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Question 3401

Topic: 10. Pathology and Oncology

A 60-year-old male presents with dull right hip pain. Radiographs show a large lytic lesion with 'popcorn' calcifications in the right ilium. Biopsy confirms a grade II chondrosarcoma. What is the mainstay of treatment for this condition?

. Neoadjuvant chemotherapy followed by wide surgical resection
. Wide surgical resection alone
. Radiation therapy and denosumab
. Intralesional curettage with adjuvant phenol and bone grafting
. Primary hemipelvectomy amputation

Correct Answer & Explanation

. Wide surgical resection alone


Explanation

Conventional chondrosarcomas are notably resistant to both chemotherapy and radiation therapy. The primary and most effective treatment for intermediate to high-grade chondrosarcoma is wide surgical resection with negative margins.

Question 3402

Topic: Bone Tumors

A 16-year-old male complains of severe, aching thigh pain that is worse at night and dramatically relieved by NSAIDs. Radiographs demonstrate a 1-cm radiolucent nidus surrounded by dense sclerotic bone in the femoral diaphysis. What is the preferred definitive treatment if medical management fails?

. Wide en bloc resection
. Intralesional curettage and bone grafting
. Radiofrequency ablation (RFA)
. Extended course of systemic chemotherapy
. External beam radiation therapy

Correct Answer & Explanation

. Radiofrequency ablation (RFA)


Explanation

The clinical and radiographic presentation is classic for an osteoid osteoma. If medical management (NSAIDs) is unsuccessful or poorly tolerated, CT-guided radiofrequency ablation (RFA) is the minimally invasive treatment of choice.

Question 3403

Topic: 10. Pathology and Oncology

A 65-year-old woman presents with severe back pain. Radiographs reveal multiple punched-out lytic lesions in the skull and a compression fracture of L2. Laboratory workup shows hypercalcemia and a monoclonal spike. What is the most common primary bone malignancy in this age group?

. Chondrosarcoma
. Osteosarcoma
. Multiple Myeloma
. Metastatic breast carcinoma
. Ewing sarcoma

Correct Answer & Explanation

. Multiple Myeloma


Explanation

Multiple myeloma is the most common primary malignancy of bone overall, particularly in adults over the age of 50. While metastatic bone disease is generally more common, myeloma represents the most common primary tumor arising from the marrow.

Question 3404

Topic: 10. Pathology and Oncology

A 14-year-old boy is diagnosed with conventional high-grade osteosarcoma of the distal femur. Genetic testing reveals a mutation in the TP53 gene. Which of the following syndromes is most closely associated with this patient's diagnosis?

. Li-Fraumeni syndrome
. Hereditary multiple exostoses
. McCune-Albright syndrome
. Maffucci syndrome
. Retinoblastoma

Correct Answer & Explanation

. Li-Fraumeni syndrome


Explanation

Li-Fraumeni syndrome is an autosomal dominant disorder caused by mutations in the TP53 tumor suppressor gene. It heavily predisposes patients to osteosarcoma, breast cancer, and soft tissue sarcomas.

Question 3405

Topic: 10. Pathology and Oncology
A 60-year-old male presents with deep thigh pain. Radiographs reveal a large, permeative lytic lesion with "popcorn" calcifications in the proximal femur. Biopsy confirms grade III conventional chondrosarcoma. What is the most appropriate management?
. Neoadjuvant chemotherapy followed by wide resection
. Wide surgical resection alone
. Radiation therapy alone
. Intralesional curettage and cementation
. Denosumab therapy followed by excision

Correct Answer & Explanation

. Wide surgical resection alone


Explanation

Conventional chondrosarcomas are notoriously resistant to both chemotherapy and radiation. The mainstay of treatment for high-grade (Grade III) conventional chondrosarcoma is wide surgical resection alone.

Question 3406

Topic: 10. Pathology and Oncology

A 12-year-old girl presents with a destructive diaphyseal lesion of her tibia with an associated "onion-skin" periosteal reaction. A biopsy is performed, and molecular analysis demonstrates a t(11;22) chromosomal translocation. Which of the following fusion proteins is highly specific to this tumor?

. SYT-SSX1
. EWS-FLI1
. TLS-CHOP
. PAX3-FKHR
. COL1A1-PDGFB

Correct Answer & Explanation

. EWS-FLI1


Explanation

Ewing sarcoma is classically characterized by the t(11;22) translocation, resulting in the EWS-FLI1 fusion transcript. SYT-SSX is seen in synovial sarcoma, and TLS-CHOP in myxoid liposarcoma.

Question 3407

Topic: 10. Pathology and Oncology

A 15-year-old male is suspected of having an osteosarcoma of the proximal tibia. A core needle biopsy is planned. To adhere to standard orthopedic oncology principles and avoid compromising definitive limb-salvage surgery, which of the following is an absolute requirement for the biopsy tract?

. It must traverse the closest joint capsule to ensure intra-articular sampling.
. It must utilize a transverse incision to follow Langer's lines.
. It must be placed in line with the planned definitive surgical incision so it can be excised entirely.
. It should pass through the adjacent major neurovascular bundle to minimize muscle damage.
. It should be closed with buried absorbable sutures to prevent tract seeding.

