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Bone Tumors MCQs

Practice Set 8 of 52

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 141

Topic: Bone Tumors

A 19-year-old male complains of severe right thigh pain that is worse at night and dramatically relieved by ibuprofen. CT demonstrates a 7mm radiolucent nidus surrounded by dense sclerotic bone in the femoral diaphysis. What is the most definitive and minimally invasive treatment?

. En bloc resection
. Observation
. Radiofrequency ablation
. Intralesional curettage
. Radiation therapy

Correct Answer & Explanation

. Radiation therapy

Explanation

Osteoid osteomas present classically with nocturnal pain relieved by NSAIDs due to high local prostaglandin levels. Radiofrequency ablation (RFA) is the treatment of choice, offering high success rates with minimal morbidity.

Question 142

Topic: Bone Tumors

A 65-year-old woman presents with multiple "punched-out" lytic bone lesions. Serum protein electrophoresis reveals an M-spike. Which of the following is the most sensitive imaging modality for detecting skeletal involvement in this specific disease?

. Technetium-99m bone scan
. Whole-body low-dose CT or PET/CT
. Plain radiographs
. Ultrasound
. Dual-energy X-ray absorptiometry

Correct Answer & Explanation

. Whole-body low-dose CT or PET/CT

Explanation

Multiple myeloma lesions are primarily osteolytic without significant osteoblastic activity, making Technetium-99m bone scans notoriously falsely negative. Whole-body low-dose CT or PET/CT are the preferred sensitive modalities.

Question 143

Topic: Bone Tumors

A 19-year-old male complains of right thigh pain that is worse at night and completely relieved by ibuprofen. Imaging reveals a 1 cm radiolucent nidus surrounded by dense reactive sclerosis in the femoral diaphysis. If left untreated, what is the natural history of this lesion?

. Malignant transformation to osteosarcoma
. Progressive enlargement leading to a pathologic fracture
. Spontaneous resolution over several years
. Transformation into an aneurysmal bone cyst
. Metastasis to the lungs

Correct Answer & Explanation

. Spontaneous resolution over several years

Explanation

The presentation is classic for an osteoid osteoma. If left untreated, the natural history of osteoid osteoma is spontaneous resolution (burnout) over a period of 2 to 6 years, though treatment is often pursued for symptom relief.

Question 144

Topic: Bone Tumors

A 16-year-old male presents with knee pain and a palpable mass. Radiographs show a "sunburst" periosteal reaction and Codman's triangle in the distal femoral metaphysis. Biopsy confirms high-grade, intramedullary osteosarcoma without systemic metastasis. What is the standard of care for definitive management?

. Primary amputation to achieve wide margins.
. Neoadjuvant chemotherapy followed by wide surgical resection and adjuvant chemotherapy.
. Wide surgical resection followed by definitive radiation therapy.
. Neoadjuvant radiation therapy followed by wide surgical resection.
. Curettage, burring, phenol application, and cementation.

Correct Answer & Explanation

. Neoadjuvant chemotherapy followed by wide surgical resection and adjuvant chemotherapy.

Explanation

The standard treatment for high-grade osteosarcoma consists of multi-agent neoadjuvant chemotherapy, followed by wide surgical resection (limb-salvage when feasible), and subsequent adjuvant chemotherapy. Osteosarcoma is highly radioresistant, making radiation therapy generally ineffective for primary local control.

Question 145

Topic: Bone Tumors

A 12-year-old female is diagnosed with a high-grade osteosarcoma of the proximal tibia. Neoadjuvant chemotherapy is initiated. During treatment, she develops severe nausea, vomiting, and acute kidney injury with elevated creatinine. Which chemotherapy agent is the most likely cause of these specific toxicities?

