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Question 381

Topic: 10. Pathology and Oncology

A 60-year-old patient requires prophylactic intramedullary nailing for an impending pathologic fracture of the femur. Preoperative staging reveals the primary tumor is a renal cell carcinoma. What is the most critical preoperative intervention to minimize intraoperative complications?

. Administration of intravenous bisphosphonates
. Preoperative embolization of the bone lesion
. Systemic administration of anti-angiogenic chemotherapy
. Local radiation therapy
. Bone marrow aspiration

Correct Answer & Explanation

. Preoperative embolization of the bone lesion


Explanation

Bone metastases from renal cell carcinoma and thyroid carcinoma are highly vascular. Preoperative embolization is critical to prevent massive, life-threatening intraoperative hemorrhage.

Question 382

Topic: 10. Pathology and Oncology

A 12-year-old boy presents with a permeative lytic lesion in the diaphysis of his femur and an associated large soft tissue mass. Biopsy demonstrates sheets of small round blue cells. Which chromosomal translocation is most characteristic of this pathology?

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

Correct Answer & Explanation

. t(11;22)


Explanation

The clinical and histological picture describes Ewing sarcoma. The classic chromosomal translocation found in about 85% of Ewing sarcoma cases is t(11;22), resulting in the EWS-FLI1 fusion protein.

Question 383

Topic: 10. Pathology and Oncology
Pneumonectomy for carcinoma of the lung is likely to be contraindicated in the presence of which one of the following?
. Adenocarcinoma
. Forced expiratory volume in 1 s (FEV1) of 1.6 l
. Mediastinal lymph nodes < 1 cm diameter
. Moderate pulmonary hypertension
. Paraneoplastic syndrome

Correct Answer & Explanation

. Forced expiratory volume in 1 s (FEV1) of 1.6 l


Explanation

Correct Answer: B - Forced expiratory volume in 1 s (FEV1) of 1.6 l. Pulmonary function assessment provides guidance on operability. The functional criteria for pneumonectomy are generally: FEV1 > 2 l, FEV1 > 50% of the observed forced vital capacity, and normal partial pressure of arterial CO2 (PaCO2) with the patient at rest. These form a starting point, backed up by global functional assessment.

Question 384

Topic: 10. Pathology and Oncology

A 15-year-old boy presents with severe thigh pain and swelling. Radiographs reveal a permeative lytic lesion in the femoral diaphysis with an "onion skin" periosteal reaction. Biopsy reveals uniform small round blue cells. What is the most common chromosomal translocation associated with this tumor?

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

Correct Answer & Explanation

. t(11;22)


Explanation

The clinical and radiographic presentation is classic for Ewing sarcoma. The t(11;22) chromosomal translocation, resulting in the EWS-FLI1 fusion protein, is present in approximately 85% to 90% of Ewing sarcoma cases.

Question 385

Topic: 10. Pathology and Oncology

A 16-year-old boy presents with worsening distal thigh pain. Radiographs reveal a mixed lytic and blastic metaphyseal lesion with a 'sunburst' periosteal reaction. Biopsy confirms a conventional high-grade intramedullary osteosarcoma. What is the most appropriate management sequence?

. Immediate amputation followed by radiation therapy
. Primary wide resection followed by adjuvant chemotherapy
. Neoadjuvant chemotherapy, wide surgical resection, and adjuvant chemotherapy
. Radiation therapy followed by intralesional curettage
. Neoadjuvant radiation therapy followed by wide surgical resection

Correct Answer & Explanation

. Neoadjuvant chemotherapy, wide surgical resection, and adjuvant chemotherapy


Explanation

The standard of care for conventional high-grade osteosarcoma is neoadjuvant chemotherapy, followed by wide surgical resection (limb salvage when possible), and concluding with adjuvant chemotherapy. Osteosarcoma is generally radioresistant, making radiation therapy a poor primary option.

