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10. Pathology and Oncology MCQs

Practice Set 51 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.

Question 1001

Topic: 10. Pathology and Oncology

During an oral boards oncology scenario, you are presented with a radiograph of a 16-year-old with a destructive, mixed lytic and sclerotic metaphyseal lesion of the distal femur with a Codman's triangle. A biopsy confirms osteosarcoma. What is the standard, evidence-based sequence of treatment for non-metastatic high-grade extremity osteosarcoma?

. Primary amputation followed by adjuvant radiation
. Neoadjuvant chemotherapy, wide surgical resection, and adjuvant chemotherapy
. Primary wide surgical resection followed by adjuvant radiation
. Neoadjuvant radiation, wide surgical resection, and adjuvant chemotherapy
. Intralesional curettage, cementation, and targeted biologic therapy

Correct Answer & Explanation

. Neoadjuvant chemotherapy, wide surgical resection, and adjuvant chemotherapy

Explanation

The standard of care for high-grade, non-metastatic extremity osteosarcoma is neoadjuvant (preoperative) chemotherapy, followed by wide surgical resection, and then adjuvant (postoperative) chemotherapy. Radiation is generally not a primary modality as osteosarcoma is relatively radioresistant.

Question 1002

Topic: 10. Pathology and Oncology

During a pathology review section, you are shown a histology slide of a bone tumor revealing uniform, small, round blue cells with scant cytoplasm. Cytogenetics reveals a t(11;22) chromosomal translocation. Which fusion gene is characteristically produced by this translocation?

. SYT-SSX
. TLS-CHOP
. PAX3-FKHR
. EWS-FLI1
. BCR-ABL

Correct Answer & Explanation

. EWS-FLI1

Explanation

Ewing sarcoma is classically characterized by the t(11;22)(q24;q12) translocation, which fuses the EWS gene on chromosome 22 with the FLI1 gene on chromosome 11, forming the EWS-FLI1 fusion protein.

Question 1003

Topic: 10. Pathology and Oncology
In the Enneking surgical staging system for musculoskeletal sarcomas, which of the following is characteristic of Stage IIB?
. Low-grade, intra-compartmental, no metastases
. Low-grade, extra-compartmental, no metastases
. High-grade, intra-compartmental, no metastases
. High-grade, extra-compartmental, no metastases
. Any grade, any compartmental, with regional metastases

Correct Answer & Explanation

. High-grade, extra-compartmental, no metastases

Explanation

The Enneking surgical staging system classifies musculoskeletal sarcomas based on Grade (G), Location (T), and Metastasis (M). Grade is either Low (G0) or High (G1). Location is either Intra-compartmental (T0) or Extra-compartmental (T1). Metastasis is either None (M0) or Present (M1). Stage II corresponds to high-grade lesions (G1). Stage IIA is high-grade, intra-compartmental (G1T0M0). Stage IIB is high-grade, extra-compartmental (G1T1M0). Stage III involves any grade with metastasis (M1). Therefore, IIB is High-grade, extra-compartmental, with no regional or distant metastases.

Question 1004

Topic: 10. Pathology and Oncology

Which of the following imaging modalities is considered the gold standard for assessing the local extent and relationship of a primary soft tissue sarcoma to neurovascular structures, critical for surgical planning?

. X-ray
. CT scan with contrast
. Bone scan
. MRI with contrast
. PET-CT scan

Correct Answer & Explanation

. MRI with contrast

Explanation

Correct Answer: DAcademic Rationale:MRI with contrast is considered the gold standard for evaluating the local extent of a soft tissue sarcoma. It provides superior soft tissue resolution compared to CT and is excellent for delineating the tumor's margins, its relationship to critical neurovascular bundles, muscle compartments, and bone, which are all crucial for planning wide surgical excision and determining the feasibility of limb salvage. While CT is useful for bone involvement and chest staging, and PET-CT for metabolic activity and distant metastases, MRI offers the best local anatomical detail for surgical planning.

Question 1005

Topic: 10. Pathology and Oncology

In the management of osteosarcoma, what is the primary purpose of obtaining a 'surgical margin' and how is it defined?

