1/68. Pseudosarcoma in Paget's disease of bone.The appearance of a sarcoma of bone is a well-recognized complication of Paget's disease. The most common type of such a sarcoma is osteosarcoma. Much less common are soft tissue lesions adjoining the pagetic bone that clinically and radiologically simulate sarcoma but histologically represent exaggerated periosteal bone formation as a manifestation of the basic pathologic process. We present a case of a bulky juxtacortical soft tissue mass in the thigh arising from a pagetic femur in a 62-year-old patient with polyostotic Paget's disease that was clinically and radiologically suspected to be a juxtacortical osteosarcoma. Microscopically, the lesion showed features of florid Paget's disease without any evidence of sarcomatous growth. It is important to be aware of this rare manifestation of Paget's disease to avoid unnecessary overtreatment.- - - - - - - - - - ranking = 1keywords = osteosarcoma, sarcoma (Clic here for more details about this article) |
2/68. Orbital chondrosarcoma developing in a patient with Paget disease.PURPOSE: To describe the radiologic, histopathologic, and cytogenetic features of an orbital chondrosarcoma developing in a patient with Paget disease. methods: A 64-year-old woman presented with rapidly progressive proptosis of her right eye. Computed tomographic scans, histopathologic examination, and cytogenetic analysis were performed. RESULTS: Computed tomographic scans disclosed osseous changes of the temporal and frontal bones, with areas of high density consistent with Paget disease. A soft-tissue tumor in the right lateral orbital wall was consistent with Paget sarcoma. On histology, a chondrosarcoma was diagnosed, which was confirmed by fluorescent in situ hybridization. CONCLUSION: This is a unique case of orbital chondrosarcoma developing in a patient with Paget disease.- - - - - - - - - - ranking = 0.080826035856871keywords = sarcoma (Clic here for more details about this article) |
3/68. Post-Paget telangiectatic osteosarcoma of the skull.Sarcomatous transformation is the most dreaded complication of Paget's disease. We report on a case of post-Paget telangiectatic osteosarcoma of the skull, a variant of osteogenic osteosarcoma, in a 79-year-old woman. We discuss the radiological pattern in relationship to the differential diagnosis.- - - - - - - - - - ranking = 3.1121062605537keywords = osteosarcoma, osteogenic, sarcoma (Clic here for more details about this article) |
4/68. Synchronous Paget's sarcoma of tibiae in which Paget's disease was limited to these bones.A 51-year-old native of Rio de Janeiro presented with bilateral synchronous Paget's sarcomas in the tibiae, which developed in the upper right tibia and in the distal third of the left tibia. There were no other areas of Paget's disease. The largest tumor spread to the right inguinal nodes and also soft tissue. The tumor in the left tibia spread dramatically in the soft tissues up the leg and only involved the medullary cavity in its inferior portion. The patient died, but there was no autopsy. Comments are made about the prevalence of Paget's disease in Rio de Janeiro.- - - - - - - - - - ranking = 0.050516272410544keywords = sarcoma (Clic here for more details about this article) |
5/68. Primary malignancy, secondary malignancy and semimalignancy of bone tumors.1. Bone tumors in contrast to tumors in soft tissue, show a wide variety of clinical behavior qualified by the expressions semimalignancy, low grade of malignancy, sarcomatous degeneration and primarily benign bone tumors and bone lesions. 2. The term semimalignancy is characterized by local invasive and destructive tumor growth with a tendency to recur locally but no hematogeneous spreading. Semimalignancy requires wide en-bloc resection of amputation. 3. The term low grade malignancy is used to describe a tumor of very slow growth and with very late metastasis. Low-grade malignancy requires resection with careful preservation of functional structures. 4. The term secondary malignancy means the sarcomatous degeneration of a primarily benign lesion or bone tumor. This transformation is enhanced by irradiation and probably by acceleration of the normal turnover of bone tissue. In Paget's disease sarcomatous degeneration is to be expected in 2 percent of cases and in fibrous dysplasia in 0.5 percent of cases. 5. Sarcomatous degeneration of bone infarcts is rare, but an increase is to be expected due to an increased frequency of bone infarcts caused by long-term treatment with cortisone. 6. Primary bone tumors and recurrences show the same structure and cytology. In a minority of cases the recurrences are less differentiated; in a very few cases the recurrences are more highly differentiated and have a better prognosis than the initial lesion.- - - - - - - - - - ranking = 0.030309763446327keywords = sarcoma (Clic here for more details about this article) |
