Myxofibrosarcoma (MFS) is a malignant fibroblastic/myofibroblastic neoplasm with the prominent myxoid area. It has the clinical features of frequent local recurrence and occasional distant metastasis. Morphologically, MFS is occasionally difficult to distinguish from undifferentiated pleomorphic sarcoma (UPS), especially in the case of high-grade MFS. Here, we reviewed clinical and histologic data of 162 MFS cases and 43 UPS cases. MFS was distinguished from UPS with the criterion of 10% myxoid area as a cutoff value. Overall, 52 MFS (34.4%) and 9 UPS (20.9%) cases showed local recurrence, 18 MFS (12.2%) and 19 UPS (44.2%) cases developed distant metastasis, and 13 MFS (9.5%) and 14 UPS (32.6%) cases resulted in tumor-related death. Statistically, MFS had a better prognosis than UPS. Moreover, MFS with less myxoid area had a tendency to present a poorer prognosis. FNCLCC grade was a statistically significant prognostic factor (distant metastasis: P=0.0021, tumor-related death: P=0.0021). Cellularity and nuclear atypia had only a statistical tendency for associations with a poorer prognosis. The overall survival rate of MFS after transformation into a UPS-like condition (<10% myxoid area) was close to that of UPS. It was suggested that MFS is a biologically distinct tumor from UPS, and MFS with less myxoid area had a tendency to present a poorer prognosis. We considered that evaluation of the amount of myxoid area, cellularity, and nuclear atypia may be important as prognostic predictors. MFS may become similar to histologic malignancy of UPS in terms of morphology and biology via local recurrence.
Departments of *Anatomic Pathology, Pathological Sciences
†Dermatology, Pathological Sciences
‡Orthopedic Surgery, Pathological Sciences, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
Conflicts of Interest and Source of Funding: Supported by a JSPS KAKEN Grant (No. 19H03444) and by funds from the Scholarship Program of the Takeda Science Foundation. The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article.
Correspondence: Yoshinao Oda, MD, PhD, Department of Anatomic Pathology, Pathological Sciences, Graduate School of Medical Sciences, Kyushu University, Maidashi 3-1-1, Higashi-ku, Fukuoka 812-8582, Japan (e-mail: firstname.lastname@example.org).