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Question 6141

Topic: Bone Tumors

A 15-year-old boy presents with severe, dull aching pain in his proximal thigh that worsens at night and is dramatically relieved by ibuprofen. Imaging shows an 8mm intracortical radiolucent nidus with dense surrounding sclerosis. What is the standard of care for definitive, minimally invasive treatment?

. En bloc resection with wide margins
. Curettage and structural bone grafting
. CT-guided Radiofrequency Ablation (RFA)
. Percutaneous cryotherapy
. External beam radiation therapy

Correct Answer & Explanation

. CT-guided Radiofrequency Ablation (RFA)


Explanation

The clinical and radiographic presentation is classic for an Osteoid Osteoma. CT-guided Radiofrequency Ablation (RFA) is the treatment of choice, offering high success rates with minimal morbidity.

Question 6142

Topic: 10. Pathology and Oncology

A 25-year-old male with Multiple Hereditary Exostoses (MHE) presents with a rapidly enlarging and increasingly painful osteochondroma on his proximal tibia. Which of the following is the most critical complication to rule out?

. Pathologic fracture through the stalk
. Compression of the common peroneal nerve
. Malignant transformation to secondary chondrosarcoma
. Pseudoaneurysm formation of the popliteal artery
. Bursitis overlying the exostosis

Correct Answer & Explanation

. Malignant transformation to secondary chondrosarcoma


Explanation

While nerve compression or bursitis can cause pain, a rapidly enlarging osteochondroma after skeletal maturity is highly suspicious for malignant transformation into a secondary peripheral chondrosarcoma.

Question 6143

Topic: Bone Tumors

The mnemonic 'CRAB' is used to recall the hallmark end-organ damage manifestations of Multiple Myeloma. Which of the following accurately represents the CRAB criteria?

. Hypercalcemia, Renal insufficiency, Anemia, Bone lesions
. Hypocalcemia, Renal hyperfiltration, Polycythemia, Bone lesions
. Hyperkalemia, Renal insufficiency, Anemia, Bone lesions
. Hypercalcemia, Hepatic failure, Leukopenia, Bone lesions
. Normal calcium, Normal renal function, Normal hemoglobin, Bone lesions

Correct Answer & Explanation

. Hypercalcemia, Renal insufficiency, Anemia, Bone lesions


Explanation

The CRAB criteria defining end-organ damage in symptomatic multiple myeloma stand for hyperCalcemia, Renal insufficiency, Anemia, and Bone lesions (lytic).

Question 6144

Topic: Bone Tumors



A 65-year-old male presents with new-onset back pain, fatigue, and hypercalcemia. Radiographs show multiple punched-out lytic lesions in the axial skeleton, as seen in the provided image. Which laboratory finding is most specific for confirming the diagnosis of this pathology?

. Elevated alkaline phosphatase
. Monoclonal spike on serum protein electrophoresis
. Low serum parathyroid hormone levels
. Elevated prostate-specific antigen
. Elevated serum phosphorus

Correct Answer & Explanation

. Monoclonal spike on serum protein electrophoresis


Explanation

The clinical picture and lytic lesions represent multiple myeloma. A monoclonal spike (M-protein) on serum protein electrophoresis (SPEP) or urine protein electrophoresis (UPEP) is a hallmark finding, confirming the clonal plasma cell proliferation.

Question 6145

Topic: 10. Pathology and Oncology



A 60-year-old male with multiple myeloma presents with severe thigh pain on weight-bearing. Radiographs show a 4 cm lytic lesion in the subtrochanteric region with 50% cortical destruction. What is the most appropriate management?

. Radiation therapy alone
. Intramedullary nailing followed by radiation therapy
. Bisphosphonate therapy and protected weight-bearing
. Percutaneous cementoplasty
. Systemic chemotherapy alone

Correct Answer & Explanation

. Intramedullary nailing followed by radiation therapy


Explanation

This lesion represents an impending pathologic fracture based on Mirels' criteria (size >2.5 cm, weight-bearing bone, pain, lytic nature). Prophylactic stabilization with an intramedullary nail followed by local radiation is the standard of care.

Question 6146

Topic: 10. Pathology and Oncology

A 32-year-old female presents with a purely lytic, eccentric lesion in the distal femoral epiphysis. Biopsy reveals multinucleated giant cells in a background of mononuclear cells. The true neoplastic cells in this tumor express high levels of which marker?

. RANK
. RANKL
. CD138
. S-100
. Vimentin

Correct Answer & Explanation

. RANKL


Explanation

In a Giant Cell Tumor of bone, the mononuclear stromal cells are the true neoplastic elements, and they overexpress RANKL. This recruits and activates reactive multinucleated giant cells that express RANK, leading to massive bone resorption.

