Low-grade fibromyxoid sarcoma is a rare tumor, which most commonly arises from the deep soft tissues of the lower extremities in young men. Diagnosis of this tumor can be difficult because of its deceptively benign histopathologic appearance. Specifically, the lesion is characterized by alternating fibrous and myxoid areas with variable cellularity and a whorled growth pattern. Like many soft tissue tumors, low-grade fibromyxoid sarcoma is also characterized by strong immunoreactivity to the human proto-oncogene BCL-2. Cytogenetically, the lesion has also been associated with the t (7,16) (q33;p11) translocation, with the characteristic resultant FUS and CREB3L2 fusion gene. In this report, we describe the rare case of a low-grade fibromyxoid sarcoma that appeared to develop as a primary malignancy in the neck of the talus of a young man who presented with recurrent ankle pain after a previous surgery for a cystic lesion at the same site.
Copyright 2010 American College of Foot and Ankle Surgeons. Published by Elsevier Inc. All rights reserved.