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Question 2541

Topic: 10. Pathology and Oncology

A 45-year-old man presents with generalized bone pain and proximal muscle weakness. Laboratory evaluation reveals severe hypophosphatemia, hyperphosphaturia, normal serum calcium, and a normal parathyroid hormone level. Imaging identifies a small mesenchymal tumor in his thigh. Which hormone is most likely being overproduced?

. Calcitriol
. Fibroblast growth factor 23 (FGF23)
. Parathyroid hormone-related peptide (PTHrP)
. Sclerostin
. Osteocalcin

Correct Answer & Explanation

. Calcitriol


Explanation

Tumor-induced osteomalacia is a paraneoplastic syndrome caused by excessive production of FGF23. FGF23 directly inhibits renal phosphate reabsorption and 1-alpha-hydroxylase activity, leading to isolated hypophosphatemia and inappropriately low active vitamin D levels.

Question 2542

Topic: 10. Pathology and Oncology

A 77-year-old man has had increasing right knee pain for the past 3 months. A radiograph and coronal T1-weighted MRI scan are shown in Figures 19a and 19b. A biopsy specimen is shown in Figure 19c. What is the most likely diagnosis?

. Metastatic prostate cancer
. Enchondroma
. Osteomyelitis
. Dedifferentiated chondrosarcoma
. Lymphoma

Correct Answer & Explanation

. Metastatic prostate cancer


Explanation

The radiograph shows a calcified lesion in the medullary canal of the distal femoral diaphysis. The MRI scan shows extensive marrow change distal to the lesion, which is not consistent with an enchondroma. The histology shows a biphasic pattern with low-grade cartilage just apposed to high-grade spindle cell sarcoma. The overall appearance is consistent with dedifferentiated chondrosarcoma. The radiographic appearance is not consistent with enchondroma, and the histologic appearance is not consistent with the other choices. Mitchell AD, Ayoub K, Mangham DC, et al: Experience in the treatment of dedifferentiated chondrosarcoma. J Bone Joint Surg Br 2000;82:55-61.

Question 2543

Topic: 10. Pathology and Oncology

An 83-year-old man has a painful mass of the great toe. Radiographs and a biopsy specimen are seen in Figures 22a and 22b. What is the most likely diagnosis?

. Gout
. Pseudogout
. Infection
. Epidermal inclusion cyst
. Charcot joint

Correct Answer & Explanation

. Gout


Explanation

Gouty arthritis, pseudogout, and infection can all present with inflammatory arthritis and periarticular erosions. Strongly negative birefringent crystals are seen in gout. The histologic image shows elongated "needle-like" crystals of gout. Epidermal inclusion cysts are rarely painful and usually have a history of localized penetrating trauma. Hamilton W, Breedman KB, Haupt HM, Lackman R: Knee pain in a 40-year-old man. Clin Orthop 2001;383:282-285,290-292.

Question 2544

Topic: 10. Pathology and Oncology

A 19-year-old man has had intermittent progressive knee pain with ambulation and pain at night following a rodeo accident 4 weeks ago. Figures 4a through 4e show the radiographs, a bone scan, CT scan, and T2-weighted MRI scan. What is the most likely diagnosis?

. Infection
. Osteosarcoma
. Osteoblastoma
. Giant cell tumor
. Distal femoral fracture

Correct Answer & Explanation

. Infection


Explanation

The imaging studies reveal a predominantly blastic lesion in the distal femur with posterolateral periosteal changes. The bone scan shows increased uptake in the distal femur, beyond that expected with radiography. Cross-sectional imaging confirms the presence of a soft-tissue mass extending from the lateral aspect of the femur, with diffuse intramedullary signal changes. This aggressive presentation, particularly in this location and in a patient of this age, is most consistent with osteosarcoma. The mineralization in the soft tissue strongly suggests neoplasm, not the reactive bony changes seen in an infectious process. Menendez LR (ed): Orthopaedic Knowledge Update: Musculoskeletal Tumors. Rosemont, IL, American Academy of Orthopaedic Surgeons, 2002, pp 175-186.

