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.
Question 5801
Topic: Bone Tumors
A 68-year-old man presents with severe back pain. Plain radiographs reveal multiple "punched-out" lytic lesions in his skull and spine. If a technetium-99m bone scan is performed, what is the most likely expected finding for these specific lesions?
Correct Answer & Explanation
. Cold spots (false-negative) due to absent osteoblastic activity
Explanation
Multiple myeloma causes bone destruction via intense, pure osteoclast activation with little to no reactive osteoblastic response. Because a technetium-99m bone scan relies on osteoblastic activity (bone formation), myeloma lesions typically appear as "cold" or false-negative spots.
Question 5802
Topic: 10. Pathology and Oncology
A 15-year-old boy presents with a painful mass around his distal femur. Radiographs show a sunburst periosteal reaction and Codman's triangle. What is the most significant prognostic factor for survival in this patient?
Correct Answer & Explanation
. Extent of tumor necrosis following neoadjuvant chemotherapy
Explanation
In osteosarcoma, the degree of tumor necrosis following neoadjuvant chemotherapy is the single most important prognostic factor. Greater than 90% necrosis indicates a good response and correlates with improved long-term survival.
Question 5803
Topic: 10. Pathology and Oncology
A 30-year-old female presents with knee pain and a lytic epiphyseal lesion extending to the articular surface of the proximal tibia. Biopsy reveals multinucleated giant cells in a background of mononuclear stromal cells. Which cell type is the true neoplastic component of this tumor?
Correct Answer & Explanation
. The mononuclear stromal cells
Explanation
In a giant cell tumor of bone, the spindle-shaped mononuclear stromal cells are the actual neoplastic cells. These stromal cells express RANKL, which recruits and stimulates the formation of the reactive, non-neoplastic multinucleated giant cells responsible for the osteolysis.
Question 5804
Topic: 10. Pathology and Oncology
Which of the following genetic alterations is most classically associated with the development of conventional osteosarcoma?
Correct Answer & Explanation
. Mutation in the p53 and Rb tumor suppressor genes
Explanation
Conventional osteosarcoma is highly associated with mutations in the retinoblastoma (Rb) and p53 tumor suppressor genes. Patients with Li-Fraumeni syndrome (p53) and hereditary retinoblastoma both have a significantly increased risk of developing osteosarcoma.
Question 5805
Topic: 10. Pathology and Oncology
A 14-year-old male presents with a permeative lytic lesion in the diaphysis of the femur with an 'onion skin' periosteal reaction. Biopsy confirms Ewing sarcoma. Which of the following is the most common chromosomal translocation associated with this diagnosis?
Correct Answer & Explanation
. t(11;22)(q24;q12)
Explanation
Ewing sarcoma is classically associated with the t(11;22)(q24;q12) translocation, resulting in the EWS-FLI1 fusion protein. t(X;18) is seen in synovial sarcoma; t(12;16) in myxoid liposarcoma; t(9;22) in CML (Philadelphia chromosome); t(2;13) in alveolar rhabdomyosarcoma.
Question 5806
Topic: 10. Pathology and Oncology
A 60-year-old male presents with a destructive diaphyseal lesion in his humerus. Biopsy reveals sheets of plasma cells with eccentric nuclei and 'clock-face' chromatin. Which of the following lab findings is most consistently associated with his primary diagnosis?
Correct Answer & Explanation
. Monoclonal spike on serum protein electrophoresis (SPEP)
Explanation
This patient has multiple myeloma, the most common primary bone malignancy in adults. Diagnosis relies on identifying a monoclonal gammopathy (M-spike) on SPEP or UPEP, along with a bone marrow biopsy confirming clonal plasma cells.
Question 5807
Topic: 10. Pathology and Oncology
Which of the following chromosomal translocations is most characteristic of Ewing sarcoma?
Correct Answer & Explanation
. t(11;22)
Explanation
Ewing sarcoma is classically associated with the t(11;22) translocation, resulting in the EWS-FLI1 fusion protein. This mutation acts as an aberrant transcription factor promoting oncogenesis.
Question 5808
Topic: 10. Pathology and Oncology
A 12-year-old boy presents with a pathologic fracture through a central, purely lytic lesion in the proximal humerus. Radiographs reveal the "fallen leaf" sign. What is the most likely composition of the fluid inside this lesion prior to the fracture?
Correct Answer & Explanation
. Clear or straw-colored fluid
Explanation
The clinical presentation and "fallen leaf" sign on radiographs are pathognomonic for a Unicameral Bone Cyst (UBC). Prior to a fracture, these benign cysts are typically filled with a clear or straw-colored serous fluid.
Question 5809
Topic: 10. Pathology and Oncology
A 60-year-old male presents with a pathologic proximal humerus fracture secondary to metastatic renal cell carcinoma. Preoperative planning for open reduction and internal fixation should strongly include which of the following?
Correct Answer & Explanation
. Preoperative selective arterial embolization
Explanation
Metastases from renal cell carcinoma and thyroid carcinoma are highly vascular. Preoperative selective arterial embolization 24 to 48 hours prior to surgery significantly reduces massive intraoperative blood loss.
Question 5810
Topic: 10. Pathology and Oncology
Ewing sarcoma is a highly aggressive bone tumor classically associated with which of the following chromosomal translocations?
Correct Answer & Explanation
. t(11;22)
Explanation
Ewing sarcoma is characterized by the t(11;22)(q24;q12) translocation in over 85% of cases, leading to the formation of the EWS-FLI1 fusion protein. Synovial sarcoma is associated with t(X;18).
