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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?

. Nidus less than 1.5 cm with extensive reactive sclerosis
. Expansile lytic lesion larger than 2 cm with a thin cortical shell
. Epiphyseal eccentric lucency with a thick sclerotic rim
. "Onion-skin" periosteal reaction in the diaphysis
. Metaphyseal sunburst periosteal reaction

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?

. Osteoid osteoma
. Osteoblastoma
. Aneurysmal bone cyst
. Eosinophilic granuloma
. Osteosarcoma

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?

. Dense, highly cellular stroma devoid of vascularity
. Loose fibrovascular connective tissue intermixed with irregular osteoid
. Abundant production of atypical malignant cartilage
. Absolute absence of any mitotic figures
. Chicken-wire calcification pattern

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?

. Codman triangle
. Sunburst periosteal reaction
. Expansile lesion with a thin shell of reactive periosteal bone
. Skip metastases in the same bone
. Cloud-like matrix mineralization in the soft tissue

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)?

. Wide en bloc resection
. Neoadjuvant chemotherapy followed by resection
. Extended intralesional curettage with adjuvant and bone grafting
. Observation and serial radiographs
. External beam radiation therapy

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?

. Size greater than 2 cm and a propensity for progressive pain unresponsive to salicylates
. Size less than 2 cm and a dense sclerotic nidus
. Presence of a t(16;17) translocation
. Malignant potential with a high rate of pulmonary metastasis
. Intense radiotracer uptake strictly limited to the central nidus

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?

. SYT-SSX1
. EWSR1-FLI1
. PAX3-FOXO1
. TLS-CHOP
. BCR-ABL

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?

. Type I collagen
. Heparan sulfate
. Hyaluronic acid
. Keratan sulfate
. Chondroitin sulfate

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?

. It will likely transform into an osteosarcoma by early adulthood
. It typically migrates toward the diaphysis and eventually heals after skeletal maturity
. It expands to involve the epiphysis, causing growth arrest
. It crosses the joint space to involve the adjacent bone
. It progressively destroys the cortex, requiring wide en bloc resection

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?

. GNAS
. USP6
. EXT1
. H3F3A
. RB1

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?

. Core needle biopsy to rule out malignancy
. Intralesional curettage and bone grafting
. Prophylactic internal fixation
. Observation and reassurance
. Radiofrequency ablation

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:
. Loose fibrovascular connective tissue intermixed with irregular osteoid and osteoblastic rimming
. Curvilinear trabeculae of woven bone lacking osteoblastic rimming in a fibrous stroma
. Multinucleated giant cells in a background of mononuclear stromal cells
. Nests of small round blue cells with positive CD99 staining
. Chondrocytes arranged in lobules with a permeative pattern

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?

. Giant Cell Tumor
. Chondroblastoma
. Clear Cell Chondrosarcoma
. Aneurysmal Bone Cyst
. Enchondroma

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:

. Weibel-Palade bodies
. Birbeck granules
. Neurosecretory granules
. Russell bodies
. Auer rods

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?

. Presence of a Codman triangle on plain radiographs
. Elevated serum alkaline phosphatase levels
. Macroscopic skip metastases within the same bone
. Location in the distal femur rather than the proximal tibia
. Age younger than 15 years

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?

. Presence of woven bone
. Absence of permeative invasion into host lamellar bone
. Presence of multinucleated giant cells
. Presence of loose fibrovascular stroma
. High levels of tartrate-resistant acid phosphatase

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?

. Presence of reactive sclerosis
. Size greater than 2 cm
. Location in the posterior elements
. Night pain entirely relieved by NSAIDs
. Histological presence of woven bone

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?

. Malignant transformation to osteosarcoma
. Metastasis to the lungs
. Local recurrence
. Spontaneous resolution
. Pathologic fracture

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?

. EXT1; 1-5%
. EXT1; 25-30%
. GNAS1; 1-5%
. GNAS1; 25-30%
. RUNX2; 1-5%

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?

. Osteoid osteoma
. Osteoblastoma
. Giant cell tumor
. Chondrosarcoma
. Ewing sarcoma

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.