Question 2545

Topic: 10. Pathology and Oncology

A 20-year-old basketball player has tenderness and bruising after sustaining a blow to the knee. A radiograph is shown in Figure 15. What is the most likely diagnosis?

. Patellar fracture
. Patellar dislocation
. Bipartite patella
. Vastus lateralis tear
. Tumor

Correct Answer & Explanation

. Patellar fracture


Explanation

The patient has a bipartite patella. The line between the fragment and the main patella is smooth and sclerotic, indicating a chronic, not acute, entity. The location is classic for a bipartite patella, not a tumor. Schmidt DR, Henry JH: Stress injuries of the adolescent extensor mechanism. Clin Sports Med 1989;8:343-355.

Question 2546

Topic: 10. Pathology and Oncology

A 12-year-old girl presents with a destructive diaphyseal lesion of the femur with a permeative pattern and an 'onion skin' periosteal reaction.

A core needle biopsy is performed, revealing sheets of uniform small, round, blue cells. Immunohistochemistry is strongly positive for CD99. Which of the following chromosomal translocations is most characteristic of this tumor?

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

Correct Answer & Explanation

. t(9;22)


Explanation

The clinical, radiographic ('onion skin' periosteal reaction, permeative diaphyseal lesion), and histologic (small round blue cells, CD99+) findings are highly characteristic of Ewing sarcoma. Ewing sarcoma is classically associated with the t(11;22)(q24;q12) chromosomal translocation, which creates the EWS-FLI1 fusion gene. t(9;22) is associated with CML (Philadelphia chromosome) and myxoid chondrosarcoma; t(12;16) is seen in myxoid liposarcoma; t(X;18) in synovial sarcoma; and t(2;13) in alveolar rhabdomyosarcoma.

Question 2547

Topic: 10. Pathology and Oncology

A 14-year-old boy presents with knee pain. Radiographs reveal a mixed sclerotic and lytic lesion in the distal femoral metaphysis with a sunburst periosteal reaction and a Codman triangle. A biopsy shows malignant spindle cells producing osteoid. What is the most significant prognostic factor for survival in this patient?

. Size of the Codman triangle
. Histologic subtype of the tumor
. Tumor response to neoadjuvant chemotherapy
. The presence of skip metastases
. Patient age at diagnosis

Correct Answer & Explanation

. Size of the Codman triangle


Explanation

Osteosarcoma is the most common primary pediatric malignant bone tumor. Standard treatment involves neoadjuvant chemotherapy followed by wide surgical resection and adjuvant chemotherapy. The most significant prognostic factor for long-term survival is the histologic response of the tumor to the neoadjuvant chemotherapy, specifically the percentage of tumor necrosis. A necrosis rate of greater than 90% is considered a favorable response and correlates strongly with improved survival.

Question 2548

Topic: 10. Pathology and Oncology

An athletic 55-year-old man reports a painless mass in the anterior aspect of the thigh that appeared 3 weeks ago and has not changed in size. The patient denies any history of trauma. Examination reveals a firm, well-defined nontender mass in the anterior thigh and no inguinal adenopathy or cutaneous changes. Plain radiographs are unremarkable. T1- and T2-weighted MRI scans are shown in Figures 44a and 44b. What is the most likely diagnosis?

