Forty-eight cases of leiomyosarcoma of bone in Japan: A multicenter study from the Japanese musculoskeletal oncology group.
BACKGROUND
Leiomyosarcoma of bone (LMSoB) is a rare malignant bone tumor. This multicenter retrospective study was conducted to investigate the diagnosis and the clinical outcome of primary LMSoB in Japan.


METHODS
Forty-eight patients (average age: 52 years [range 14-88 years]) with primary LMSoB who were treated at registered institutes in Japan between 1991 and 2014 were recruited. The median follow-up period was 44 months (range: 2-273).


RESULTS
The 5-year overall survival rates and disease-free survival rates were 78.3% and 44.9%, respectively. Surgical treatment was performed in 42 patients, and R0 resection was achieved in 31 patients. Neoadjuvant chemotherapy was administered in 18 patients. The most common regimen (cisplatin-based chemotherapy) was administered in 15 patients, however, no patient achieved a good response in both radiological and histological evaluations. The presence of metastasis at the first visit and a lack of definitive surgery were significantly correlated with poor overall survival, and the surgical margin was a significant prognostic factor for disease-free survival.


CONCLUSIONS
This study is the largest LMSoB case series ever reported. Surgical treatment with wide margins was the only treatment that proved to be effective, whereas adjuvant chemotherapy in the present setting did not improve the overall survival. J. Surg. Oncol. 2016;114:495-500. © 2016 Wiley Periodicals, Inc.
Department of Orthopaedic Surgery, School of Medicine, Keio University, Tokyo, Japan.|Division of Musculoskeletal Oncology, National Cancer Center Hospital, Tokyo, Japan.
Tomoaki
T
Mori
Department of Orthopaedic Surgery, School of Medicine, Keio University, Tokyo, Japan.
Robert
R
Nakayama
Department of Orthopaedic Surgery, Kyushu University, Fukuoka, Japan.
Makoto
M
Endo
Department of Orthopaedic Surgery, Hokkaido Cancer Center, Hokkaido, Japan.
Hiroaki
H
Hiraga
Department of Orthopaedic Surgery, Nagasaki University, Nagasaki, Japan.
Masato
M
Tomita
Department of Orthopaedic Surgery, Hyogo Cancer Center, Akashi, Japan.
Naomasa
N
Fukase
Division of Musculoskeletal Oncology, National Cancer Center Hospital, Tokyo, Japan.
Eisuke
E
Kobayashi
Division of Musculoskeletal Oncology, National Cancer Center Hospital, Tokyo, Japan.
Akira
A
Kawai
Department of Orthopaedic Surgery, National Hospital Organization Osaka National Hospital, Osaka, Japan.
Takafumi
T
Ueda
Department of Orthopaedic Surgery, School of Medicine, Keio University, Tokyo, Japan.
Hideo
H
Morioka
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001859:Bone Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D017024:Chemotherapy, Adjuvant; D005260:Female; D006801:Humans; D007890:Leiomyosarcoma / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies
chemotherapy; cisplatin; leiomyosarcoma of bone; prognostic factors; surgery