Menu

Question 4681

Topic: Bone Tumors

A 22-year-old male presents with dull, aching thoracic back pain that is not significantly relieved by NSAIDs. CT imaging demonstrates a 3.5 cm expansile, lytic lesion in the posterior elements of T11 with a thin sclerotic rim. Histology reveals interlacing trabeculae of woven bone lined by prominent osteoblasts in a highly vascular stroma. What is the most likely diagnosis?

. Osteoid osteoma
. Osteoblastoma
. Aneurysmal bone cyst
. Giant cell tumor
. Chondromyxoid fibroma

Correct Answer & Explanation

. Osteoid osteoma


Explanation

The clinical presentation, anatomic location (posterior elements of the spine), size (>2 cm), and histologic appearance (woven bone, prominent osteoblasts, vascular stroma) are diagnostic of osteoblastoma. Osteoid osteoma shares identical histology but is defined clinically and radiographically as being smaller (typically <1.5-2.0 cm) and the pain is classically dramatically relieved by NSAIDs.

Question 4682

Topic: 10. Pathology and Oncology

A 25-year-old male presents with a deep, slow-growing soft tissue mass in his foot. Biopsy reveals a biphasic tumor with both epithelial and spindle cell components. Which chromosomal translocation is most characteristic of this lesion?

. t(11;22)(q24;q12)
. t(X;18)(p11;q11)
. t(12;16)(q13;p11)
. t(2;13)(q35;q14)
. t(9;22)(q22;q12)

Correct Answer & Explanation

. t(11;22)(q24;q12)


Explanation

The clinical and histological description (biphasic tumor with epithelial and spindle cells in a young adult's distal extremity) is classic for synovial sarcoma. Synovial sarcoma is characterized by the t(X;18) translocation, resulting in the SYT-SSX fusion gene. Options 0, 2, 3, and 4 refer to Ewing sarcoma, myxoid liposarcoma, alveolar rhabdomyosarcoma, and extraskeletal myxoid chondrosarcoma, respectively.

Question 4683

Topic: 10. Pathology and Oncology
A 55-year-old man is diagnosed with a conventional Grade II chondrosarcoma of the proximal femur. What is the most appropriate definitive surgical management?
. Intralesional curettage with adjuvant argon beam coagulation
. Neoadjuvant chemotherapy followed by wide resection
. Wide en bloc resection and reconstruction
. Radiation therapy followed by intralesional curettage
. Primary amputation

Correct Answer & Explanation

. Wide en bloc resection and reconstruction


Explanation

Conventional chondrosarcoma is notoriously resistant to both chemotherapy and radiation. Thus, surgical resection is the primary modality of treatment. While Grade I (atypical cartilaginous tumors) in the appendicular skeleton can sometimes be managed with extended curettage, Grade II and Grade III (high-grade) conventional chondrosarcomas require wide en bloc resection to minimize local recurrence. Reconstruction follows.

Question 4684

Topic: 10. Pathology and Oncology

A 15-year-old boy presents with progressive knee pain and a palpable mass in the distal femoral diaphysis. Radiographs demonstrate a permeative, destructive bone lesion with an "onion skin" periosteal reaction. Biopsy confirms small round blue cells. Which of the following cytogenetic abnormalities is most characteristic of this tumor?

. t(X;18) translocation
. t(9;22) translocation
. t(11;22) translocation
. MDM2 gene amplification
. Mutation in the EXT1 gene

Correct Answer & Explanation

. t(X;18) translocation


Explanation

The clinical presentation, "onion skin" periosteal reaction, and small round blue cell histology are characteristic of Ewing sarcoma. This tumor is strongly associated with the t(11;22) chromosomal translocation, which creates the EWS-FLI1 fusion protein.

Question 4685

Topic: Bone Tumors

A 19-year-old male presents with severe, progressive mid-tibial pain that is worse at night and dramatically relieved by over-the-counter ibuprofen. Radiographs demonstrate a diaphyseal cortical thickening with a small radiolucent nidus. What is the primary mechanism by which non-steroidal anti-inflammatory drugs (NSAIDs) relieve pain in this condition?

. Inhibition of histamine release from local mast cells
. Inhibition of high levels of prostaglandin E2 production by the nidus
. Reversal of local tissue ischemia
. Reduction of mechanical expansion of the periosteum
. Suppression of an inflammatory cytokine cascade from lymphocytes

Correct Answer & Explanation

. Inhibition of histamine release from local mast cells


Explanation

The clinical presentation is classic for an osteoid osteoma. The dramatic pain relief with NSAIDs is highly characteristic, as the central nidus produces large amounts of prostaglandin E2 (PGE2), which is directly inhibited by cyclooxygenase blockers.

