Malignant pigmented villonodular synovitis (PVNS) is an extremely rare lesion. Approximately 30 cases of malignant PVNS have been reported to date and of these, only 1 case involved the temporomandibular joint. Owing to the rarity of well-documented cases and the heterogeneous histologic features of this group of tumors, there has been some confusion regarding its diagnosis. The heterogeneous features of the sarcomatous areas contain fibrosarcomatous, myxosarcomatous, malignant fibrous histiocytomalike or giant cell tumorlike patterns. However, despite the absence of frank sarcomatous change in the histopathogy of PVNS, there have been 3 reported cases of metastatic lesions in the lung or lymph nodes. Here we present an additional case of clinically malignant PVNS with pulmonary metastasis after recurrence. A 29-year-old man presented in our hospital with a recurrent swelling and pain in the right preauricular area, where benign tumor had been previously resected. MRI demonstrated a large mass with a low signal intensity that seemed to demonstrate a ferromagnetic effect. Surgical resection of the lesion was performed and the diagnosis of PVNS with focal atypical cells was made. Unfortunately, at 30 months post surgery, a thoracic CT found a metastatic nodule in the left lower lobe of the lung.
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