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.
Question 3261
Topic: 10. Pathology and Oncology
A 40-year-old patient presents with pain in the proximal humerus. MRI shows a cartilaginous lesion. Which of the following MRI findings most strongly supports a diagnosis of low-grade chondrosarcoma rather than a benign enchondroma?
Correct Answer & Explanation
. Endosteal scalloping > 2/3 of the cortical thickness
Explanation
Differentiating enchondroma from low-grade chondrosarcoma can be difficult. MRI findings favoring chondrosarcoma include deep endosteal scalloping (greater than two-thirds of the cortical thickness), extensive marrow edema, soft tissue extension, and large size (>5 cm).
Question 3262
Topic: 10. Pathology and Oncology
A 30-year-old female presents with dull aching in her distal femur. Imaging shows an ill-defined, sclerotic, intramedullary lesion. Biopsy reveals woven bone stroma lacking osteoblastic rimming, forming a permeative pattern. Which of the following is an absolute contraindication for limb-salvage surgery in this disease?
Correct Answer & Explanation
. Major neurovascular bundle involvement preventing a functional limb
Explanation
This patient has an osteosarcoma. The absolute contraindication to limb-salvage surgery is the inability to achieve negative margins while preserving a functional limb, typically due to major neurovascular bundle encasement. Pathologic fractures and lung metastases are relative, not absolute, contraindications.
Question 3263
Topic: 10. Pathology and Oncology
A 12-year-old boy presents with a permeative, diaphyseal lesion in his femur with a periosteal 'onion-skin' reaction. A biopsy is performed. Which specific chromosomal translocation is most strongly associated with this tumor?
Correct Answer & Explanation
. t(11;22)
Explanation
The presentation is classic for Ewing sarcoma. Over 85% of Ewing sarcomas are characterized by the t(11;22)(q24;q12) translocation, resulting in the EWSR1-FLI1 fusion gene.
Question 3264
Topic: 10. Pathology and Oncology
A 15-year-old male is diagnosed with conventional high-grade osteosarcoma of the distal femur. He undergoes 10 weeks of neoadjuvant chemotherapy followed by wide surgical resection. Pathologic analysis of the resected tumor is performed. Which of the following is the most significant prognostic factor for long-term survival?
Correct Answer & Explanation
. Percentage of tumor necrosis in response to chemotherapy
Explanation
The histologic response to neoadjuvant chemotherapy, defined by the percentage of tumor necrosis, is the most important prognostic factor in osteosarcoma. Greater than 90% necrosis is considered a good response and correlates with improved survival.
Question 3265
Topic: 10. Pathology and Oncology
A 55-year-old male presents with a proximal humerus lesion displaying characteristic 'rings and arcs' of calcification on radiographs. Biopsy confirms a grade 2 chondrosarcoma. What is the most appropriate definitive management?
Correct Answer & Explanation
. Wide surgical resection
Explanation
Grade 2 (intermediate) and Grade 3 (high-grade) chondrosarcomas are locally aggressive with metastatic potential and do not respond well to chemotherapy or radiation. Wide surgical resection is the standard of care to minimize local recurrence.
Question 3266
Topic: Bone Tumors
An 18-year-old male complains of deep, aching pain in his proximal femur that is worse at night and dramatically relieved by ibuprofen. Imaging shows a 1 cm radiolucent nidus surrounded by dense reactive sclerosis. The pain is primarily mediated by high local concentrations of which of the following?
Correct Answer & Explanation
. Prostaglandin E2 (PGE2)
Explanation
Osteoid osteomas produce extremely high levels of Prostaglandin E2 (PGE2) and cyclooxygenase (COX-2) within the nidus. This accounts for the intense, night-time pain that is characteristically relieved by NSAIDs.
Question 3267
Topic: 10. Pathology and Oncology
When evaluating a cartilaginous lesion in the distal femur of a 45-year-old patient, which radiographic feature most strongly suggests a low-grade chondrosarcoma rather than a benign enchondroma?
