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Low-grade fibromyxoid sarcoma (LGFMS) is a relatively uncommon tumor with distinctive histologic features, which most commonly presents as a solitary mass. A 54-year-old man with a suspected renal tumor underwent a partial nephrectomy after a biopsy revealed findings consistent with a high-grade sarcoma. Two years later, an abdominopelvic computed tomographic scan revealed a 4.5-cm mass in the lower pole of his right kidney. This mass was biopsied, and the diagnosis of LGFMS was confirmed. Histologically, the tumor consisted of a proliferation of bland spindled tumor cells arranged in an alveolar pattern with brisk mitotic activity. Microscopic perirenal and periureteral lymphovascular invasion was present, and tumor cells had spread to the renal sinus and perinephric adipose tissue. The literature was reviewed, and only four cases were identified in which LGFMS presented as perinephric or retroperitoneal tumor with either local (n = 2) or distant (n = 2) invasion. Immunohistochemical studies showed diffuse staining for vimentin. Importantly, the majority of cases reported were characterized by a biphasic pattern with islands of myofibroblastic spindle cells surrounded by a collagenized, myxoid stroma. The neoplastic cells in our case demonstrated a similar presentation. Our findings support the previously mentioned biphasic histologic pattern, along with their propensity for perinephric, retroperitoneal
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