Question 386

Topic: 10. Pathology and Oncology

An 18-year-old male presents with a painful, enlarging mass about the right knee. Radiographs reveal a mixed lytic and sclerotic lesion in the distal femoral metaphysis with a Codman triangle. If a biopsy is planned to confirm the suspected diagnosis of osteosarcoma, what is the most critical principle of the biopsy technique?

. Using a transverse incision to minimize tension
. Placing the biopsy tract in line with the planned definitive resection incision
. Performing an excisional biopsy to obtain the entire tumor margin
. Raising extensive flaps to fully visualize the neurovascular bundle
. Tourniquet exsanguination prior to incision

Correct Answer & Explanation

. Placing the biopsy tract in line with the planned definitive resection incision


Explanation

The biopsy tract must be placed longitudinally and in line with the planned definitive surgical incision so that the entire tract can be excised en bloc with the tumor. Exsanguination with an Esmarch bandage is contraindicated to prevent tumor spread.

Question 387

Topic: 10. Pathology and Oncology

A 15-year-old boy presents with knee pain and a destructive lesion in the distal femoral metaphysis exhibiting a "sunburst" periosteal reaction. Biopsy confirms high-grade osteosarcoma. What is the standard sequence of treatment?

. Wide surgical resection alone
. Neoadjuvant chemotherapy followed by wide resection and adjuvant chemotherapy
. Radiation therapy followed by wide resection
. Neoadjuvant radiation and chemotherapy followed by amputation
. Intralesional curettage and bone grafting

Correct Answer & Explanation

. Neoadjuvant chemotherapy followed by wide resection and adjuvant chemotherapy


Explanation

The gold standard treatment for high-grade osteosarcoma is neoadjuvant multi-agent chemotherapy, followed by definitive wide surgical resection and adjuvant chemotherapy.

Question 388

Topic: 10. Pathology and Oncology

A 15-year-old boy presents with progressive knee pain and a palpable mass in the distal femur. Radiographs show a destructive metaphyseal lesion with a 'sunburst' periosteal reaction. Biopsy confirms classic high-grade intramedullary osteosarcoma. What is the defining histological hallmark of this tumor?

. Small round blue cells with Homer-Wright rosettes
. Multinucleated giant cells in a mononuclear stroma
. Production of malignant osteoid by spindle cells
. Chondroid matrix with hyperchromatic binucleated cells
. Nests of epithelioid cells with fibrous septa

Correct Answer & Explanation

. Production of malignant osteoid by spindle cells


Explanation

The defining histological characteristic of osteosarcoma is the production of malignant osteoid by atypical, malignant spindle cells. Homer-Wright rosettes describe Ewing sarcoma, while a prominent chondroid matrix without osteoid points to chondrosarcoma.

Question 389

Topic: 10. Pathology and Oncology

A 65-year-old man with a history of lung cancer presents with progressively worsening right thigh pain. Radiographs reveal a large destructive lytic lesion in the proximal femur with a Mirels' score of 10. What is the most appropriate next step in management?

. Observation and protected weight-bearing
. Initiation of systemic chemotherapy
. Immediate localized radiation therapy
. Prophylactic internal fixation
. Intravenous bisphosphonate therapy

Correct Answer & Explanation

. Prophylactic internal fixation


Explanation

A Mirels' score of 9 or greater indicates a high risk of impending pathological fracture. Prophylactic internal fixation is indicated prior to initiating localized radiation therapy.

Question 390

Topic: 10. Pathology and Oncology

A 15-year-old boy presents with worsening knee pain and swelling. Radiographs of the distal femur show a mixed lytic and blastic lesion with a "sunburst" periosteal reaction and Codman's triangle. If distant metastasis is present, what is the most likely location?

. Brain
. Liver
. Lungs
. Regional lymph nodes
. Lumbar spine

Correct Answer & Explanation

. Lungs


Explanation

Osteosarcoma is the most common primary malignant bone tumor in youths. It metastasizes almost exclusively hematogenously, with the lungs being the most common site of distant spread.

