Diffuse-type tenosynovial giant cell tumor (D-TGCT), otherwise known as pigmented villonodular synovitis, is a locally aggressive tumor which can show multiple recurrences but is rarely associated with metastasis. A handful of studies have elucidated the imaging features and clinical course in metastatic D-TGCT with malignant transformation on histology. However, only 5 cases of metastatic D-TGCT with benign histological features have been reported in the literature, with the clinical course and prognosis reported in only 1 case. Therefore, relatively little is known about the implications of histologically benign metastasis on the role of imaging, management, and clinical outcomes. We report a case of a 51-year-old female with recurrent D-TGCT localized to the knee that metastasized to the lymph nodes and soft tissue 3 years after above-the-knee amputation and 16 years after initial diagnosis of localized D-TGCT, despite benign histologic features on lymph node excision. This case highlights the necessity of timely MRI imaging to prevent delayed diagnosis, the role of histological findings on treatment response, and clinical outcomes associated with metastasized D-TGCT. Keywords: D-TGCT, PVNS, Metastasis, Histologically benign, Tenosynovial giant cell tumor
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