Correct Answer & Explanation
. Endosteal scalloping involving greater than 2/3 of the cortical thickness
Explanation
Deep endosteal scalloping (greater than 2/3 of the cortical thickness), cortical breakthrough, and pain in the absence of a fracture are key features differentiating a low-grade chondrosarcoma from a benign enchondroma.
Question 3268
Topic: 10. Pathology and Oncology
A 58-year-old male presents with a painful, destructive osteolytic lesion in the distal phalanx of his right thumb. Biopsy confirms metastatic adenocarcinoma. What is the most common primary malignancy to metastasize to locations distal to the knee or elbow (acrometastasis)?
Correct Answer & Explanation
. Lung carcinoma
Explanation
Metastases to the hands or feet (acrometastases) are rare and account for about 0.1% of all bone metastases. Lung carcinoma is by far the most common primary source for acrometastases, accounting for approximately 50% of cases.
Question 3269
Topic: Bone Tumors
A 16-year-old boy presents with progressive right thigh pain that is noticeably worse at night and dramatically relieved by taking ibuprofen. Radiographs demonstrate a diaphyseal cortical thickening with a central radiolucent nidus measuring 8 mm. Which of the following is the most likely diagnosis?
Correct Answer & Explanation
. Osteoid osteoma
Explanation
Osteoid osteoma classically presents with nocturnal pain that is exquisitely sensitive to NSAIDs due to high local prostaglandin production. Imaging typically shows a small (<1.5 cm) radiolucent nidus surrounded by dense reactive sclerosis.
Question 3270
Topic: 10. Pathology and Oncology
A 14-year-old boy is diagnosed with a permeative diaphyseal lesion of the femur with an 'onion-skin' periosteal reaction. Biopsy confirms small round blue cells. Which of the following chromosomal translocations is most characteristic of this tumor?
Correct Answer & Explanation
. t(11;22)
Explanation
Ewing sarcoma is characterized by small round blue cells and is classically driven by the t(11;22) chromosomal translocation. This creates the EWS-FLI1 fusion protein, which acts as an aberrant transcription factor.
Question 3271
Topic: 10. Pathology and Oncology
A 55-year-old male presents with a painful, enlarging mass in his proximal femur. Radiographs show a destructive lytic lesion with 'popcorn' intralesional calcifications. Biopsy confirms a grade II conventional chondrosarcoma. What is the most appropriate primary management?
Correct Answer & Explanation
. Wide surgical resection
Explanation
Conventional chondrosarcomas are largely resistant to both chemotherapy and radiation therapy. Wide surgical resection with negative margins is the definitive and most effective treatment modality.
Question 3272
Topic: 10. Pathology and Oncology
A 28-year-old female presents with an expansile, purely lytic epiphyseal lesion of the distal femur extending to the subchondral bone. Biopsy confirms a Giant Cell Tumor. If the tumor is deemed surgically unresectable due to its size and location, which of the following pharmacological agents is most appropriate to downstage the tumor?
Correct Answer & Explanation
. Denosumab
Explanation
Giant cell tumors of bone are characterized by neoplastic stromal cells that express high levels of RANKL, driving the proliferation of multinucleated giant cells. Denosumab, a monoclonal antibody against RANKL, is highly effective for downstaging unresectable lesions.
Question 3273
Topic: 10. Pathology and Oncology
A 15-year-old male is suspected of having an osteosarcoma of the distal femur. A core needle biopsy is planned. Which of the following is a critical orthopedic oncology principle regarding the placement of the biopsy tract?
Correct Answer & Explanation
. The tract must be placed in line with the planned definitive surgical resection incision.
Explanation
Biopsy tracts must be placed longitudinally and strictly in line with the planned definitive surgical incision. This ensures that the entire biopsy tract, which is considered contaminated with tumor cells, can be excised en bloc during the definitive tumor resection.
Question 3274
Topic: Bone Tumors
A 9-year-old boy sustains a minor fall and presents with arm pain. Radiographs reveal a central, purely radiolucent metaphyseal lesion in the proximal humerus with a cortical fragment resting at the bottom of the cyst. What is the most likely diagnosis?
