Menu

Question 4781

Topic: Bone Tumors

A 16-year-old male is receiving neoadjuvant chemotherapy for osteosarcoma using the standard MAP regimen (Methotrexate, Doxorubicin, Cisplatin). He subsequently develops irreversible congestive heart failure. Which of the chemotherapeutic agents is most likely responsible for this specific toxicity?

. Methotrexate
. Doxorubicin
. Cisplatin
. Ifosfamide
. Vincristine

Correct Answer & Explanation

. Doxorubicin


Explanation

Doxorubicin (Adriamycin) is an anthracycline known for its dose-dependent, cumulative, and potentially irreversible cardiotoxicity (dilated cardiomyopathy and congestive heart failure). Cisplatin is associated with ototoxicity and nephrotoxicity. Methotrexate is associated with mucositis, hepatotoxicity, and renal toxicity.

Question 4782

Topic: 10. Pathology and Oncology

A 55-year-old male presents with chronic back pain and bowel/bladder dysfunction. Imaging reveals a destructive, midline lesion centered in the sacrum. Biopsy demonstrates lobules of large cells with abundant vacuolated cytoplasm (physaliferous cells) set in a myxoid background. The tumor cells are most likely to show strong immunohistochemical positivity for which of the following markers?

. Cytokeratin only
. S100 only
. Brachyury
. CD34
. Myogenin

Correct Answer & Explanation

. Brachyury


Explanation

The clinical picture and histology (physaliferous cells) describe a chordoma, which arises from notochord remnants. Brachyury is a transcription factor critical for notochord development and is a highly sensitive and specific immunohistochemical marker for chordoma.

Question 4783

Topic: 10. Pathology and Oncology

A 20-year-old female presents with mild chronic leg pain. Radiographs show an eccentric, lytic, expansile lesion in the proximal tibial metaphysis with a well-defined, scalloped sclerotic margin. Histology reveals a pseudolobular architecture with stellate to spindle-shaped cells in a myxoid stroma, and osteoclast-like giant cells at the lobule peripheries. What is the diagnosis?

. Chondroblastoma
. Chondromyxoid fibroma
. Non-ossifying fibroma
. Giant cell tumor of bone
. Enchondroma

Correct Answer & Explanation

. Chondromyxoid fibroma


Explanation

Chondromyxoid fibroma (CMF) is a rare benign bone tumor typically presenting as an eccentric, expansile metaphyseal lesion with a sclerotic, scalloped rim. Histologically, it has a classic pseudolobular pattern with stellate cells in a myxoid background, and hypercellular peripheries containing giant cells.

Question 4784

Topic: Bone Tumors

A patient diagnosed with polyostotic fibrous dysplasia presents with a newly discovered, painless, deep soft tissue mass in the thigh. Excisional biopsy of the mass reveals a well-circumscribed lesion composed of bland spindle cells in an abundant myxoid stroma with decreased vascularity. The presence of this soft tissue lesion in conjunction with fibrous dysplasia is known as:

. McCune-Albright syndrome
. Mazabraud syndrome
. Ollier disease
. Maffucci syndrome
. Jaffe-Campanacci syndrome

Correct Answer & Explanation

. Mazabraud syndrome


Explanation

Mazabraud syndrome is a rare disorder characterized by the association of fibrous dysplasia (usually polyostotic) and one or more intramuscular myxomas. McCune-Albright involves fibrous dysplasia, precocious puberty, and cafe-au-lait spots.

Question 4785

Topic: 10. Pathology and Oncology

A 28-year-old female presents with a painless mass behind her knee. Radiographs reveal a densely ossified mass attached to the posterior cortex of the distal femur by a broad base, with a radiolucent cleft between the tumor and the underlying bone cortex (string sign). Molecular analysis of this tumor is most likely to reveal an amplification of which of the following?