. Doxorubicin
. Ifosfamide
. Methotrexate
. Cisplatin
. Etoposide

Correct Answer & Explanation

. Cisplatin

Explanation

Correct Answer: DExplanation:Cisplatinis a platinum-based alkylating-like agent commonly used in osteosarcoma regimens. Its well-known dose-limiting toxicities include severe nausea and vomiting (highly emetogenic), nephrotoxicity (acute kidney injury), and ototoxicity (hearing loss, tinnitus). Therefore, the patient's symptoms are highly suggestive of cisplatin toxicity.Doxorubicin(Adriamycin) is an anthracycline associated with cumulative dose-dependent cardiotoxicity (dilated cardiomyopathy) and myelosuppression.Ifosfamideis an alkylating agent known for causing hemorrhagic cystitis (prevented by Mesna) and neurotoxicity.Methotrexate, especially high-dose, is associated with myelosuppression, mucositis, and nephrotoxicity, but severe nausea/vomiting are less prominent compared to cisplatin. Leucovorin is used for rescue.Etoposideis a topoisomerase II inhibitor primarily causing myelosuppression and mucositis.

Question 146

Topic: Bone Tumors

Which of the following conditions is most strongly associated with the development of secondary osteosarcoma in the craniofacial bones, particularly the jaw, in older adults?

. Fibrous dysplasia
. Hereditary retinoblastoma
. Multiple hereditary exostoses
. Paget's disease of bone
. Ollier's disease

Correct Answer & Explanation

. Paget's disease of bone

Explanation

Correct Answer: DExplanation:Paget's disease of boneis a well-recognized predisposing factor for the development of secondary osteosarcoma, especially in older adults. The craniofacial bones, including the maxilla and mandible (jaw), are common sites for Paget's disease. Therefore, osteosarcoma arising in the jaw in an older adult should raise suspicion for underlying Paget's disease. These secondary osteosarcomas generally carry a worse prognosis than conventional osteosarcomas.A. Fibrous dysplasia:While fibrous dysplasia can rarely undergo malignant transformation into osteosarcoma, it is not the most common predisposing factor for jaw osteosarcoma in older adults.B. Hereditary retinoblastoma:This condition is associated with germline RB1 mutations and a significantly increased risk of osteosarcoma, but typically in younger individuals and not specifically localized to the jaw.C. Multiple hereditary exostoses (HME):HME is a genetic disorder predisposing to the development of multiple osteochondromas, which can transform into chondrosarcoma, not osteosarcoma.E. Ollier's disease (multiple enchondromatosis):Similar to HME, Ollier's disease is associated with multiple enchondromas and carries a risk of malignant transformation into chondrosarcoma, not osteosarcoma.

Question 147

Topic: Bone Tumors

Which of the following best describes the typical radiographic appearance of a Non-Ossifying Fibroma on a plain X-ray?

. Central calcification with a periosteal reaction.
. Permeative osteolytic lesion with soft tissue mass.
. Well-defined, eccentric, lytic lesion with a sclerotic margin and often multiloculated appearance.
. Sunburst periosteal reaction with Codman's triangle.
. Ground-glass matrix with endosteal scalloping.

Correct Answer & Explanation

. Well-defined, eccentric, lytic lesion with a sclerotic margin and often multiloculated appearance.

Explanation

Correct Answer: CNOFs typically present as well-defined, eccentric, purely lytic lesions with a characteristic sclerotic margin. They often have a lobulated or 'bubbly' appearance, indicating fibrous septa. Cortical thinning and mild expansion are also common. The other options describe features of other bone tumors (osteosarcoma for B and D, fibrous dysplasia for E, and calcification for enchondroma).

Question 148

Topic: Bone Tumors

A 9-year-old girl is evaluated for multiple eccentrically located, radiolucent, metaphyseal lesions with sclerotic margins in her bilateral lower extremities. Physical examination reveals multiple cafe-au-lait macules. She has a documented history of developmental delay. Which of the following is the most likely diagnosis?

. Neurofibromatosis type 1
. McCune-Albright syndrome
. Jaffe-Campanacci syndrome
. Mazabraud syndrome
. Ollier disease

Correct Answer & Explanation

. Jaffe-Campanacci syndrome

Explanation

Jaffe-Campanacci syndrome is characterized by the triad of multiple non-ossifying fibromas (NOFs), cafe-au-lait macules, and intellectual disability or hypogonadism. While neurofibromatosis type 1 also presents with cafe-au-lait spots, it is not classically associated with multiple NOFs.