. To assess tumor grade; defined by the degree of tumor necrosis.
. To predict chemotherapy response; defined by tumor shrinkage.
. To achieve local tumor control; defined by the microscopic distance between the tumor and the cut edge of the resected specimen.
. To determine metastatic potential; defined by lymph node involvement.
. To select the type of limb reconstruction; defined by bone involvement.

Correct Answer & Explanation

. To achieve local tumor control; defined by the microscopic distance between the tumor and the cut edge of the resected specimen.

Explanation

Correct Answer: CAcademic Rationale:The primary purpose of obtaining a 'surgical margin' in osteosarcoma (and other sarcomas) is to achieve local tumor control. It is defined by the microscopic distance (or lack thereof) between the tumor cells and the cut edge of the resected surgical specimen. A 'wide' or 'negative' margin (R0 resection) means no tumor cells are seen at the cut edge, which significantly reduces the risk of local recurrence. This is distinct from tumor grade, chemotherapy response, metastatic potential, or reconstruction choice, although all are interrelated aspects of oncology.

Question 1006

Topic: 10. Pathology and Oncology

Which of the following statements regarding osteosarcoma is FALSE?

. It is the most common primary malignant bone tumor in children and adolescents.
. The most common sites are the metaphysis of long bones (distal femur, proximal tibia, proximal humerus).
. Chemotherapy is an essential part of treatment, both neoadjuvantly and adjuvantly.
. Pulmonary metastasis is the most common site of metastatic disease.
. Radiation therapy is the primary treatment modality for localized disease.

Correct Answer & Explanation

. Radiation therapy is the primary treatment modality for localized disease.

Explanation

Correct Answer: ERadiation therapy is generally NOT the primary treatment modality for localized osteosarcoma. Surgical resection with wide margins is the cornerstone of local control. Osteosarcoma is relatively radioresistant. Chemotherapy, both neoadjuvant (pre-operative) and adjuvant (post-operative), is critical for controlling micrometastatic disease and improving survival. The other statements are true: osteosarcoma is the most common primary malignant bone tumor in children/adolescents, commonly affects the metaphysis of long bones, and frequently metastasizes to the lungs.

Question 1007

Topic: 10. Pathology and Oncology

A 24-year-old male presents with a deep-seated mass near his knee joint. Biopsy reveals a biphasic spindle cell tumor. What is the characteristic chromosomal translocation associated with this pathology?

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

Correct Answer & Explanation

. t(X;18)

Explanation

Synovial sarcoma is characterized by the t(X;18)(p11;q11) translocation, resulting in the SYT-SSX fusion gene. It commonly presents in young adults as a deep-seated soft tissue mass near a joint.

Question 1008

Topic: 10. Pathology and Oncology

A 16-year-old female with classic osteosarcoma of the distal femur completes neoadjuvant chemotherapy followed by wide surgical resection. Histological analysis of the resected specimen shows 95% tumor necrosis. What is the most significant prognostic implication of this finding?

. Increased risk of local recurrence
. Decreased need for adjuvant chemotherapy
. Improved overall survival
. Indication to switch to radiation therapy
. High likelihood of synchronous skip metastases

Correct Answer & Explanation

. Improved overall survival

Explanation

Tumor necrosis of 90% or greater following neoadjuvant chemotherapy in osteosarcoma indicates a good histologic response. This is the single most important prognostic factor for improved overall survival.

Question 1009

Topic: Soft Tissue Tumors & Metastasis

A 45-year-old male is diagnosed with a massive deep myxoid liposarcoma of the thigh. Staging chest CT is negative. Which additional imaging study is uniquely critical for complete staging of this specific histologic subtype?

. PET-CT scan
. Bone scan
. MRI of the total spine
. CT of the brain
. Ultrasound of regional lymph nodes

Correct Answer & Explanation

. MRI of the total spine

Explanation

Myxoid liposarcoma has a unique propensity for extrapulmonary metastases, particularly to the spine and other bone marrow sites. An MRI of the total spine (or whole-body MRI) is recommended for accurate staging.

Question 1010

Topic: 10. Pathology and Oncology
According to the Enneking surgical staging system for malignant bone tumors, how is a high-grade osteosarcoma that has broken through the cortex into the surrounding soft tissue but lacks regional or distant metastases classified?
. Stage IA
. Stage IB
. Stage IIA
. Stage IIB
. Stage III

Correct Answer & Explanation

. Stage IIB

Explanation

In the Enneking staging system, Stage II denotes a high-grade tumor. The suffix "B" indicates an extracompartmental lesion, making a tumor extending into surrounding soft tissue a Stage IIB.

