Benign Bone Lesions
A 12-year-old girl presents with progressive leg pain and a limp. Examination reveals leg length discrepancy with the right leg shorter, and a varus deformity of the proximal femur. Radiographs show an expansile lesion in the proximal femur with a "ground glass" appearance and a characteristic "shepherd's crook" deformity. She also has irregular café-au-lait spots with serrated ("coast of Maine") borders on her trunk. Her bone age is advanced and she had precocious puberty. Regarding fibrous dysplasia:
Mark each as TRUE or FALSE
Fibrous dysplasia is a developmental disorder caused by activating GNAS mutations (G-protein signali...
Radiographically, fibrous dysplasia appears as a well-defined intramedullary lesion with a "ground g...
Fibrous dysplasia is caused by NF1 gene mutations; monostotic disease is rare; the lesions appear pu...
Histologically, fibrous dysplasia shows a fibrous stroma with irregularly shaped immature woven bone...
Management is observation for asymptomatic lesions; bisphosphonates may reduce pain and bone turnove...
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Click T (True) or F (False) for each option