Question 5811
Topic: 10. Pathology and Oncology
A 16-year-old boy presents with severe right thigh pain that is worse at night and dramatically relieved by NSAIDs. Imaging reveals a 1 cm radiolucent nidus with surrounding reactive sclerosis in the femoral diaphysis. What is the characteristic histological finding of the nidus?
Correct Answer & Explanation
. Interlacing woven bone trabeculae lined by osteoblasts without atypia
Explanation
The clinical and radiographic presentation is classic for an osteoid osteoma. Histologically, the nidus consists of a complex, interlacing network of woven bone trabeculae (osteoid) lined by prominent, yet entirely benign, osteoblasts, set within a highly vascularized fibrovascular connective tissue stroma.
Question 5812
Topic: 10. Pathology and Oncology
According to the Enneking surgical staging system for musculoskeletal tumors, a high-grade osteosarcoma that has broken through the bone cortex into the surrounding soft tissue without distant metastases is classified as:
Correct Answer & Explanation
. Stage IIB
Explanation
In the Enneking staging system, Stage II indicates a high-grade tumor. The "B" designation signifies that the lesion is extracompartmental, meaning it has extended outside its compartment of origin.
Question 5813
Topic: 10. Pathology and Oncology
Molecular analysis of a suspected bone tumor reveals the specific chromosomal translocation t(11;22), resulting in the EWS-FLI1 fusion gene. Which of the following is the most likely diagnosis?
Correct Answer & Explanation
. Ewing sarcoma
Explanation
Ewing sarcoma is classically characterized by the t(11;22) chromosomal translocation. This creates the EWS-FLI1 fusion protein, which acts as an aberrant transcription factor driving oncogenesis.
Question 5814
Topic: 10. Pathology and Oncology
The Mirels criteria are used to assess the risk of a pathological fracture in a long bone affected by a metastatic lesion. Which of the following is NOT one of the four parameters evaluated in the Mirels scoring system?
The Mirels scoring system uses four parameters to predict pathological fracture risk: Site (upper limb, lower limb, peritrochanteric), Pain (mild, moderate, functional), Lesion nature (blastic, mixed, lytic), and Size (<1/3, 1/3-2/3, >2/3 of cortical diameter). Primary tumor histology is not one of the criteria scored.
Question 5815
Topic: 10. Pathology and Oncology
An 18-year-old male presents with deep knee pain and a mixed lytic/sclerotic lesion in the distal femur. Biopsy confirms high-grade intramedullary osteosarcoma. What is the standard treatment algorithm?
Correct Answer & Explanation
. Neoadjuvant chemotherapy, wide surgical resection, and adjuvant chemotherapy
Explanation
High-grade osteosarcoma is typically treated with neoadjuvant chemotherapy, followed by surgical resection with wide margins, and then adjuvant chemotherapy to address systemic micrometastases.
Question 5816
Topic: Bone Tumors
A 60-year-old female with back pain has punched-out lytic skull lesions and a monoclonal protein spike. What is the most appropriate imaging modality to evaluate the extent of her skeletal involvement?
Correct Answer & Explanation
. Low-dose whole-body CT or skeletal survey
Explanation
Multiple myeloma lesions are purely lytic and lack reactive osteoblastic activity. Therefore, a Technetium-99m bone scan is often falsely negative; a low-dose whole-body CT or skeletal survey is the standard.
Question 5817
Topic: Bone Tumors
A 15-year-old boy presents with aching knee pain that is noticeably worse at night and dramatically relieved by ibuprofen. Radiographs show a small radiolucent nidus with surrounding sclerosis in the proximal tibia. What is the primary molecular driver of his pain?
Correct Answer & Explanation
. High local production of Prostaglandin E2 by the nidus
Explanation
The clinical and radiographic presentation is classic for an osteoid osteoma. The osteoblastic cells within the nidus produce extremely high levels of prostaglandins (particularly PGE2), mediating the characteristic night pain that responds to NSAIDs.
Question 5818
Topic: 10. Pathology and Oncology
A 60-year-old man presents with a destructive sacral mass causing bowel and bladder dysfunction. Biopsy reveals cells arranged in cords with abundant bubbly cytoplasm and distinct physaliferous features. What is the most likely diagnosis?
Correct Answer & Explanation
. Chordoma
Explanation
Chordomas are rare, low-grade malignant bone tumors arising from embryonic remnants of the notochord, most commonly located in the sacrococcygeal and spheno-occipital regions. The presence of large, vacuolated "physaliferous" cells is the pathognomonic histologic hallmark.
Question 5819
Topic: 10. Pathology and Oncology
A 14-year-old boy presents with a painful mass in his distal femur. Biopsy reveals a small round blue cell tumor. Molecular testing is positive for a t(11;22) translocation. What is the most likely diagnosis and its associated fusion gene?
Correct Answer & Explanation
. Ewing sarcoma (EWS-FLI1)
Explanation
Ewing sarcoma is classically characterized by the t(11;22) chromosomal translocation, which results in the EWS-FLI1 fusion gene. It typically presents as a small round blue cell tumor in children and young adults.
Question 5820
Topic: 10. Pathology and Oncology
A 12-year-old boy presents with progressive diaphyseal leg pain. Radiographs reveal a permeative lytic lesion in the tibial diaphysis with an "onion skin" periosteal reaction. Cytogenetic analysis is most likely to reveal which of the following translocations?
Correct Answer & Explanation
. t(11;22)
Explanation
The clinical and radiographic presentation is classic for Ewing sarcoma. This tumor is strongly associated with the t(11;22) chromosomal translocation, which results in the EWS-FLI1 fusion protein.
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