Question 391

Topic: 10. Pathology and Oncology

A 12-year-old boy presents with localized thigh pain, low-grade fever, and an elevated ESR. Radiographs demonstrate a diaphyseal lytic lesion of the femur with an "onion-skin" periosteal reaction. Cytogenetic testing of the biopsy will most likely show which translocation?

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

Correct Answer & Explanation

. t(11;22)


Explanation

Ewing sarcoma often mimics osteomyelitis with systemic symptoms and an "onion-skin" lamellated periosteal reaction. It is driven by the t(11;22) chromosomal translocation, which forms the pathognomonic EWS-FLI1 fusion protein.

Question 392

Topic: 10. Pathology and Oncology

A 32-year-old female presents with progressive knee pain. Radiographs reveal an eccentric, purely lytic lesion in the distal femoral epiphysis extending to the subchondral bone with no sclerotic border. What is the most appropriate definitive surgical management?

. Intralesional curettage alone
. Wide en bloc resection and endoprosthetic reconstruction
. Intralesional curettage, high-speed burring, and use of local adjuvants with cementation
. Primary amputation above the knee
. Core needle biopsy followed by primary radiotherapy

Correct Answer & Explanation

. Intralesional curettage, high-speed burring, and use of local adjuvants with cementation


Explanation

Giant cell tumors of bone are benign but locally aggressive epiphyseal lesions. Standard treatment is extended intralesional curettage using a high-speed burr, local adjuvants (e.g., phenol or argon beam), and packing with polymethylmethacrylate (PMMA) bone cement to minimize recurrence.

Question 393

Topic: 10. Pathology and Oncology

A 35-year-old immigrant presents with progressive back pain, night sweats, and lower extremity weakness. MRI reveals a destructive lesion of the T8-T9 vertebral bodies with relative sparing of the disc space and a large paraspinal abscess. What is the most appropriate next step in management?

. Immediate laminectomy and decompression
. CT-guided needle biopsy and culture
. Empiric initiation of vancomycin and ceftriaxone
. Radiation therapy
. Anterior spinal fusion

Correct Answer & Explanation

. CT-guided needle biopsy and culture


Explanation

The clinical picture is classic for Pott's disease (spinal tuberculosis). A tissue biopsy with culture and PCR is required to confirm the diagnosis and guide appropriate multidrug antitubercular therapy.

Question 394

Topic: Bone Tumors

A 65-year-old male with a 40-pack-year smoking history presents with bilateral knee and ankle pain. Radiographs reveal symmetric, solid periosteal new bone formation along the diaphyses and metaphyses of the tibiae and fibulae. Which of the following is the most likely underlying diagnosis?

. Osteosarcoma
. Osteoid osteoma
. Bronchogenic carcinoma
. Rheumatoid arthritis
. Syphilis

Correct Answer & Explanation

. Bronchogenic carcinoma


Explanation

Hypertrophic osteoarthropathy (HOA) is characterized by clubbing, polyarthritis, and symmetric periostitis of tubular bones. It is a paraneoplastic syndrome strongly associated with bronchogenic carcinoma.

Question 395

Topic: 10. Pathology and Oncology

A 58-year-old female with known breast cancer presents with thigh pain. Radiographs show a 3.5 cm lytic lesion in the peritrochanteric region of the proximal femur. She complains of severe pain with weight-bearing. What does her Mirels' score dictate regarding management?

. Score 7, Radiation therapy only
. Score 8, Observation
. Score 9, Prophylactic internal fixation
. Score 11, Prophylactic internal fixation
. Score 10, Radiation therapy only

Correct Answer & Explanation

. Score 11, Prophylactic internal fixation


Explanation

Mirels' criteria assess pathologic fracture risk based on site (peritrochanteric = 3), pain (functional = 3), lesion type (lytic = 3), and size (>2/3 cortical diameter = 3). A score of 11 or 12 strongly indicates prophylactic internal fixation.

