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Question 21

Topic: 10. Pathology and Oncology

Which of the following is considered to be a malignant primary spine tumor:

. Osteoblastoma
. Eosinophilic granuloma
. Giant cell tumor
. C hordoma
. Aneurysmal bone cyst

Correct Answer & Explanation

. C hordoma


Explanation

I. Primary benign tumors of the spine are: A. Slow-growing B. Well-circumscribed C . Usually occur in patients younger than 21 years of age D. Involve the vertebral body and posterior elements of the spine 1. Overall slight predilection for the posterior elements 2. Location of tumor is an important factor in determining the type of tumor E. Examples include: 1. Osteochondroma 2. Osteoid osteoma 3. Osteoblastoma 4. Aneurysmal bone cyst 5. Giant cell tumor 6. Eosinophilic granuloma II. Primary malignant tumors of the spine are: A. Fast-growing B. Permeative C . Usually occur in patients older than 21 years of age D. Examples include: 1. Multiple myeloma/solitary plasmacytoma 2. Osteosarcoma 3. C hondrosarcoma 4. Ewing's sarcoma/primitive neuroectodermal tumor 5. Chordoma 6. Lymphoma

Question 22

Topic: 10. Pathology and Oncology

Which of the following tumors is considered to be a benign primary spine tumor:

. Osteosarcoma
. C hordoma
. Multiple myeloma
. Osteoblastoma
. Lymphoma

Correct Answer & Explanation

. Osteoblastoma


Explanation

I. Primary benign tumors of the spine are: A. Slow-growing B. Well-circumscribed C . Usually occur in patients younger than 21 years of age D. Involve the vertebral body and posterior elements of the spine 1. Overall slight predilection for the posterior elements 2. Location of tumor is an important factor in determining the type of tumor E. Examples include: 1. Osteochondroma 2. Osteoid osteoma 3. Osteoblastoma 4. Aneurysmal bone cyst 5. Giant cell tumor 6. Eosinophilic granuloma II. Primary malignant tumors of the spine are: A. Fast-growing B. Permeative C . Usually occur in patients older than 21 years of age D. Examples include: 1. Multiple myeloma/solitary plasmacytoma 2. Osteosarcoma 3. C hondrosarcoma 4. Ewing's sarcoma/primitive neuroectodermal tumor 5. Chordoma 6. Lymphoma

Question 23

Topic: 10. Pathology and Oncology

Primary malignant tumors of the spine have which of the following characteristics:

. Slow-growing, well-circumscribed, and usually occur in patients older than 21 years of age
. Slow-growing, well-circumscribed, and usually occur in patients younger than 21 years of age
. Fast-growing, permeative, and usually occur in patients older than 21 years of age
. Fast-growing, permeative, and usually occur in patients younger than 21 years of age
. Fast-growing, permeative, and usually occur in patients older than 40 years of age

Correct Answer & Explanation

. Fast-growing, permeative, and usually occur in patients older than 21 years of age


Explanation

I. Primary benign tumors of the spine are: A. Slow-growing B. Well-circumscribed C . Usually occur in patients younger than 21 years of age D. Involve the vertebral body and posterior elements of the spine 1. Overall slight predilection for the posterior elements 2. Location of tumor is an important factor in determining the type of tumor E. Examples include: 1. Osteochondroma 2. Osteoid osteoma 3. Osteoblastoma 4. Aneurysmal bone cyst 5. Giant cell tumor 6. Eosinophilic granuloma II. Primary malignant tumors of the spine are: A. Fast-growing B. Permeative C . Usually occur in patients older than 21 years of age D. Examples include: 1. Multiple myeloma/solitary plasmacytoma 2. Osteosarcoma 3. C hondrosarcoma 4. Ewing's sarcoma/primitive neuroectodermal tumor 5. C hordoma 6. Lymphoma

Question 24

Topic: Bone Tumors

A 22-year-old male presents with chronic, dull back pain that is not relieved by NSAIDs. Imaging reveals a 3.5 cm expansile radiolucent lesion in the posterior elements of L3. What is the most likely diagnosis?

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

Correct Answer & Explanation

. Osteoblastoma


Explanation

Osteoblastomas are typically larger than 2 cm and usually occur in the posterior elements of the spine. Unlike osteoid osteomas, the pain is less likely to be strictly nocturnal and is poorly relieved by NSAIDs.

Question 25

Topic: Bone Tumors

Secondary aneurysmal bone cysts (ABCs) are most frequently associated with which of the following underlying primary bone tumors?

. Osteosarcoma
. Osteochondroma
. Osteoblastoma
. Enchondroma
. Multiple myeloma

Correct Answer & Explanation

. Osteoblastoma


Explanation

Secondary ABCs can develop in the setting of other primary bone tumors, most notably giant cell tumors, chondroblastomas, and osteoblastomas. Identifying the underlying precursor lesion is critical for appropriate surgical management.

Question 26

Topic: 10. Pathology and Oncology

Unlike osteoblastoma, which prefers the posterior elements, Giant Cell Tumor (GCT) of the spine most commonly originates in which anatomic location?

