Fourteen cases of sarcomatous degeneration in Paget's disease.

ABSTRACT

OBJECTIVE: To describe characteristics of cases of sarcomatous degeneration in Paget's disease (SDP) recorded over 10 years in 2 French university hospitals, with particular emphasis on the pattern of this entity on magnetic resonance imaging (MRI). METHODS: Fourteen cases are described. Epidemiological, clinical, radiological, histological, and MRI features (in 3 cases) are compared to others in the literature. RESULTS: Nine men and 5 women (mean age 75.2+/-7.7 yrs) were studied. Paget's disease was monostotic in 5 cases and polyostotic in 9. Neurological complications were present in 10 cases. The mean interval between the first clinical signs and diagnosis was 4.4+/-4.7 months. The locations were limbs 2, skull 2, pelvis 7, sacrum 3. The radiological pattern was lytic in 9 cases, sclerotic in 3, and mixed in 2. MRI revealed a similar pattern in all 3 cases studied (heterogeneous low signal intensity of the tumor on T1 weighted images increased after intravenous gadolinium administration and high signal intensity on T2 weighted images), whereas the radiological pattern was lytic in 2 cases and sclerotic in one case. The diagnosis was proved by histological investigation in 11/14 patients (osteogenic sarcoma of different appearance in 7 patients, fibroblastic sarcoma in one, fibrous histiocytoma-like sarcoma in one, and undifferentiated sarcoma in 2). Four patients had pulmonary metastases and 4 patients are still alive with followup ranging from 5 months to 5 years. CONCLUSION: Paget sarcomas remain the most threatening sarcomas of bone, their prognosis being far more negative than primary sarcomas. Improvement in therapy strategies including surgery, radiation therapy, and chemotherapy might together provide better prognosis for SDP.