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10. Pathology and Oncology MCQs

Practice Set 111 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 2201

Topic: 10. Pathology and Oncology

A 66-year-old man reports a 2-week history of worsening low back and leg pain. He reports that his pain is aggravated by lying down and relieved by standing and walking. He notes that he has been losing weight recently and that his pain has been awakening him during the night. His medical history is significant for hypertension, coronary artery disease, and prostate cancer. His physical examination is essentially unremarkable. Lumbar radiographs are within normal limits. What is the most appropriate management for this patient?

. MRI of chest
. Laboratory studies, including a complete blood cell (CBC) count, erythrocyte sedimentation rate (ESR), and urinalysis, PSA, CEA
. Activity alterations to avoid undue back irritation
. Comfort measures, including medications
. Spinal manipulative therapy within the first 6 weeks

Correct Answer & Explanation

. Laboratory studies, including a complete blood cell (CBC) count, erythrocyte sedimentation rate (ESR), and urinalysis, PSA, CEA

Explanation

In the initial assessment of acute low back pain in adults, no diagnostic testing is indicated during the first 4 weeks in the absence of "red flags" for a serious underlying condition. The purpose of the initial assessment of acute low back pain in adults is to rule out serious underlying conditions presenting as low back pain. The Agency for Healthcare Policy and Research, in its 1994 clinical practice guideline, identified four serious conditions that may present with low back pain, including fracture, tumor, infection, and cauda equina syndrome. This patient has five "red flags" for a spinal tumor as a possible etiology of his low back pain, including age of older than 50 years, constitutional symptoms (recent weight loss), pain worse when supine, severe nighttime pain, and a history of cancer. Of these, his history of cancer is most significant, as greater than 90% of spinal tumors are metastatic. In order of frequency, breast, prostate, lung, and kidney make up approximately 80% of all secondary spread to the spine. In the presence of "red flags" for tumor or infection, it is recommended that the clinician obtain a CBC count, ESR, and a urinalysis. If these are within normal limits and suspicions still remain, consider consultation or seek further evidence with a bone scan, radiographs, or additional laboratory studies. Negative radiographs alone are insufficient to rule out disease. If radiographs are positive, the anatomy can be better defined with MRI. Agency for Health Care Policy and Research, Bigos SJ (ed): Acute Low Back Problems in Adults. Rockville, MD, US Department of Health and Human Services, AHCPR Publication 95-0642, Clinical Practice Guideline #14, 1994.

Question 2202

Topic: 10. Pathology and Oncology

A 60-year-old woman has a mass in the right scapula. Figures 25a and 25b show a CT scan and a biopsy specimen. The cells are lymphocyte common antigen positive, Ewing's specific antigen (CD99) negative, and keratin negative. What is the next step in management?

. Skeletal survey
. PET scan
. Indium-labeled WBC scan
. Bone marrow aspiration and biopsy
. Mammography

Correct Answer & Explanation

. Bone marrow aspiration and biopsy

Explanation

The clinical history, CT scan, and histology are most consistent with a lymphoma of bone. An important part of the staging is bone marrow aspiration and biopsy. The other studies listed are not indicated. Lymphoma of bone, when localized, is usually treated with chemotherapy and radiation therapy and has excellent survival rates. Widespread lymphoma has a worse prognosis. Finiewicz K, van Biesen K: Non-Hodgkins lymphoma, in Golomb H, Vokes E (eds): Oncologic Therapies, ed 2. Berlin, Germany, Springer, 2003, pp 295-318.

Question 2203

Topic: 10. Pathology and Oncology

The biopsy specimens seen in Figures 55a and 55b are from a lytic lesion in the sacrum of a 58-year-old man. What is the most likely diagnosis?

