shows a spontaneous pneumothorax. What is the most likely underlying cause?
Correct Answer & Explanation
. Rupture of a cavitating pulmonary metastasis
Explanation
Spontaneous pneumothorax in a pediatric patient with osteosarcoma is highly suspicious for the rupture of a cavitating subpleural pulmonary metastasis. Osteosarcoma frequently metastasizes to the lungs.
Question 108
Topic: Bone Tumors
A 65-year-old man presents with severe mid-back pain. Laboratory tests reveal anemia, hypercalcemia, and elevated serum creatinine. Serum protein electrophoresis shows a monoclonal spike. Which test is most appropriate to evaluate the full extent of skeletal involvement?
Correct Answer & Explanation
. Whole-body low-dose CT or skeletal survey
Explanation
Multiple myeloma lesions are purely lytic and often lack osteoblastic activity, leading to false-negative results on standard Technetium-99m bone scans. A whole-body low-dose CT or conventional skeletal survey is the standard for detecting these lesions.
Question 109
Topic: Bone Tumors
A 15-year-old boy presents with progressive distal thigh pain that awakens him at night. Radiographs of the distal femur reveal an ill-defined, destructive, bone-forming lesion in the metaphysis accompanied by a sunburst periosteal reaction and a Codman's triangle. A core needle biopsy confirms a high-grade conventional osteosarcoma. What is the current standard of care sequence for this patient?
Correct Answer & Explanation
. Neoadjuvant chemotherapy, wide surgical resection, and adjuvant chemotherapy
Explanation
The standard treatment for high-grade conventional osteosarcoma includes neoadjuvant chemotherapy, followed by wide surgical resection (limb-salvage or amputation), and subsequent adjuvant chemotherapy. This multidisciplinary approach significantly improves overall survival and limb preservation rates.
Question 110
Topic: Bone Tumors
An orthopedic researcher wants to investigate the association between a rare primary bone malignancy (e.g., osteosarcoma) and a specific prior environmental exposure. Which epidemiological study design is the most appropriate and efficient for this scenario?
Correct Answer & Explanation
. Case-control study
Explanation
A case-control study is the most efficient design for evaluating rare diseases. It retrospectively compares previous exposures between a group with the disease (cases) and a group without the disease (controls).
Question 111
Topic: Bone Tumors
A 65-year-old man presents with a pathologic subtrochanteric femur fracture. Laboratory workup reveals hypercalcemia, renal insufficiency, and anemia. Serum protein electrophoresis shows an M-spike. Which of the following radiographic findings is most characteristic of this patient's underlying disease?
Correct Answer & Explanation
. "Punched-out" lytic lesions without sclerotic margins
Explanation
The patient's clinical presentation is classic for multiple myeloma. The characteristic radiographic appearance includes multiple "punched-out" lytic bone lesions lacking a reactive sclerotic rim due to simultaneous osteoclast activation and osteoblast inhibition.
Question 112
Topic: Bone Tumors
A 65-year-old man with hypercalcemia and an M-spike on protein electrophoresis has multiple "punched-out" skull lesions. Which is the most appropriate initial screening test to evaluate for impending pathologic appendicular fractures?
Correct Answer & Explanation
. Skeletal survey with plain radiographs
Explanation
Multiple myeloma lesions are purely lytic and often lack an osteoblastic response, leading to "cold" or false-negative results on a bone scan. A complete plain radiographic skeletal survey is the standard initial imaging modality.
Question 113
Topic: Bone Tumors
Aneurysmal bone cyst of the spine is most likely in this age group:
Correct Answer & Explanation
. Second decade
Explanation
The most common age is the second decade; the mean age is 13 years old for patients with this disorder.
