Practice Set 201 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 of the following specific cytogenetic translocations is characteristically diagnostic for synovial sarcoma?
. t(X;18)(p11;q11)
When performing an open biopsy for a suspected primary malignant bone tumor of the distal femur, which of the following surgical principles must be strictly adhered to?
. Place the biopsy tract longitudinally and in line with the planned definitive resection incision
A 14-year-old boy presents with a destructive diaphyseal lesion of the femur with a permeative pattern and an "onion skin" periosteal reaction. Cytogenetic analysis is most likely to reveal which of the following translocations?
. t(11;22)
Which of the following is the most common primary malignant bone tumor in adults over the age of 40?
. Multiple myeloma
A 24-year-old male presents with a slow-growing, deep-seated soft tissue mass in his thigh. Core needle biopsy demonstrates a biphasic pattern of epithelial and spindle cells. Molecular analysis is most likely to reveal which of the following chromosomal translocations?
. t(X;18)(p11;q11) SYT-SSX
A 15-year-old boy complains of localized knee pain that is worse at night and dramatically relieved by ibuprofen. Radiographs show a 1.5 cm radiolucent nidus surrounded by dense reactive sclerosis in the proximal tibial diaphysis. This tumor produces pain primarily through the localized secretion of which of the following mediators?
. Prostaglandin E2
A 15-year-old male presents with severe knee pain and a palpable distal femoral mass. Biopsy confirms classic high-grade intramedullary osteosarcoma. Which of the following genetic mutations or conditions is most strongly associated with the development of this tumor?
. Li-Fraumeni syndrome (TP53 mutation)
A 12-year-old girl presents with pain in her mid-thigh. Radiographs show a permeative, lytic lesion in the diaphyseal region of the femur with a prominent 'onion-skin' periosteal reaction. Histology reveals small, round, blue cells. Which specific fusion gene product is most likely driving this malignancy?
. EWS-FLI1
A 14-year-old boy presents with a destructive diaphyseal lesion of the femur and an associated large soft-tissue mass. Biopsy reveals sheets of small round blue cells. Cytogenetic testing of this tumor is most likely to demonstrate a translocation resulting in which of the following fusion proteins?
. EWS-FLI1
A 14-year-old boy presents with a painful, enlarging mass on his proximal humerus. Biopsy reveals uniform small blue cells. Molecular analysis demonstrates an EWS-FLI1 fusion protein. Which chromosomal translocation is pathognomonic for this tumor?
. t(11;22)
A 14-year-old boy presents with a diaphyseal femur lesion with an "onion-skin" periosteal reaction. Molecular analysis confirms a t(11;22)(q24;q12) translocation. The resulting EWS-FLI1 fusion protein primarily functions as which of the following?
. Aberrant transcription factor
A 45-year-old male presents with a large, deep intramuscular mass in his thigh. Biopsy shows a prominent myxoid stroma, lipoblasts, and a delicate "chicken-wire" capillary network. The diagnosis is confirmed by identifying the t(12;16)(q13;p11) translocation. This sarcoma is clinically notable for which of the following?
. Excellent response to radiation therapy and a propensity for bone metastases
A 14-year-old boy presents with a painful diaphyseal femur lesion. Radiographs demonstrate an ill-defined destructive lesion with 'onion-skin' periosteal reaction. A biopsy is performed. Which of the following is the most common cytogenetic translocation and resulting fusion protein associated with this diagnosis?
. t(11;22) resulting in EWS-FLI1
A 16-year-old male complains of severe, aching leg pain that awakens him at night but is reliably completely relieved by ibuprofen. Radiographs reveal a thickened anterior tibial cortex surrounding a 5-mm radiolucent nidus. If this nidus were excised and examined histologically, what would be the classic finding?
. Interlacing trabeculae of woven bone lined by plump osteoblasts within a highly vascular stroma
A 30-year-old female presents with a deep, painless mass in the plantar aspect of her foot. Biopsy reveals a malignant proliferation of cells, and cytogenetic analysis demonstrates a t(12;22)(q13;q12) chromosomal translocation. Which of the following fusion genes is most likely associated with this lesion?
. EWS-ATF1
A 50-year-old male undergoes wide resection of a proximal femur lesion. Pathology describes a cartilaginous tumor with abundant myxoid stroma, high cellularity, marked nuclear atypia, and prominent mitotic figures. Which grade of chondrosarcoma does this best represent, and what is its expected metastatic potential?
. Grade 3; high metastatic potential
A 15-year-old female is diagnosed with conventional high-grade intramedullary osteosarcoma of the distal femur. Staging reveals no macroscopic metastatic disease. What is the most standard, evidence-based treatment algorithm for this patient?
. Neoadjuvant chemotherapy, wide surgical resection, and adjuvant chemotherapy
A 19-year-old male complains of severe, progressively worsening pain in his left thigh that is notably worse at night. The pain is rapidly relieved by ibuprofen. A CT scan reveals a 0.8 cm radiolucent nidus surrounded by reactive sclerosis in the femoral diaphysis. What is the primary mechanism by which nonsteroidal anti-inflammatory drugs (NSAIDs) relieve this patient's pain?
. Inhibition of cyclooxygenase, decreasing high levels of Prostaglandin E2
A 30-year-old man undergoes excision of a deep-seated, slow-growing soft tissue mass in his thigh. Histopathology reveals a biphasic pattern of spindle cells and epithelial cells. Molecular testing identifies a t(X;18)(p11;q11) chromosomal translocation. What is the diagnosis?
. Synovial sarcoma
A 15-year-old girl presents with a distal femur osteosarcoma. Which of the following tumor suppressor genes, frequently mutated in osteosarcoma, directly controls the G1 to S phase transition in the cell cycle?
. Rb