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Question 101

Topic: 10. Pathology and Oncology

The Weinstein-Boriani-Biagini (WBB) surgical staging system for spinal tumors is primarily designed to:

. Determine the radiosensitivity of metastatic lesions
. Determine the feasibility and anatomical approach for en bloc resection of primary spinal tumors
. Predict the systemic survival rate of patients with multiple myeloma
. Classify the degree of epidural spinal cord compression
. Assess the neurological recovery potential after trauma

Correct Answer & Explanation

. Determine the feasibility and anatomical approach for en bloc resection of primary spinal tumors


Explanation

The WBB staging system divides the vertebra into 12 radiating clock-face zones and multiple tissue layers. It is used specifically to plan complex en bloc resections for primary malignant or aggressive benign spinal tumors.

Question 102

Topic: Bone Tumors

A 62-year-old man presents with diffuse bone pain and fatigue. Radiographs show multiple punched-out lytic lesions in the skull and spine. A technetium-99m bone scan is performed. What is the expected finding on the bone scan?

. Intensely 'hot' diffuse skeletal uptake
. Superscan appearance
. Normal uptake or 'cold' spots in the areas of lytic lesions
. Increased uptake specifically localized to the appendicular skeleton
. Asymmetric uptake limited to the joints

Correct Answer & Explanation

. Normal uptake or 'cold' spots in the areas of lytic lesions


Explanation

Multiple myeloma characteristically lacks osteoblastic activity. Therefore, despite extensive lytic bone destruction, a standard technetium-99m bone scan often appears normal or shows 'cold' (photopenic) defects rather than increased uptake.

Question 103

Topic: 10. Pathology and Oncology

A 45-year-old man is diagnosed with a solitary plasmacytoma of the L3 vertebra without systemic involvement (normal bone marrow biopsy and no M-spike on SPEP). He has minimal localized pain and no neurological deficits. What is the definitive treatment?

. En bloc resection
. Local definitive radiation therapy
. Observation and sequential MRI
. High-dose systemic chemotherapy with autologous stem cell rescue
. Intralesional curettage

Correct Answer & Explanation

. Local definitive radiation therapy


Explanation

Solitary bone plasmacytomas are highly radiosensitive. Definitive local radiation therapy is the standard of care and provides excellent local control, though many patients eventually progress to multiple myeloma.

Question 104

Topic: 10. Pathology and Oncology

A 50-year-old man presents with severe mid-back pain. CT shows a large lytic mass in the T10 vertebral body with areas of 'rings and arcs' calcifications extending into the epidural space. Biopsy confirms primary conventional chondrosarcoma. What is the most appropriate management?

. Intralesional excision and radiation
. Neoadjuvant chemotherapy followed by curettage
. En bloc wide excision
. Primary stereotactic radiosurgery
. Palliative decompression only

Correct Answer & Explanation

. En bloc wide excision


Explanation

Conventional chondrosarcoma is both radioresistant and chemoresistant. The only curative treatment option is complete en bloc wide excision with negative margins.

Question 105

Topic: Bone Tumors

Osteoblastoma and osteoid osteoma of the spine share similar histologic appearances. Which of the following clinical or radiographic features most reliably distinguishes an osteoblastoma from an osteoid osteoma?

. Location exclusively in the anterior vertebral body
. Lesion size greater than 2 centimeters
. Complete relief of pain with NSAIDs
. Presence of a dense reactive bone sclerosis out of proportion to the nidus
. Predominance in elderly females

Correct Answer & Explanation

. Lesion size greater than 2 centimeters


Explanation

Osteoblastoma is histologically identical to osteoid osteoma but is distinguished by a nidus larger than 1.5 to 2.0 cm. It is also less likely to be completely relieved by NSAIDs and has a higher tendency for local aggression.

Question 106

Topic: 10. Pathology and Oncology

A 30-year-old patient with neurofibromatosis type 1 (NF-1) is evaluated for a new spinal nerve root tumor. Biopsy reveals a spindle cell neoplasm. Which of the following histologic or anatomical characteristics differentiates a neurofibroma from a schwannoma?

. Antoni A and Antoni B tissue patterns
. Verocay bodies
. Exclusive origin from the ventral root
. Incorporation of the parent nerve fibers within the tumor mass
. Strong and uniform S-100 positivity throughout all cells

Correct Answer & Explanation

. Incorporation of the parent nerve fibers within the tumor mass


Explanation

Neurofibromas intertwine with and incorporate the native nerve fibers, making them difficult to resect without sacrificing the nerve. Schwannomas are eccentrically located and push the nerve fibers aside, allowing for potentially nerve-sparing enucleation.

Question 107

Topic: 10. Pathology and Oncology

A 12-year-old boy presents with progressive paraparesis, fever, and weight loss. Imaging demonstrates a permeative destructive lesion of the T6 vertebra with a large paraspinal soft tissue mass. Biopsy reveals sheets of uniform, small round blue cells that are strongly positive for CD99. What cytogenetic abnormality is characteristic of this tumor?

