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10. Pathology and Oncology MCQs

Practice Set 56 of 351

This practice set contains high-yield board review questions covering key concepts in 10. Pathology and Oncology. Each clinical scenario is designed to test your diagnostic and management skills relevant to this subspecialty.

Question 1101

Topic: 10. Pathology and Oncology

A 15-year-old boy presents with progressive knee pain. Radiographs reveal a mixed lytic and sclerotic lesion in the distal femur metaphysis with a 'sunburst' periosteal reaction. Core needle biopsy confirms high-grade, intramedullary osteosarcoma without evidence of metastasis. What is the most appropriate management strategy?

. Immediate primary amputation
. Neoadjuvant chemotherapy followed by wide surgical resection and adjuvant chemotherapy
. Wide surgical resection followed by adjuvant radiation therapy
. Intralesional curettage, bone grafting, and prophylactic internal fixation
. Neoadjuvant radiation therapy followed by wide surgical resection

Correct Answer & Explanation

. Neoadjuvant chemotherapy followed by wide surgical resection and adjuvant chemotherapy

Explanation

High-grade osteosarcoma is managed with neoadjuvant chemotherapy, followed by wide surgical resection, and then adjuvant chemotherapy. The histologic response to neoadjuvant chemotherapy is the most critical prognostic indicator.

Question 1102

Topic: 10. Pathology and Oncology

A 12-year-old girl presents with a destructive diaphyseal lesion in her fibula with an 'onion-skin' periosteal reaction. A biopsy is performed, and molecular studies are requested. Which of the following chromosomal translocations is diagnostic for this patient's most likely condition?

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

Correct Answer & Explanation

. t(11;22)

Explanation

The patient's presentation is classic for Ewing sarcoma. The t(11;22) translocation, resulting in the EWS-FLI1 fusion protein, is found in approximately 85% of Ewing sarcoma cases.

Question 1103

Topic: 10. Pathology and Oncology

A 60-year-old male presents with deep groin pain. Radiographs reveal a large destructive lesion in the right ilium with stippled 'popcorn' calcifications extending into the soft tissues. Biopsy confirms a grade II (intermediate grade) conventional chondrosarcoma. What is the recommended definitive treatment?

. Systemic chemotherapy alone
. External beam radiation therapy alone
. Neoadjuvant chemotherapy followed by wide surgical resection
. Wide surgical resection alone
. Intralesional curettage, adjuvant phenol, and cementation

Correct Answer & Explanation

. Wide surgical resection alone

Explanation

Conventional chondrosarcomas are notoriously resistant to both chemotherapy and radiation therapy. Wide surgical resection with negative margins is the definitive and most effective treatment for intermediate- and high-grade chondrosarcomas.

Question 1104

Topic: Bone Tumors

A 68-year-old female presents with persistent back pain and generalized fatigue. Laboratory tests show hypercalcemia and mild anemia. Radiographs demonstrate multiple 'punched-out' lytic lesions in her skull and pelvis. Which of the following laboratory tests is the most sensitive initial screening tool for her most likely diagnosis?

. Serum and urine protein electrophoresis (SPEP/UPEP)
. Serum alkaline phosphatase
. Prostate-specific antigen (PSA)
. Carcinoembryonic antigen (CEA)
. Cancer antigen 125 (CA-125)

Correct Answer & Explanation

. Serum and urine protein electrophoresis (SPEP/UPEP)

Explanation

Multiple myeloma is the most common primary bone malignancy in adults. Serum and urine protein electrophoresis (SPEP/UPEP) with immunofixation are the most appropriate initial diagnostic tests to detect the characteristic monoclonal protein spike.

Question 1105

Topic: 10. Pathology and Oncology

A 32-year-old female presents with knee pain. Radiographs reveal an eccentric, expansile, lytic lesion in the epiphysis of her distal femur extending to the subchondral bone. Biopsy confirms a giant cell tumor of bone. For massive or unresectable giant cell tumors, which targeted pharmacologic therapy has shown significant efficacy in arresting tumor progression?

. Imatinib (tyrosine-kinase inhibitor)
. Denosumab (RANKL monoclonal antibody)
. Rituximab (anti-CD20 monoclonal antibody)
. Methotrexate (folate antimetabolite)
. Bevacizumab (anti-VEGF monoclonal antibody)

Correct Answer & Explanation

. Denosumab (RANKL monoclonal antibody)

Explanation

Giant cell tumors of bone are driven by the overexpression of RANK-ligand (RANKL) by the neoplastic stromal cells, leading to osteoclast recruitment. Denosumab, a monoclonal antibody against RANKL, is highly effective for unresectable or recurrent giant cell tumors.