. Hematoma
. Lipoma
. Soft-tissue sarcoma
. Pyomyositis
. Hemangioma

Correct Answer & Explanation

. Hematoma


Explanation

The presence of a painless soft-tissue mass that is greater than 5 cm and deep to the fascia should be considered a soft-tissue sarcoma until proven otherwise. The diagnosis of a hematoma should be made with great caution because the absence of a history of trauma, pain, or presence of ecchymosis makes it unlikely. A diagnosis of pyomyositis is unlikely because of the absence of warmth, erythema, or adenopathy. The MRI scans are not consistent with lipoma or hemangioma. The MRI signal characteristics of a lipoma should be the same as subcutaneous fat on all sequences. Soft-tissue hemangiomas are not well defined and have an infiltrative appearance on MRI scans, as does pyomyositis. Sim FH, Frassica FJ, Frassica DA: Soft-tissue tumors: Diagnosis, evaluation and management. J Am Acad Orthop Surg 1994;2:202-211.

Question 2549

Topic: 10. Pathology and Oncology

What is the most common primary malignant tumor of bone in childhood?

. Osteochondroma
. Ewing's sarcoma
. Osteosarcoma
. Chondrosarcoma
. Rhabdomyosarcoma

Correct Answer & Explanation

. Osteochondroma


Explanation

Osteosarcoma is the most common primary malignant tumor of bone in childhood, followed by Ewing's sarcoma. Rhabdomyosarcoma is a soft-tissue sarcoma of childhood. Chondrosarcoma rarely occurs in childhood. Osteochondroma is a benign tumor of bone. Simon MA, Springfield DS, et al: Osteogenic Sarcoma: Surgery for Bone and Soft Tissue Tumors. Philadelphia, PA, Lippincott Raven, 1998, p 266.

Question 2550

Topic: 10. Pathology and Oncology

Figures 21a and 21b show the radiograph and CT scan of a 14-year-old patient with thigh pain. The next most appropriate step in management should consist of

. blood cultures.
. oral anti-inflammatory drugs.
. a biopsy.
. MRI.
. a SPECT bone scan.

Correct Answer & Explanation

. blood cultures.


Explanation

The radiographs show increased density and reactive bone formation. A faint nidus can be seen on the radiograph but is obvious on the CT scan. The initial therapy for an osteoid osteoma should be oral anti-inflammatory drugs. A biopsy or SPECT bone scan is not indicated because the osteoid osteoma is clearly seen on the CT scan. If the patient fails to respond to nonsurgical therapy, CT-guided radiofrequency ablation or surgical excision is indicated depending on the anatomic location. Frassica FJ, Waltrip RL, Sponseller PD, Ma LD, McCarthy EF Jr: Clinicopathologic features and treatment of osteoid osteoma and osteoblastoma in children and adolescents. Orthop Clin North Am 1996;27:559-574. Kneisl JS, Simon MA: Medical management compared with operative treatment for osteoid osteoma. J Bone Joint Surg Am 1992;74:179-185.

Question 2551

Topic: 10. Pathology and Oncology

Mutations in the retinoblastoma (Rb) gene are strongly associated with the subsequent development of which of the following primary bone tumors?

. Ewing sarcoma
. Chondrosarcoma
. Osteosarcoma
. Giant cell tumor of bone
. Adamantinoma

Correct Answer & Explanation

. Ewing sarcoma


Explanation

Patients with hereditary retinoblastoma have a germline mutation in the Rb gene on chromosome 13q14, giving them a significantly increased risk of developing osteosarcoma. Alterations in p53 (Li-Fraumeni syndrome) are also closely associated with osteosarcoma.

Question 2552

Topic: 10. Pathology and Oncology

The characteristic chromosomal translocation t(11;22)(q24;q12) found in the majority of Ewing sarcoma cases results in the fusion of which two genes?

. SYT-SSX
. EWS-FLI1
. TLS-CHOP
. COL1A1-PDGFB
. PAX3-FKHR

Correct Answer & Explanation

. SYT-SSX


Explanation

The t(11;22) translocation is present in approximately 85% of Ewing sarcomas, resulting in the EWS-FLI1 fusion transcript. This creates an aberrant transcription factor that acts as an oncogene driving tumor proliferation.