Correct Answer & Explanation

. It must be placed in line with the planned definitive surgical incision so it can be excised entirely.


Explanation

Biopsy tracts for suspected malignant bone tumors are considered contaminated with tumor cells. They must be placed longitudinally and strictly in line with the planned definitive surgical approach so the entire tract can be excised en bloc during resection.

Question 3408

Topic: 10. Pathology and Oncology

A 25-year-old male presents with a slow-growing, deep mass near his knee. Biopsy reveals a biphasic pattern of epithelial and spindle cells. Which of the following cytogenetic abnormalities is pathognomonic for this tumor?

. t(11;22)(q24;q12)
. t(X;18)(p11;q11)
. t(12;16)(q13;p11)
. t(2;13)(q35;q14)
. t(9;22)(q22;q12)

Correct Answer & Explanation

. t(X;18)(p11;q11)


Explanation

Synovial sarcoma is characterized by the t(X;18)(p11;q11) translocation, resulting in the SYT-SSX fusion gene. It classically presents as a deep mass near a joint and exhibits either biphasic or monophasic histology.

Question 3409

Topic: 10. Pathology and Oncology

A 45-year-old male complains of knee pain, swelling, and mechanical locking. Radiographs show multiple intra-articular radiopaque bodies of uniform size.

What is the primary underlying pathophysiology of this condition?

. Synovial metaplasia
. Fragmentation of osteoarthritic spurs
. Malignant transformation of synovium
. Deposition of calcium pyrophosphate
. Overgrowth of Hoffa's fat pad

Correct Answer & Explanation

. Synovial metaplasia


Explanation

Primary synovial chondromatosis results from benign metaplasia of the synovial membrane, producing multiple uniform cartilaginous nodules. These nodules frequently ossify, making them visible on standard radiographs.

Question 3410

Topic: 10. Pathology and Oncology

A 35-year-old female undergoes marginal excision of a large intramuscular mass in her anterior thigh. Pathology reveals myxoid stroma, a prominent capillary network, and lipoblasts. Which genetic abnormality is most likely present in this tumor?

. MDM2 amplification
. t(11;22)(q24;q12)
. t(12;16)(q13;p11)
. t(X;18)(p11;q11)
. APC gene mutation

Correct Answer & Explanation

. t(12;16)(q13;p11)


Explanation

Myxoid liposarcoma is defined by the t(12;16)(q13;p11) translocation, which creates the FUS-DDIT3 fusion protein. It features a characteristic chicken-wire capillary network and myxoid background on histology.

Question 3411

Topic: 10. Pathology and Oncology

A 22-year-old male presents with a painless, ulcerating nodule on his volar forearm that has been enlarging over several months. It was initially excised under the presumed diagnosis of a ganglion cyst but rapidly recurred. Immunohistochemistry of the lesion is expected to show loss of expression of which of the following markers?

. INI1
. MDM2
. Beta-catenin
. S100
. CD99

Correct Answer & Explanation

. INI1


Explanation

Epithelioid sarcoma classically presents as an ulcerating, slow-growing nodule in the distal extremities of young adults and is frequently misdiagnosed. It is immunohistochemically characterized by the loss of INI1 (SMARCB1) expression.

Question 3412

Topic: 10. Pathology and Oncology

While most soft tissue sarcomas metastasize hematogenously to the lungs, a specific subset has a higher propensity for regional lymph node metastasis. Which of the following soft tissue tumors requires the most vigilant clinical examination of regional lymph nodes?

. Myxoid liposarcoma
. Pleomorphic liposarcoma
. Clear cell sarcoma
. Undifferentiated pleomorphic sarcoma
. Fibrosarcoma

Correct Answer & Explanation

. Clear cell sarcoma


Explanation

Clear cell sarcoma has a high propensity for lymph node metastasis. The mnemonic 'SCARE' (Synovial, Clear cell, Angiosarcoma, Rhabdomyosarcoma, Epithelioid) identifies the sarcomas most likely to spread to lymph nodes.

Question 3413

Topic: 10. Pathology and Oncology

In the multidisciplinary management of a high-grade soft tissue sarcoma of the extremity, a decision is made to utilize preoperative rather than postoperative radiation therapy.

What is the primary disadvantage of this specific approach?

. Higher total radiation dose required
. Increased rate of major wound complications
. Greater long-term joint fibrosis
. Decreased local recurrence control
. Increased risk of secondary malignancy

Correct Answer & Explanation

. Increased rate of major wound complications


Explanation

Preoperative radiation therapy is associated with a significantly higher rate of major wound complications compared to postoperative radiation. However, it requires a lower total dose and results in less long-term limb edema and fibrosis.

Question 3414

Topic: 10. Pathology and Oncology

A 28-year-old female presents with a slow-growing right thigh mass and new-onset focal seizures. Brain MRI demonstrates multiple contrast-enhancing lesions. Which soft tissue sarcoma is notoriously associated with early brain metastases despite a relatively small primary tumor?