Question 1106

Topic: Bone Tumors

A 19-year-old male complains of severe, progressively worsening mid-thigh pain that awakens him at night but is dramatically relieved by ibuprofen. A CT scan reveals a 1 cm radiolucent nidus surrounded by dense reactive sclerosis in the femoral diaphysis. What is the most appropriate, minimally invasive definitive treatment?

. Wide en bloc resection and intercalary allograft
. Intralesional curettage and bone grafting
. Neoadjuvant chemotherapy followed by curettage
. Radiofrequency ablation (RFA) of the nidus
. External beam radiation therapy

Correct Answer & Explanation

. Radiofrequency ablation (RFA) of the nidus

Explanation

The clinical and radiographic presentation is classic for an osteoid osteoma. Percutaneous radiofrequency ablation (RFA) is the current gold standard treatment, providing excellent symptom relief with minimal morbidity compared to open surgical excision.

Question 1107

Topic: 10. Pathology and Oncology

A 62-year-old female with a history of breast cancer presents with increasing right thigh pain. Radiographs demonstrate a large, lytic lesion in the subtrochanteric region of the femur causing a 60% loss of cortical diameter. Her Mirels score is calculated at 10. What is the most appropriate management?

. Immediate initiation of systemic bisphosphonates and strict non-weight-bearing
. External beam radiation therapy alone
. Prophylactic cephalomedullary nailing followed by radiation therapy
. Wide en bloc resection and proximal femoral replacement
. Observation with serial radiographs every 3 months

Correct Answer & Explanation

. Prophylactic cephalomedullary nailing followed by radiation therapy

Explanation

A Mirels score of 9 or greater indicates a high risk for an impending pathologic fracture. Prophylactic surgical stabilization (e.g., cephalomedullary nailing for a subtrochanteric lesion) followed by adjuvant local radiation is the standard of care to prevent fracture and relieve pain.

Question 1108

Topic: 10. Pathology and Oncology

A 15-year-old boy presents with progressive knee pain. Radiographs reveal a destructive metaphyseal lesion in the distal femur with a "sunburst" periosteal reaction. Biopsy confirms high-grade intramedullary osteosarcoma. What is the most appropriate treatment sequence?

. Neoadjuvant chemotherapy, wide surgical resection, and adjuvant chemotherapy
. Wide surgical resection followed by radiation therapy
. Neoadjuvant radiation therapy followed by wide surgical resection
. Primary amputation and adjuvant chemotherapy
. Intralesional curettage, cementation, and adjuvant chemotherapy

Correct Answer & Explanation

. Neoadjuvant chemotherapy, wide surgical resection, and adjuvant chemotherapy

Explanation

Standard of care for high-grade conventional osteosarcoma includes neoadjuvant chemotherapy, wide surgical resection, and adjuvant chemotherapy. Osteosarcoma is largely radioresistant, and amputation is reserved for cases where limb salvage is not possible.

Question 1109

Topic: 10. Pathology and Oncology

A 12-year-old boy presents with progressive distal thigh pain. Radiographs demonstrate a permeative diaphyseal lesion with a lamellated periosteal reaction.

Biopsy reveals sheets of small round blue cells. Which of the following translocations is most characteristic of this diagnosis?

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

Correct Answer & Explanation

. t(11;22)

Explanation

The clinical and radiographic presentation is classic for Ewing sarcoma. This tumor is most commonly associated with the t(11;22) chromosomal translocation, resulting in the EWS-FLI1 fusion protein.

Question 1110

Topic: 10. Pathology and Oncology

A 55-year-old male presents with deep, aching thigh pain. Radiographs reveal a lytic lesion with 'popcorn' calcifications in the proximal femur. Biopsy reveals atypical chondrocytes in a hyaline cartilage matrix with permeation into the surrounding marrow spaces. What is the most appropriate definitive treatment?

. Neoadjuvant chemotherapy followed by wide resection
. Intralesional curettage, phenol adjuvant, and bone grafting
. Wide surgical resection alone
. External beam radiation therapy
. Radiofrequency ablation

Correct Answer & Explanation

. Wide surgical resection alone

Explanation

The clinical and histologic findings are diagnostic of a conventional chondrosarcoma, indicated by matrix calcifications and marrow permeation. Because conventional chondrosarcomas are notoriously resistant to both chemotherapy and radiation therapy, wide surgical resection with negative margins is the definitive standard of care.

