Malignant transformation in paget disease of bone

Abstract

Background. The previously reported incidence of malignant transformation of Paget disease up to 5.5% and its dismal prognosis have prompted the clinical investigation of a large population with Paget disease. Methods. A chart review of symptomatic and asymptomatic patients with a diagnosis of Paget disease between 1970 and 1985 at four large Montreal Hospitals revealed 1078 patients. Eight patients with malignant transformation were studied in detail. Results. The incidence of malignant transformation was 0.770, and the most frequent histologic type was osteogenic sarcoma. The most prevalent site was the femur, and pathologic fracture with focal osteolysis was present in 50% of patients at the initial appearance of the tumor. Healing at the fracture site was demonstrated in one patient. Another patient died of uncontrollable hemorrhage. A case of malignant lymphoma in Paget disease is recorded. Conclusions. Surgery or biopsy should be preceded by preoperative scintigraphic bone blood flow evaluation and, if necessary, administration of a preoperative course of mithramycin and selective embolization to minimize bleeding. The prognosis of malignant transformation in Paget disease is poor, although one patient with malignant lymphoma survived after aggressive treatment. Early biopsy and aggressive treatment should not be delayed.

Genetic variation in the TNFRSF11A gene encoding RANK is associated with susceptibility to Paget's disease of bone

“…Moreover, PDB patients have an increased risk to develop osteosarcomas. (10)(11)(12) However, up to 70% of PDB patients are asymptomatic. (4,7,13) At the histologic level, osteoclasts are most likely the primary defective cells.…”

Paget's disease of bone

“…The most frequent complications include the following: pathological fractures, bone deformities, degenerative arthritis, loss of hearing, basilar invagination, nerve root or cord compression, and rarely hypercalcemia during immobilization, increased cardiac output in cases with severe bone involvement and osteosarco- ma, the latter being a rare complication that occurs in only 0.7-1% of the cases (30). Deafness may be of the conduction type, owing to the involvement of the ossicles of the middle ear, of central origin, from compression of the auditory nerve, or a mixed form.…”

Principles of management of osteometabolic disorders affecting the aging spine

“…The spine is the second most commonly affected site in PD [2,30,95], predisposing patients to low back pain and spinal stenosis [4,52,64,137]. Hartman and Dohn have shown that 15.2% of patients with PD had involvement of the vertebrae, and 26% of these patients had symptoms of spinal stenosis [59].…”

“…Fortunately, this complication is relatively rare, occurring in about 0.7% [52] of cases. In our series of PD patients [52,53] we have not seen any cases with sarcomatous degeneration in the spine. In Schajowicz et al [120], of 62 patients with sarcomatous transformation, five of the sarcomas occurred in the spine.…”