. Chondrosarcoma
. Metastatic renal cell carcinoma
. Chondromyxoid fibroma
. Chordoma
. Giant cell tumor

Correct Answer & Explanation

. Chordoma

Explanation

The lesion is a chordoma and the other listed choices can be eliminated based on the histology. Many tumors can occur in the sacrum including chordoma, multiple myeloma, giant cell tumor, aneurysmal bone cyst, and metastatic disease. The histology in this patient shows a lobulated lesion on low power with fibrous septae separating the lobules. At higher magnification, the cells have eosinophilic vacuolated cytoplasm and are called physaliferous cells. Chordoma is a low-grade neoplasm that most commonly occurs in the sacrum and rarely in the base of the skull. The diagnosis is often delayed. Chordoma is thought to originate from notochordal remnants. Chordoma typically occurs in the midline and has an associated soft-tissue mass. Wold LE, Adler CP, Sim FH, et al: Atlas of Orthopedic Pathology, ed 2. Philadelphia, PA, WB Saunders, 2003, p 372. Fuchs B, Dickey ID, Yaszemski MJ, et al: Operative management of sacral chordoma. J Bone Joint Surg Am 2005;87:2211-2216.

Question 2204

Topic: 10. Pathology and Oncology

Figures 12a through 12e show the radiograph, MRI scans, and biopsy specimens of a 17-year-old boy. What is the most likely diagnosis?

. Giant cell tumor
. Chondroblastoma
. Clear cell chondrosarcoma
. Osteosarcoma
. Tuberculous septic arthritis

Correct Answer & Explanation

. Chondroblastoma

Explanation

The images show an epiphyseal lesion. The MRI scan shows extensive bone edema surrounding the lesion, consistent with chondroblastoma. Histology shows polygonal chondroblasts in a cobblestone-like pattern and areas of calcification consistent with chondroblastoma. Although some giant cells are seen, the age of the patient and the polygonal chondroblasts differentiate this lesion from giant cell tumor. Clear cell chondrosarcoma is an epiphyseal lesion that occurs in an older population, and the cells have clear cytoplasm. This lesion is not producing bone on imaging or histologic specimen, eliminating osteosarcoma. Tuberculous septic arthritis can be an epiphyseal lesion, but granulomas would be seen on histology. Menendez LR (ed): Orthopaedic Knowledge Update: Musculoskeletal Tumors. Rosemont, IL, American Academy of Orthopaedic Surgeons, 2002, pp 103-111.

Question 2205

Topic: 10. Pathology and Oncology

A 38-year-old man who is an avid runner reports a several month history of right hip pain. Based on the radiograph and cross-sectional CT scan shown in Figures 33a and 33b, what is the most likely diagnosis for the lesions seen on the femoral neck?

. Synovial herniation pits
. Osteoid osteoma
. Fibrous dysplasia
. Metastatic bone disease
. Multiple enchondromas

Correct Answer & Explanation

. Synovial herniation pits

Explanation

Synovial herniation pits or Pitt's pits are tumor simulators and are incidentally identified on radiographs obtained for either pain or trauma. The main diagnostic pitfall with this lesion is mistakenly identifying it as an osteoid osteoma. Accurate diagnosis is achieved by knowledge of the location and the characteristic imaging appearance. These are common lesions in individuals with femoroacetabular impingement. Pitt MJ, Graham AR, Shipman JH, et al: Herniation pit of the femoral neck. Am J Roentgenol 1982;138:1115-1121.

Question 2206

Topic: 10. Pathology and Oncology

A 39-year-old man has had a foot mass for the past several months. MRI scans are shown in Figures 78a through 78c. A core biopsy specimen reveals synovial sarcoma, and a staging chest CT scan is normal. Which of the following treatments offers the best local tumor control and expedites the patient's return to normal function?