Question 2553

Topic: Soft Tissue Tumors & Metastasis

According to the Seddon classification, a peripheral nerve injury characterized by complete disruption of the axon and myelin sheath, but with preservation of the surrounding endoneurial, perineurial, and epineurial connective tissue is termed:

. Neuropraxia
. Axonotmesis
. Neurotmesis
. Neurofibroma
. Wallerian degeneration

Correct Answer & Explanation

. Neuropraxia


Explanation

Axonotmesis involves the loss of axonal continuity while the structural connective tissue framework remains intact, allowing for spontaneous nerve regeneration at about 1 mm/day. Neuropraxia is a local conduction block without structural disruption.

Question 2554

Topic: 10. Pathology and Oncology

A 10-year-old boy who plays baseball reports acute pain after throwing a softball from the outfield to second base. A radiograph is shown in Figure 26. Management should consist of

. observation during healing of the fracture, followed by bone marrow or corticosteroid injection.
. biopsy and appropriate chemotherapy.
. curettage and bone grafting.
. high-dose systemic corticosteroids.
. pulsed electromagnetic fields.

Correct Answer & Explanation

. observation during healing of the fracture, followed by bone marrow or corticosteroid injection.


Explanation

The patient has a fracture through a unicameral bone cyst, as evidenced by the "falling leaf" sign on the radiograph. Following healing of the fracture, treatment should consist of corticosteroid injection or bone marrow injection. Some cysts heal with the fracture and do not require injections. Biopsy is unnecessary because the radiograph shows that the cyst is benign. Curettage and bone grafting are seldom necessary because these cysts regularly heal with injections. Corticosteroids are useful when injected into the cyst, but are not used systemically. Pulsed electromagnetic fields have not been used therapeutically in this condition.

Question 2555

Topic: 10. Pathology and Oncology

A 10-year-old boy presents with knee pain and inability to actively extend his knee after a jumping injury. Radiographs reveal a high-riding patella and a small bony avulsion from the inferior pole of the patella. What is the most appropriate management?

. Cylinder cast in extension for 6 weeks
. Hinged knee brace and immediate weight-bearing
. Open reduction and internal fixation
. Patellar tendon lengthening
. Observation as this is a benign apophysitis

Correct Answer & Explanation

. Cylinder cast in extension for 6 weeks


Explanation

This clinical picture represents a patellar sleeve fracture, a severe pediatric injury involving a large cartilaginous avulsion from the patella that disrupts the extensor mechanism. It mandates surgical repair (ORIF) to restore active extension.

Question 2556

Topic: 10. Pathology and Oncology

In a patient sustaining multiple injuries, Damage Control Orthopedics (DCO) relies on early temporary stabilization followed by definitive fixation. Which inflammatory marker is thought to peak on day 2-3 and correlates with the "second hit" phenomenon?

. Interleukin-1
. Interleukin-6
. Tumor Necrosis Factor-alpha
. C-reactive protein
. Erythrocyte sedimentation rate

Correct Answer & Explanation

. Interleukin-1


Explanation

Interleukin-6 (IL-6) is a well-established marker for the systemic inflammatory response in trauma. It peaks 2-3 days post-injury and helps predict the magnitude of the immune "second hit."

Question 2557

Topic: Bone Tumors

An otherwise healthy 16-year-old boy who has had thoracolumbar pain with an increasingly worse deformity for the past 2 years now reports that the pain is worse at night. He responded well to nonsteroidal anti-inflammatory drugs initially, but they have become less effective. He denies any neurologic or constitutional symptoms. Examination is consistent with a mild thoracolumbar scoliosis and is otherwise normal. Laboratory studies show a normal CBC, erythrocyte sedimentation rate, and C-reactive protein. Standing radiographs show a 20 degree left thoracolumbar scoliosis, and he has a Risser stage of 4. A bone scan shows increased uptake at L2; a CT scan through this level is shown in Figure 18. Management should now consist of

. percutaneous aspiration and appropriate antibiotic therapy.
. an underarm Boston brace for 23 hours per day.
. a referral for radiation therapy.
. posterior instrumented arthrodesis from one level above to one level below the deformity.
. removal of the lesion and local arthrodesis if necessary.