Question 1011

Topic: 10. Pathology and Oncology

A 10-year-old boy presents with fever, elevated ESR, and thigh pain. Radiographs reveal a permeative diaphyseal lesion in the femur with a laminated periosteal reaction. Biopsy shows small round blue cells strongly positive for CD99. Which of the following characterizes the optimal definitive management of this condition?

. Wide surgical resection alone
. Neoadjuvant chemotherapy followed by local control and adjuvant chemotherapy
. Primary radiation therapy alone
. Intralesional curettage with cementation
. Amputation without adjuvant therapy

Correct Answer & Explanation

. Neoadjuvant chemotherapy followed by local control and adjuvant chemotherapy

Explanation

Ewing sarcoma is a highly aggressive, systemic disease from the outset. Standard management involves neoadjuvant systemic chemotherapy, followed by local control via wide surgical resection or radiation, and then adjuvant chemotherapy.

Question 1012

Topic: 10. Pathology and Oncology

A 55-year-old male presents with deep shoulder pain. Imaging shows a lytic lesion in the proximal humerus with popcorn calcifications, endosteal scalloping, and soft tissue extension. Biopsy confirms a grade 3 conventional chondrosarcoma. What is the recommended treatment?

. Intralesional curettage with phenol and bone cement
. Wide surgical resection
. Neoadjuvant chemotherapy followed by wide resection
. Primary radiation therapy
. Radiofrequency ablation

Correct Answer & Explanation

. Wide surgical resection

Explanation

High-grade (Grade 2 and 3) conventional chondrosarcomas are generally resistant to chemotherapy and radiotherapy. The standard treatment is wide surgical resection to achieve negative margins.

Question 1013

Topic: 10. Pathology and Oncology

A 12-year-old boy presents with a diaphyseal femur lesion accompanied by an "onion skin" periosteal reaction. Biopsy reveals small round blue cells that are strongly positive for CD99. Which chromosomal translocation is most commonly associated with this pathology?

. 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

Ewing sarcoma is characterized by small round blue cells, CD99 positivity, and an "onion skin" periosteal reaction. It is defined by the t(11;22) translocation, which creates the EWS-FLI1 fusion protein.

Question 1014

Topic: 10. Pathology and Oncology

A 30-year-old male presents with a slowly enlarging, painful mass near his ankle joint. MRI shows a deep mass with heterogeneous signal intensity. Core needle biopsy reveals a biphasic tumor with both spindle and epithelial cell components. Which genetic abnormality is diagnostic for this tumor?

. MDM2 amplification
. t(X;18) (p11;q11)
. t(12;16) (q13;p11)
. t(2;13) (q35;q14)
. GNAS mutation

Correct Answer & Explanation

. t(X;18) (p11;q11)

Explanation

The clinical presentation and biphasic histology are classic for synovial sarcoma. This aggressive soft tissue sarcoma is genetically defined by the t(X;18) translocation, resulting in the SYT-SSX fusion gene.

Question 1015

Topic: 10. Pathology and Oncology
A 15-year-old male with conventional osteosarcoma of the proximal tibia undergoes 10 weeks of neoadjuvant MAP (methotrexate, doxorubicin, cisplatin) chemotherapy, followed by wide surgical resection. Which histological finding in the resected specimen is most strongly associated with a favorable overall survival?
. Tumor necrosis > 90%
. Presence of prominent chondroblastic matrix
. Development of a thick fibrous pseudocapsule
. Tumor necrosis between 50% and 90%
. High vascular endothelial growth factor (VEGF) expression

Correct Answer & Explanation

. Tumor necrosis > 90%

Explanation

The degree of tumor necrosis following neoadjuvant chemotherapy is the single most important prognostic factor in osteosarcoma. Greater than 90% necrosis (Huvos Grade III or IV) indicates an excellent histologic response and correlates with significantly improved survival.

Question 1016

Topic: 10. Pathology and Oncology

A 28-year-old female presents with a slow-growing mass in her popliteal fossa. Biopsy reveals a biphasic tumor with both epithelial and spindle cell components. Which chromosomal translocation is most characteristic of this specific sarcoma?

