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All my patients get bone spicules10/27/2022 ![]() ![]() It is characterized by an 11:22 translocation, which helps differentiate it from similarly appearing tumors. Histologically, it is known as a small, round, blue-cell tumor. It presents with pain and has a unique onion-skin effect on x-ray. The other choices are not associated with Paget disease.Įwing sarcoma (choice A) occurs mainly in the diaphysis of long bones and axial skeleton of children and adolescents. Importantly, the high rate of cell turnover in the bones of these patients may predispose them to developing osteosarcoma, typically in the jaw pelvis or femur. Overgrowth of affected bone can produce nerve impingement, hearing loss (due to involvement of bone surrounding cochlea), increased hat size, and bone fractures (due to weakened bone). As a result, patients can rarely develop high output cardiac failure (due to increased vascularity). Marrow spaces are also filled with excess fibrous tissue and blood vessels. This highly dynamic process of bone resorption and bone formation results in elevated alkaline phosphatase activity, a marker for osteoblast activity. The osteolytic phase is followed by a secondary osteoblastic phase, which results in compensatory new bone formation, producing the mosaic pattern. In its early stages, Paget disease is characterized by excessive osteolysis, producing patchwork areas of bone resorption with bizarre, large osteoclasts. This unique disease process occurs in several stages: osteolytic stage, mixed stage, and osteoblastic stage. The initial trigger is unknown, but the hypothesis potentially associates a prior paramyxovirus infection. The disease is affects older adults and may present with monostotic (1 bone) or polyostotic (more than 2 bones) involvement. The phrase "mosaic pattern" of newly formed woven bone is a specific tip-off for Paget disease of bone and is not seen in other bone conditions. ![]()
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