Question 149

Topic: Bone Tumors

A 16-year-old boy is receiving standard neoadjuvant chemotherapy (MAP regimen) for an osteosarcoma of the distal femur. During the course of his treatment, he develops signs of congestive heart failure. Which of the following agents is most likely responsible for this complication?

. Methotrexate
. Cisplatin
. Doxorubicin
. Ifosfamide
. Etoposide

Correct Answer & Explanation

. Doxorubicin

Explanation

Doxorubicin (Adriamycin) is an anthracycline known for its dose-dependent, cumulative cardiotoxicity, which can lead to dilated cardiomyopathy and congestive heart failure. Cisplatin is primarily nephrotoxic and ototoxic, while methotrexate can cause hepatotoxicity and mucositis.

Question 150

Topic: Bone Tumors

A 15-year-old boy presents with severe knee pain. MRI of the distal femur shows a destructive metaphyseal lesion with multiple fluid-fluid levels, mimicking an aneurysmal bone cyst. Biopsy reveals highly pleomorphic cells producing delicate lace-like osteoid in the septa. Which of the following is true regarding this variant of osteosarcoma compared to conventional osteosarcoma?

. It has a substantially worse prognosis and overall survival rate.
. It is managed primarily with wide resection without the need for neoadjuvant chemotherapy.
. It presents with similar overall survival and is treated with identical modern multimodal protocols.
. It predominantly affects patients over the age of 40.
. It is characterized by the t(11;22) chromosomal translocation.

Correct Answer & Explanation

. It presents with similar overall survival and is treated with identical modern multimodal protocols.

Explanation

Telangiectatic osteosarcoma frequently mimics an aneurysmal bone cyst on imaging due to fluid-fluid levels. Despite its aggressive radiographic appearance, when treated with standard modern chemotherapy and wide resection, its prognosis and overall survival are similar to conventional osteosarcoma.

Question 151

Topic: Bone Tumors
A 12-year-old boy is evaluated for multiple non-ossifying fibromas in his lower extremities. Physical examination reveals several café-au-lait spots with smooth margins, axillary freckling, and mild intellectual disability. Which of the following conditions is most consistent with this presentation?
. Neurofibromatosis type 1
. McCune-Albright syndrome
. Jaffe-Campanacci syndrome
. Mazabraud syndrome
. Ollier disease

Correct Answer & Explanation

. Jaffe-Campanacci syndrome

Explanation

Jaffe-Campanacci syndrome is characterized by multiple non-ossifying fibromas (NOFs), café-au-lait spots, mental retardation, hypogonadism, and cardiovascular abnormalities. The café-au-lait spots can mimic those seen in Neurofibromatosis type 1.

Question 152

Topic: Bone Tumors

A 55-year-old male is diagnosed with a conventional Grade II chondrosarcoma of the ilium. Compared to an identical histologic lesion located in the distal femur, what is the primary reason this pelvic lesion portends a significantly poorer prognosis?

. Higher likelihood of early hematogenous pulmonary metastasis
. Increased intrinsic cellular resistance to systemic chemotherapy
. Difficulty in achieving wide and adequate surgical margins
. Higher rate of spontaneous dedifferentiation in flat bones
. Tendency to spread to regional lymph nodes early in the disease course

Correct Answer & Explanation

. Difficulty in achieving wide and adequate surgical margins

Explanation

Chondrosarcomas of the pelvis typically have a worse prognosis than those in the appendicular skeleton primarily because they grow to a massive size before becoming symptomatic and involve complex anatomy, making wide surgical margins difficult to achieve.

Question 153

Topic: Bone Tumors

The standard neoadjuvant chemotherapy regimen (MAP) utilized for high-grade conventional osteosarcoma in pediatric and young adult patients typically consists of which combination of agents?

