Question 581
Topic: Bone TumorsCorrect Answer & Explanation
. Constitutive activation of adenylyl cyclase leading to elevated cAMP
Practice Set 30 of 52
This practice set contains high-yield board review questions covering key concepts in Bone Tumors. Each clinical scenario is designed to test your diagnostic and management skills relevant to this subspecialty.
. Constitutive activation of adenylyl cyclase leading to elevated cAMP
An 8-year-old boy presents with mild right arm pain after throwing a ball. Radiographs reveal a central, lytic, well-circumscribed lesion in the proximal humeral metaphysis with a "fallen leaf" sign. Assuming the fracture has healed and the lesion remains asymptomatic, what is the most appropriate natural history expectation and management?
. Progression to osteosarcoma requires en bloc resection
A 16-year-old male presents with a 6-month history of well-localized mid-back pain that is worse at night and dramatically improves with ibuprofen. Imaging reveals a 1.2 cm sclerotic lesion with a central lucent nidus in the left lamina of T8. What is the most appropriate definitive management if conservative measures are poorly tolerated?
. Wide en bloc resection and fusion
A 22-year-old male presents with dull, aching pain in his mid-back that does not completely resolve with NSAIDs. Imaging reveals a 2.8 cm radiolucent lesion in the posterior elements of the L3 vertebra with an surrounding zone of reactive sclerosis. Histological evaluation shows a nidus of irregular, interconnected woven bone trabeculae lined by prominent osteoblasts, highly vascular loose connective tissue, and no atypical cells. Based on clinical and pathologic features, what is the most likely diagnosis?
. Osteoblastoma
A 15-year-old male undergoes curettage of a 3 cm lytic lesion located exclusively in the greater tuberosity epiphysis of the proximal humerus. Histological analysis reveals sheets of mononuclear cells with characteristic nuclear grooves, prominent eosinophilic cytoplasm, and focal areas of fine, intercellular pericellular calcifications. Molecular testing of this lesion is most likely to reveal a mutation in which of the following genes?
. GNAS
A 55-year-old male is referred for an asymptomatic calcified lesion discovered incidentally on a radiograph of his proximal humerus. The radiologist suggests distinguishing between an enchondroma and a low-grade (Grade 1) central chondrosarcoma. Which of the following MRI or radiographic findings is the most reliable indicator favoring a diagnosis of low-grade chondrosarcoma over enchondroma?
. Popcorn-like intralesional calcifications
A 15-year-old patient with multiple enchondromatosis presents with extensive cartilaginous lesions primarily localized to one side of the body, sparing the soft tissues. Recent literature has identified that the pathogenesis of this syndrome (Ollier disease) is most closely linked to a somatic mosaic mutation in which of the following genes?
. EXT1
A 10-year-old boy presents with thigh pain after a minor fall. Radiographs show a transverse fracture through a purely lytic, centrally located metadiaphyseal lesion of the femur with cortical thinning. What is the most likely underlying diagnosis?
. Osteosarcoma
A 10-year-old girl is brought in for a mild limp. Radiographs show a centrally located, lytic lesion in the proximal humerus metaphysis with a 'fallen leaf' sign. She has minimal pain after a minor fall. What is the most likely diagnosis?
. Aneurysmal bone cyst
. Polyostotic fibrous dysplasia
A 16-year-old boy presents with severe right thigh pain that is distinctly worse at night and dramatically relieved by taking ibuprofen. Radiographs reveal a cortical thickening with a small 8 mm radiolucent nidus in the diaphysis of the femur. If a trial of conservative medical management fails, what is the most appropriate definitive treatment?
. Wide surgical resection
A 16-year-old male presents with severe right thigh pain that is uniquely worse at night and dramatically relieved by ibuprofen. Radiographs demonstrate a radiolucent nidus surrounded by dense reactive sclerosis in the proximal femur. Which of the following inflammatory mediators is produced in high levels by this lesion and is responsible for the classic clinical presentation?
. Tumor necrosis factor alpha (TNF-alpha)
A 55-year-old male presents with a painful mass in his proximal humerus. Plain radiographs demonstrate an intramedullary lytic lesion with cortical destruction and an internal matrix mineralization pattern described as "rings and arcs" or "popcorn-like."
This matrix pattern is most characteristic of which type of tissue?
. Osteoid matrix
A 10-year-old boy presents with multiple cafe-au-lait spots, polyostotic fibrous dysplasia, and precocious puberty. What is the underlying genetic mutation associated with this syndrome?
. GNAS1 gene activating mutation
Which of the following histologic variants of osteosarcoma typically carries the most favorable prognosis?
. Telangiectatic
A 19-year-old male complains of dull, aching back pain that is notably worse at night and dramatically relieved by ibuprofen. Radiographs and CT show a 2.5 cm radiolucent nidus in the posterior elements of L4. Histologically, the lesion consists of woven bone trabeculae lined by prominent osteoblasts. What is the most likely diagnosis?
. Osteoid osteoma
A 65-year-old male presents with deep bone pain in his proximal femur. Blood work reveals hypercalcemia and mild renal insufficiency. Radiographs show a large solitary lytic lesion in the subtrochanteric region without a sclerotic rim. What is the most likely primary diagnosis?
. Osteosarcoma
A 22-year-old male presents with dull, aching thoracic back pain that is not significantly relieved by NSAIDs. CT imaging demonstrates a 3.5 cm expansile, lytic lesion in the posterior elements of T11 with a thin sclerotic rim. Histology reveals interlacing trabeculae of woven bone lined by prominent osteoblasts in a highly vascular stroma. What is the most likely diagnosis?
. Osteoid osteoma
A 19-year-old male presents with severe, progressive mid-tibial pain that is worse at night and dramatically relieved by over-the-counter ibuprofen. Radiographs demonstrate a diaphyseal cortical thickening with a small radiolucent nidus. What is the primary mechanism by which non-steroidal anti-inflammatory drugs (NSAIDs) relieve pain in this condition?
. Inhibition of histamine release from local mast cells
A 24-year-old male complains of severe dull pain in his thoracic spine that is not reliably relieved by NSAIDs. CT scan shows a 2.5 cm radiolucent nidus with posterior element expansion. Histology shows interlacing trabeculae of woven bone lined by prominent osteoblasts. What is the most likely diagnosis?
. Osteoid osteoma