Practice Set 23 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.
The most common benign tumor of vertebral bodies is:
. Aneurysmal bone cyst
A 10-year-old patient has a painful lytic lesion replacing 75% of the distal femoral metaphysis. The lesion has indistinct margins, but there is not any periosteal reaction. Biopsy reveals a benign histiocytic tumor with multiple eosinophils. Physical exam and skeletal survey reveal no other abnormalities. Which of the following would be the most appropriate treatment method:
. C urettage and bone graft
A 15-year-old girl presents with pain and a 17°-scoliosis curve. The film suggests an enlargement and sclerosis of the transverse process of L2. The best study to further evaluate the nature and anatomic extent of the lesion is:
. Computed tomography
A 15-year-old boy presents with progressive knee pain. Radiographs reveal a mixed lytic and sclerotic lesion in the distal femoral metaphysis with a sunburst periosteal reaction. Core needle biopsy confirms a high-grade intramedullary osteosarcoma. Staging studies are negative for metastasis. What is the standard of care for this patient?
. Neoadjuvant chemotherapy, wide surgical excision, and adjuvant chemotherapy
A 12-year-old boy presents with thigh pain and systemic symptoms including low-grade fever and weight loss. Radiographs show a permeative diaphyseal lesion of the femur with "onion-skin" periosteal reaction. Biopsy confirms Ewing sarcoma. Which of the following chromosomal translocations is most characteristic of this diagnosis?
. t(11;22)
A 14-year-old boy presents with a painful mass in his diaphyseal femur with a "periosteal onion-skin" appearance on X-ray. Biopsy reveals small round blue cells. Cytogenetic analysis is most likely to show which of the following translocations?
. t(11;22)
A 12-year-old boy presents with progressive knee pain and a palpable mass in the distal femur. Radiographs reveal a destructive metaphyseal lesion with a 'sunburst' periosteal reaction. Biopsy confirms the diagnosis of osteosarcoma. Which of the following genetic alterations is most consistently associated with the pathogenesis of this tumor?
. Mutations in the RB1 and TP53 tumor suppressor genes
A 12-year-old patient with osteogenic sarcoma metastatic to the spine is noted to have new onset of weakness of both lower extremities. Magnetic resonance imaging shows a mass expanding posteriorly and encroaching on the spinal cord. The recommended initial step is:
. Surgical resection
A 12-year-old boy with hemophilia A has a painless mass in his thigh. The femur is eroded anterolaterally and there is a large overlying soft tissue mass. Magnetic resonance imaging shows a 5 cm x 7 cm mass arising from the bone. The most likely diagnosis is:
. Pseudotumor
Which of the following is the most common final attribution of back pain in children and adolescents after all appropriate diagnostic studies are performed:
. No identifiable cause
A 9-year-old girl presents with precocious puberty, irregular hyperpigmented macules, and a limp. Radiographs of her femur reveal a ground-glass appearance and a "shepherd's crook" deformity. Which of the following best describes the underlying genetic defect?
. Activating mutation of the GNAS1 gene
A 4-year-old boy presents with recurrent, painful soft tissue swellings on his back that eventually turn into hard masses. He has a history of congenital bilateral hallux valgus and shortened great toes. What is the most appropriate management?
. Avoidance of trauma and supportive care
An asymptomatic 25-year-old man undergoes pelvic radiographs after a minor fall. The x-rays show numerous small, well-defined circular radiodensities clustered around the pelvic joints and proximal femurs. A mutation in the LEMD3 gene is suspected. What is the most appropriate management?
. Reassurance and observation
A 7-year-old girl presents with precocious puberty and a large cafe-au-lait macule with an irregular "coast of Maine" border. Radiographs of her proximal femur show a lytic, expansile, "ground-glass" lesion with a shepherd's crook deformity. What is the underlying genetic mechanism of this disease?
. GNAS1 gene mutation
A 4-year-old boy presents with a painless, hard mass on the medial aspect of his right knee, causing a progressive asymmetric valgus deformity. Radiographs show an irregular, ossified mass arising from the medial half of the distal femoral epiphysis. What is the most likely diagnosis?
. Dysplasia epiphysealis hemimelica
A 3-year-old boy presents with painful soft tissue swellings on his back and neck. Physical exam reveals bilateral short great toes with marked hallux valgus. Radiographs show early heterotopic ossification in the paraspinal muscles. What is the most appropriate next step in management?
. Genetic testing for an ACVR1 mutation
Adolescent girls with multiple radiographs for idiopathic scoliosis are statistically at increased risk for which of the following problems later in life:
. Breast cancer
. Intravenous hydration and analgesia
A 12-year-old boy presents with knee pain. Radiographs show a sunburst periosteal reaction and a Codman triangle in the distal femur. Biopsy reveals malignant spindle cells producing osteoid. What is the most appropriate initial step in treatment?
. Neoadjuvant chemotherapy
A 14-year-old boy presents with a permeative lytic lesion in the femoral diaphysis, an associated soft tissue mass, and an "onion-skin" periosteal reaction. Biopsy reveals uniform small, round blue cells. Which chromosomal translocation is most characteristic of this diagnosis?
. t(11;22)