Malignant pigmented villonodular synovitis: a brief literature review and a case report
Case report, 181 - 185Tag this article
Pigmented villonodular synovitis (PVNS) is a benign lesion that usually occurs in tendon sheaths, in burses or in the large joints synovia of the extremities. The pathogenesis of PVNS is still not clear, recently defined as a benign clonal neoplastic proliferation. PVNS is often asymptomatic and it is incidentally diagnosed in the majority of cases; given its local aggressiveness and the high rate of local recurrences it requires wide excision to be successfully treated. In rare cases PVNS can have malignant features sometimes with metastatic diffusion. The malignant form is often associated to the presence of colony-stimulating factor 1 (CSF1) gene mutation. In this report we present a brief literature review and the first case of recurrent hip PVNS with vertebral metastases, associated to an unusual genetic mutation.
KEY WORDS: PVNS; bone tumor; vertebral metastases; Imatinib; malignant synovitis