. t(11;22)
. t(X;18)
. t(9;22)
. t(2;13)
. t(12;16)

Correct Answer & Explanation

. t(11;22)


Explanation

Ewing sarcoma is characterized by small round blue cells expressing CD99. The hallmark cytogenetic abnormality is the t(11;22)(q24;q12) translocation, resulting in the EWS-FLI1 fusion protein.

Question 108

Topic: 10. Pathology and Oncology

Regarding the neurological outcome following surgery for spinal intramedullary tumors, which of the following preoperative factors is the most consistent predictor of a patient's long-term functional status?

. Age of the patient at the time of presentation
. Preoperative neurological grade or status
. Duration of symptoms prior to diagnosis
. The specific histologic grade of the tumor
. Use of intraoperative neurophysiological monitoring

Correct Answer & Explanation

. Preoperative neurological grade or status


Explanation

The preoperative neurological status is the strongest and most consistent predictor of functional outcome after the resection of intramedullary spinal cord tumors. Patients with minimal deficits before surgery are most likely to maintain good function postoperatively.

Question 109

Topic: 10. Pathology and Oncology

A 60-year-old woman is found to have an intradural extramedullary mass in the thoracic spine causing mild cord compression. MRI demonstrates a "dural tail" sign and homogeneous enhancement with gadolinium. What is the most appropriate initial surgical management?

. Incisional biopsy and radiotherapy
. Preoperative embolization followed by en bloc resection
. Gross total resection with dural excision or coagulation
. Stereotactic radiosurgery alone
. Laminectomy without tumor resection

Correct Answer & Explanation

. Gross total resection with dural excision or coagulation


Explanation

The clinical and radiographic presentation is classic for a spinal meningioma. The gold standard treatment is gross total resection, which should include excision or extensive coagulation of the dural attachment to minimize recurrence.

Question 110

Topic: Soft Tissue Tumors & Metastasis

A 35-year-old man presents with progressive back pain and lower extremity paresthesias. MRI demonstrates an enhancing intramedullary nodule with a large associated cyst. He also has a history of retinal angiomas. Which of the following genetic syndromes is most closely associated with this patient's spinal lesion?

. Neurofibromatosis type 1
. Neurofibromatosis type 2
. Tuberous sclerosis
. Von Hippel-Lindau disease
. Li-Fraumeni syndrome

Correct Answer & Explanation

. Von Hippel-Lindau disease


Explanation

The patient's presentation suggests a spinal hemangioblastoma, characterized by an enhancing mural nodule and an associated cyst. Hemangioblastomas are a hallmark of Von Hippel-Lindau (VHL) disease, which also presents with retinal angiomas and renal cell carcinomas.

Question 111

Topic: 10. Pathology and Oncology

A 12-year-old boy presents with severe thoracic back pain and progressive myelopathy. MRI reveals a solid, poorly circumscribed intramedullary lesion extending over five vertebral levels with patchy enhancement. What is the most appropriate primary surgical treatment?

. En bloc resection with clear margins
. Radiosurgery without biopsy
. Biopsy, maximal safe debulking, and adjuvant therapies
. Preoperative embolization followed by observation
. Observation and bracing until skeletal maturity

Correct Answer & Explanation

. Biopsy, maximal safe debulking, and adjuvant therapies


Explanation

Astrocytomas are the most common intramedullary tumors in children and are typically infiltrative and poorly circumscribed. Gross total resection is rarely possible without significant neurologic morbidity, so biopsy, maximal safe debulking, and adjuvant therapy are the standard of care.

Question 112

Topic: 10. Pathology and Oncology

A 55-year-old man presents with intractable mechanical lower back pain. Imaging shows a lytic lesion involving the L3 vertebral body, and biopsy confirms metastatic renal cell carcinoma. The Spinal Instability Neoplastic Score (SINS) is 14. Which of the following is the most crucial step prior to surgical decompression and stabilization?

. Systemic chemotherapy
. Preoperative selective arterial embolization
. Stereotactic body radiation therapy
. Administration of high-dose corticosteroids
. Proton beam therapy

Correct Answer & Explanation

. Preoperative selective arterial embolization


Explanation

Renal cell carcinoma and thyroid carcinoma metastases to the spine are highly vascular. Preoperative selective arterial embolization is critically important to minimize life-threatening intraoperative blood loss during decompression and stabilization.

Question 113

Topic: Bone Tumors

A 16-year-old girl presents with a rigid thoracic scoliosis and night pain relieved by ibuprofen. Imaging reveals a sclerotic lesion with a central lucent nidus in the left T9 pedicle. In which direction will the apex of her scoliosis typically point?

. Anteriorly, creating an extreme focal kyphosis
. Away from the side of the lesion
. Towards the side of the lesion
. Posteriorly, creating a focal lordosis
. Randomly, with no predictable correlation to the lesion side

Correct Answer & Explanation

. Away from the side of the lesion


Explanation

The lesion is an osteoid osteoma. Muscle spasm secondary to the inflammatory mediators (prostaglandins) released by the nidus causes a convex deformity, so the apex of the scoliosis points away from the side of the lesion.