Question 6147

Topic: 10. Pathology and Oncology
In the treatment of conventional high-grade osteosarcoma, which specific finding is the most important independent prognostic factor for long-term survival?
. Tumor size less than 10 cm at presentation
. Alkaline phosphatase normalization prior to surgery
. Greater than 90% tumor necrosis following neoadjuvant chemotherapy
. Absence of a detectable p53 mutation
. Negative surgical margins exceeding 5 cm

Correct Answer & Explanation

. Greater than 90% tumor necrosis following neoadjuvant chemotherapy


Explanation

A good histologic response to neoadjuvant chemotherapy, defined as >90% tumor necrosis (Huvos Grade III or IV), is the most reliable predictor of overall and disease-free survival in patients with conventional osteosarcoma.

Question 6148

Topic: 10. Pathology and Oncology

A 14-year-old boy presents with a permeative lytic lesion in the femoral diaphysis with an "onion skin" periosteal reaction. A biopsy shows small round blue cells. Molecular analysis is most likely to reveal which of the following translocations?

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

Correct Answer & Explanation

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


Explanation

The clinical and radiographic presentation is classic for Ewing sarcoma. Over 85% of Ewing sarcomas are characterized by the t(11;22)(q24;q12) translocation, which creates the EWS-FLI1 fusion oncogene.

Question 6149

Topic: 10. Pathology and Oncology

An 11-year-old boy presents with a painful, swollen mid-thigh. Radiographs demonstrate a destructive diaphyseal lesion with an 'onion skin' periosteal reaction. Histology shows sheets of small, round, blue cells. Which chromosomal translocation is most classically associated with this malignancy?

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

Correct Answer & Explanation

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


Explanation

Ewing sarcoma is classically characterized by the t(11;22)(q24;q12) translocation. This fuses the EWS gene on chromosome 22 with the FLI1 gene on chromosome 11, creating an oncogenic transcription factor.

Question 6150

Topic: 10. Pathology and Oncology
A 16-year-old girl with distal femur osteosarcoma undergoes 10 weeks of neoadjuvant chemotherapy, followed by a wide surgical resection. Pathological analysis of the resected specimen is performed. Which of the following is the most important prognostic factor for her long-term survival?
. Preoperative serum alkaline phosphatase normalization
. Achieving greater than 90% tumor necrosis
. Initial tumor volume less than 150 cubic centimeters
. Absence of skip metastases on preoperative MRI
. Negative surgical margins of at least 2 centimeters

Correct Answer & Explanation

. Achieving greater than 90% tumor necrosis


Explanation

The degree of tumor necrosis in response to neoadjuvant chemotherapy is the single most important prognostic factor in osteosarcoma. A 'good response' is defined as >90% tumor necrosis (Huvos Grade III or IV) and correlates with significantly higher survival rates.

Question 6151

Topic: 10. Pathology and Oncology

A 62-year-old man presents with an impending pathologic fracture of the proximal femur secondary to a biopsy-proven metastatic renal cell carcinoma lesion. Prophylactic intramedullary nailing is planned. What is the most critical step in management immediately prior to surgical fixation?

. Administration of denosumab
. Preoperative angiogram and selective arterial embolization
. Neoadjuvant external beam radiation therapy
. Administration of systemic bisphosphonates
. Placement of a prophylactic inferior vena cava filter

Correct Answer & Explanation

. Preoperative angiogram and selective arterial embolization


Explanation

Metastatic lesions from renal cell carcinoma and thyroid cancer are notoriously hypervascular. Preoperative selective arterial embolization is critical to prevent catastrophic intraoperative hemorrhage during orthopedic stabilization.

Question 6152

Topic: 10. Pathology and Oncology

A 32-year-old man presents with recurrent, spontaneous hemarthroses of the knee. MRI demonstrates a nodular synovial mass with prominent 'blooming artifact' on gradient-echo sequences. The pathophysiology of this condition involves the overexpression of which of the following factors?

. Vascular Endothelial Growth Factor (VEGF)
. Fibroblast Growth Factor 23 (FGF-23)
. Colony Stimulating Factor 1 (CSF1)
. Transforming Growth Factor Beta (TGF-beta)
. Bone Morphogenetic Protein 2 (BMP-2)

Correct Answer & Explanation

. Colony Stimulating Factor 1 (CSF1)


Explanation

Tenosynovial Giant Cell Tumor (formerly Pigmented Villonodular Synovitis) demonstrates 'blooming artifact' due to hemosiderin. It is driven by a t(1;2) translocation that causes overexpression of CSF1, which recruits inflammatory macrophages that form the bulk of the tumor mass.

Question 6153

Topic: 10. Pathology and Oncology

A 70-year-old man presents with severe back pain and anemia. Radiographs demonstrate multiple lytic skull lesions.

Serum electrophoresis shows an M-spike. Which of the following is the most likely finding on bone marrow biopsy?

. Sheets of uniform round cells with CD99 positivity
. Spindle cells producing osteoid matrix
. Greater than 10% monoclonal plasma cells
. Giant cells in a background of mononuclear cells
. Chondrocytes with abundant hyaline cartilage

Correct Answer & Explanation

. Greater than 10% monoclonal plasma cells


Explanation

The classic presentation and radiographic "punched-out" lesions denote multiple myeloma. The diagnosis is confirmed by finding greater than 10% clonal plasma cells on a bone marrow biopsy.