6/68. Sarcomatous transformation of the orbit in a patient with Paget's disease.OBJECTIVE: To report the clinical and pathologic features of sarcomatous transformation of the skull with involvement of the orbit, in Paget's disease. DESIGN: Interventional case report. PARTICIPANT: An 83-year-old woman with Paget's disease who experienced progressive proptosis of the left eye. INTERVENTION: Fine-needle aspiration biopsy of the orbital mass. RESULTS: Cytologic examination revealed round to oval malignant cells with wispy cytoplasm, consistent with the diagnosis of sarcoma. The patient died shortly after institution of orbital radiation therapy and systemic steroid therapy. CONCLUSIONS: Proptosis related to Paget's disease is uncommon, and only rarely is it a result of sarcomatous transformation. Sarcomatous transformation of the skull involving the orbit should be included in the differential diagnosis of progressive proptosis in patients with Paget's disease.- - - - - - - - - - ranking = 0.030309763446327keywords = sarcoma (Clic here for more details about this article) |
7/68. Multifocal osteogenic sarcoma in Paget's disease.The most serious complication of Paget's disease is sarcomatous degeneration of pagetic bone. Multifocal sarcomatous degeneration occurs mainly in polyostotic Paget's disease. Multifocal Paget's sarcoma is uncommon and can arise in any site. We report two cases of synchronous multifocal sarcomatous degeneration. The two patients were elderly women (aged 77 and 86 years, respectively) who developed sarcomatous lesions concomitantly, in the first case report in left ilium, left tibia, and first lumbar vertebra and in the second case report in the skull, right ilium, and sacrum. Whether these cases are due to the simultaneous development of several primaries or to metastases from a single primary remains unclear.- - - - - - - - - - ranking = 1.5092667628363keywords = osteogenic, sarcoma (Clic here for more details about this article) |
8/68. Case of an ivory vertebra.The differential diagnosis of an osteoblastic vertebral lesion (ivory vertebra) includes metastatic prostate cancer, lung cancer, lymphoma, osteosarcoma and Paget's disease. We report a case of a man who was initially diagnosed with Paget's disease on vertebral biopsy. He failed to respond to conventional bisphosphate therapy. The review of the original biopsy specimen showed metastatic carcinoid tumor involving the bone marrow. The various features of carcinoid tumors metastasizing to the skeleton are briefly reviewed.- - - - - - - - - - ranking = 0.45958698207156keywords = osteosarcoma, sarcoma (Clic here for more details about this article) |
9/68. Pseudosarcoma in Paget's disease.The authors describe the case of a male patient who had had Paget's disease since 1973 which led to the diffuse swelling of the proximal femur in 1993. The elevated alkaline phosphatase level and the destructive lytic lesions on the X-ray of the femur raised the possibility of malignancy. The bone scan showed increased accumulation in the proximal part of the left femur. On the MR the low T1 and the elevated T2 signal intensity suggested malignity despite that in some regions signals typical of adipose tissue could also be seen, which is usually interpreted as a sign of benignity. In the histological specimens no signs of malignant tumour cells were identified. The patient received bisphosphonate treatment which decreased his pain and swelling and also the alkaline phosphatase level. The patient died because of a rapidly progressing retroperitoneal fibrosarcoma and lung metastasis in 1996. The autopsy did not prove any malignant transformation of Paget's disease of bone in the proximal femur.- - - - - - - - - - ranking = 0.050516272410544keywords = sarcoma (Clic here for more details about this article) |
10/68. Benign tumefactive soft tissue extension from Paget's disease of bone simulating malignancy.osteosarcoma is a frequently fatal complication of Paget's disease of bone typically manifesting radiographically as a lytic lesion with soft tissue extension. A clinically worrisome, but benign manifestation of Paget's disease simulating malignancy because of an extraosseous mass is reported.- - - - - - - - - - ranking = 0.010103254482109keywords = sarcoma (Clic here for more details about this article) |
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