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Question 621

Topic: Bone Tumors

A 19-year-old male presents with persistent, dull aching back pain that is worse at night. The pain is not relieved by ibuprofen. CT imaging reveals a 2.5 cm radiolucent nidus with surrounding sclerosis in the posterior elements of L4. What is the most likely diagnosis?

. Osteoid osteoma
. Osteoblastoma
. Aneurysmal bone cyst
. Ewing sarcoma
. Chondromyxoid fibroma

Correct Answer & Explanation

. Osteoblastoma


Explanation

Osteoblastoma is histologically similar to osteoid osteoma but is distinguished clinically by a nidus larger than 1.5-2.0 cm. Additionally, the pain from osteoblastoma is typically less responsive to NSAIDs compared to osteoid osteoma.

Question 622

Topic: Bone Tumors

A 24-year-old male presents with chronic axial back pain. He notes that the pain is dull, constant, and only mildly relieved by non-steroidal anti-inflammatory drugs (NSAIDs). Radiographs and a subsequent CT scan reveal a 3.0 cm expansile, lytic lesion in the posterior elements of L4. Histologically, the lesion shows a loose fibrovascular stroma with interlacing trabeculae of osteoid lined by prominent osteoblasts. What is the most likely diagnosis?

. Osteoid osteoma
. Osteoblastoma
. Aneurysmal bone cyst
. Osteosarcoma
. Ewing sarcoma

Correct Answer & Explanation

. Osteoblastoma


Explanation

The clinical and radiographic presentation is classic for an osteoblastoma. While histologically identical to an osteoid osteoma, an osteoblastoma is larger (by definition >2 cm), typically involves the posterior elements of the spine, and causes pain that is often NOT dramatically relieved by NSAIDs or salicylates.

Question 623

Topic: Bone Tumors

Which of the following histologic variants of osteosarcoma typically carries the most favorable prognosis following wide surgical resection without neoadjuvant chemotherapy?

. Telangiectatic osteosarcoma
. Parosteal osteosarcoma
. Periosteal osteosarcoma
. High-grade surface osteosarcoma
. Conventional osteosarcoma

Correct Answer & Explanation

. Parosteal osteosarcoma


Explanation

Parosteal osteosarcoma is a low-grade surface osteosarcoma that carries an excellent prognosis with wide surgical resection alone. Telangiectatic, high-grade surface, and conventional osteosarcomas are high-grade lesions that require systemic neoadjuvant and adjuvant chemotherapy.

Question 624

Topic: Bone Tumors

A 9-year-old boy incidentally undergoes radiography for mild shoulder pain, revealing a centrally located, well-circumscribed, purely lytic lesion in the proximal humeral metaphysis. An MRI demonstrates a single fluid-filled cavity with no fluid-fluid levels. During a diagnostic aspiration, clear yellow fluid is obtained. Which of the following is true regarding the biochemical fluid contents of this specific lesion?

. It will demonstrate high levels of basic fibroblast growth factor (bFGF)
. It will have high concentrations of prostaglandins (PGE2) and interleukin-1 (IL-1)
. It will clot rapidly upon exposure to room air due to high fibrinogen
. It will be heavily nucleated with neoplastic spindle cells
. It will show an abundance of multinucleated giant cells on cytology

Correct Answer & Explanation

. It will have high concentrations of prostaglandins (PGE2) and interleukin-1 (IL-1)


Explanation

The lesion described is a Unicameral Bone Cyst (UBC), which typically yields clear, yellowish serous fluid upon aspiration. Analysis of UBC fluid demonstrates high levels of bone-resorbing factors, specifically prostaglandins (such as PGE2) and inflammatory cytokines like Interleukin-1 (IL-1), IL-6, and nitrogen oxides. This local biochemical environment promotes osteoclast activation and cyst expansion.

Question 625

Topic: Bone Tumors

A 19-year-old male presents with chronic, dull back pain that profoundly worsens at night. He notes that naproxen provides significant, albeit temporary, relief. CT scan reveals a 3.0 cm lytic defect in the posterior elements of L3, surrounded by extensive sclerosis. Histologically, the lesion consists of interconnecting woven bone trabeculae lined by prominent single layers of osteoblasts with a vascular stroma. Based on the overall presentation and imaging, what is the most likely diagnosis?

