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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?

. Ewing sarcoma
. Osteosarcoma
. Chondrosarcoma
. Osteoid osteoma
. Multiple myeloma

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?

. t(11;22)(q24;q12)
. t(9;22)(q34;q11)
. t(X;18)(p11;q11)
. t(12;16)(q13;p11)
. t(2;13)(q35;q14)

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?

. Wide resection of affected bones
. Empiric broad-spectrum antibiotics
. Surgical curettage and bone grafting
. Systemic chemotherapy and bisphosphonates
. Prolonged immobilization

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?

. Observation and prolonged NSAID therapy
. Radiofrequency ablation
. En bloc resection with margins
. Radiation therapy
. Intralesional chemotherapy

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?

. Elevated serum alkaline phosphatase
. Monoclonal spike on serum protein electrophoresis
. Polyclonal gammopathy on serum protein electrophoresis
. Profound hypocalcemia
. Elevated parathyroid hormone levels

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?

. Osteosarcoma
. Osteoid osteoma
. Bronchogenic carcinoma
. Rheumatoid arthritis
. Syphilis

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?

. Chemotherapy-induced pulmonary fibrosis
. Rupture of a cavitating pulmonary metastasis
. Pulmonary embolism
. Myocardial infarction
. Rib fracture

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?

. Technetium-99m bone scan
. Whole-body low-dose CT or skeletal survey
. Dual-energy X-ray absorptiometry (DEXA)
. Ultrasound
. Single-photon emission computed tomography (SPECT)

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?

. Primary limb amputation followed by definitive radiotherapy
. Neoadjuvant chemotherapy, wide surgical resection, and adjuvant chemotherapy
. Wide surgical resection alone with negative margins
. Neoadjuvant radiotherapy followed by limb-salvage surgery
. Intralesional curettage, bone grafting, and cryotherapy

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?

. Randomized controlled trial
. Prospective cohort study
. Case-control study
. Cross-sectional study
. Ecological study

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?

. Diffuse osteoblastic lesions
. "Punched-out" lytic lesions without sclerotic margins
. Sunburst periosteal reaction
. Ground-glass matrix
. Soap-bubble expansile lesion

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?

. Technetium-99m bone scan
. Whole-body MRI
. Skeletal survey with plain radiographs
. PET-CT scan
. Dual-energy X-ray absorptiometry (DEXA)

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:

. First decade
. Second decade
. Third decade
. Fourth decade
. Fifth decade

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:

. Coned radiographs
. Bone scintigraphy
. Bone scan with SPEC T (single photon emission computed tomography)
. Magnetic resonance imaging
. Computed tomography

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:

. Telangiectatic osteosarcoma
. Aneurysmal bone cyst
. Infection
. Pseudotumor
. Lymphangioma

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:

. Spondylolysis
. Osteoid osteoma
. Infection
. Herniated nucleus pulposus
. No identifiable cause

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?

. Inactivating mutation of the COMP gene
. Activating mutation of the GNAS1 gene
. Mutation of the EXT1 gene
. Mutation of the FGFR3 gene
. Mutation of the RUNX2 gene

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?

. GNAS1 gene mutation
. EXT1 gene mutation
. NF1 gene mutation
. COL1A1 gene mutation
. RUNX2 gene mutation

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?

. Osteochondroma
. Dysplasia epiphysealis hemimelica
. Ollier disease
. Maffucci syndrome
. Chondroblastoma

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:

. Bilateral
. Right
. Left
. The side more involved with fibrous dysplasia
. The side with the proximal focal femoral dysplasia

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.