. Transtibial amputation
. Transmetatarsal amputation
. Wide resection and radiation therapy
. Ray resection and radiation therapy
. Transfemoral amputation

Correct Answer & Explanation

. Transtibial amputation

Explanation

Certain histologic subtypes of soft-tissue sarcoma have been noted to arise preferentially in the hand and the foot, such as epithelioid sarcoma, clear cell sarcoma, and synovial sarcoma. Synovial sarcoma is the most common foot sarcoma. Frequently there is a delay in diagnosis because the lesions are rare. The lesions tend to occur in younger adults, typically between the ages of 15 and 40 years. Patients with hand and foot sarcomas have been described as having improved overall survival, but this is likely a result of the smaller size of tumors arising in these locations. In this patient, the tumor has grown to a substantial size and involves many of the bones of the midfoot. Limb salvage may be a possibility when incorporated into a multidisciplinary treatment program, but this will entail months of adjuvant treatment and significant morbidity. Amputation and early prosthetic fitting still have a role in management of some soft-tissue sarcomas, most frequently in the foot. Ferguson PC: Surgical considerations for management of distal extremity soft tissue sarcomas. Curr Opin Oncol 2005;17:366-369.

Question 2207

Topic: 10. Pathology and Oncology

In addition to radiographs of the primary lesion and chest, MRI of the primary lesion, and CT of the chest, staging studies for Ewing's sarcoma should include which of the following?

. Bone scan and gallium scan
. Bone scan and sentinel node biopsy
. Bone scan and bone marrow biopsy
. Bone marrow and sentinel node biopsy
. Gallium scan and sentinel node biopsy

Correct Answer & Explanation

. Bone scan and bone marrow biopsy

Explanation

A bone scan and bone marrow biopsy are part of the staging studies for Ewing's sarcoma. Whole body MRI and PET scans are investigational and show promise of greater sensitivity than a bone scan. Schleiermacher G, Peter M, Oberlin O, Philip T, Rubie H, Mechinaud F, et al: Increased risk of systemic relapses associated with bone marrow micrometastasis and circulating tumor cells in localized ewing tumor. J Clin Oncol 2003;21:85-91.

Question 2208

Topic: 10. Pathology and Oncology

A 33-year-old woman reports a mass on the right hand that has been enlarging for 1 year. An intraoperative photograph is shown in Figure 28a, and a biopsy specimen is shown in Figure 28b. What is the most likely diagnosis?

. Ganglion cyst
. Abscess
. Hematoma
. Giant cell tumor of tendon sheath
. Synovial sarcoma

Correct Answer & Explanation

. Giant cell tumor of tendon sheath

Explanation

Giant cell tumor of the tendon sheath is the most common solid soft-tissue mass in the hand. These tumors are slow-growing and may be present for months or years before coming to medical attention. Patients usually report mechanical difficulties because of the size or position of the tumor. The gross appearance is that of a lobulated mass that may be multicolored; typically yellow, brown, red, and gray. Histologically the lesion consists of multinucleated giant cells, polygonal mononuclear cells, and histiocytes that may contain abundant hemosiderin or lipid. Walsh EF, Mechrefe A, Akelman E, et al: Giant cell tumor of tendon sheath. Am J Orthop 2005;34;116-121.

Question 2209

Topic: 10. Pathology and Oncology

A 15-year-old boy presents with knee pain. Imaging reveals a destructive metaphyseal lesion of the distal femur with a sunburst periosteal reaction. Staging shows no metastasis. Following neoadjuvant chemotherapy and wide surgical resection, what is the most significant prognostic factor for overall survival?

. Tumor size at presentation
. Serum alkaline phosphatase levels
. Percentage of tumor necrosis on histologic map
. Type of surgical reconstruction performed
. Distance of the tumor from the joint line

Correct Answer & Explanation

. Percentage of tumor necrosis on histologic map

Explanation

In osteosarcoma, the histologic response to neoadjuvant chemotherapy, measured as the percentage of tumor necrosis in the resected specimen, is the most powerful predictor of disease-free and overall survival. A good response is typically defined as greater than 90% tumor necrosis.

