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 6061
Topic: 10. Pathology and Oncology
A 20-year-old male presents with chronic knee pain. Radiographs demonstrate an eccentric, well-circumscribed, lytic lesion with a sclerotic margin in the proximal tibial metaphysis. Biopsy reveals lobules of spindle or stellate cells in a myxoid background separated by highly cellular fibrous bands containing osteoclast-like giant cells. What is the most likely diagnosis?
Correct Answer & Explanation
. Chondromyxoid fibroma
Explanation
Chondromyxoid fibroma typically presents as an eccentric, lytic metaphyseal lesion. The classic histologic appearance consists of lobules of myxoid and chondroid tissue separated by fibrous septa containing multinucleated giant cells.
Question 6062
Topic: 10. Pathology and Oncology
A 45-year-old woman is evaluated for a destructive diaphyseal lesion in the humerus. Histology reveals a diffuse infiltrate of small to medium-sized round blue cells. Immunohistochemistry is positive for CD45 (LCA) and CD20, but negative for CD99 and cytokeratin. Which diagnosis is confirmed?
Correct Answer & Explanation
. Diffuse large B-cell lymphoma of bone
Explanation
Primary lymphoma of bone is a round blue cell tumor that stains positive for CD45 (Leukocyte Common Antigen) and B-cell markers like CD20. It is negative for CD99 (Ewing sarcoma) and cytokeratin (metastatic carcinoma).
Question 6063
Topic: Bone Tumors
A 4-year-old boy presents with a painless, asymmetric swelling on the medial aspect of his ankle. Radiographs show an irregular, ossified mass arising from the medial epiphysis of the distal tibia. Histologically, the lesion strongly resembles an osteochondroma. What is the most likely diagnosis?
Dysplasia epiphysealis hemimelica (Trevor disease) is characterized by an osteochondroma-like outgrowth arising from the epiphysis, most commonly on the medial side of the knee or ankle. It results in asymmetric epiphyseal enlargement and joint deformity.
Question 6064
Topic: Bone Tumors
A 48-year-old male presents with severe right thigh pain. Plain radiographs appear largely unremarkable except for a very subtle permeative radiolucency in the diaphysis. MRI demonstrates extensive marrow replacement and a massive soft tissue mass surrounding the bone. Which of the following is the most likely diagnosis?
Correct Answer & Explanation
. Primary lymphoma of bone
Explanation
The combination of minimal cortical destruction on plain radiographs with an extensive soft tissue mass on MRI is a hallmark presentation of primary lymphoma of bone. The tumor permeates through the Haversian canals without completely destroying the cortex.
Question 6065
Topic: Bone Tumors
A 6-year-old girl is noted to have early breast development and vaginal bleeding. She complains of left hip pain, and radiographs reveal a ground-glass, expansile lytic lesion in the proximal femur with a "shepherd's crook" deformity. Physical exam reveals large, irregular hyperpigmented skin macules. A mutation in which of the following genes is responsible for this condition?
Correct Answer & Explanation
. GNAS1
Explanation
This patient has McCune-Albright syndrome, characterized by polyostotic fibrous dysplasia, precocious puberty, and cafe-au-lait spots. It is caused by an activating post-zygotic mutation in the GNAS1 gene, resulting in persistent adenylyl cyclase activity.
Question 6066
Topic: 10. Pathology and Oncology
A 22-year-old male presents with persistent left knee pain. Radiographs reveal an eccentric, purely lytic lesion in the proximal tibial metaphysis with a distinct sclerotic inner margin.
A biopsy demonstrates lobules of myxoid and chondroid tissue with hypercellular peripheries. What is the most likely diagnosis?
Correct Answer & Explanation
. Chondromyxoid fibroma
Explanation
Chondromyxoid fibroma (CMF) is a rare, benign cartilage tumor that classically presents as an eccentric metaphyseal radiolucency with a sclerotic inner margin. Histologically, it features biphasic lobules with hypocellular centers and hypercellular peripheries containing stellate cells.
Question 6067
Topic: 10. Pathology and Oncology
A 55-year-old male presents with thigh pain. Radiographs show a large permeative, 'moth-eaten' lesion in the femoral diaphysis with minimal cortical expansion.