Question 1111

Topic: 10. Pathology and Oncology

A 14-year-old boy presents with progressive knee pain and swelling. Imaging

reveals a permeative diaphyseal lesion with an 'onion skin' periosteal reaction. Biopsy reveals sheets of uniform small round blue cells. Cytogenetic analysis is pending. Which of the following chromosomal translocations is most pathognomonic for this suspected diagnosis?

. t(11;22)(q24;q12)
. t(9;22)(q34;q11)
. t(X;18)(p11;q11)
. t(2;13)(q35;q14)
. t(12;16)(q13;p11)

Correct Answer & Explanation

. t(11;22)(q24;q12)

Explanation

The presentation, imaging, and histology are classic for Ewing sarcoma. The t(11;22)(q24;q12) translocation, which results in the EWS-FLI1 fusion transcript, is found in approximately 85% to 90% of all Ewing sarcoma cases.

Question 1112

Topic: 10. Pathology and Oncology

A 62-year-old female with known metastatic breast cancer presents with severe back pain and myelopathic symptoms. Imaging reveals a lytic metastasis in the T8 vertebral body causing high-grade epidural spinal cord compression (ESCC). Her neurologic exam demonstrates 4/5 strength in the bilateral lower extremities with hyperreflexia. According to the NOMS framework, what is the most appropriate management?

. Conventional external beam radiation therapy alone
. Stereotactic body radiation therapy (SBRT) alone
. Surgical decompression and stabilization followed by radiation therapy
. Bisphosphonate therapy and rigid TLSO bracing
. Systemic chemotherapy and close clinical observation

Correct Answer & Explanation

. Surgical decompression and stabilization followed by radiation therapy

Explanation

The Neurologic, Oncologic, Mechanical, and Systemic (NOMS) framework guides treatment for spinal metastases. For a solid tumor (radioresistant compared to myeloma/lymphoma) causing high-grade epidural spinal cord compression with myelopathic deficit, surgical decompression and stabilization are indicated prior to adjuvant radiation therapy.

Question 1113

Topic: 10. Pathology and Oncology

A 14-year-old boy with Duchenne Muscular Dystrophy is scheduled for posterior spinal fusion. Which of the following anesthetic agents is strictly contraindicated due to the risk of severe hyperkalemia and a malignant hyperthermia-like reaction?

. Propofol
. Sevoflurane
. Succinylcholine
. Rocuronium
. Fentanyl

Correct Answer & Explanation

. Succinylcholine

Explanation

Succinylcholine, a depolarizing muscle relaxant, is contraindicated in patients with DMD due to the risk of massive potassium release leading to fatal hyperkalemia and rhabdomyolysis.

Question 1114

Topic: 10. Pathology and Oncology

Which of the following is the leading cause of mortality in patients with Duchenne Muscular Dystrophy who survive into early adulthood?

. Renal failure secondary to myoglobinuria
. Hepatic failure from corticosteroid toxicity
. Cardiopulmonary failure
. Malignant hyperthermia
. Overwhelming sepsis from pressure ulcers

Correct Answer & Explanation

. Cardiopulmonary failure

Explanation

As pulmonary and cardiac care has improved, cardiopulmonary failure (specifically respiratory insufficiency and dilated cardiomyopathy) remains the leading cause of death in older DMD patients.

Question 1115

Topic: 10. Pathology and Oncology

A 15-year-old boy presents with progressive, worsening knee pain. Radiographs reveal a mixed lytic and sclerotic lesion in the distal femoral metaphysis with a sunburst periosteal reaction. Biopsy confirms high-grade osteosarcoma. Which tumor suppressor gene mutation is most strongly associated with the Li-Fraumeni syndrome variant of this disease?

. RB1
. PTEN
. TP53
. APC
. NF1

Correct Answer & Explanation

. TP53

Explanation

Li-Fraumeni syndrome is an autosomal dominant condition caused by a germline mutation in the TP53 tumor suppressor gene. It predisposes patients to multiple early-onset malignancies, including osteosarcoma.

Question 1116

Topic: 10. Pathology and Oncology

A patient with Li-Fraumeni syndrome develops an aggressive primary bone sarcoma. This syndrome is characterized by a germline mutation in which of the following genes?

. p53
. Retinoblastoma (Rb)
. EXT1
. GNAS
. NF1

Correct Answer & Explanation

. p53

Explanation

Li-Fraumeni syndrome is an autosomal dominant disorder caused by a germline mutation in the TP53 tumor suppressor gene. It places patients at high risk for multiple malignancies, most notably osteosarcoma, breast cancer, and soft tissue sarcomas.