. TP53
. RB1
. MDM2
. EWSR1
. MYC

Correct Answer & Explanation

. MDM2


Explanation

The presentation is classic for a parosteal osteosarcoma (surface, low-grade, posterior distal femur, 'string sign'). Genetically, parosteal osteosarcomas (and low-grade central osteosarcomas) are characterized by supernumerary ring chromosomes containing amplifications of the 12q13-15 region, which includes the MDM2 and CDK4 genes.

Question 4786

Topic: 10. Pathology and Oncology

A 40-year-old male undergoes wide excision of a deep thigh mass, which is diagnosed histologically as a myxoid liposarcoma (t(12;16) translocation). Unlike most soft tissue sarcomas of the extremities, which primarily metastasize to the lungs, myxoid liposarcoma has a uniquely high propensity to metastasize to which of the following locations?

. Liver
. Brain
. Lymph nodes
. Bone and spine
. Skin

Correct Answer & Explanation

. Bone and spine


Explanation

Myxoid liposarcoma has a well-known, unique propensity for extrapulmonary metastasis, specifically to the skeletal system (especially the spine), often before pulmonary metastases occur. Staging should include whole-spine MRI or bone scintigraphy/PET-CT to rule out bony disease.

Question 4787

Topic: 10. Pathology and Oncology

A 28-year-old female presents with a painless mass on the posterior aspect of her distal thigh. Radiographs show a densely ossified mass attached to the posterior femoral cortex with a broad base and no medullary involvement. Which genetic amplification is most specific to this lesion?

. t(11;22) translocation
. USP6 rearrangement
. MDM2 and CDK4 amplification
. GNAS mutation
. t(X;18) translocation

Correct Answer & Explanation

. MDM2 and CDK4 amplification


Explanation

Parosteal osteosarcoma typically presents as a densely ossified mass on the posterior distal femur. It is a low-grade surface osteosarcoma characterized by a ring chromosome 12p leading to MDM2 and CDK4 amplification.

Question 4788

Topic: 10. Pathology and Oncology

A 24-year-old male presents with a slowly enlarging, painless mass near his knee joint. MRI reveals a deep soft-tissue mass adjacent to the joint capsule with small areas of calcification. Biopsy confirms a malignant biphasic tumor. Which chromosomal abnormality is diagnostic?

. 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(X;18) (p11;q11)


Explanation

Synovial sarcoma frequently occurs in young adults near large joints and can exhibit calcifications. The pathognomonic chromosomal translocation is t(X;18)(p11;q11), creating the SYT-SSX fusion gene.

Question 4789

Topic: 10. Pathology and Oncology

A 65-year-old male with a history of renal cell carcinoma presents with a painful destructive lesion in his subtrochanteric femur. Mirels' score is 10. Prophylactic fixation is planned. What is the most critical step prior to surgical intervention?

. Neoadjuvant chemotherapy
. Preoperative selective arterial embolization
. Neoadjuvant radiation therapy
. Systemic administration of bisphosphonates
. Core needle biopsy

Correct Answer & Explanation

. Preoperative selective arterial embolization


Explanation

Renal cell carcinoma and thyroid carcinoma metastases to bone are highly vascular. Preoperative selective arterial embolization is critical to minimize life-threatening intraoperative blood loss.

Question 4790

Topic: 10. Pathology and Oncology

A 55-year-old male presents with bowel and bladder dysfunction and sacral pain. Imaging reveals a midline destructive sacral lesion. Histology shows physaliferous cells with vacuolated cytoplasm. Which immunohistochemical marker is highly specific for this diagnosis?

. Cytokeratin
. S-100
. Vimentin
. Brachyury
. CD99

Correct Answer & Explanation

. Brachyury


Explanation

Chordoma is a locally aggressive tumor arising from notochordal remnants, typically occurring in the sacrum or clivus. Brachyury is a transcription factor critical for notochord development and is highly sensitive and specific for chordomas.

Question 4791

Topic: Bone Tumors

An 18-year-old female presents with a rapidly expanding, painful lytic lesion in her distal fibula. MRI shows an expansile lesion with multiple fluid-fluid levels. Genetic analysis reveals a characteristic rearrangement. Which gene is primarily involved?

