Recurrence patterns and survival for patients with intermediate- and high-grade myxofibrosarcoma.
OBJECTIVE
Myxofibrosarcoma (MFS) is a rare sarcoma with a predilection for multiple local recurrences (LR), for which optimal treatment has not been defined. We reviewed our experience to determine the impact of surgery and radiation therapy (RT) on pattern of recurrence, limb salvage, and overall survival (OS).


METHODS
Between 1995 and 2005, 36 patients with localized intermediate- or high-grade MFS were treated at our institution. Data on clinicopathologic features, treatments, and patient outcomes were reviewed and analyzed.


RESULTS
Median age was 72.5 years (range, 42-96 years). Median tumor size was 7.5 cm, and 34 tumors (94%) were high grade. All patients underwent surgery at our institution, including re-resections in 20 patients (56%) after initial surgery elsewhere. Margins were microscopically positive in 9 patients (25%). RT was given to 28 patients (78%) pre - and/or postoperatively. After a median follow-up of 3.5 years (range, 0.4-12.4 years), 11 patients (31%) developed LR. There were no significant predictors for LR on univariate analysis, including margin status or use of RT. Limb salvage was ultimately achieved in only 5 of 11 LRs (45%) because of multiple subsequent LRs. Distant recurrence (DR) occurred in 6 patients (17%). Median and 4-year OS were 96 months and 65%, respectively. Seven patients (19%) died of tumor-related causes, 6 of whom had DRs. On univariate analysis, tumor size was associated with OS.


CONCLUSIONS
Despite aggressive surgery and RT, intermediate- and high-grade MFS are associated with a high rate of LR that adversely affects limb preservation. More aggressive local treatment strategies are necessary.
Harvard Radiation Oncology Program, Brigham and Women's Hospital, Boston, MA 02115, USA.
Karl E
KE
Haglund
Chandrajit P
CP
Raut
Alessandra F
AF
Nascimento
Qian
Q
Wang
Suzanne
S
George
Elizabeth H
EH
Baldini
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000671:Amputation, Surgical / Q000706:statistics & numerical data; D000704:Analysis of Variance; D005260:Female; D005354:Fibrosarcoma / Q000401:mortality / Q000473:pathology / Q000532:radiotherapy* / Q000556:secondary / Q000601:surgery*; D006801:Humans; D023821:Limb Salvage / Q000706:statistics & numerical data; D035002:Lower Extremity / Q000601:surgery; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000401:mortality / Q000601:surgery; D018365:Neoplasm, Residual; D011879:Radiotherapy Dosage; D035583:Rare Diseases / Q000401:mortality / Q000473:pathology / Q000532:radiotherapy* / Q000601:surgery*; D016896:Treatment Outcome; D047368:Tumor Burden; D034941:Upper Extremity / Q000601:surgery