Question 4686

Topic: 10. Pathology and Oncology

A 15-year-old male presents with persistent knee pain. Radiographs reveal a mixed lytic and sclerotic lesion in the distal femoral metaphysis with a 'sunburst' periosteal reaction. Biopsy confirms high-grade intramedullary osteosarcoma. What is the standard treatment protocol?

. Wide surgical resection followed by radiation therapy
. Neoadjuvant chemotherapy, wide surgical resection, and adjuvant chemotherapy
. Primary amputation without systemic therapy
. Radiation therapy alone
. Curettage, cryotherapy, and cementation

Correct Answer & Explanation

. Wide surgical resection followed by radiation therapy


Explanation

The standard of care for high-grade classic intramedullary osteosarcoma involves multi-agent neoadjuvant chemotherapy, followed by limb-salvage surgery (wide surgical resection), and then adjuvant chemotherapy. Radiation therapy is rarely used as osteosarcoma is highly radioresistant.

Question 4687

Topic: 10. Pathology and Oncology

A 68-year-old male presents with severe back pain and generalized fatigue. Laboratory studies reveal hypercalcemia, anemia, and an elevated total protein with a monoclonal spike on serum protein electrophoresis (SPEP). Radiographs demonstrate multiple 'punched-out' lytic lesions in the skull and vertebral bodies. Which of the following is the most definitive diagnostic test for the underlying condition?

. Bone scan
. Skeletal survey
. Bone marrow biopsy
. MRI of the whole spine
. Urine immunofixation

Correct Answer & Explanation

. Bone scan


Explanation

The patient's presentation is classic for Multiple Myeloma (CRAB criteria: hyperCalcemia, Renal involvement, Anemia, Bone lesions). While SPEP, urine immunofixation, and skeletal surveys are crucial in the workup, the definitive diagnostic test is a bone marrow aspirate and biopsy demonstrating >10% clonal plasma cells.

Question 4688

Topic: 10. Pathology and Oncology

An 11-year-old boy presents with a painful mass in his diaphyseal femur. Biopsy reveals small round blue cells. Cytogenetic analysis is pending. Which of the following chromosomal translocations is most characteristic of this lesion?

. t(11;22)
. t(9;22)
. t(X;18)
. t(12;16)
. t(2;13)

Correct Answer & Explanation

. t(11;22)


Explanation

Ewing sarcoma is characterized by the t(11;22) translocation, resulting in the EWS-FLI1 fusion protein. t(9;22) is seen in extraskeletal myxoid chondrosarcoma, t(X;18) in synovial sarcoma, t(12;16) in myxoid liposarcoma, and t(2;13) in alveolar rhabdomyosarcoma.

Question 4689

Topic: Bone Tumors

A 24-year-old male complains of severe dull pain in his thoracic spine that is not reliably relieved by NSAIDs. CT scan shows a 2.5 cm radiolucent nidus with posterior element expansion. Histology shows interlacing trabeculae of woven bone lined by prominent osteoblasts. What is the most likely diagnosis?

. Osteoid osteoma
. Osteoblastoma
. Aneurysmal bone cyst
. Osteosarcoma
. Chondroblastoma

Correct Answer & Explanation

. Osteoid osteoma


Explanation

Osteoblastoma is histologically identical to osteoid osteoma but is clinically differentiated by a nidus size >2 cm, less reliable response to NSAIDs, and progressive growth. It frequently involves the posterior elements of the spine.

Question 4690

Topic: Bone Tumors

A 72-year-old woman presents with severe back pain. Laboratory testing shows anemia, hypercalcemia, and renal insufficiency. Radiographs reveal multiple punched-out lytic lesions in her skull and pelvis. Which of the following laboratory findings is most specific for confirming her likely diagnosis?

. Elevated alkaline phosphatase
. Elevated serum prostate-specific antigen
. Monoclonal spike on serum protein electrophoresis (SPEP)
. Elevated erythrocyte sedimentation rate (ESR)
. Decreased serum parathyroid hormone (PTH)

Correct Answer & Explanation

. Elevated alkaline phosphatase


Explanation

The patient's presentation of back pain, anemia, hypercalcemia, renal issues, and lytic bone lesions is highly characteristic of multiple myeloma (CRAB criteria). A monoclonal spike (M-protein) on Serum Protein Electrophoresis (SPEP) or Urine Protein Electrophoresis (UPEP) is a specific diagnostic hallmark.

Question 4691

Topic: 10. Pathology and Oncology

A 35-year-old woman presents with knee pain. Radiographs reveal an eccentric, lytic, epiphyseal lesion in the proximal tibia that extends to the subchondral bone without a sclerotic margin. What is the most likely diagnosis?

