Management of acral myxoinflammatory fibroblastic sarcoma.
BACKGROUND
Acral myxoinflammatory fibroblastic sarcoma (AMFS) is a rare, low-grade sarcoma that commonly affects the distal extremities. From the published cases, therapy for AMFS to date has been comprised of excision or amputation, with limited use of radiotherapy (RT) or chemotherapy. In this report, the outcome of 17 patients with AMFS treated at the study institution was reported.


METHODS
A retrospective review of all cases of AMFS identified in the Sarcoma Database in the Department of Radiation Oncology at the study institution was conducted. Treatment records and data from follow-up visits of patients were reviewed.


RESULTS
Seventeen patients were identified. All the patients underwent surgical resection (15 excisions and 2 amputations). Positive surgical margins after excisions were noted in 5 patients and were widely positive in 1 patient. Of the 17 patients, 14 patients received some form of RT. The average total dose was 56.4 Gray (Gy). Eight patients received preoperative RT alone, 5 patients received preoperative RT and postoperative RT, and 1 patient received preoperative RT and intraoperative RT. Median follow-up was 24.5 months. One patient presented with recurrent disease and was treated with resection, and both pre- and postoperative RT. He was free of disease 23 months after his last treatment. No local recurrence was noted in the remaining patients. Of the 14 patients undergoing preoperative RT, complete pathologic necrosis or no tumor was noted in 1 of the patients. No metastatic disease was observed in any of the patients. There was no significant radiation toxicity observed in any of the patients.


CONCLUSIONS
Data were consistent with local control of distal extremity sarcomas with resection and RT, suggesting that limb-sparing surgery with this treatment combination is an appropriate option in the limb-sparing control of patients with AMFS, even those with positive surgical margins.
Tejwani
Ajay
A
Center for Sarcoma and Connective Tissue Oncology, Massachusetts General Hospital, Boston, Massachusetts, USA.
Kobayashi
Wendy
W
Chen
Yen-Lin Evelyn
YL
Rosenberg
Andrew E
AE
Yoon
Sam
S
Raskin
Kevin A
KA
Rosenthal
Daniel I
DI
Nielsen
Gunnlaugur P
GP
Hornicek
Francis J
FJ
Delaney
Thomas F
TF
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005121:Extremities; D005260:Female; D051677:Histiocytoma, Malignant Fibrous / Q000532:radiotherapy* / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012509:Sarcoma / Q000532:radiotherapy* / Q000601:surgery*