. Synovial sarcoma
. Alveolar soft part sarcoma
. Undifferentiated pleomorphic sarcoma
. Desmoplastic small round cell tumor
. Epithelioid sarcoma

Correct Answer & Explanation

. Alveolar soft part sarcoma


Explanation

Alveolar soft part sarcoma is uniquely known for its high propensity to metastasize to the brain, often presenting with neurologic symptoms before the primary soft tissue mass is identified. It is characterized by the ASPSCR1-TFE3 fusion gene.

Question 3415

Topic: 10. Pathology and Oncology

A 32-year-old female notes a deep palpable mass in her abdominal wall. Biopsy reveals uniform spindle cells in a collagenous stroma without nuclear atypia, and immunohistochemistry is strongly positive for nuclear beta-catenin. What is the most appropriate initial systemic therapy if the mass is symptomatic and progressively enlarging?

. Doxorubicin and Ifosfamide
. Sorafenib or Pazopanib
. High-dose methotrexate
. Denosumab
. Surgical amputation

Correct Answer & Explanation

. Sorafenib or Pazopanib


Explanation

Desmoid tumors (aggressive fibromatoses) are locally invasive, non-metastasizing tumors characterized by beta-catenin mutations. Tyrosine kinase inhibitors (like Sorafenib) or low-dose chemotherapy are favored for progressive, symptomatic disease.

Question 3416

Topic: 10. Pathology and Oncology

A 50-year-old patient undergoes a diagnostic core needle biopsy for a suspicious 8 cm deep mass in the vastus lateralis.

Which of the following principles MUST be adhered to during the biopsy?

. The biopsy tract should be transverse to allow maximal healing
. The biopsy should traverse adjacent uninvolved muscle compartments to safely reach the mass
. The biopsy tract must be planned so it can be excised en bloc during definitive resection
. A tourniquet should be inflated and exsanguinated prior to needle insertion
. The sample should exclusively target the necrotic center of the mass

Correct Answer & Explanation

. The biopsy tract must be planned so it can be excised en bloc during definitive resection


Explanation

The core biopsy tract is considered contaminated with tumor cells and must be excised en bloc with the primary tumor during definitive surgery. Therefore, the tract must be carefully planned in line with the future longitudinal surgical incision.

Question 3417

Topic: 10. Pathology and Oncology

A 60-year-old male undergoes excision of a large, deep fatty mass in his thigh. The pathologist must differentiate between a deep lipoma and an atypical lipomatous tumor. Amplification of which of the following genes confirms the diagnosis of an atypical lipomatous tumor?

. P53
. MDM2
. c-MYC
. RUNX2
. SOX9

Correct Answer & Explanation

. MDM2


Explanation

Atypical lipomatous tumors (well-differentiated liposarcomas) are defined by the amplification of MDM2 and CDK4 genes on chromosome 12. This molecular signature reliably distinguishes them from benign deep lipomas.

Question 3418

Topic: 10. Pathology and Oncology

A 6-year-old boy presents with a rapidly expanding mass in his forearm. Biopsy confirms an alveolar rhabdomyosarcoma. Which of the following best describes the typical fusion gene associated with this specific histologic subtype?

. PAX3-FOXO1
. SYT-SSX1
. EWSR1-FLI1
. FUS-DDIT3
. ETV6-NTRK3

Correct Answer & Explanation

. PAX3-FOXO1


Explanation

Alveolar rhabdomyosarcoma is driven by t(2;13) or t(1;13) translocations, which result in PAX3-FOXO1 or PAX7-FOXO1 fusion proteins. The alveolar subtype generally carries a worse prognosis than the embryonal variant.

Question 3419

Topic: 10. Pathology and Oncology

A patient with a 10-year history of known stable primary synovial chondromatosis of the hip presents with rapid onset of severe resting pain and cortical bone erosion on radiographs. What is the most likely complication driving these new symptoms?

. Joint space infection
. Malignant transformation to secondary chondrosarcoma
. Spontaneous hemarthrosis
. Severe rapidly progressive osteoarthritis
. Transformation to synovial sarcoma

Correct Answer & Explanation

. Malignant transformation to secondary chondrosarcoma


Explanation

While rare, primary synovial chondromatosis can undergo malignant transformation to secondary chondrosarcoma. Rapid growth, new severe rest pain, and uncharacteristic bone destruction are hallmark signs warranting immediate biopsy.

Question 3420

Topic: 10. Pathology and Oncology

A 65-year-old woman presents with a rapidly growing mass in her left axilla. She had breast-conserving surgery and localized radiation therapy for breast cancer 12 years ago. Biopsy reveals a high-grade undifferentiated pleomorphic sarcoma. According to Cahan's criteria, which of the following is essential for diagnosing a radiation-induced sarcoma?

. The sarcoma must be of a different histologic type than the primary tumor
. The latent period must be less than 2 years
. The patient must possess an underlying p53 mutation
. The new tumor must occur outside the prior radiation field
. The tumor must exhibit a low-grade histology

Correct Answer & Explanation

. The sarcoma must be of a different histologic type than the primary tumor


Explanation

Cahan's criteria for radiation-induced sarcomas require that the sarcoma arises within the prior radiation field, has a different histology than the primary tumor, and has a sufficient latency period (typically greater than 5 years).