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 3161
Topic: 10. Pathology and Oncology
The image demonstrates the gross curettage specimen of a primary bone tumor excised from the posterior elements of a 17-year-old's cervical spine. The tumor typically presents with dull pain that is not completely relieved by NSAIDs. Which of the following best describes the classical radiographic finding of this condition?
Correct Answer & Explanation
. Expansile lytic lesion larger than 2 cm with a thin cortical shell
Explanation
Osteoblastomas typically appear radiographically as expansile, predominantly lytic lesions larger than 2 cm with a thin rim of reactive bone. They are most commonly located in the posterior elements of the spine.
Question 3162
Topic: Bone Tumors
A 17-year-old male presents with chronic dull, aching back pain that is not completely relieved by NSAIDs. Imaging shows a 2.5 cm expansile lytic lesion in the posterior elements of L3.
What is the most likely diagnosis?
Correct Answer & Explanation
. Osteoblastoma
Explanation
Osteoblastoma typically presents as an expansile lesion greater than 2 cm in the posterior elements of the spine. Unlike osteoid osteoma, the pain is less classically nocturnal and less completely responsive to NSAIDs.
Question 3163
Topic: 10. Pathology and Oncology
A biopsy is taken from a painful expansile lesion in the C2 lamina. Histology demonstrates the following features.
Which histologic finding is most characteristic of this condition?
Correct Answer & Explanation
. Loose fibrovascular connective tissue intermixed with irregular osteoid
Explanation
Osteoblastomas typically feature loose fibrovascular connective tissue intermixed with irregular osteoid. Mitotic figures may be present, differentiating it somewhat from entirely quiescent lesions, but lacks malignant anaplasia.
Question 3164
Topic: Bone Tumors
Which of the following radiographic findings is most characteristic of an aggressive osteoblastoma rather than conventional osteosarcoma?
Correct Answer & Explanation
. Expansile lesion with a thin shell of reactive periosteal bone
Explanation
Aggressive osteoblastoma often presents as a large, expansile lesion with a thin peripheral shell of reactive bone. Conventional osteosarcoma classically shows more destructive features like Codman's triangle or a sunburst reaction.
Question 3165
Topic: 10. Pathology and Oncology
A 10-year-old girl presents with a rapidly enlarging mass over her proximal humerus. Radiographs show an eccentric, expansile, purely lytic lesion. MRI demonstrates multiple fluid-fluid levels. What is the most appropriate definitive surgical management after biopsy confirms an active aneurysmal bone cyst (ABC)?
Correct Answer & Explanation
. Extended intralesional curettage with adjuvant and bone grafting
Explanation
The standard surgical treatment for an active aneurysmal bone cyst is extended intralesional curettage utilizing an adjuvant (e.g., high-speed burr, phenol, cryotherapy) followed by bone grafting or cementation.
Question 3166
Topic: 10. Pathology and Oncology
A 16-year-old male presents with dull, aching pain in his cervical spine. Radiographs and MRI demonstrate an expansile 3.5 cm lesion in the posterior elements of C4.
During curettage, the lesion appears hypervascular and granular. Which of the following best describes the distinguishing features of this lesion compared to an osteoid osteoma?
Correct Answer & Explanation
. Size greater than 2 cm and a propensity for progressive pain unresponsive to salicylates
Explanation
Osteoblastomas are typically larger than 2 cm, show progressive pain not reliably relieved by NSAIDs/salicylates, and predominantly affect the posterior elements of the spine. Osteoid osteomas are smaller (<2 cm) and respond well to NSAIDs.
Question 3167
Topic: 10. Pathology and Oncology
A biopsy from a diaphyseal femur lesion in an 11-year-old boy reveals sheets of uniform, small, round, blue cells. Molecular testing is positive for a t(11;22)(q24;q12) translocation. Which of the following fusion proteins is most likely driving this malignancy?
Correct Answer & Explanation
. EWSR1-FLI1
Explanation
The t(11;22)(q24;q12) translocation is the hallmark of Ewing Sarcoma. It results in the EWSR1-FLI1 fusion protein, which acts as an aberrant transcription factor promoting tumorigenesis.
