Myxofibrosarcoma of unusual sites

Abstract

Introduction

Myxofibrosarcoma classically presents as a painless mass in the proximal extremities. Cutaneous myxofibrosarcomas arising in the head and neck and distal extremities are extremely uncommon. We present a series of six cases of myxofibrosarcoma presenting in the head and neck and acral locations.

Methods

Archives were searched using the term "myxofibrosarcoma" over a 6-year period (2009–2015). The clinicopathologic features of myxofibrosarcoma were recorded. Cases in the head and neck or acral locations were retrieved. When available, the patient's medical records were reviewed.

Results

Ninety-five cases of myxofibrosarcoma were identified over a 6-year period. Six patients were identified with myxofibrosarcoma arising in the head (n = 4, M:F 3:1), hand (n = 1, F), and foot (n = 1, F). Each had typical features of myxofibrosarcoma. Two of the tumors on the head were high-grade and had multiple recurrences, while the remaining two were intermediate grade. Both acral tumors were intermediate grade and one recurred locally within a year of diagnosis.

Conclusions

Myxofibrosarcoma may rarely involve the head and neck and acral locations, and presentation in these sites is a potential source of diagnostic difficulty. Recognition of the characteristic histologic features of myxofibrosarcoma in conjunction with judicial use of immunohistochemical stains should allow for accurate diagnosis.