. Spinous process
. Lamina
. Pedicle
. Vertebral body
. Transverse process

Correct Answer & Explanation

. Vertebral body


Explanation

Giant Cell Tumors of the spine predominantly affect the vertebral body, often in the sacral region. This contrasts with osteoid osteomas, osteoblastomas, and ABCs, which typically arise in the posterior elements.

Question 27

Topic: 10. Pathology and Oncology

A 55-year-old male presents with bowel and bladder dysfunction. MRI shows a destructive midline mass in the sacrum. Biopsy reveals cells with abundant bubbly, vacuolated cytoplasm. What is the diagnosis?

. Chondrosarcoma
. Giant cell tumor
. Chordoma
. Ependymoma
. Plasmacytoma

Correct Answer & Explanation

. Chordoma


Explanation

Chordomas are locally aggressive malignant tumors arising from notochordal remnants, most commonly in the sacrum and clivus. Histology classically shows physaliferous cells with bubbly, vacuolated cytoplasm.

Question 28

Topic: 10. Pathology and Oncology

A 6-year-old boy presents with back pain. Radiographs demonstrate a symmetrically flattened vertebral body (vertebra plana) at T10 with preserved adjacent disc spaces. What is the most likely diagnosis?

. Ewing sarcoma
. Hemangioma
. Langerhans cell histiocytosis
. Osteoblastoma
. Osteomyelitis

Correct Answer & Explanation

. Langerhans cell histiocytosis


Explanation

Langerhans cell histiocytosis (eosinophilic granuloma) is the classic cause of vertebra plana in young children. The adjacent disc spaces are characteristically preserved, unlike in infectious disciitis.

Question 29

Topic: Bone Tumors

A 65-year-old male presents with a pathologic compression fracture of L4. Laboratory tests show a monoclonal gammopathy. Which imaging modality is least likely to show increased uptake in the affected vertebra?

. MRI with gadolinium
. FDG-PET scan
. Technetium-99m bone scan
. CT scan with contrast
. Sestamibi scan

Correct Answer & Explanation

. Technetium-99m bone scan


Explanation

Multiple myeloma and solitary plasmacytoma are characterized by purely osteolytic lesions with minimal reactive bone formation. Consequently, a standard Technetium-99m bone scan is often falsely negative (cold).

Question 30

Topic: 10. Pathology and Oncology

According to Enneking's principles of tumor resection, what is the most appropriate surgical treatment for an aggressive (Enneking Stage 3) osteoblastoma of the spine?

. Intralesional curettage alone
. Intralesional curettage with burr down and bone grafting
. En bloc resection with marginal or wide margins
. Radiation therapy alone
. Embolization followed by serial observation

Correct Answer & Explanation

. En bloc resection with marginal or wide margins


Explanation

Aggressive osteoblastomas (Stage 3) have a high rate of local recurrence and can behave unpredictably. En bloc resection with marginal or wide margins is recommended to minimize recurrence, provided neurologic morbidity is acceptable.

Question 31

Topic: 10. Pathology and Oncology

A 72-year-old male with a long history of Paget's disease develops worsening pain and a rapidly enlarging mass in his pelvis. What is the most likely malignant transformation?

. Chondrosarcoma
. Ewing sarcoma
. Osteosarcoma
. Fibrosarcoma
. Angiosarcoma

Correct Answer & Explanation

. Osteosarcoma


Explanation

Paget's disease carries an approximately 1% risk of malignant transformation. Secondary osteosarcoma is the most common malignancy and carries a significantly worse prognosis than primary osteosarcoma in younger patients.

Question 32

Topic: Bone Tumors

Primary aneurysmal bone cysts (ABCs) are neoplastic lesions driven by a specific genetic translocation. Which of the following genes is typically rearranged in primary ABCs?

. EXT1
. USP6
. GNAS
. RUNX2
. EWSR1

Correct Answer & Explanation

. USP6


Explanation

Primary ABCs are characterized by a t(16;17) translocation that causes upregulation of the USP6 gene. This true neoplastic nature distinguishes them from secondary ABCs, which lack this mutation.

Question 33

Topic: 10. Pathology and Oncology

Which of the following histologic features is most reliable for differentiating an aggressive osteoblastoma from a well-differentiated osteosarcoma?

. Presence of osteoid matrix
. High cellularity
. Permeation of tumor cells into surrounding host trabecular bone
. Prominent vascular stroma
. Presence of multinucleated giant cells

Correct Answer & Explanation

. Permeation of tumor cells into surrounding host trabecular bone


Explanation

Osteosarcomas classically demonstrate a destructive, permeative growth pattern invading the surrounding host trabeculae. In contrast, even aggressive osteoblastomas tend to have a distinct border and push rather than permeate host bone.

Question 34

Topic: Bone Tumors

A 14-year-old girl has a large, expansile aneurysmal bone cyst in the posterior elements of C5 causing neck pain but no neurologic deficit. What is the most appropriate initial surgical management?

