Transcript SPINDLE CELL SARCOMA OF BONE AN ASSESSMENT OF OUTCOME David PEAKE Robert J GRIMER, David SPOONER, Simon R CARTER, Roger M TILLMAN, Seggy ABUDU, Mark.

SPINDLE CELL SARCOMA OF BONE
AN ASSESSMENT OF OUTCOME
David PEAKE
Robert J GRIMER, David SPOONER,
Simon R CARTER, Roger M TILLMAN,
Seggy ABUDU, Mark DAVIES
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
THE STUDY
• To investigate treatment, outcome and
prognostic factors for “spindle cell
sarcomas of bone”
• Retrospective review of prospective
database (1970 – 2001)
• Pathology diagnosis as made at the time
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
SPINDLE CELL SARCOMA OF BONE
DEFINITION
All primary bone sarcomas that
were NOT
Osteosarcoma
Ewing’s sarcoma
Chondrosarcoma
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
THE DATABASE
• Prospective from 1986 (but includes all
tumour cases since 1970)
• >15,000 patients
• Records patient details, diagnosis,
treatment, outcome, function etc
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
RESULTS
• 183 patients - 116 male : 67 Female
• 179 primary : 4 secondary
–3 RT / 1 Paget’s
• Stage:
–10 low grade
–173 high grade
– 26 metastases at presentation
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
AGE
35
Mean 45 yrs
30
Count
25
20
76
<40
15
60
40-60
47
>60
10
5
0
0
20
40
60
80
100
AGE
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
SITE
157 limb : 26 axial
– 78
– 26
– 23
– 21
– 10
– 25
distal femur
pelvis
proximal femur
proximal tibia
humerus
other
34 had a
pathological
fracture at
presentation
(18%)
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
HISTOLOGY
MFH
Spindle cell sarcoma
Leiomyosarcoma
Fibrosarcoma
Angiosarcoma
74
56
24
18
11
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
HISTOLOGY VARIED WITH TIME !
100%
90%
80%
70%
Sarcoma
MFH
Leio
Fibro
Angio
60%
50%
40%
30%
20%
10%
0%
1980
1985
1990
1995
2000
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
MANAGEMENT
• As per osteosarcoma
• Chemotherapy when appropriate
• Surgical excision
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
CHEMOTHERAPY
131 (71%) received chemotherapy
Doxorubicin / Cisplatin
Doxorubicin / HDMTX
Doxorubicin / Ifosfamide
Others
Unknown
84
10
10
15
12
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
CHEMOTHERAPY - 2
• Chemotherapy dependant on age
–84% under age 40
–56% aged 40-60
–18% over age 60
• Neoadjuvant in 83% of those who had it
• 30% had better than 90% necrosis
• No conclusion possible about best regime
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
SURGERY
• 150 patients had surgery
–37 amputation (25%)
–113 LSS (75%)
• Trend towards increased amputation in
older patients and those with
pathological fracture
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
OUTCOME
• Stage 3 disease
– median survival 13 months
• High grade, non axial, non metastatic
disease (n = 125):
–Overall survival
62% at 5 years 58% at 10 years
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
OVERALL SURVIVAL (no mets, limb)
1
62% at 5 years (+ 4%)
58% at 10 years (+ 4%)
Cum. Survival
.8
.6
.4
.2
0
0
5
10
15
20
25
30
Time in yrs
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
PROGNOSTIC FACTORS (p<0.05)
•
•
•
•
•
Limb salvage > amputation
Necrosis > 90%
Limb > central
Age < 40
Not significant
HR 5.4
HR 5.4
HR 1.9
HR 2.0
– Diagnosis
– Type of chemotherapy
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
Survival according to age
1
< 40 yrs
Cum. Survival
.8
.6
40 – 60 yrs
.4
.2
> 60 yrs
0
0
2
4
6
8
10
12
14
16
18
20
Time in yrs
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
Survival according to histology
1
Cum. Survival
.8
LMS
.6
MFH
.4
Fibrosarcoma
Angiosarcoma
Sarcoma
.2
0
0
2
4
6
8
10
12
14
16
18
20
N.S.
Time in yrs
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
LOCAL RECURRENCE
• 15% overall
• 20% in LSS cases
• 7% after amputation
– Highest in distal femur (28%)
– Decreasing risk by decade
– Not increased with pathological fracture
• 73% died after LR
– Mean survival 11 months after LR
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
RISK FACTORS FOR
LOCAL RECURRENCE
• Marginal margins HR 3.5 (1.5-8.0)
p = 0.002
• < 90% necrosis
p = 0.026
HR 11.6 (1.3 – 100)
Univariate
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
COMPARISON (63% at 5 yrs)
Huvos, 1985
Little, 1993
Yokoyama, 1993
Picci, 1997
Bielack, 1997
Bramwell,1999
MFH (n=130)
MFH (n=39)
MFH(n=34)
MFH (n=51)
MFH (n=125)
MFH (n=41)
53%
53%
43%
67%
59%
59%
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
CONCLUSION
“Spindle cell sarcoma of bone”
• describes a heterogeneous group of primary
bone sarcomas (cf soft tissue sarcoma)
• behaves like, if not better than, osteosarcoma
matched for age
• should be treated the same as osteosarcoma
• why not register for same trials (especially for
OS > 40)?
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.