Question 2210

Topic: 10. Pathology and Oncology

A 15-year-old male presents with severe, aching night pain in his distal femur. Radiographs reveal an aggressive osseous lesion with a 'sunburst' periosteal reaction and a Codman triangle. Biopsy confirms a diagnosis of high-grade conventional osteosarcoma. Pathogenesis of this primary bone tumor is most frequently linked to a mutation in which of the following genes?

. EWS-FLI1
. TP53
. BRAF V600E
. GNAS
. EXT1

Correct Answer & Explanation

. TP53

Explanation

High-grade conventional osteosarcoma is strongly associated with mutations in major tumor suppressor genes, particularly TP53 (associated with Li-Fraumeni syndrome) and RB1 (associated with hereditary retinoblastoma). EWS-FLI1 is the translocation found in Ewing sarcoma. BRAF V600E mutations are seen in Langerhans cell histiocytosis and certain ameloblastomas. GNAS is mutated in fibrous dysplasia. EXT1 is associated with hereditary multiple exostoses (osteochondromas).

Question 2211

Topic: 10. Pathology and Oncology

A 65-year-old male with a known history of advanced renal cell carcinoma presents with a solitary, highly destructive lytic lesion in the proximal diaphysis of the right femur. He complains of excruciating pain with weight-bearing. The Mirels score is 11, and an impending fracture is diagnosed. A wide local excision and endoprosthetic reconstruction is planned. Given the specific primary histology, which of the following preoperative interventions is most critical?

. Preoperative radiation therapy to the entire femur
. Preoperative selective arterial embolization of the lesion
. Initiation of intravenous bisphosphonates 4 weeks prior to surgery
. Preoperative neoadjuvant systemic chemotherapy
. Prophylactic stabilization of the contralateral femur

Correct Answer & Explanation

. Preoperative selective arterial embolization of the lesion

Explanation

Bone metastases from renal cell carcinoma (RCC) and thyroid carcinoma are notoriously hypervascular. Surgical intervention on these lesions can result in massive, life-threatening intraoperative hemorrhage. Preoperative selective arterial embolization performed 24 to 48 hours prior to surgical resection or stabilization is critically indicated to safely minimize intraoperative blood loss.

Question 2212

Topic: 10. Pathology and Oncology
A 55-year-old man presents with chronic, dull, aching shoulder pain. Radiographs reveal an aggressive lytic lesion with 'popcorn' calcifications in the proximal humerus. A core needle biopsy is obtained and the histology slide is shown below. Assuming the biopsy demonstrates hypercellular atypical chondrocytes in a myxoid stroma consistent with a Grade II Chondrosarcoma, what is the most appropriate management strategy?
. Neoadjuvant chemotherapy followed by wide surgical resection
. Wide surgical resection alone
. Intralesional curettage and filling with polymethylmethacrylate (PMMA)
. Primary external beam radiation therapy
. Neoadjuvant radiation therapy followed by curettage

Correct Answer & Explanation

. Wide surgical resection alone

Explanation

Chondrosarcomas are generally characterized as chemoresistant and radioresistant tumors. Therefore, the mainstay of treatment for intermediate- (Grade II) and high-grade (Grade III) conventional chondrosarcomas is wide surgical resection alone to achieve negative margins. Intralesional curettage with local adjuvants (like phenol or cryotherapy) is reserved for low-grade (Grade I) or atypical cartilaginous tumors in the appendicular skeleton. Neoadjuvant chemotherapy and radiation play no primary role in conventional chondrosarcoma.

Question 2213

Topic: 10. Pathology and Oncology

A 16-year-old patient diagnosed with high-grade, intramedullary osteosarcoma of the distal femur completes a course of neoadjuvant multi-agent chemotherapy. Subsequently, he undergoes a wide surgical resection of the tumor with endoprosthetic reconstruction. Which of the following is considered the most significant prognostic factor for long-term survival in this patient?