. En bloc vertebrectomy
. Preoperative embolization followed by extended curettage and bone grafting
. Primary radiotherapy
. Serial observation
. Denosumab therapy

Correct Answer & Explanation

. Preoperative embolization followed by extended curettage and bone grafting


Explanation

Preoperative embolization significantly reduces intraoperative blood loss for spinal ABCs. Extended intralesional curettage with high-speed burring and bone grafting achieves a low recurrence rate while preserving spinal stability.

Question 35

Topic: 10. Pathology and Oncology

A 12-year-old boy presents with progressive back pain and fever. MRI shows a destructive mass in the L2 vertebral body with a large paraspinal soft tissue component. Biopsy shows small round blue cells expressing CD99. What is the most common initial treatment?

. Neoadjuvant multi-agent chemotherapy
. Immediate en bloc resection
. Primary radiation therapy alone
. High-dose corticosteroids
. Denosumab

Correct Answer & Explanation

. Neoadjuvant multi-agent chemotherapy


Explanation

Ewing sarcoma is a highly chemosensitive, systemic disease. The standard of care begins with neoadjuvant multi-agent chemotherapy, followed by local control (surgery or radiation), and subsequent adjuvant chemotherapy.

Question 36

Topic: 10. Pathology and Oncology

A 16-year-old boy presents with right thigh pain that is worse at night and dramatically relieved by ibuprofen. Plain radiographs show a dense cortical sclerosis with a 7-mm central radiolucent nidus. The intense pain associated with this lesion is primarily mediated by local production of which of the following?

. Tumor necrosis factor-alpha
. Interleukin-1
. Prostaglandin E2
. Bradykinin
. Substance P

Correct Answer & Explanation

. Prostaglandin E2


Explanation

Osteoid osteomas produce high levels of prostaglandins, specifically Prostaglandin E2, which is responsible for the characteristic severe, nocturnal pain. NSAIDs provide dramatic relief by inhibiting cyclooxygenase and subsequent prostaglandin synthesis.

Question 37

Topic: 10. Pathology and Oncology

A 14-year-old female presents with an expansile, eccentric lytic lesion in the distal femur metaphysis. MRI shows multiple fluid-fluid levels. Biopsy confirms an aneurysmal bone cyst (ABC). Primary ABCs are characterized by a genetic rearrangement involving which of the following genes?

. GNAS1
. EXT1
. USP6
. RUNX2
. COL1A1

Correct Answer & Explanation

. USP6


Explanation

Primary aneurysmal bone cysts frequently demonstrate rearrangements of the USP6 gene on chromosome 17p13. This genetic marker helps differentiate primary ABCs from secondary ABCs or other lytic bone lesions.

Question 38

Topic: 10. Pathology and Oncology

A 15-year-old male presents with chronic knee pain. Radiographs reveal a well-circumscribed, 1.5 cm radiolucent lesion in the epiphysis of the proximal tibia with a thin sclerotic rim. Histological examination reveals chondroblasts and "chicken-wire" calcification. What is the most appropriate definitive management?

. Observation with serial radiographs
. Radiofrequency ablation
. Intralesional curettage and bone grafting
. Wide surgical resection
. Radiation therapy

Correct Answer & Explanation

. Intralesional curettage and bone grafting


Explanation

Chondroblastoma is a benign cartilage tumor characteristically located in the epiphysis of skeletally immature patients. The standard treatment is extended intralesional curettage, burring, and bone grafting, which provides excellent local control.

Question 39

Topic: Bone Tumors

A 9-year-old girl is evaluated for a shepherd's crook deformity of the proximal femur, multiple cafe-au-lait spots with irregular borders, and precocious puberty. Which of the following mutations is responsible for this condition?

. Post-zygotic activating mutation of the GNAS1 gene
. Germline mutation of the RB1 gene
. Inactivating mutation of the EXT1 gene
. Translocation t(11;22)
. Mutation in the FGFR3 gene

Correct Answer & Explanation

. Post-zygotic activating mutation of the GNAS1 gene


Explanation

The patient has McCune-Albright syndrome, characterized by polyostotic fibrous dysplasia, cafe-au-lait macules, and endocrine abnormalities. It is caused by a somatic, post-zygotic activating mutation in the GNAS1 gene leading to increased intracellular cAMP.

Question 40

Topic: 10. Pathology and Oncology

A 5-year-old boy presents with anterior bowing of his tibia. Radiographs demonstrate a multilocular, lucent lesion within the anterior cortex of the tibial diaphysis. Biopsy shows fibrous tissue with spicules of woven bone lined by osteoblasts. Which of the following malignant tumors is most closely associated with this condition and must be ruled out?

. Osteosarcoma
. Ewing sarcoma
. Chondrosarcoma
. Adamantinoma
. Chordoma

Correct Answer & Explanation

. Adamantinoma


Explanation

Osteofibrous dysplasia (Campanacci disease) typically affects the anterior tibial cortex in children under 10. It is histologically and clinically linked to adamantinoma, a low-grade malignant bone tumor that must be excluded via biopsy.