The benign exostoses in TRPS have
The benign exostoses in TRPS have a recognized malignant potential which have been shown to develop into chondrosarcomas [4,13]. Both our index case and his mother demonstrated de novo high grade osteosarcoma. To our knowledge, this occurrence has not been described anywhere within the literature.
Our cases highlight that TRPS in general also has the potential for de novo osteosarcoma. We would suggest plain radiographs and magnetic resonance imaging of the affected limb, along with staging bone scintigraphy and computer topography of the chest, abdomen and DNA Damage DNA Repair Library as the first radiological investigations of choice at any center managing a patient with a suspected sinister exostosis or bone pain in TRPS. Bone biopsy is the gold standard method of determining the exact nature of any bony lesion as it provides a tissue diagnosis. Biopsy should be performed at a reference bone tumor unit that is familiar with the care of musculoskeletal malignancies and where specialist orthopedic, radiology, oncology and histopathology expertise can be rapidly accessed under the auspices of a fully accredited bone sarcoma multi-disciplinary team.
We would advocate that any unit treating a patient with TRPS who has a suspect bone malignancy follow published guidelines for the management of bone sarcoma .
No recommendations have been published regarding the long term surveillance of patients with TRPS. Follow-up should take into account the multi-systemic involvement of the syndrome and any potential malignant transformation within a pre-existing exostosis or, as in our cases, de novo malignancy, should be recognized early thereby promoting the possibility of limb salvage surgery as a viable, curative option.
Conflict of Interest Statement