. The predominant histologic subtype (e.g., osteoblastic versus chondroblastic)
. The absolute tumor volume at the time of initial diagnosis
. The percentage of tumor necrosis in the resected specimen following neoadjuvant chemotherapy
. The presence of a skip metastasis within the ipsilateral femur at diagnosis
. The width (in millimeters) of the healthy tissue margin obtained during resection

Correct Answer & Explanation

. The percentage of tumor necrosis in the resected specimen following neoadjuvant chemotherapy

Explanation

The percentage of tumor necrosis evaluated in the resected specimen after neoadjuvant chemotherapy is the single most important prognostic indicator for overall survival in patients with high-grade osteosarcoma. This is formalized by the Huvos grading system. A good response is defined as greater than 90% tumor necrosis, which is strongly correlated with improved long-term disease-free survival. While wide margins are necessary to prevent local recurrence, the chemotherapeutic response (>90% necrosis) remains the best predictor of systemic control and overall survival.

Question 2214

Topic: 10. Pathology and Oncology

A 15-year-old male presents with deep thigh pain. A biopsy of a diaphyseal femur lesion reveals sheets of uniform small round blue cells. Cytogenetic analysis demonstrates a t(11;22)(q24;q12) chromosomal translocation. Which fusion protein, which acts as an aberrant transcription factor, is produced by this translocation?

. SYT-SSX1
. EWS-FLI1
. PAX3-FOXO1
. TLS-CHOP
. COL1A1-PDGFB

Correct Answer & Explanation

. EWS-FLI1

Explanation

The scenario describes Ewing sarcoma, characterized histologically by small round blue cells. The hallmark cytogenetic abnormality is the t(11;22)(q24;q12) translocation, which fuses the EWS gene on chromosome 22 with the FLI1 gene on chromosome 11, creating the EWS-FLI1 fusion protein. Other translocations: SYT-SSX (synovial sarcoma); PAX3-FOXO1 (alveolar rhabdomyosarcoma); TLS-CHOP (myxoid liposarcoma).

Question 2215

Topic: 10. Pathology and Oncology
A 15-year-old male is diagnosed with high-grade osteosarcoma of the distal femur without evidence of metastatic disease. What is the single most important prognostic factor for his long-term survival?
. Histologic response to neoadjuvant chemotherapy
. Surgical margins achieved during resection
. Anatomic location of the tumor
. Serum alkaline phosphatase levels at presentation
. Subtype of osteosarcoma (e.g., osteoblastic vs. chondroblastic)

Correct Answer & Explanation

. Histologic response to neoadjuvant chemotherapy

Explanation

In non-metastatic high-grade osteosarcoma, the extent of tumor necrosis following neoadjuvant chemotherapy is the single most important prognostic indicator. A good response is defined as >90% necrosis (Huvos grade III or IV) and correlates highly with increased overall and disease-free survival.

Question 2216

Topic: 10. Pathology and Oncology
A 16-year-old male presents with knee pain. Radiographs and a subsequent MRI reveal a high-grade conventional osteosarcoma of the distal femur. He undergoes neoadjuvant chemotherapy followed by wide surgical resection. Pathologic evaluation of the resected specimen is performed. Which of the following is the most significant independent prognostic factor for long-term overall survival in this patient?
. The specific chemotherapeutic agents used in the neoadjuvant regimen
. Histologic evidence of >90% tumor necrosis in the resection specimen
. The anatomic size of the tumor (volume > 200 mL)
. The degree of soft tissue extension outside the bony cortex
. Alkaline phosphatase levels at the time of initial diagnosis

Correct Answer & Explanation

. Histologic evidence of >90% tumor necrosis in the resection specimen

Explanation

The degree of tumor necrosis following neoadjuvant chemotherapy is the single most important prognostic factor for overall survival in patients with localized high-grade osteosarcoma. A 'good response' is defined as >90% tumor necrosis (Huvos grade III or IV) and is associated with significantly higher 5-year survival rates compared to poor responders (<90% necrosis). While tumor size and initial lab markers have prognostic value, histologic response is the most highly predictive metric.