Correct Answer & Explanation

. percutaneous aspiration and appropriate antibiotic therapy.


Explanation

The findings and radiographic appearance are most consistent with osteoid osteoma involving the medial pedicle. Scoliosis is commonly seen with this lesion and usually does not need surgical intervention. Excellent results have been reported with surgical excision as well as with percutaneous thermocoagulation. Nonsurgical treatment also has been described in peripheral osteoid osteoma but is not well described for lesions within the spine. Cove JA, Taminiau AH, Obermann WR, Vanderschueren GM: Osteoid osteoma of the spine treated with percutaneous computed tomography-guided thermocoagulation. Spine 2000;25:1283-1286. Kneisl JS, Simon MA: Medical management compared with operative treatment for osteoid-osteoma. J Bone Joint Surg Am 1992;74:179-185.

Question 2558

Topic: 10. Pathology and Oncology

A 45-year-old woman with chronic forefoot pain and a positive Mulder's click in the third webspace undergoes surgical excision of the symptomatic lesion after failing conservative management. Histologic examination of the excised specimen is most likely to reveal which of the following?

. Proliferation of Schwann cells forming a true neoplasm
. Perineural fibrosis, endoneurial edema, and axonal degeneration
. Malignant peripheral nerve sheath tumor
. A synovial cyst compressing the interdigital nerve
. Epithelioid granulomas surrounding a foreign body

Correct Answer & Explanation

. Proliferation of Schwann cells forming a true neoplasm


Explanation

Despite the name, a Morton's neuroma is not a true tumor. Histologically, it is characterized by reactive perineural fibrosis, demyelination, and axonal degeneration resulting from repetitive mechanical irritation.

Question 2559

Topic: 10. Pathology and Oncology

A 45-year-old woman presents with progressive diffuse bone pain, muscle weakness, and multiple stress fractures. Lab tests reveal severe hypophosphatemia, normal serum calcium, and markedly elevated FGF-23 levels. Which of the following best describes the pathogenesis of her condition?

. Decreased intestinal absorption of calcium
. Impaired hepatic hydroxylation of vitamin D
. Excessive osteoclastic bone resorption driven by PTH
. Decreased renal tubular reabsorption of phosphate and impaired 1-alpha-hydroxylase activity
. Mutations in the COL1A1 gene

Correct Answer & Explanation

. Decreased intestinal absorption of calcium


Explanation

Tumor-induced osteomalacia is caused by excessive FGF-23 secretion by a mesenchymal tumor. FGF-23 decreases phosphate reabsorption in the proximal renal tubules and inhibits 1-alpha-hydroxylase, leading to low 1,25-dihydroxyvitamin D and hypophosphatemia.

Question 2560

Topic: 10. Pathology and Oncology

A 30-year-old male presents with a slow-growing, painless mass on the dorsum of his wrist, which transilluminates. It fluctuates in size and becomes more prominent with wrist flexion. What is the MOST likely diagnosis?

. Lipoma
. Giant cell tumor of the tendon sheath
. Epidermoid cyst
. Ganglion cyst
. Enchondroma

Correct Answer & Explanation

. Lipoma


Explanation

The classic description of a slow-growing, painless, transilluminating, fluctuating mass on the dorsum of the wrist that becomes more prominent with wrist flexion is highly indicative of a dorsal wrist ganglion cyst. These are benign, fluid-filled sacs originating from a joint capsule or tendon sheath. Lipomas are soft, fatty tumors that do not transilluminate. Giant cell tumors of the tendon sheath are solid, firm, and do not transilluminate. Epidermoid cysts are typically subcutaneous, not directly associated with joint capsules, and generally do not transilluminate. Enchondromas are intraosseous tumors. Therefore, a ganglion cyst is the most likely diagnosis.