Question 396

Topic: 10. Pathology and Oncology

A 7-year-old boy presents with a painful, solitary lytic lesion in the skull and a mild chronic cough. Biopsy of the skull lesion demonstrates cells with grooved nuclei expressing CD1a and S-100. Electron microscopy reveals Birbeck granules. Which of the following organs should also be carefully evaluated?

. Heart
. Lungs
. Kidneys
. Intestines
. Bladder

Correct Answer & Explanation

. Lungs


Explanation

Langerhans cell histiocytosis (eosinophilic granuloma) presents with lytic bone lesions and characteristic Birbeck granules. It can involve the lungs, particularly in systemic forms or older patients, necessitating pulmonary evaluation.

Question 397

Topic: 10. Pathology and Oncology

A 30-year-old female undergoes curettage and cementing of a giant cell tumor of the distal femur. Two years later, routine follow-up imaging reveals multiple asymptomatic, small, peripheral lung nodules. Biopsy of a lung nodule confirms histopathology identical to the primary tumor. What is the standard management for this pulmonary condition?

. Palliative care only
. Urgent systemic chemotherapy with methotrexate and doxorubicin
. Observation or surgical resection
. High-dose radiation therapy to the lungs
. Amputation of the affected leg

Correct Answer & Explanation

. Observation or surgical resection


Explanation

"Benign" pulmonary metastases occur in 2-3% of giant cell tumors of bone. They are often indolent and can be managed with observation, denosumab therapy, or local surgical resection.

Question 398

Topic: Bone Tumors

A 15-year-old male is undergoing chemotherapy for conventional osteosarcoma of the proximal tibia. He presents to the emergency department with sudden onset pleuritic chest pain and shortness of breath. Chest radiograph shows a spontaneous pneumothorax. What is the most likely underlying cause?

. Chemotherapy-induced pulmonary fibrosis
. Rupture of a cavitating pulmonary metastasis
. Pulmonary embolism
. Myocardial infarction
. Rib fracture

Correct Answer & Explanation

. Rupture of a cavitating pulmonary metastasis


Explanation

Spontaneous pneumothorax in a pediatric patient with osteosarcoma is highly suspicious for the rupture of a cavitating subpleural pulmonary metastasis. Osteosarcoma frequently metastasizes to the lungs.

Question 399

Topic: 10. Pathology and Oncology

An 11-year-old boy presents with a 2-month history of thigh pain and low-grade fever. Radiographs show a permeative diaphyseal lesion in the femur with a prominent "onion-skin" periosteal reaction. Which of the following cytogenetic abnormalities is most diagnostic for this tumor?

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

Correct Answer & Explanation

. t(11;22)


Explanation

Ewing sarcoma is classically characterized by the t(11;22)(q24;q12) translocation, which results in the EWS-FLI1 fusion protein. It typically presents with a permeative diaphyseal lesion and a multi-lamellated periosteal reaction.

Question 400

Topic: Bone Tumors

A 65-year-old man presents with severe mid-back pain. Laboratory tests reveal anemia, hypercalcemia, and elevated serum creatinine. Serum protein electrophoresis shows a monoclonal spike. Which test is most appropriate to evaluate the full extent of skeletal involvement?

. Technetium-99m bone scan
. Whole-body low-dose CT or skeletal survey
. Dual-energy X-ray absorptiometry (DEXA)
. Ultrasound
. Single-photon emission computed tomography (SPECT)

Correct Answer & Explanation

. Whole-body low-dose CT or skeletal survey


Explanation

Multiple myeloma lesions are purely lytic and often lack osteoblastic activity, leading to false-negative results on standard Technetium-99m bone scans. A whole-body low-dose CT or conventional skeletal survey is the standard for detecting these lesions.