Biopsy confirms primary diffuse large B-cell lymphoma of bone. What is the standard definitive treatment?
Correct Answer & Explanation
. Systemic chemotherapy and localized radiotherapy
Explanation
Primary bone lymphoma is highly responsive to non-operative treatment. The standard of care is systemic multidrug chemotherapy (e.g., CHOP-R) combined with localized external beam radiotherapy, reserving surgery strictly for impending or actual pathologic fractures.
Question 6068
Topic: Bone Tumors
Which histologic feature is considered the hallmark of a chondromyxoid fibroma, aiding in differentiating it from chondrosarcoma?
Correct Answer & Explanation
. Lobular architecture with hypercellular margins and hypocellular centers
Explanation
Chondromyxoid fibroma exhibits a distinct pseudo-lobular pattern with central hypocellular myxoid tissue and hypercellular peripheries containing multinucleated giant cells. Chondrosarcomas typically lack this organized lobular zonation and show permeation of native bone.
Question 6069
Topic: 10. Pathology and Oncology
A classic imaging characteristic that strongly suggests primary bone lymphoma over other aggressive sarcomas (such as Ewing sarcoma or osteosarcoma) is:
Correct Answer & Explanation
. A large soft-tissue mass with relatively preserved cortical bone contour
Explanation
Primary bone lymphoma often presents with a massive soft-tissue component that seems disproportionately large compared to the relatively preserved cortical bone structure. The tumor typically spreads through Haversian canals without completely destroying the cortex.
Question 6070
Topic: 10. Pathology and Oncology
What is the recommended surgical management for a symptomatic chondromyxoid fibroma in the proximal tibia of a 25-year-old patient?
Correct Answer & Explanation
. Extended intralesional curettage with high-speed burring and bone grafting
Explanation
Chondromyxoid fibroma is a benign but locally aggressive tumor. The standard of care is extended intralesional curettage utilizing a high-speed burr (with or without local adjuvants) followed by bone grafting or cementation.
Question 6071
Topic: 10. Pathology and Oncology
During the immunohistochemical evaluation of a suspected primary bone lymphoma biopsy, which of the following surface markers is most likely to be strongly positive?
Correct Answer & Explanation
. CD20
Explanation
The vast majority of primary bone lymphomas are diffuse large B-cell lymphomas. Therefore, they will strongly express B-cell markers such as CD20, along with the leukocyte common antigen CD45.
Question 6072
Topic: 10. Pathology and Oncology
A 55-year-old man presents with dull, aching thigh pain for 3 months. Radiographs demonstrate a permeative, moth-eaten radiolucent lesion in the femoral diaphysis with minimal periosteal reaction.
Biopsy reveals sheets of uniform, small round blue cells. Immunohistochemistry is positive for CD20 and CD45. What is the standard initial treatment?
Correct Answer & Explanation
. Systemic chemotherapy and localized radiation therapy
Explanation
The diagnosis is primary bone lymphoma (diffuse large B-cell lymphoma), supported by the permeative lesion, age, and CD20/CD45 positivity. The primary treatment is multi-agent chemotherapy (e.g., R-CHOP) combined with localized radiation; surgery is reserved for impending or actual pathologic fractures.
Question 6073
Topic: 10. Pathology and Oncology
A 60-year-old male presents with deep thigh pain.
Biopsy reveals sheets of uniform, small to medium-sized cells with prominent nucleoli. The cells are positive for CD45 and CD20. Which of the following immunohistochemical markers is also most likely to be positive, confirming the diagnosis?
Correct Answer & Explanation
. PAX5
Explanation
Primary bone lymphoma is most commonly a diffuse large B-cell lymphoma (DLBCL). These tumors typically express B-cell markers such as CD20, CD45 (LCA), and PAX5, distinguishing them from other small round blue cell tumors.
Question 6074
Topic: Bone Tumors
A 45-year-old male completes definitive radiation therapy for primary diffuse large B-cell lymphoma of the femoral diaphysis. Which of the following is the most significant orthopedic complication directly associated with treating appendicular primary bone lymphoma exclusively with local high-dose radiation?