. Osteoid osteoma
. Osteoblastoma
. Aneurysmal bone cyst
. Giant cell tumor
. Chondromyxoid fibroma

Correct Answer & Explanation

. Osteoblastoma


Explanation

Osteoid osteoma and osteoblastoma share identical histologic features (woven bone trabeculae lined by prominent osteoblasts). They are differentiated primarily by size: osteoid osteomas are less than 1.5 - 2.0 cm in diameter, whereas osteoblastomas are larger (> 2.0 cm). Osteoblastomas also have a strong predilection for the posterior elements of the spine. While both can cause pain responsive to NSAIDs (due to prostaglandin production), the large size (3.0 cm) defines this as an osteoblastoma.

Question 626

Topic: Bone Tumors

A 9-year-old girl presents with a pathologic fracture of the proximal femur with a "shepherd's crook" deformity. She also has precocious puberty and cafe-au-lait spots with irregular borders. The underlying pathophysiology involves a post-zygotic activating mutation in which of the following?

. Gs alpha protein (GNAS)
. Fibroblast growth factor receptor 3 (FGFR3)
. Patched 1 (PTCH1)
. Runx2
. EXT1

Correct Answer & Explanation

. Gs alpha protein (GNAS)


Explanation

McCune-Albright syndrome consists of polyostotic fibrous dysplasia, endocrine abnormalities (e.g., precocious puberty), and cafe-au-lait spots. It is caused by a somatic activating mutation in the GNAS gene encoding the Gs alpha protein.

Question 627

Topic: Bone Tumors

A 68-year-old woman presents with generalized bone pain, anemia, and hypercalcemia. Skeletal survey reveals multiple punched-out lytic lesions in the skull and pelvis. Which of the following lab findings is most likely present?

. Elevated alkaline phosphatase
. Elevated parathyroid hormone (PTH)
. Monoclonal spike on serum protein electrophoresis
. Decreased serum creatinine
. Profound hypocalcemia

Correct Answer & Explanation

. Monoclonal spike on serum protein electrophoresis


Explanation

The patient's presentation is classic for multiple myeloma, defined by the CRAB criteria (hypercalcemia, renal failure, anemia, bone lesions). Diagnosis is supported by identifying a monoclonal (M) spike on serum or urine protein electrophoresis.

Question 628

Topic: Bone Tumors

A 20-year-old male complains of severe nocturnal back pain relieved by NSAIDs. Imaging reveals a 2.5 cm lytic lesion with surrounding sclerosis in the posterior elements of L4. What makes this lesion most likely an osteoblastoma rather than an osteoid osteoma?

. Location in the posterior elements
. Symptomatic relief with NSAIDs
. Size greater than 2 cm
. Presence of central calcification
. Age of the patient

Correct Answer & Explanation

. Size greater than 2 cm


Explanation

Both osteoid osteoma and osteoblastoma can occur in the posterior elements of the spine and cause pain. However, osteoblastomas are distinguished primarily by their larger size, typically defined as greater than 1.5 to 2.0 cm.

Question 629

Topic: Bone Tumors

A 22-year-old male presents with a 6-month history of a dull, aching pain in his posterior thoracic spine that is not relieved by aspirin. CT imaging reveals a 3.5 cm expansile, radiolucent lesion in the T8 posterior elements. What is the most likely diagnosis?

. Osteoid osteoma
. Osteoblastoma
. Aneurysmal bone cyst
. Giant cell tumor
. Chordoma

Correct Answer & Explanation

. Osteoblastoma


Explanation

Osteoblastoma typically presents in the posterior elements of the spine in young adults. It is histologically similar to osteoid osteoma but is distinguished by being larger (>2 cm), exhibiting progressive growth, and being less responsive to NSAIDs.

Question 630

Topic: Bone Tumors

A 14-year-old girl presents with a rapidly enlarging, painful mass in her proximal humerus. Radiographs show an eccentric, expansile radiolucent lesion. MRI demonstrates multiple fluid-fluid levels. If a primary neoplastic process is confirmed, which gene rearrangement is characteristic of this lesion?

. GNAS
. USP6
. EXT1
. RUNX2
. COL1A1

Correct Answer & Explanation

. USP6


Explanation

Primary aneurysmal bone cysts (ABCs) are now known to be true neoplasms driven by the USP6 (TRE17) gene rearrangement. While fluid-fluid levels on MRI are characteristic of ABCs, they can also be seen in telangiectatic osteosarcoma and giant cell tumors.