Question 114

Topic: 10. Pathology and Oncology

A 25-year-old woman has an expansile, radiolucent lesion involving the L2 posterior elements. MRI shows multiple fluid-fluid levels. A biopsy confirms a benign, blood-filled cystic lesion. Which genetic alteration is commonly associated with this primary pathology?

. USP6 gene rearrangement
. GNAS somatic mutation
. H3F3A mutation
. EXT1 mutation
. NF2 gene microdeletion

Correct Answer & Explanation

. USP6 gene rearrangement


Explanation

The lesion is an aneurysmal bone cyst (ABC), characterized by fluid-fluid levels on MRI. Primary ABCs are neoplasms frequently driven by a translocation involving the USP6 gene on chromosome 17.

Question 115

Topic: 10. Pathology and Oncology

A 65-year-old man presents with progressive lower extremity paraparesis. MRI reveals an extradural mass causing severe cord compression at T10. Histology confirms multiple myeloma. According to the Patchell criteria, which of the following is an absolute indication for surgical decompression over primary radiotherapy?

. The presence of a single, radiosensitive lesion
. A known primary tissue diagnosis of myeloma
. Spinal instability or bone fragments compressing the cord
. Life expectancy of less than 3 months
. Paraplegia present and stable for more than 48 hours

Correct Answer & Explanation

. Spinal instability or bone fragments compressing the cord


Explanation

While multiple myeloma is highly radiosensitive, indications for surgery over primary radiation include spinal instability, bony compression of the cord, progression despite radiation, or need for open biopsy. Mechanical instability always requires surgical stabilization.

Question 116

Topic: 10. Pathology and Oncology

A 42-year-old male undergoes surgery for a mass of the filum terminale. Histology confirms a myxopapillary ependymoma. What is the most critical intraoperative technical factor to prevent tumor recurrence?

. Avoidance of somatosensory evoked potentials (SSEPs) monitoring
. Performing a wide dural resection above the conus
. Maintaining the integrity of the tumor capsule during en bloc resection
. Intraoperative injection of intrathecal chemotherapy
. Use of a high-speed burr to completely morcellate the mass

Correct Answer & Explanation

. Maintaining the integrity of the tumor capsule during en bloc resection


Explanation

Myxopapillary ependymomas are WHO grade I tumors but have a high propensity for local recurrence and cerebrospinal fluid (CSF) seeding if the tumor capsule is violated. En bloc resection with an intact capsule is paramount for minimizing recurrence.

Question 117

Topic: 10. Pathology and Oncology

A 55-year-old female with breast cancer presents with a metastatic lesion to the L1 vertebral body compressing the thecal sac without a neurological deficit (Bilsky grade 1c). What is the accepted paradigm for "separation surgery" in this patient prior to stereotactic body radiation therapy (SBRT)?

. En bloc total spondylectomy with clear margins
. Circumferential epidural decompression to create a 2-3 mm gap from the dura
. Laminectomy alone without instrumentation
. Percutaneous vertebroplasty followed by standard external beam radiation
. Intralesional injection of radioactive isotopes

Correct Answer & Explanation

. Circumferential epidural decompression to create a 2-3 mm gap from the dura


Explanation

Separation surgery aims to decompress the spinal cord, creating a safe margin (usually 2-3 mm) between the dura and the tumor. This allows for the subsequent delivery of ablative high-dose SBRT without causing radiation necrosis to the spinal cord.

Question 118

Topic: 10. Pathology and Oncology

Which of the following intradural extramedullary spinal tumors is classically described histologically by alternating regions of high cellularity (Antoni A) and low cellularity (Antoni B) with palisading Verocay bodies?

. Meningioma
. Ependymoma
. Schwannoma
. Neurofibroma
. Hemangioblastoma

Correct Answer & Explanation

. Schwannoma


Explanation

Schwannomas are benign peripheral nerve sheath tumors that commonly present as intradural extramedullary spinal masses. Their classic histologic appearance features dense Antoni A areas containing Verocay bodies, alongside looser, myxoid Antoni B areas.

Question 119

Topic: 10. Pathology and Oncology

Which of the following is the most common primary intramedullary spinal cord tumor in adults?

. Astrocytoma
. Ependymoma
. Schwannoma
. Meningioma
. Hemangioblastoma

Correct Answer & Explanation

. Ependymoma


Explanation

Ependymomas are the most common primary intramedullary spinal cord tumors in adults, whereas astrocytomas are the most common in children.

Question 120

Topic: 10. Pathology and Oncology

A 14-year-old boy presents with a painful thoracic scoliosis. He reports the pain is worse at night and dramatically improves with ibuprofen. Radiographs and CT are most likely to show a nidus located in the:

. Vertebral body on the convex side of the curve
. Vertebral body on the concave side of the curve
. Posterior elements on the convex side of the curve
. Posterior elements on the concave side of the curve
. Intervertebral disc space

Correct Answer & Explanation

. Posterior elements on the concave side of the curve


Explanation

Osteoid osteomas in the spine typically present with painful scoliosis. The tumor is almost always located in the posterior elements on the concave side of the scoliotic curve.