Myxoinflammatory Fibroblastic Sarcoma of the Nose: First Reported Case at an Unusual Location (Nasal Dorsum), with a Review of the Literature

Abstract

Myxoinflammatory fibroblastic sarcoma (MIFS) is a rare, low-grade, malignant, soft-tissue tumor that typically affects the distal extremities of middle-aged patients. In most cases, it presents as a painless, slowly growing mass within the subcutaneous tissue. It is associated with a low rate of metastasis but a high rate of local recurrence. In addition to the distal extremities, MIFS has been reported in the thigh, arm, forearm, groin, upper back, neck, and temporal area. As far as we know, no case has been previously reported in the nasal area. We report for the first time a case of MIFS presenting on the dorsum of the nose. The painless, 3.0-cm tumor was initially mistaken for reticular erythematous mucinosis, a benign skin condition that occurs when fibroblasts produce abnormally large amounts of mucopolysaccharides. The tumor was surgically removed in its entirety with surgical margins of 3 to 5 mm. During 4 years of follow-up, no clinical or radiologic evidence of a recurrence or metastasis was seen. We discuss the imaging and histologic features of MIFS, as well as its clinical management and follow-up, and we review related reports in the literature.