. Vincristine, doxorubicin, and cyclophosphamide
. Methotrexate, doxorubicin, and cisplatin
. Ifosfamide and etoposide
. Imatinib and sunitinib
. Denosumab and zoledronic acid

Correct Answer & Explanation

. Methotrexate, doxorubicin, and cisplatin

Explanation

The MAP regimen consists of high-dose Methotrexate, doxorubicin (Adriamycin), and Cisplatin. It is the gold standard neoadjuvant and adjuvant chemotherapy protocol for conventional high-grade osteosarcoma.

Question 154

Topic: Bone Tumors

A 20-year-old male presents with leg pain. Imaging shows a radiolucent surface lesion on the anterior diaphysis of the tibia with a 'sunburst' periosteal reaction and adjacent cortical thickening, but no medullary involvement. Histology reveals predominantly chondroblastic matrix with intermediate-grade malignant cells. Which of the following is the most likely diagnosis?

. Parosteal osteosarcoma
. Conventional osteosarcoma
. Periosteal osteosarcoma
. Periosteal chondrosarcoma
. Osteochondroma

Correct Answer & Explanation

. Periosteal osteosarcoma

Explanation

Periosteal osteosarcoma is an intermediate-grade surface tumor that typically arises on the diaphyseal surface of the tibia or femur. It is characterized by a predominantly chondroblastic matrix and has a slightly better prognosis than conventional osteosarcoma.

Question 155

Topic: Bone Tumors

A 55-year-old male is diagnosed with an intermediate-grade (Grade 2) conventional chondrosarcoma located entirely within the iliac wing. What is the most appropriate surgical treatment?

. Intralesional curettage with phenol adjuvant
. Radiofrequency ablation
. Wide surgical resection
. Observation
. Neoadjuvant chemotherapy followed by wide resection

Correct Answer & Explanation

. Wide surgical resection

Explanation

For chondrosarcomas of the pelvis, wide surgical resection is mandatory, even for lower-grade lesions. Curettage in the pelvis yields unacceptably high local recurrence rates, which are extremely difficult to salvage.

Question 156

Topic: Bone Tumors

A 16-year-old girl presents with a destructive, expansile lytic lesion in the proximal humerus. MRI shows multiple fluid-fluid levels within the lesion, resembling an aneurysmal bone cyst (ABC), but with solid septal nodularity on contrast sequences. Which histologic feature most reliably differentiates this lesion from a primary ABC?

. The presence of hemosiderin-laden macrophages
. Expansion of the overlying cortical bone
. Identification of highly pleomorphic cells producing osteoid in the septa
. The presence of multinucleated giant cells
. Lack of active periosteal reaction

Correct Answer & Explanation

. Identification of highly pleomorphic cells producing osteoid in the septa

Explanation

Telangiectatic osteosarcoma frequently mimics an aneurysmal bone cyst radiographically and on MRI due to fluid-fluid levels. The definitive differentiating feature is the histologic presence of highly pleomorphic, malignant cells producing fine lace-like osteoid within the septal walls.

Question 157

Topic: Bone Tumors

Following initial radiographs, an MRI of the left femur was performed to further characterize the lesion and rule out secondary aneurysmal bone cyst (ABC) or malignant transformation. The MRI findings included heterogeneous low-to-intermediate signal on T1-weighted images, high signal intensity on T2-weighted images, and heterogeneous, predominantly peripheral enhancement post-contrast. Crucially, no definitive fluid-fluid levels were identified. An example MRI image is provided.

Which of the following MRI findings, if present, would have most strongly suggested an alternative diagnosis of an Aneurysmal Bone Cyst (ABC) rather than Fibrous Dysplasia?

