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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.

Bone Neoplasms↗

Changing concepts in the pathological basis of soft tissue and bone sarcoma treatment.

Though soft tissue sarcomas are rare considerable progress has been made in the clinical and biological understanding of these neoplasms. This has led to the launch of a new WHO classification of soft tissue tumours in 2002, which integrate morphological data with tumour specific (cyto-) genetics. Moreover worldwide consensus has grown how to predict clinical behaviour based on a specific grading system and which specific types of tumours seem not to obey these rules. As a consequence entry criteria for multi-institute prospective trials have changed over the last few years. The recent identification of tumour specific drug targets by immunohistochemistry has had impact on specimen requirements and handling as well as laboratory standards. These changes in concepts, classification, and processing of soft tissue sarcomas have had impact on patient selection and treatment and formats of multi-institute trials.

Biomarkers, Tumor↗