Correct Answer & Explanation
. Pathologic fracture
Explanation
Radiation therapy alone for primary bone lymphoma in weight-bearing bones carries a high risk of subsequent pathologic fracture due to radiation-induced osteonecrosis and delayed bone remodeling. This has shifted modern paradigms toward combined chemoradiation or prophylactic stabilization.
Question 6075
Topic: 10. Pathology and Oncology
A 22-year-old female undergoes a core needle biopsy of an eccentric, lytic metaphyseal lesion in the distal femur. The pathology report confirms chondromyxoid fibroma. What is the most appropriate surgical treatment to minimize recurrence?
Correct Answer & Explanation
. Extended curettage with high-speed burring and filling with bone graft or cement
Explanation
Chondromyxoid fibroma is a benign but locally aggressive tumor with a high recurrence rate if treated with simple curettage. Extended intralesional curettage with high-speed burring and adjuvant treatment (e.g., phenol, cement) is the gold standard.
Question 6076
Topic: 10. Pathology and Oncology
A 28-year-old male presents with a permeative lytic lesion of the humerus. A biopsy yields a small round blue cell tumor. The pathologist performs a reticulin stain to help differentiate between primary bone lymphoma and Ewing sarcoma. What is the classic reticulin staining pattern for primary bone lymphoma?
Correct Answer & Explanation
. Reticulin fibers individually surround each tumor cell
Explanation
In primary bone lymphoma, reticulin staining typically highlights a dense network of fibers surrounding individual tumor cells. In contrast, Ewing sarcoma shows reticulin fibers surrounding groups or nests of cells rather than individual cells.
Question 6077
Topic: 10. Pathology and Oncology
A 60-year-old man presents with dull thigh pain. Plain radiographs show a subtle permeative osteolytic lesion in the femoral diaphysis with minimal periosteal reaction. MRI reveals extensive marrow replacement extending far beyond the radiographic abnormalities.
Histological evaluation of a core biopsy will most likely reveal cells that strongly express which of the following markers?
Correct Answer & Explanation
. CD20
Explanation
The clinical and imaging findings are highly characteristic of primary bone lymphoma, often appearing as a 'ghost tumor' with extensive MRI marrow involvement. Primary bone lymphoma is typically a diffuse large B-cell lymphoma, which strongly expresses the B-cell marker CD20.
Question 6078
Topic: 10. Pathology and Oncology
A 22-year-old presents with chronic proximal tibial pain. Radiographs demonstrate a well-defined, eccentric, lucent metaphyseal lesion with a sclerotic rim.
Biopsy shows lobules of myxoid tissue separated by fibrous septa containing giant cells. What is the recommended treatment?
Correct Answer & Explanation
. Intralesional curettage with high-speed burr and bone grafting
Explanation
The diagnosis is a chondromyxoid fibroma (CMF), a rare benign cartilage tumor. The standard treatment is intralesional extended curettage (using a high-speed burr) followed by bone grafting or cementation to minimize the risk of local recurrence.
Question 6079
Topic: 10. Pathology and Oncology
A 45-year-old male is diagnosed with primary diffuse large B-cell lymphoma of the humerus based on an image-guided core needle biopsy. The cortical continuity is preserved, and the calculated Mirels score is 6. What is the most appropriate initial management?
Correct Answer & Explanation
. Multi-agent chemotherapy combined with Rituximab
Explanation
The primary treatment for primary bone lymphoma (diffuse large B-cell) is multi-agent chemotherapy (such as R-CHOP) with or without consolidative radiation. Surgery is strictly reserved for actual or impending fractures, which are unlikely with a Mirels score of 6.
Question 6080
Topic: 10. Pathology and Oncology
A 50-year-old woman complains of persistent knee pain. Plain radiographs show only subtle, ill-defined diaphyseal permeation. MRI reveals uniform low T1 signal replacing the entire distal femoral marrow cavity.
Which core needle biopsy finding would definitively confirm the most likely diagnosis?
Correct Answer & Explanation
. CD45 and CD20 positive sheets of small round blue cells
Explanation
The striking mismatch between minimal plain radiograph findings and extensive MRI marrow replacement is a hallmark of primary bone lymphoma ('ghost tumor'). Biopsy will yield small round blue cells strongly positive for leukocyte common antigen (CD45) and B-cell markers (CD20).
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