Question 3168
Topic: Bone Tumors
A 12-year-old boy with Multiple Hereditary Exostoses (MHE) is evaluated for a rapidly growing, painful lesion on his proximal humerus. Mutations in EXT1 and EXT2 genes are implicated in MHE. These genes normally encode enzymes responsible for the synthesis of which molecule?
Correct Answer & Explanation
. Heparan sulfate
Explanation
EXT1 and EXT2 genes encode glycosyltransferases essential for the polymerization of heparan sulfate. Defects in this pathway disrupt normal physeal signaling, leading to osteochondroma formation.
Question 3169
Topic: 10. Pathology and Oncology
A 9-year-old girl sustains a pathologic fracture through a proximal humerus lesion. Radiographs demonstrate a centrally located, lytic metaphyseal lesion with a "fallen leaf" sign. Which of the following best describes the natural history of this lesion if left untreated?
Correct Answer & Explanation
. It typically migrates toward the diaphysis and eventually heals after skeletal maturity
Explanation
Unicameral bone cysts (UBCs) are benign, fluid-filled lesions typically found in the metaphysis of growing children. They migrate diaphyseally away from the physis over time and usually resolve spontaneously upon skeletal maturity.
Question 3170
Topic: 10. Pathology and Oncology
Aneurysmal bone cysts (ABCs) can occur as primary lesions or secondary to other bone tumors. Primary ABCs have been shown to be true neoplasms driven by a specific genetic rearrangement. Which gene is typically upregulated due to translocation in primary ABCs?
Correct Answer & Explanation
. USP6
Explanation
Primary aneurysmal bone cysts are neoplastic and characterized by translocations involving the USP6 gene on chromosome 17 (e.g., t(16;17)). This leads to increased matrix metalloproteinase activity and cystic bone destruction.
Question 3171
Topic: 10. Pathology and Oncology
A 10-year-old boy undergoes knee radiographs following minor trauma, revealing an incidental, eccentric, cortically based, multiloculated lucent lesion in the distal femoral metaphysis. He is completely asymptomatic. What is the most appropriate management?
Correct Answer & Explanation
. Observation and reassurance
Explanation
The radiographic description is classic for a Non-Ossifying Fibroma (NOF). Because the patient is asymptomatic and the lesion is an incidental finding, the standard of care is observation. NOFs typically ossify and resolve by skeletal maturity.
Question 3172
Topic: Bone Tumors
A 14-year-old girl presents with precocious puberty, café-au-lait macules with irregular 'coast of Maine' borders, and multiple lytic bone lesions with a ground-glass appearance. Histologic examination of the bone lesions would most likely reveal:
Correct Answer & Explanation
. Curvilinear trabeculae of woven bone lacking osteoblastic rimming in a fibrous stroma
Explanation
The clinical presentation is classic for McCune-Albright syndrome, which features polyostotic fibrous dysplasia. Histologically, fibrous dysplasia exhibits irregular trabeculae of woven bone (Chinese character pattern) in a fibrous stroma, characteristically lacking osteoblastic rimming.
Question 3173
Topic: 10. Pathology and Oncology
A 15-year-old boy complains of chronic knee pain. Radiographs demonstrate a well-circumscribed, eccentrically located, lytic lesion exclusively within the proximal tibial epiphysis. Biopsy reveals mononuclear cells with longitudinal nuclear grooves and areas of "chicken-wire" calcification. What is the diagnosis?
Correct Answer & Explanation
. Chondroblastoma
Explanation
Chondroblastoma is a rare, benign cartilaginous tumor that characteristically arises in the epiphysis or apophysis of growing children. The histologic hallmarks are chondroblasts with grooved nuclei (coffee-bean shape) and pericellular 'chicken-wire' calcifications.
