Abate, Veronica (2025) Primary And Metastatic Neoplasms Arising In Pagetic Bones. [Tesi di dottorato]

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Abstract

Background. Paget’s disease of bone (PDB) is a focal metabolic bone disorder. In adulthood, bones affected by PDB are predisposed to neoplastic degeneration and resistant to metastatic invasion. In this article, a clinical survey of patients enrolled in the Italian PDB registry and a systematic review with individual patient data analysis (IPD, PROSPERO database number CRD42020151209) were performed to describe the characteristic of PDB patients with primary (BC-PDB) or metastatic (BM-PDB) neoplasms arising in PDB bones. Methods. BC-PDB were classified as osteosarcoma (Sar-PDB), giant cell tumor (GCT-PDB), and miscellaneous malignancies (MM-PDB). Patients from the Italian PDB Registry without BC-PDB and/or BM-PDB were classified as Italian PDB Controls. Results. Among 742 patients enrolled in the Italian PDB Registry, we observed 11 Sar-PDB, 6 GCT-PDB, and 1 MM-PDB, with a prevalence of 1.35%, 0.81%, and <0.01%, respectively. The IPD identified 1468 Sar-PDB, 122 GCT-PDB, 57 MM-PDB, and 70 BM-PDB. Overall, BC-PDB arises exclusively in pagetic bones and affect preferentially Caucasian men with multiple skeletal sites affected by PDB. The occurrence of cancer increases the 5-years mortality rate of affected patients. Sar-PDB and MM-PDB showed a 5-years mortality rate higher than GCT-PDB, which in turn showed a lower mean age at PDB onset compared to Sar-PDB and MM-PDB. Among 149 PDB patients enrolled in the Italian PDB Registry showing a non-bone cancer (56 with metastatic disorder), 6 showed a BM-PDB. Conclusion. BC and BM arising in PDB patients show unique characteristics compared to non PDB BC and BM.

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