Question 1117

Topic: 10. Pathology and Oncology

A 35-year-old female presents with progressive knee pain. Radiographs reveal an eccentric, lytic lesion in the distal femoral epiphysis extending to the subchondral bone, with no sclerotic margin. Biopsy shows multinucleated giant cells and mononuclear stromal cells. What is the standard initial surgical treatment?

. Wide en bloc resection and megaprosthesis reconstruction
. Extended intralesional curettage and filling with bone cement (PMMA)
. Preoperative radiation followed by marginal excision
. Amputation
. Observation with serial radiographs

Correct Answer & Explanation

. Extended intralesional curettage and filling with bone cement (PMMA)

Explanation

Giant cell tumor of bone is typically treated with extended intralesional curettage using high-speed burrs and adjuvants (like phenol or argon beam) followed by packing with PMMA. Wide resection is generally reserved for massive joint destruction or recurrent, aggressive lesions.

Question 1118

Topic: 10. Pathology and Oncology

A 14-year-old boy is diagnosed with a destructive diaphyseal bone lesion with an "onion skin" periosteal reaction. Cytogenetic analysis reveals a t(11;22) chromosomal translocation. Which fusion protein is characteristic of this tumor?

. EWS-FLI1
. SYT-SSX1
. PAX3-FOXO1
. TLS-CHOP
. MYC-N

Correct Answer & Explanation

. EWS-FLI1

Explanation

The clinical picture describes Ewing sarcoma, characterized by the t(11;22) translocation. This specific genetic aberration results in the EWS-FLI1 fusion protein, which acts as an aberrant transcription factor.

Question 1119

Topic: 10. Pathology and Oncology

A 33-year-old male presents with intermittent painful locking of his right elbow. Radiographs show a single loose body in the anterior aspect of the joint with well-maintained joint space. He reports a history of unexplained painful elbow for 18 months when he was 17 years old, which resolved spontaneously. No history of acute trauma. Based on this clinical presentation and history, what is the most likely diagnosis?

. Primary synovial chondromatosis
. Post-traumatic loose body
. Osteochondritis dissecans (OCD) with loose body formation
. Early osteoarthritis of the elbow
. Myositis ossificans
. Synovial sarcoma

Correct Answer & Explanation

. Osteochondritis dissecans (OCD) with loose body formation

Explanation

Correct Answer: CExplanation:The most likely diagnosis is Osteochondritis Dissecans (OCD) with loose body formation. The key elements supporting this diagnosis are:Age of onset of initial symptoms:The patient experienced unexplained painful elbow symptoms at age 17, which is a classic age range for the presentation of OCD of the capitellum.Nature of current symptoms:Intermittent painful locking is highly characteristic of a symptomatic intra-articular loose body.Radiographic findings:A single loose body with well-maintained joint space points away from advanced osteoarthritis as the primary cause. The absence of calcification in the muscle or capsule rules out myositis ossificans and synovial sarcoma.Absence of trauma:The patient denies any history of acute injury, ruling out a purely post-traumatic loose body.Single loose body:While synovial chondromatosis can cause loose bodies, it typically presents with multiple loose bodies, often described as 'rice bodies,' rather than a single one.Therefore, the history of adolescent elbow pain, followed by the current presentation of a single loose body causing locking in a joint with preserved space, strongly indicates a sequela of untreated or unrecognized OCD.

Question 1120

Topic: 10. Pathology and Oncology

The candidate notes that the radiographs show 'well-maintained joint space' and 'no calcification in the muscle or capsule.' What specific diagnoses are primarily ruled out or made less likely by these observations?

. Osteochondritis dissecans and Panner's disease
. Acute traumatic fracture and dislocation
. Advanced osteoarthritis and myositis ossificans
. Synovial chondromatosis and rheumatoid arthritis
. Septic arthritis and gout

Correct Answer & Explanation

. Synovial chondromatosis and rheumatoid arthritis

Explanation

Correct Answer: CExplanation:The observation of 'well-maintained joint space' makesadvanced osteoarthritisless likely, as osteoarthritis is characterized by joint space narrowing, osteophyte formation, and subchondral sclerosis. The presence of a single loose body with preserved joint space points away from widespread degenerative changes. 'No calcification in the muscle or capsule' specifically helps to rule outmyositis ossificans, which involves heterotopic ossification within muscle, and also makes conditions like synovial sarcoma (which can have calcification) less likely. While the case mentions ruling out synovial sarcoma, myositis ossificans is a more direct exclusion based on the lack of muscle/capsule calcification. Therefore, advanced osteoarthritis and myositis ossificans are primarily ruled out or made less likely.

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