Question 3174
Topic: 10. Pathology and Oncology
A 4-year-old boy presents with a painful, solitary lytic lesion of the skull. A biopsy demonstrates sheets of cells with indented nuclei intermixed with eosinophils. Immunohistochemistry is positive for CD1a and S-100. Electron microscopy of these cells would most likely show:
Correct Answer & Explanation
. Birbeck granules
Explanation
The clinical and histologic findings describe Langerhans Cell Histiocytosis (Eosinophilic Granuloma). The diagnostic ultrastructural feature on electron microscopy is the presence of Birbeck granules, which resemble tennis rackets.
Question 3175
Topic: Bone Tumors
A 13-year-old girl is diagnosed with high-grade intramedullary osteosarcoma of the distal femur. Which of the following is considered the most significant adverse prognostic factor at the time of initial diagnosis?
Correct Answer & Explanation
. Macroscopic skip metastases within the same bone
Explanation
While an elevated alkaline phosphatase is a poor prognostic factor, the presence of macroscopic skip metastases within the same bone indicates advanced disease and is associated with a significantly worse prognosis and higher risk of systemic spread.
Question 3176
Topic: 10. Pathology and Oncology
During curettage of a suspected benign spinal lesion in a 20-year-old female, the surgeon notes the gross appearance shown in the provided atlas image.
The tissue is red, granular, and heavily bleeding. Histologic analysis is ordered. Which feature is crucial to differentiate this lesion (osteoblastoma) from a well-differentiated osteosarcoma?
Correct Answer & Explanation
. Absence of permeative invasion into host lamellar bone
Explanation
Osteoblastomas are benign and distinctively well-circumscribed (pushing margins), despite their hypercellularity and occasional atypia. The hallmark differentiating them from osteosarcoma is the absence of permeative invasion trapping host lamellar bone trabeculae.
Question 3177
Topic: Bone Tumors
A 16-year-old male presents with localized cervical spine pain. Radiographs reveal a 3.5 cm expansile, radiolucent lesion in the posterior elements of C4.
Which of the following characteristics most reliably differentiates this lesion from an osteoid osteoma?
Correct Answer & Explanation
. Size greater than 2 cm
Explanation
Osteoblastoma and osteoid osteoma are histologically identical, primarily consisting of woven bone trabeculae and fibrovascular stroma. The main clinical and diagnostic differentiating factor is size, with osteoblastomas defined as being larger than 2 cm in diameter.
Question 3178
Topic: 10. Pathology and Oncology
A 19-year-old female undergoes curettage for an expansile, painful lesion in the posterior elements of T12. The gross pathological specimen is shown.
Histological analysis shows rich vascularity and prominent osteoblasts surrounding irregular osteoid. What is the most common potential complication if this lesion is treated with standard intralesional curettage alone?
Correct Answer & Explanation
. Local recurrence
Explanation
Osteoblastoma is a benign but locally aggressive tumor. When treated with intralesional curettage alone, local recurrence rates can be high (up to 20%), often necessitating extended curettage with a high-speed burr or en bloc resection.
Question 3179
Topic: 10. Pathology and Oncology
A 12-year-old boy presents with multiple painless, bony prominences around his knees, ankles, and shoulders. Radiographs reveal multiple osteochondromas pointing away from the joint lines. He is diagnosed with Multiple Hereditary Exostoses (MHE). Which of the following genes is most likely mutated, and what is the estimated risk of malignant transformation in his lifetime?
Correct Answer & Explanation
. EXT1; 1-5%
Explanation
Multiple Hereditary Exostoses is inherited in an autosomal dominant pattern and is most often caused by mutations in the EXT1 or EXT2 tumor suppressor genes. The lifetime risk of an osteochondroma undergoing malignant transformation to a secondary chondrosarcoma is approximately 1% to 5%.
Question 3180
Topic: 10. Pathology and Oncology
An 18-year-old male undergoes curettage of a 3.5 cm posterior spinal lesion. The gross specimen demonstrates hemorrhagic, gritty red tissue.
Based on the gross appearance and size of the lesion, what is the most likely diagnosis?
Correct Answer & Explanation
. Osteoblastoma
Explanation
Osteoblastoma is histologically identical to osteoid osteoma but is distinguished by a size greater than 2 cm. Grossly, it appears as a vascular, gritty, and reddish mass due to abundant osteoid and prominent vascularity.
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