Question 2217

Topic: 10. Pathology and Oncology
A 55-year-old male presents with deep, aching pain in his proximal humerus. Radiographs demonstrate a large, permeative lytic lesion with intralesional 'popcorn' calcifications and significant endosteal scalloping. Core needle biopsy confirms a Grade II (intermediate grade) chondrosarcoma. What is the standard treatment of choice for this lesion?
. Intralesional curettage and cryotherapy
. Neoadjuvant chemotherapy followed by wide excision
. Wide surgical excision alone
. Radiation therapy followed by wide excision
. Intralesional curettage, phenol, and cementation

Correct Answer & Explanation

. Wide surgical excision alone

Explanation

Conventional chondrosarcomas are generally resistant to both chemotherapy and radiation therapy. The standard of care for Grade II and Grade III chondrosarcomas is wide, en bloc surgical excision. Extended intralesional curettage with local adjuvants is generally reserved for low-grade (Grade I/atypical cartilaginous tumors) in the appendicular skeleton.

Question 2218

Topic: Bone Tumors

A 15-year-old boy complains of intense, unrelenting right thigh pain that is noticeably worse at night and dramatically relieved within 30 minutes of taking ibuprofen. A CT scan reveals a 7mm radiolucent nidus surrounded by dense, reactive cortical sclerosis in the proximal femur. Which of the following factors produced within the nidus is most directly responsible for this characteristic pain pattern?

. High concentration of interleukins
. Extensive infiltration of eosinophils
. Elevated levels of prostaglandins
. Accumulation of lactic acid
. Overexpression of RANK ligand

Correct Answer & Explanation

. Elevated levels of prostaglandins

Explanation

The nidus of an osteoid osteoma produces extraordinarily high levels of prostaglandins (particularly Prostaglandin E2, or PGE2) due to high expression of cyclooxygenase (COX) enzymes. This localized prostaglandin production causes profound vasodilation and is the direct cause of the intense, nocturnal pain that is classically and rapidly relieved by NSAIDs or salicylates.

Question 2219

Topic: Bone Tumors

A 15-year-old male presents with worsening knee pain. Radiographs reveal a metaphyseal aggressive bone lesion with a 'sunburst' periosteal reaction and Codman's triangle.

Biopsy confirms a high-grade intramedullary osteosarcoma. What is the standard algorithmic approach to treatment for this patient?

. Wide surgical resection alone
. Neoadjuvant chemotherapy followed by wide surgical resection and adjuvant chemotherapy
. Neoadjuvant radiotherapy followed by wide surgical resection
. Marginal excision followed by adjuvant chemotherapy
. Primary amputation without chemotherapy

Correct Answer & Explanation

. Neoadjuvant chemotherapy followed by wide surgical resection and adjuvant chemotherapy

Explanation

The standard of care for high-grade classic intramedullary osteosarcoma is neoadjuvant (pre-operative) chemotherapy, followed by wide surgical resection (either limb salvage or amputation), and then adjuvant (post-operative) chemotherapy. The percentage of histologic necrosis seen in the resected specimen after neoadjuvant chemotherapy is one of the strongest prognostic indicators for long-term survival.

Question 2220

Topic: 10. Pathology and Oncology

A 14-year-old boy presents with severe thigh pain, low-grade fever, and unintentional weight loss.

Radiographs show a permeative, destructive diaphyseal lesion of the femur with a multi-layered 'onion skin' periosteal reaction. A core needle biopsy is performed. Which of the following chromosomal translocations is most highly diagnostic for this malignancy?

. 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. Ewing sarcoma is a small round blue cell tumor characterized genetically by a balanced reciprocal translocation, most commonly t(11;22)(q24;q12), which is present in 85-90% of cases and results in the fusion of the EWS and FLI1 genes. The t(X;18) translocation is seen in synovial sarcoma, t(12;16) in myxoid liposarcoma, and t(2;13) in alveolar rhabdomyosarcoma.

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