. Heterogeneous low-to-intermediate signal on T1-weighted images
. High signal intensity on T2-weighted images
. Prominent fluid-fluid levels
. Heterogeneous peripheral enhancement post-contrast
. Absence of aggressive soft tissue invasion

Correct Answer & Explanation

. Prominent fluid-fluid levels

Explanation

Correct Answer: CAneurysmal Bone Cysts (ABCs) are characterized by blood-filled spaces separated by fibrous septa. On MRI, the classic and most diagnostic feature of an ABC is the presence of prominent fluid-fluid levels, which represent the sedimentation of blood products within these cystic spaces. While fibrous dysplasia can sometimes have cystic degeneration or hemorrhage, leading to areas of high T2 signal and heterogeneous enhancement, it typically lacks definitive fluid-fluid levels unless a secondary ABC has developed within the fibrous dysplastic lesion. The other options (heterogeneous T1 signal, high T2 signal, and heterogeneous enhancement) are non-specific and can be seen in both fibrous dysplasia and ABCs, as well as other bone lesions. The absence of aggressive soft tissue invasion is a feature that helps rule out high-grade malignancy but does not differentiate between fibrous dysplasia and ABC.

Question 158

Topic: Bone Tumors
The patient's initial systemic examination and laboratory investigations were unremarkable. Specifically, there was no café-au-lait pigmentation, and serum calcium, phosphate, and alkaline phosphatase levels were within normal ranges. The absence of these findings in this patient helps to rule out which of the following associated syndromes or conditions?
. Neurofibromatosis Type 1
. Ollier's disease
. McCune-Albright syndrome
. Maffucci syndrome
. Paget's disease of bone

Correct Answer & Explanation

. McCune-Albright syndrome

Explanation

McCune-Albright syndrome is a classic triad characterized by polyostotic fibrous dysplasia, café-au-lait skin pigmentation, and various endocrine dysfunctions (e.g., precocious puberty, hyperthyroidism, acromegaly, Cushing's syndrome). The absence of café-au-lait spots and normal endocrine labs effectively rules out McCune-Albright syndrome in this patient, confirming his monostotic presentation without systemic involvement. Neurofibromatosis Type 1 is associated with café-au-lait spots but typically presents with neurofibromas and other skeletal dysplasias, not fibrous dysplasia as its primary bone lesion. Ollier's disease and Maffucci syndrome are characterized by multiple enchondromas, not fibrous dysplasia. Paget's disease of bone is a metabolic bone disease of older adults, characterized by disorganized bone remodeling and typically elevated alkaline phosphatase, which was normal in this patient.

Question 159

Topic: Bone Tumors

A 45-year-old female with known polyostotic fibrous dysplasia presents with a painless, slow-growing soft tissue mass in her right thigh. Magnetic resonance imaging reveals a well-circumscribed, lobulated intramuscular mass that is T1-hypointense and markedly T2-hyperintense with mild peripheral enhancement. What is the most likely diagnosis associated with this syndrome?

. Jaffe-Campanacci syndrome
. Maffucci syndrome
. Ollier disease
. Mazabraud syndrome
. McCune-Albright syndrome

Correct Answer & Explanation

. Mazabraud syndrome

Explanation

Mazabraud syndrome is characterized by the association of fibrous dysplasia with single or multiple intramuscular myxomas. The MRI findings described are classic for an intramuscular myxoma.

Question 160

Topic: Bone Tumors

A 16-year-old male with fibrous dysplasia presents with increasing thigh pain and a progressive varus deformity of the proximal femur, characteristic of a 'Shepherd's crook' deformity. Non-operative management has failed. Which of the following is the most appropriate surgical management?

. Curettage and bone grafting alone
. Open reduction and internal fixation with a dynamic hip screw
. Varus-producing proximal femoral osteotomy with plate fixation
. Valgus-producing proximal femoral osteotomy and intramedullary nailing
. Resection and endoprosthetic reconstruction

Correct Answer & Explanation

. Valgus-producing proximal femoral osteotomy and intramedullary nailing

Explanation

Surgical management of a Shepherd's crook deformity requires a valgus-producing osteotomy to restore mechanical alignment, followed by fixation with a load-sharing intramedullary nail. Rigid plate fixation or cortical bone grafting alone has a high failure rate in fibrous dysplasia.

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