Question 631

Topic: Bone Tumors
A 10-year-old girl is found to have a diaphyseal femur fracture after a minor fall. Radiographs show a "shepherd's crook" deformity with an intramedullary "ground-glass" appearance. She also has precocious puberty and large café-au-lait spots. What is the underlying genetic mutation?
. Rb1
. TP53
. GNAS
. FGFR3
. SH3BP2

Correct Answer & Explanation

. GNAS


Explanation

McCune-Albright syndrome consists of polyostotic fibrous dysplasia, endocrine abnormalities (such as precocious puberty), and "coast of Maine" café-au-lait spots. It is caused by a somatic activating missense mutation in the GNAS gene.

Question 632

Topic: Bone Tumors

An 18-year-old boy complains of nocturnal lower back pain that is completely relieved by ibuprofen. A CT scan reveals a 1 cm sclerotic lesion with a central radiolucent nidus in the L4 pedicle. What is the most appropriate initial minimally invasive treatment?

. Intralesional curettage and bone grafting
. CT-guided radiofrequency ablation
. En bloc spondylectomy
. Selective arterial embolization
. External beam radiation

Correct Answer & Explanation

. CT-guided radiofrequency ablation


Explanation

The clinical presentation and imaging are classic for an osteoid osteoma. CT-guided radiofrequency ablation (RFA) is highly successful, minimally invasive, and is considered the treatment of choice for typical osteoid osteomas.

Question 633

Topic: Bone Tumors

A 9-year-old boy experiences acute right shoulder pain after a minor fall. X-rays show a central, purely lytic lesion in the proximal humerus metaphysis extending to the physis, with a small piece of cortical bone resting at the dependent portion of the cyst. What is the most likely diagnosis?

. Aneurysmal bone cyst
. Osteosarcoma
. Non-ossifying fibroma
. Unicameral bone cyst
. Eosinophilic granuloma

Correct Answer & Explanation

. Unicameral bone cyst


Explanation

A central, lytic metaphyseal lesion in a child with a 'fallen leaf' sign (a fragment of fractured cortex at the bottom of the cyst) is pathognomonic for a fractured unicameral bone cyst (UBC).

Question 634

Topic: Bone Tumors

A 35-year-old woman with an unresectable giant cell tumor of the sacrum is initiated on denosumab therapy. Which of the following best describes the expected histologic and radiographic response to this medication?

. Cystic degeneration with the development of prominent fluid-fluid levels
. Intense peripheral rim enhancement with central liquefactive necrosis
. Depletion of multinucleated giant cells and replacement with dense osteosclerosis
. Massive intralesional hemorrhage mimicking an aneurysmal bone cyst
. Rapid dedifferentiation into a high-grade osteosarcoma

Correct Answer & Explanation

. Depletion of multinucleated giant cells and replacement with dense osteosclerosis


Explanation

Denosumab is a RANKL inhibitor that prevents the maturation of osteoclast-like giant cells. Treatment results in the rapid depletion of giant cells and the laying down of woven bone, appearing as dense central osteosclerosis on radiographs.

Question 635

Topic: Bone Tumors
A 10-year-old girl is evaluated for a limp. Radiographs demonstrate a 'ground-glass' expansile lesion with a 'shepherd's crook' deformity in the proximal femur. Physical exam reveals unilateral café-au-lait spots with irregular 'coast of Maine' borders. This syndrome is caused by a post-zygotic mutation in which gene?
. EXT1
. NF1
. GNAS
. RB1
. TP53

Correct Answer & Explanation

. GNAS


Explanation

This patient has McCune-Albright syndrome, featuring polyostotic fibrous dysplasia, café-au-lait spots, and precocious puberty. It is caused by an activating missense mutation in the GNAS gene, which encodes the alpha subunit of the Gs protein.

Question 636

Topic: Bone Tumors

Which characteristic typically distinguishes Ewing's Sarcoma from osteosarcoma on a plain radiograph?