. EWSR1
. USP6
. GNAS
. EXT1
. RB1

Correct Answer & Explanation

. USP6


Explanation

Primary aneurysmal bone cysts (ABCs) are benign but locally aggressive neoplasms driven by USP6 gene rearrangements. MRI typically shows characteristic fluid-fluid levels indicating blood components of varying ages.

Question 4792

Topic: 10. Pathology and Oncology

A 32-year-old male presents with an anterior tibial bowing deformity and pain. Radiographs reveal a multiloculated, expansile, eccentric intracortical radiolucency in the tibial diaphysis. Histology shows biphasic spindle and epithelial cells. What is the most appropriate definitive surgical treatment?

. Intralesional curettage with local adjuvants
. Wide resection and segmental reconstruction
. Amputation
. Radiation therapy followed by curettage
. Systemic chemotherapy alone

Correct Answer & Explanation

. Wide resection and segmental reconstruction


Explanation

Adamantinoma is a low-grade malignant bone tumor that almost exclusively occurs in the tibial diaphysis. Because it is resistant to radiation and chemotherapy, wide surgical resection with segmental reconstruction is the standard of care to prevent local recurrence.

Question 4793

Topic: Bone Tumors

A 68-year-old female complains of generalized bone pain and fatigue. Radiographs show multiple "punched-out" lytic lesions in her skull and pelvis. A bone scan shows no increased uptake in these areas. Which of the following laboratory findings is most likely to confirm the diagnosis?

. Elevated serum alkaline phosphatase
. Monoclonal spike on serum protein electrophoresis
. Positive Bence-Jones protein but normal serum calcium
. Elevated prostate-specific antigen
. High titer of antinuclear antibodies

Correct Answer & Explanation

. Monoclonal spike on serum protein electrophoresis


Explanation

Multiple myeloma frequently presents with punched-out lytic bone lesions that are characteristically "cold" on technetium bone scans due to osteoblast suppression. Diagnosis is confirmed by demonstrating a monoclonal paraprotein spike on serum or urine protein electrophoresis.

Question 4794

Topic: 10. Pathology and Oncology

A 50-year-old male undergoes wide resection of a high-grade pleomorphic sarcoma of the thigh. He receives preoperative radiation therapy. Compared to postoperative radiation, preoperative radiation is associated with which of the following?

. Higher total radiation dose
. Increased long-term fibrosis and joint contracture
. Higher rate of acute wound healing complications
. Lower overall survival rate
. Increased risk of local recurrence

Correct Answer & Explanation

. Higher rate of acute wound healing complications


Explanation

Preoperative radiation therapy for soft tissue sarcomas uses a lower total dose and has less long-term tissue fibrosis compared to postoperative radiation. However, it is strongly associated with a significantly higher rate of acute postoperative wound complications.

Question 4795

Topic: 10. Pathology and Oncology

A 14-year-old boy presents with chronic knee pain. Radiographs reveal a well-circumscribed, 2 cm lytic lesion in the proximal tibial epiphysis with a thin sclerotic margin and central calcifications. What is the most common histological finding associated with this tumor?

. Chicken-wire calcification surrounding mononuclear cells
. Spindle cells with a herringbone pattern
. Malignant cartilage with cortical destruction
. Physaliferous cells with mucinous background
. Hyperchromatic round cells in a pseudo-rosette pattern

Correct Answer & Explanation

. Chicken-wire calcification surrounding mononuclear cells


Explanation

Chondroblastoma is a rare benign cartilage tumor characteristically arising in the epiphysis of skeletally immature patients. Histology typically shows chondroblasts, multinucleated giant cells, and fine pericellular "chicken-wire" calcifications.

Question 4796

Topic: 10. Pathology and Oncology

A 45-year-old male has a large, deep mass in his thigh. Core biopsy confirms myxoid liposarcoma. Which chromosomal translocation is classically associated with this tumor, and where is the most common site of metastasis?

