Fourteen cases of sarcomatous degeneration in Paget's disease

Objective. To describe characteristics of cases of sarcomatous degeneration in Pager's disease (SDP) recorded over 10 years in 2 French university hos pitals, with particular emphasis on the pattern of this entity on magnetic resonance imaging (MRI). Methods. Fourteen cases are described. Epidemiological, clinical, radiologi cal, 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. Pag et's disease was monostotic in 5 cases and polyostotic in 9. Neurological c omplications were present in 10 cases. The mean interval between the first clinical signs and diagnosis was 4.4 +/- 4.7 months. The locations were: li mbs 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 a ll 3 cases studied (heterogeneous low signal intensity of the tumor on T1 w eighted images increased after intravenous gadolinium administration and hi gh signal intensity on T2 weighted images), whereas the radiological patter n was lyric in 2 cases and sclerotic in one case. The diagnosis was proved by histological investigation in 11/14 patients (osteogenic sarcoma of diff erent appearance in 7 patients, fibroblastic sarcoma in one, fibrous histio cytoma-like sarcoma in one, and undifferentiated sarcoma in 2), Four patien ts had pulmonary metastases and 1 patients are still alive with followup ra nging from 5 months to 5 years. Conclusion. Paget sarcomas remain the most threatening sarcomas of bone, th eir 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.