Correct Answer & Explanation
. Unicameral bone cyst
Explanation
A Unicameral Bone Cyst (UBC) is a benign, fluid-filled, central metaphyseal lesion typically found in the proximal humerus or femur of children. The 'fallen leaf' or 'fallen fragment' sign is pathognomonic and indicates a pathologic fracture through the cyst.
Question 3275
Topic: 10. Pathology and Oncology
A 65-year-old male presents with generalized bone pain, fatigue, and renal insufficiency. Radiographs of the skull and pelvis demonstrate numerous 'punched-out' purely lytic lesions without reactive sclerosis. What is the most common primary malignant bone tumor in this age group?
Correct Answer & Explanation
. Multiple myeloma
Explanation
Multiple myeloma is the most common primary malignancy of bone in adults. It classically presents with the CRAB criteria (Hypercalcemia, Renal failure, Anemia, Bone lesions) and sharply demarcated, purely lytic 'punched-out' lesions on imaging.
Question 3276
Topic: Bone Tumors
A 12-year-old girl presents with knee pain. MRI of the distal femur demonstrates an eccentric, expansile metaphyseal lesion containing multiple cystic spaces with distinct fluid-fluid levels. Histology shows blood-filled spaces separated by cellular septa. What is the most likely diagnosis?
Correct Answer & Explanation
. Aneurysmal bone cyst
Explanation
Aneurysmal bone cysts (ABCs) are benign, expansile, eccentric metaphyseal lesions. MRI classically demonstrates fluid-fluid levels due to the settling of blood components within the cystic spaces.
Question 3277
Topic: 10. Pathology and Oncology
A 22-year-old patient presents with multiple palpable, hard masses on his hands and severe deformities. Imaging reveals multiple enchondromas. Physical examination also reveals numerous bluish soft tissue nodules which are identified as hemangiomas. What is the diagnosis?
Correct Answer & Explanation
. Maffucci syndrome
Explanation
Maffucci syndrome is a rare nonhereditary disorder characterized by multiple enchondromas associated with soft tissue hemangiomas. Patients with Maffucci syndrome have a significantly higher risk of malignant transformation to chondrosarcoma compared to those with Ollier disease.
Question 3278
Topic: 10. Pathology and Oncology
A 35-year-old male with a known long-standing asymptomatic distal femoral osteochondroma presents with new-onset, progressive pain and enlargement of the mass. An MRI is performed. Which of the following findings is most highly suspicious for secondary malignant transformation to a chondrosarcoma?
Correct Answer & Explanation
. A cartilaginous cap thickness greater than 2.0 cm
Explanation
Osteochondromas (exostoses) typically stop growing when the skeleton matures. In an adult, new pain, growth, and an MRI demonstrating a cartilage cap thicker than 2.0 cm are highly suspicious for malignant transformation to a secondary chondrosarcoma.
Question 3279
Topic: 10. Pathology and Oncology
In the management of high-grade intramedullary osteosarcoma, which of the following factors is considered the most significant predictor of overall patient survival?
Correct Answer & Explanation
. Histologic response to neoadjuvant chemotherapy
Explanation
The degree of tumor necrosis following neoadjuvant chemotherapy (Huvos grading) is the single most important prognostic factor for overall survival in high-grade osteosarcoma. Greater than 90% necrosis indicates a favorable response and improved prognosis.
Question 3280
Topic: 10. Pathology and Oncology
A 35-year-old female presents with knee pain. Radiographs show an eccentric, lytic lesion in the distal femur extending to the subchondral bone. Biopsy confirms Giant Cell Tumor (GCT). If medical management is considered prior to intralesional curettage to downstage the tumor, which agent is most appropriate?
Correct Answer & Explanation
. Denosumab
Explanation
Denosumab is a monoclonal antibody against RANKL, which is heavily expressed by the stromal cells in Giant Cell Tumor of bone. It inhibits osteoclast-like giant cells, leading to tumor ossification and facilitating subsequent intralesional curettage.
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