Question 6154

Topic: 10. Pathology and Oncology

A 14-year-old boy has a permeative, destructive lesion in the distal femur with a "sunburst" periosteal reaction. Biopsy confirms high-grade intramedullary osteosarcoma. What is the single most important prognostic factor for his long-term survival?

. Tumor size at presentation
. Serum alkaline phosphatase level
. Anatomic location of the tumor
. Histologic response to neoadjuvant chemotherapy
. Presence of a pathologic fracture

Correct Answer & Explanation

. Histologic response to neoadjuvant chemotherapy


Explanation

The degree of tumor necrosis following neoadjuvant chemotherapy is the most critical prognostic factor for long-term survival in high-grade osteosarcoma. Greater than 90% necrosis indicates a favorable response.

Question 6155

Topic: 10. Pathology and Oncology

A 12-year-old boy presents with thigh pain and fever. Radiographs show a diaphyseal lytic lesion of the femur with an "onion-skin" periosteal reaction. Biopsy shows small blue round cells. Which chromosomal translocation is most characteristically associated with this tumor?

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

Correct Answer & Explanation

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


Explanation

Ewing sarcoma is classically characterized by the t(11;22)(q24;q12) translocation. This specific genetic alteration results in the formation of the EWS-FLI1 fusion protein.

Question 6156

Topic: 10. Pathology and Oncology

A 65-year-old man presents with chronic hip pain. Radiographs show a large destructive lesion in the ilium with "rings and arcs" calcification. Biopsy confirms a grade II conventional chondrosarcoma. What is the most appropriate primary treatment?

. Chemotherapy followed by radiation
. Wide surgical resection
. Neoadjuvant chemotherapy followed by wide resection
. Intralesional curettage and cementation
. Radiation therapy alone

Correct Answer & Explanation

. Wide surgical resection


Explanation

Conventional chondrosarcomas are largely resistant to both chemotherapy and radiation therapy. Wide surgical resection with negative margins is the mainstay of treatment for intermediate and high-grade lesions.

Question 6157

Topic: 10. Pathology and Oncology

A 45-year-old woman undergoes an MRI for knee pain, which reveals a 4 cm cartilage tumor in the distal femur. Which of the following features is the most reliable indicator of a grade I chondrosarcoma rather than a benign enchondroma?

. Presence of popcorn calcifications on radiograph
. High T2 signal on MRI
. Deep endosteal scalloping greater than two-thirds of the cortical thickness
. Lesion size less than 3 cm
. Metaphyseal location

Correct Answer & Explanation

. Deep endosteal scalloping greater than two-thirds of the cortical thickness


Explanation

Deep endosteal scalloping (>2/3 of the cortical thickness), cortical breakthrough, and soft tissue extension are key radiographic signs. These findings help differentiate a low-grade chondrosarcoma from a benign enchondroma.

Question 6158

Topic: Bone Tumors

A 16-year-old boy presents with a 6-month history of right thigh pain that is worse at night and completely relieved by ibuprofen. Radiographs show a 1 cm radiolucent nidus surrounded by dense sclerotic bone. The profound pain relief from NSAIDs is due to inhibition of which substance produced by the nidus?

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

Correct Answer & Explanation

. Prostaglandin E2


Explanation

Osteoid osteomas classically cause night pain relieved by NSAIDs. This occurs because the nidus produces extremely high levels of Prostaglandin E2 (PGE2), which is inhibited by cyclooxygenase (COX) inhibitors.

Question 6159

Topic: 10. Pathology and Oncology

A 15-year-old male presents with enlarging bumps around his knees and ankles. Radiographs demonstrate multiple sessile and pedunculated osteochondromas. He carries a mutation in the EXT1 gene. What is the approximate lifetime risk of malignant transformation to chondrosarcoma in this condition?

. Less than 1%
. 1-5%
. 15-20%
. 30-40%
. Greater than 50%

Correct Answer & Explanation

. 1-5%


Explanation

Multiple Hereditary Exostoses (MHE) is caused by mutations in the EXT1 or EXT2 genes. The lifetime risk of an osteochondroma undergoing malignant transformation to secondary chondrosarcoma in MHE is approximately 1-5%.

Question 6160

Topic: Bone Tumors

A 68-year-old male presents with fatigue, diffuse bone pain, and hypercalcemia. A representative radiograph is shown.

Serum protein electrophoresis confirms a monoclonal gammopathy. When evaluating the extent of this patient's skeletal disease, which of the following imaging modalities is most likely to yield false-negative results due to the underlying pathophysiology of the bone lesions?

. Whole-body MRI
. Low-dose whole-body CT
. Technetium-99m bone scintigraphy
. F-18 FDG PET/CT
. Conventional skeletal survey

Correct Answer & Explanation

. Technetium-99m bone scintigraphy


Explanation

Technetium-99m bone scintigraphy relies on osteoblastic activity for radiotracer uptake. In multiple myeloma, neoplastic cells secrete factors (such as DKK-1) that profoundly suppress osteoblasts, leading to purely lytic lesions that frequently do not demonstrate uptake on traditional bone scans.