. t(X;18); lung
. t(12;16); bone/spine
. t(2;13); lymph nodes
. t(11;22); liver
. t(1;2); brain

Correct Answer & Explanation

. t(12;16); bone/spine


Explanation

Myxoid liposarcoma is characterized by the t(12;16) translocation (FUS-DDIT3 fusion). Unlike most other soft tissue sarcomas that metastasize primarily to the lungs, myxoid liposarcoma has a unique propensity to metastasize to extrapulmonary sites, notably the spine and other bones.

Question 4797

Topic: Bone Tumors

A 19-year-old male presents with persistent, dull aching back pain that is worse at night. The pain is not relieved by ibuprofen. CT imaging reveals a 2.5 cm radiolucent nidus with surrounding sclerosis in the posterior elements of L4. What is the most likely diagnosis?

. Osteoid osteoma
. Osteoblastoma
. Aneurysmal bone cyst
. Ewing sarcoma
. Chondromyxoid fibroma

Correct Answer & Explanation

. Osteoblastoma


Explanation

Osteoblastoma is histologically similar to osteoid osteoma but is distinguished clinically by a nidus larger than 1.5-2.0 cm. Additionally, the pain from osteoblastoma is typically less responsive to NSAIDs compared to osteoid osteoma.

Question 4798

Topic: 10. Pathology and Oncology

A 10-year-old girl is evaluated for multiple bony deformities and multiple subcutaneous soft-tissue hemangiomas. Radiographs show multiple cartilaginous lesions within the medullary cavity of her tubular bones. She is at highest risk for developing which of the following malignancies?

. Osteosarcoma
. Chondrosarcoma
. Angiosarcoma
. Leukemia
. Ewing sarcoma

Correct Answer & Explanation

. Chondrosarcoma


Explanation

This clinical presentation describes Maffucci syndrome, characterized by multiple enchondromas and soft-tissue hemangiomas. Patients have a nearly 100% lifetime risk of malignant transformation, most commonly to chondrosarcoma.

Question 4799

Topic: 10. Pathology and Oncology

A 20-year-old male presents with swelling over the anterior mid-tibia. Imaging reveals a surface lesion with sunburst periosteal reaction, cortical thickening, and no intramedullary involvement. Biopsy shows an intermediate-grade tumor with predominantly chondroblastic matrix. What is the appropriate treatment?

. Wide local excision only
. Neoadjuvant chemotherapy and wide local excision
. Intralesional curettage with cryotherapy
. Radiation therapy followed by wide excision
. Amputation

Correct Answer & Explanation

. Neoadjuvant chemotherapy and wide local excision


Explanation

Periosteal osteosarcoma is an intermediate-grade surface osteosarcoma that predominantly produces a chondroblastic matrix. Because of its intermediate grade and higher risk of metastasis compared to parosteal variants, standard treatment includes neoadjuvant chemotherapy followed by wide surgical resection.

Question 4800

Topic: Bone Tumors

A 24-year-old male presents with chronic axial back pain. He notes that the pain is dull, constant, and only mildly relieved by non-steroidal anti-inflammatory drugs (NSAIDs). Radiographs and a subsequent CT scan reveal a 3.0 cm expansile, lytic lesion in the posterior elements of L4. Histologically, the lesion shows a loose fibrovascular stroma with interlacing trabeculae of osteoid lined by prominent osteoblasts. What is the most likely diagnosis?

. Osteoid osteoma
. Osteoblastoma
. Aneurysmal bone cyst
. Osteosarcoma
. Ewing sarcoma

Correct Answer & Explanation

. Osteoblastoma


Explanation

The clinical and radiographic presentation is classic for an osteoblastoma. While histologically identical to an osteoid osteoma, an osteoblastoma is larger (by definition >2 cm), typically involves the posterior elements of the spine, and causes pain that is often NOT dramatically relieved by NSAIDs or salicylates.