. Ewing's often presents with an 'onion-skin' periosteal reaction, while osteosarcoma commonly shows a 'sunburst' or Codman's triangle.
. Ewing's is usually a purely sclerotic lesion, while osteosarcoma is purely lytic.
. Ewing's primarily affects the epiphysis, whereas osteosarcoma affects the diaphysis.
. Ewing's lesions typically have sharp, well-defined margins, unlike osteosarcoma.
. Osteosarcoma rarely has a soft tissue mass, while Ewing's always does.

Correct Answer & Explanation

. Ewing's often presents with an 'onion-skin' periosteal reaction, while osteosarcoma commonly shows a 'sunburst' or Codman's triangle.


Explanation

Ewing's Sarcoma classically presents with a lamellated ('onion-skin') periosteal reaction due to its rapid, infiltrative growth pattern. Osteosarcoma, in contrast, frequently demonstrates a 'sunburst' periosteal reaction (spicules perpendicular to the cortex) or a Codman's triangle (triangular elevation of the periosteum). Ewing's is typically lytic, and osteosarcoma can be lytic, sclerotic, or mixed. Ewing's usually affects diaphysis/metadiaphysis, while osteosarcoma typically affects the metaphysis. Both often have poorly defined margins and a significant soft tissue mass.

Question 637

Topic: Bone Tumors

Which age group is most commonly affected by Aneurysmal Bone Cysts?

. Infants (0-1 year)
. Children and adolescents (5-20 years)
. Young adults (20-40 years)
. Middle-aged adults (40-60 years)
. Elderly (>60 years)

Correct Answer & Explanation

. Children and adolescents (5-20 years)


Explanation

Aneurysmal Bone Cysts primarily affect children and adolescents, with the vast majority of cases occurring between the ages of 5 and 20 years. They are rare in individuals over 30 and almost unheard of in the elderly. This age demographic is an important distinguishing factor from other lesions like Giant Cell Tumors, which typically affect skeletally mature young adults.

Question 638

Topic: Bone Tumors

Which imaging modality is most sensitive for demonstrating the characteristic 'fluid-fluid levels' within an Aneurysmal Bone Cyst?

. Plain Radiography
. Computed Tomography (CT)
. Magnetic Resonance Imaging (MRI)
. Ultrasound
. Bone Scintigraphy

Correct Answer & Explanation

. Magnetic Resonance Imaging (MRI)


Explanation

Magnetic Resonance Imaging (MRI) is the imaging modality of choice for demonstrating fluid-fluid levels, which are highly characteristic of Aneurysmal Bone Cysts (ABCs). These levels represent sedimentation of blood products (serum, red blood cells, fibrin) within the cystic cavities. While CT can sometimes show them, MRI is far more sensitive and also provides excellent soft tissue detail for evaluating lesion extension.

Question 639

Topic: Bone Tumors

When distinguishing a primary Aneurysmal Bone Cyst from a Unicameral Bone Cyst (UBC), which feature is more typical of a primary ABC?

. Centrally located in the metaphysis.
. More common in patients over 20 years old.
. Fluid-fluid levels on MRI.
. Asymptomatic until pathological fracture.
. Response to intralesional corticosteroid injection.

Correct Answer & Explanation

. Fluid-fluid levels on MRI.


Explanation

Fluid-fluid levels on MRI are a hallmark feature of Aneurysmal Bone Cysts, reflecting the presence of blood and serum within the cystic cavities. UBCs are typically centrally located within the metaphysis, often asymptomatic until a pathological fracture, and can respond to intralesional corticosteroid injection (though efficacy is variable). ABCs are typically eccentric, more likely symptomatic with pain and swelling, and primarily affect children/adolescents.

Question 640

Topic: Bone Tumors

Which of the following locations for an Aneurysmal Bone Cyst is most challenging to treat surgically and often requires consideration of alternative or multi-modal therapies?

. Distal radius
. Proximal tibia
. Lumbar vertebral body
. Femoral diaphysis
. Phalanges of the hand

Correct Answer & Explanation

. Lumbar vertebral body


Explanation

Aneurysmal Bone Cysts in the axial skeleton, particularly the sacrum and vertebral bodies (like the lumbar vertebral body in this option), are notoriously challenging to treat surgically due to their deep location, proximity to vital neurovascular structures, and often high vascularity. These locations frequently require pre-operative embolization, careful surgical planning, and sometimes less aggressive intralesional approaches or even non-surgical management. Long bones and appendicular skeleton locations are generally more accessible.