. Osteosarcoma
. Ewing sarcoma
. Aneurysmal bone cyst
. Giant cell tumor of bone
. Chondroblastoma

Correct Answer & Explanation

. Osteosarcoma


Explanation

Giant cell tumors (GCT) of bone classically occur in skeletally mature patients (usually 20-40 years old) and present as eccentric, lytic, epiphyseal/metaphyseal lesions that uniquely extend right up to the subchondral bone plate without a sclerotic rim.

Question 4692

Topic: 10. Pathology and Oncology

A 16-year-old boy presents with severe right shin pain that is significantly worse at night and dramatically improves with oral ibuprofen. Radiographs show a thickened cortical area in the tibial diaphysis surrounding a central 5mm radiolucent nidus. Which of the following best describes the expected histological appearance of the central nidus?

. A disorganized cartilage cap with underlying mature trabecular bone
. Spindle cells arranged in a storiform pattern interspersed with multinucleated giant cells
. An interlacing network of immature woven bone trabeculae lined by prominent, plump osteoblasts
. Solid sheets of small round blue cells demonstrating positive PAS staining
. Benign chondrocytes in lacunae exhibiting focal myxoid changes

Correct Answer & Explanation

. A disorganized cartilage cap with underlying mature trabecular bone


Explanation

The clinical and radiographic presentation is classic for an osteoid osteoma. Histologically, the central nidus of an osteoid osteoma is characterized by a sharply demarcated, highly vascularized area of interlacing networks of immature woven bone trabeculae rimmed by prominent, plump, but benign-appearing osteoblasts. Option 0 describes an osteochondroma; Option 1 describes a non-ossifying fibroma or GCT; Option 3 describes Ewing sarcoma.

Question 4693

Topic: 10. Pathology and Oncology

A 15-year-old boy presents with severe thigh pain. Radiographs reveal a destructive, permeative diaphyseal lesion of the femur with a prominent 'onion-skin' periosteal reaction. Histological evaluation confirms a small round blue cell tumor. Which of the following chromosomal translocations is most strongly associated with this patient's diagnosis?

. t(11;22)(q24;q12)
. t(X;18)(p11;q11)
. t(12;16)(q13;p11)
. t(2;13)(q35;q14)
. t(9;22)(q34;q11)

Correct Answer & Explanation

. t(11;22)(q24;q12)


Explanation

The clinical and radiographic presentation is classic for Ewing Sarcoma, a small round blue cell tumor. Ewing sarcoma is characterized by the t(11;22)(q24;q12) translocation in about 85-90% of cases, resulting in the EWS-FLI1 fusion protein. Synovial sarcoma is associated with t(X;18), myxoid liposarcoma with t(12;16), and alveolar rhabdomyosarcoma with t(2;13).

Question 4694

Topic: 10. Pathology and Oncology

A 14-year-old boy presents with progressive knee pain. Plain radiographs reveal an aggressive, ill-defined metaphyseal radiolucent lesion in the distal femur with a 'sunburst' periosteal reaction and a Codman's triangle. A core needle biopsy is obtained. Which of the following histological descriptions is the hallmark finding for the most likely diagnosis?

. Sheets of uniform small round blue cells expressing CD99
. Malignant spindle cells directly producing an amorphous, eosinophilic osteoid matrix
. Abundant multinucleated giant cells interspersed within uniform mononuclear stromal cells
. A disorganized hyaline cartilage cap undergoing irregular endochondral ossification
. Chicken-wire calcifications surrounding mononuclear cells with clear cytoplasm

Correct Answer & Explanation

. Sheets of uniform small round blue cells expressing CD99


Explanation

The clinical and radiographic presentation (metaphyseal lesion, sunburst periosteal reaction, Codman's triangle in a teenager) is classic for osteosarcoma. The defining histological hallmark of osteosarcoma is the production of malignant osteoid by atypical, malignant spindle-shaped mesenchymal cells. Option A describes Ewing sarcoma. Option C describes Giant Cell Tumor. Option D describes an Osteochondroma. Option E describes a Chondroblastoma.

Question 4695

Topic: 10. Pathology and Oncology

A 14-year-old boy presents with progressive knee pain and a palpable distal femoral mass. Biopsy confirms high-grade conventional osteosarcoma. Which of the following underlying genetic mutations is most strongly associated with the pathogenesis of this tumor?

. t(11;22) translocation
. t(X;18) translocation
. Rb1 and p53 tumor suppressor gene mutations
. EXT1 and EXT2 gene mutations
. GNAS1 mutation

Correct Answer & Explanation

. t(11;22) translocation


Explanation

High-grade conventional osteosarcoma is strongly associated with mutations in the Rb1 and p53 tumor suppressor genes, as seen in hereditary retinoblastoma and Li-Fraumeni syndrome. The t(11;22) translocation is characteristic of Ewing sarcoma.

