Question 4661
Topic: 10. Pathology and OncologyWhich primary malignant bone tumor is classically driven by a t(11;22)(q24;q12) chromosomal translocation, resulting in the EWS-FLI1 fusion gene?
Correct Answer & Explanation
. Osteosarcoma
Practice Set 234 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.
Which primary malignant bone tumor is classically driven by a t(11;22)(q24;q12) chromosomal translocation, resulting in the EWS-FLI1 fusion gene?
. Osteosarcoma
A 15-year-old presents with a high-grade osteosarcoma of the distal femur. What is the classic genetic mutation associated with the pathogenesis of this tumor in familial syndromes?
. t(11;22) translocation
A 12-year-old boy presents with a painful, swollen thigh. Radiographs show a permeative diaphyseal lesion with an onion-skin periosteal reaction. Histology reveals small round blue cells. Which chromosomal translocation is most characteristically associated with this tumor?
. t(9;22)
An 18-year-old male presents with deep knee pain. Radiographs show a lytic lesion in the distal femur with a "sunburst" periosteal reaction. Biopsy reveals malignant spindle cells producing osteoid. Which genetic mutations are most commonly associated with this pathology?
. t(11;22) translocation
Which of the following histologic variants of osteosarcoma typically carries the most favorable prognosis?
. Telangiectatic
. Wide surgical resection
A 28-year-old male is 6 months status-post anterior cruciate ligament (ACL) reconstruction. He complains of an audible clunk and a painful block to terminal knee extension. MRI is likely to reveal a lesion consisting primarily of what tissue type?
. Chondroid metaplasia
An 18-year-old male undergoes neoadjuvant chemotherapy followed by wide local excision of a distal femur osteosarcoma. Which of the following histologic findings in the resected specimen is the most significant predictor of long-term survival?
. Presence of Codman's triangle
A 14-year-old boy presents with a painful, swollen thigh. Radiographs reveal a permeative diaphyseal lesion of the femur with a prominent 'onion-skin' periosteal reaction. Core biopsy is performed. Which of the following cytogenetic abnormalities is most likely to be identified in the neoplastic cells?
. t(11;22)(q24;q12)
A 19-year-old male complains of dull, aching back pain that is notably worse at night and dramatically relieved by ibuprofen. Radiographs and CT show a 2.5 cm radiolucent nidus in the posterior elements of L4. Histologically, the lesion consists of woven bone trabeculae lined by prominent osteoblasts. What is the most likely diagnosis?
. Osteoid osteoma
A 15-year-old male presents with deep knee pain and a mass in the distal femur. Biopsy reveals highly pleomorphic cells producing a malignant osteoid matrix. This condition is classically associated with a germline mutation in which of the following tumor suppressor genes?
. TP53
A 14-year-old boy presents with a painful mass in his diaphyseal femur. Biopsy reveals small round blue cells. Cytogenetic analysis demonstrates a t(11;22) chromosomal translocation. Which fusion gene product is most likely responsible for this malignancy?
. EWS-FLI1
A 14-year-old boy presents with a painful, swollen thigh. Radiographs reveal a permeative diaphyseal lesion with an 'onion-skin' periosteal reaction. Biopsy confirms Ewing sarcoma. Which chromosomal translocation is most characteristic of this tumor?
. t(11;22)
A 16-year-old girl is diagnosed with a high-grade osteosarcoma of the distal femur and begins treatment. What is the most critical local prognostic factor for her long-term overall survival?
. Size of the primary tumor at diagnosis
A 15-year-old boy presents with progressive knee pain. Radiographs show a destructive, bone-forming lesion in the distal femur with a "sunburst" periosteal reaction. A biopsy confirms conventional osteosarcoma. Which of the following genetic abnormalities is most commonly associated with this tumor?
. t(11;22) translocation
A 65-year-old male presents with deep bone pain in his proximal femur. Blood work reveals hypercalcemia and mild renal insufficiency. Radiographs show a large solitary lytic lesion in the subtrochanteric region without a sclerotic rim. What is the most likely primary diagnosis?
. Osteosarcoma
. The percentage of tumor necrosis observed in the resection specimen
A 15-year-old male presents with a painful mass in the mid-diaphysis of his humerus. Radiographs show a permeative, "moth-eaten" lytic lesion with an "onion skin" periosteal reaction. Biopsy reveals small, round, blue cells. Molecular genetic testing is performed. Which of the following chromosomal translocations is the hallmark of this malignancy?
. t(9;22)(q34;q11)
A 35-year-old male presents with chronic, progressive right hip pain. Radiographs reveal a well-defined lytic lesion in the proximal femur epiphysis with central calcifications. Biopsy demonstrates sheets of cells with abundant clear cytoplasm, distinct cytoplasmic borders, and an underlying cartilaginous matrix. What is the most appropriate definitive treatment?
. Intralesional curettage and bone grafting
A 30-year-old female presents with severe knee pain. Imaging reveals an eccentric, expansile lytic lesion in the distal femoral epiphysis that extends down to the subchondral bone. Biopsy confirms a Giant Cell Tumor (GCT) of bone. To reduce tumor size, minimize surgical morbidity, and limit recurrence, the multidisciplinary tumor board recommends preoperative therapy with a systemic agent that directly targets the RANK ligand. Which of the following drugs is indicated?
. Zoledronic acid