Question 5761
Topic: 10. Pathology and OncologyAccording to the Enneking surgical staging system for musculoskeletal tumors, what defines a Stage IIB sarcoma?
Correct Answer & Explanation
. High-grade, intracompartmental
Practice Set 289 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.
According to the Enneking surgical staging system for musculoskeletal tumors, what defines a Stage IIB sarcoma?
. High-grade, intracompartmental
During an emergent open reduction and internal fixation, the patient develops sudden tachycardia, muscle rigidity, and a rapidly rising core body temperature. The anesthesia team administers a life-saving medication. What is the mechanism of this drug?
. Binds to ryanodine receptors, inhibiting calcium release from the sarcoplasmic reticulum
A 15-year-old male presents with night pain in his femur that is dramatically relieved by NSAIDs. A CT scan shows a small radiolucent nidus surrounded by dense reactive sclerosis. What is the primary mediator secreted by this lesion that causes the characteristic pain?
. Prostaglandin E2 (PGE2)
A 14-year-old female is diagnosed with conventional high-grade osteosarcoma of the distal femur. Genetic analysis of the tumor is most likely to reveal a somatic mutation or inactivation in which of the following genes?
. TP53
A 15-year-old boy presents with pain and swelling around his distal femur. Radiographs reveal a sunburst periosteal reaction and a Codman triangle. Biopsy confirms osteosarcoma. Germline mutations in which of the following tumor suppressor genes are most classically associated with an increased risk for this malignancy?
. Rb1 and p53
A 28-year-old male presents with a deep, slow-growing soft tissue mass in the popliteal fossa. Biopsy reveals a biphasic tumor with both spindle cells and epithelial cells. Which of the following chromosomal translocations is highly specific to this diagnosis?
. t(X;18)
A 15-year-old boy presents with severe night pain in his proximal femur that is dramatically relieved by oral ibuprofen. Imaging confirms a cortically based lesion with a small radiolucent nidus. The symptomatic relief provided by NSAIDs is directly related to the inhibition of which of the following?
. Prostaglandin E2 (PGE2) production by the nidus
A 14-year-old girl is diagnosed with osteosarcoma of the distal femur. Her family history is notable for her mother having premenopausal breast cancer and her brother having a soft tissue sarcoma. A germline mutation in which gene is the most likely underlying cause?
. TP53
Which of the following chromosomal translocations is classically associated with Ewing sarcoma, leading to the formation of a chimeric transcription factor?
. t(11;22)
A 14-year-old boy presents with progressive knee pain and swelling. Radiographs show a permeative, destructive diaphyseal lesion of the femur with a large soft tissue mass and an 'onion skin' periosteal reaction. Biopsy reveals small round blue cells. Which chromosomal translocation is the diagnostic hallmark of this tumor?
. t(11;22)
A 19-year-old male reports persistent nocturnal diaphyseal tibial pain that is dramatically relieved by aspirin. Radiographs reveal a cortically based sclerotic lesion with a central radiolucent nidus measuring 8 mm. What is the primary biochemical mediator produced in high quantities by the nidus responsible for the patient's specific pain pattern?
. Prostaglandin E2
A 55-year-old male with a history of renal cell carcinoma presents with a large, solitary, highly destructive lytic lesion in the proximal diaphyseal femur with an impending pathologic fracture. What is the most critical step prior to proceeding with prophylactic intramedullary stabilization?
. Preoperative angiographic embolization of the lesion
A 14-year-old boy presents with a painful, swollen mass on his left diaphyseal femur. Radiographs reveal a permeative, destructive lesion with an "onion-skin" periosteal reaction. Biopsy confirms small round blue cells. Immunohistochemistry is most likely to be strongly positive for which of the following markers?
. CD99
A 15-year-old boy presents with a destructive diaphyseal lesion of the femur with a large soft tissue mass. Biopsy reveals small round blue cells. Which of the following chromosomal translocations is most pathognomonic for this tumor?
. t(11;22)(q24;q12)
A 60-year-old female with a history of breast cancer presents with severe thigh pain. Radiographs reveal a 3 cm lytic lesion in the peritrochanteric region of the femur involving 50% of the cortical diameter. What is her Mirels' score, and what is the recommended treatment?
. Score 10; prophylactic internal fixation
A 14-year-old boy presents with a diaphyseal femur lesion with an "onion skin" periosteal reaction. Biopsy reveals small round blue cells. Which of the following chromosomal translocations is most pathognomonic for this tumor?
. t(11;22)
A 45-year-old female with a history of breast cancer presents with progressively worsening proximal femur pain. Radiographs demonstrate a large lytic lesion. You calculate a Mirels score to determine the need for prophylactic fixation. Which of the following is NOT a parameter used in the Mirels scoring system?
. Primary tumor histology
. Osteoid osteoma
A 14-year-old boy presents with a painful, swollen thigh. Radiographs reveal a permeative, diaphyseal lesion with an 'onion-skin' periosteal reaction. A biopsy is planned. Which of the following chromosomal translocations is most pathognomonic for the suspected diagnosis?
. t(11;22)
A 19-year-old male complains of severe, aching pain in his proximal femur that is notably worse at night and dramatically relieved by ibuprofen. Radiographs demonstrate a 1-cm radiolucent nidus surrounded by dense reactive sclerosis. What is the most appropriate initial management?
. Observation and nonsteroidal anti-inflammatory drugs (NSAIDs)