Question 4696

Topic: 10. Pathology and Oncology

A 14-year-old boy presents with a destructive diaphyseal lesion of the femur with an associated periosteal "onion skin" reaction. Biopsy confirms Ewing sarcoma. Which of the following genetic translocations is most characteristic of this diagnosis?

. t(11;22)(q24;q12)
. t(X;18)(p11;q11)
. t(12;16)(q13;p11)
. t(9;22)(q34;q11)
. t(2;13)(q35;q14)

Correct Answer & Explanation

. t(11;22)(q24;q12)


Explanation

Ewing sarcoma is classically associated with the t(11;22)(q24;q12) translocation, which creates the EWS-FLI1 fusion protein. The t(X;18) translocation is seen in synovial sarcoma, and t(12;16) in myxoid liposarcoma.

Question 4697

Topic: 10. Pathology and Oncology

A 15-year-old boy presents with severe, unrelenting mid-thigh pain. Radiographs reveal a permeative, destructive diaphyseal bone lesion in the femur with a prominent 'onion-skin' periosteal reaction. Biopsy demonstrates a population of small, round, blue cells. Which chromosomal translocation is most characteristically associated with this pathology?

. t(11;22)
. t(X;18)
. t(9;22)
. t(12;16)
. t(2;13)

Correct Answer & Explanation

. t(11;22)


Explanation

The clinical presentation and histology are classic for Ewing sarcoma. Ewing sarcoma is characterized by the t(11;22)(q24;q12) chromosomal translocation in about 85% of cases, resulting in the EWS-FLI1 fusion protein. Synovial sarcoma is t(X;18), myxoid liposarcoma is t(12;16), and alveolar rhabdomyosarcoma is t(2;13).

Question 4698

Topic: Bone Tumors

An 8-year-old boy incidentally undergoes radiography of the humerus, revealing a centrally located, well-circumscribed, radiolucent lesion in the proximal metaphysis with a cortical fragment resting at the dependent aspect of the cyst ('fallen leaf' sign). If aspirated, what is the most likely composition of the fluid contained within this lesion?

. Blood with numerous multi-nucleated giant cells
. Clear, serous fluid with high levels of prostaglandins
. Purulent fluid with abundant polymorphonuclear leukocytes
. Thick, mucin-rich viscous fluid
. Lipid-rich fluid containing visible cholesterol crystals

Correct Answer & Explanation

. Blood with numerous multi-nucleated giant cells


Explanation

The 'fallen leaf' sign and the location are pathognomonic for a Unicameral Bone Cyst (UBC), also known as a simple bone cyst. Aspiration of a UBC typically yields clear, yellow, serous fluid that is biochemically characterized by high levels of prostaglandins, particularly PGE2. Aneurysmal bone cysts (ABCs), in contrast, yield blood.

Question 4699

Topic: 10. Pathology and Oncology

A 15-year-old female presents with a distal femoral mass. MRI suggests an aggressive primary bone sarcoma. A biopsy is planned. Which of the following is an absolute principle of a musculoskeletal oncology biopsy?

. Use a transverse incision to minimize tension on the skin
. Ensure the biopsy tract is located outside the planned definitive resection bed
. Use a longitudinal incision in line with the planned definitive resection
. Exsanguinate the limb prior to tourniquet inflation
. Always obtain a frozen section for definitive staging before closing

Correct Answer & Explanation

. Use a transverse incision to minimize tension on the skin


Explanation

A biopsy for a suspected bone sarcoma must utilize a longitudinal incision that is perfectly in line with the planned definitive excision. This allows the entire biopsy tract to be excised en bloc with the tumor to prevent local recurrence.

Question 4700

Topic: 10. Pathology and Oncology

A 14-year-old boy presents with a painful, swollen thigh. Radiographs show a permeative, 'moth-eaten' lesion in the femoral diaphysis with an 'onion-skin' periosteal reaction. Cytogenetic testing of the biopsy specimen is most likely to reveal which of the following chromosomal translocations?

. t(X;18)
. t(12;16)
. t(11;22)
. t(9;22)
. t(2;13)

Correct Answer & Explanation

. t(X;18)


Explanation

The presentation is classic for Ewing sarcoma. Over 90% of Ewing sarcomas are characterized by the t(11;22)(q24;q12) translocation, which results in the EWS-FLI1 fusion protein. Synovial sarcoma is associated with t(X;18), and myxoid liposarcoma with t(12;16).