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

Correct Answer & Explanation

. t(X;18)(p11;q11)

Explanation

The patient has a synovial sarcoma, which is classically associated with the t(X;18)(p11;q11) translocation resulting in the SYT-SSX fusion gene. The t(11;22) translocation is characteristic of Ewing sarcoma, while t(12;16) is seen in myxoid liposarcoma.

Question 1017

Topic: 10. Pathology and Oncology
A 50-year-old male presents with dull right shoulder pain. Radiographs reveal a lytic lesion in the proximal humerus with 'popcorn' calcifications. MRI demonstrates endosteal scalloping greater than two-thirds of the cortical thickness. Biopsy confirms a Grade II chondrosarcoma. What is the mainstay of treatment?
. Reassurance and clinical observation
. Intralesional curettage with phenol adjuvant and bone grafting
. Wide surgical resection
. Neoadjuvant chemotherapy followed by wide resection
. Definitive external beam radiation therapy

Correct Answer & Explanation

. Wide surgical resection

Explanation

The standard of care for Grade II (intermediate) and Grade III (high-grade) conventional chondrosarcomas is wide surgical resection. Conventional chondrosarcomas are generally resistant to both chemotherapy and radiation therapy.

Question 1018

Topic: 10. Pathology and Oncology
A 15-year-old boy completes neoadjuvant chemotherapy and undergoes wide resection for conventional high-grade osteosarcoma of the distal femur. Pathological examination of the resected specimen reveals 95% tumor necrosis. What does this histopathologic finding indicate regarding his prognosis?
. It is a poor prognostic sign requiring an immediate change to a salvage chemotherapy regimen.
. It indicates a favorable prognosis (Huvos Grade III/IV), but the patient must still complete the planned adjuvant chemotherapy.
. It indicates an excellent prognosis, allowing for the complete cessation of all further adjuvant chemotherapy.
. It suggests a high likelihood of local recurrence due to soft tissue seeding.
. It implies the original biopsy was misdiagnosed and the tumor is actually an Ewing sarcoma.

Correct Answer & Explanation

. It indicates a favorable prognosis (Huvos Grade III/IV), but the patient must still complete the planned adjuvant chemotherapy.

Explanation

Tumor necrosis of 90% or greater (Huvos Grade III or IV) following neoadjuvant chemotherapy is the most significant positive prognostic indicator for overall survival in osteosarcoma. However, patients must still complete their standardized postoperative adjuvant chemotherapy course.

Question 1019

Topic: 10. Pathology and Oncology

A 9-year-old boy presents with a 1-month history of progressive thigh pain and low-grade fevers. Radiographs demonstrate a permeative diaphyseal lesion with a classic 'onion-skin' periosteal reaction. A core needle biopsy confirms Ewing sarcoma. Which fusion gene is most likely present in this patient's tumor cells?

. SYT-SSX
. EWS-FLI1
. PAX3-FOXO1
. FUS-DDIT3
. EWS-CHN

Correct Answer & Explanation

. EWS-FLI1

Explanation

Ewing sarcoma is driven by a balanced translocation, most commonly t(11;22)(q24;q12), which fuses the EWS gene on chromosome 22 to the FLI1 gene on chromosome 11. This creates the EWS-FLI1 fusion oncoprotein, present in approximately 85-90% of cases.

Question 1020

Topic: 10. Pathology and Oncology

A 12-year-old boy presents with a diaphyseal femur lesion. Biopsy shows a proliferation of small blue round cells. Which chromosomal translocation and resulting fusion gene are most characteristic of this diagnosis?

. t(11;22)(q24;q12) EWS-FLI1
. t(2;13)(q35;q14) PAX3-FOXO1
. t(X;18)(p11;q11) SYT-SSX1
. t(12;16)(q13;p11) FUS-CHOP
. t(9;22)(q22;q12) EWS-CHN

Correct Answer & Explanation

. t(11;22)(q24;q12) EWS-FLI1

Explanation

Ewing sarcoma is classically associated with the t(11;22)(q24;q12) translocation, resulting in the EWS-FLI1 fusion protein. This acts as an aberrant transcription factor that drives tumorigenesis.

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