Question 114
Topic: Bone Tumors
A 15-year-old girl presents with pain and a 17°-scoliosis curve. The film suggests an enlargement and sclerosis of the transverse process of L2. The best study to further evaluate the nature and anatomic extent of the lesion is:
Correct Answer & Explanation
. Computed tomography
Explanation
This lesion is most likely to be an osteoid osteoma or an osteoblastoma. The best study to further evaluate the nature and anatomic extent is with computed tomography. Magnetic resonance imaging may overestimate the lesion because of sensitivity to edema in the marrow and surrounding soft tissue. Bone scintigraphy with or without SPEC T does not provide enough anatomic detail. C oned radiographs do not provide the needed multidimensional detail for this condition, as well as many other spinal problems.
Question 115
Topic: Bone Tumors
A 12-year-old boy with hemophilia A has a painless mass in his thigh. The femur is eroded anterolaterally and there is a large overlying soft tissue mass. Magnetic resonance imaging shows a 5 cm x 7 cm mass arising from the bone. The most likely diagnosis is:
Correct Answer & Explanation
. Pseudotumor
Explanation
A pseudotumor is a hemophilic subperiosteal hematoma. The pseudotumor expands by repeated bleeds and increasing osmotic pressure. There was no periosteal reaction or intralesional calcification. The bone wall itself is not expanded as in aneurysmal bone cyst. There is nothing in the physical examination or patient history to point to infection.
Question 116
Topic: Bone Tumors
Which of the following is the most common final attribution of back pain in children and adolescents after all appropriate diagnostic studies are performed:
Correct Answer & Explanation
. No identifiable cause
Explanation
The majority of children and adolescents do not have an identifiable cause of back pain after all appropriate tests are performed. Many times the diagnosis is "musculo-ligamentous strain." the most common identified cause is spondylolysis.
Question 117
Topic: Bone Tumors
A 9-year-old girl presents with precocious puberty, irregular hyperpigmented macules, and a limp. Radiographs of her femur reveal a ground-glass appearance and a "shepherd's crook" deformity. Which of the following best describes the underlying genetic defect?
Correct Answer & Explanation
. Activating mutation of the GNAS1 gene
Explanation
The patient has McCune-Albright syndrome, defined by polyostotic fibrous dysplasia, coast of Maine cafe-au-lait spots, and endocrine abnormalities. It is caused by a somatic activating mutation in the GNAS1 gene.
Question 118
Topic: Bone Tumors
A 7-year-old girl presents with precocious puberty and a large cafe-au-lait macule with an irregular "coast of Maine" border. Radiographs of her proximal femur show a lytic, expansile, "ground-glass" lesion with a shepherd's crook deformity. What is the underlying genetic mechanism of this disease?
Correct Answer & Explanation
. GNAS1 gene mutation
Explanation
McCune-Albright syndrome consists of polyostotic fibrous dysplasia, precocious puberty, and cafe-au-lait spots. It is caused by a somatic activating mutation in the GNAS1 gene, leading to overproduction of cAMP.
Question 119
Topic: Bone Tumors
A 4-year-old boy presents with a painless, hard mass on the medial aspect of his right knee, causing a progressive asymmetric valgus deformity. Radiographs show an irregular, ossified mass arising from the medial half of the distal femoral epiphysis. What is the most likely diagnosis?
Correct Answer & Explanation
. Dysplasia epiphysealis hemimelica
Explanation
Dysplasia epiphysealis hemimelica (Trevor disease) is an osteochondroma-like overgrowth of an epiphysis, typically affecting one side (usually medial) of a joint, most commonly the knee or ankle. It leads to asymmetric joint deformity and restricted range of motion.
Question 120
Topic: Bone Tumors
C ongenital pseudarthrosis of the clavicle occurs most commonly on which side:
Correct Answer & Explanation
. Right
Explanation
Ninety percent of cases are noted on the right side. Ten percent of cases are bilateral and have been associated with bilateral cervical ribs. Only a few cases of left-sided pseudarthrosis have been described and have been associated with dextrocardia. Only <5% of cases of congenital pseudarthrosis are on the left. Congenital pseudarthrosis is not related to fibrous dysplasia. Congenital pseudarthrosis is not related to proximal focal femoral dysplasia.
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