royal orthopaedic hospital oncology service, birmingham, u.k. spindle cell sarcoma of bone an...
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Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
SPINDLE CELL SARCOMA OF BONE AN ASSESSMENT OF OUTCOME
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 STUDYTHE 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
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 DATABASETHE 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.
RESULTSRESULTS
• 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.
AGEAGE
Mean 45 yrs
76 <40
60 40-60
47 >60
0
5
10
15
20
25
30
35
Cou
nt
0 20 40 60 80 100AGE
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
SITESITE
157 limb : 26 axial– 78 distal femur– 26 pelvis– 23 proximal femur– 21 proximal tibia– 10 humerus– 25 other
34 had a pathological fracture at presentation (18%)
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
HISTOLOGYHISTOLOGY
MFH 74
Spindle cell sarcoma 56
Leiomyosarcoma 24
Fibrosarcoma 18
Angiosarcoma 11
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
HISTOLOGY VARIED WITH TIME !HISTOLOGY VARIED WITH TIME !
0%
10%
20%
30%
40%
50%
60%
70%
80%
90%
100%
1980 1985 1990 1995 2000
Sarcoma
MFH
Leio
Fibro
Angio
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
MANAGEMENTMANAGEMENT
• As per osteosarcoma
• Chemotherapy when appropriate
• Surgical excision
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
CHEMOTHERAPYCHEMOTHERAPY
131 (71%) received chemotherapy
Doxorubicin / Cisplatin 84
Doxorubicin / HDMTX 10
Doxorubicin / Ifosfamide 10
Others 15
Unknown 12
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
CHEMOTHERAPY - 2 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.
SURGERYSURGERY
• 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.
OUTCOMEOUTCOME
• 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)OVERALL SURVIVAL (no mets, limb)
62% at 5 years (+ 4%)58% at 10 years (+ 4%)
0
.2
.4
.6
.8
1C
um. S
urvi
val
0 5 10 15 20 25 30
Time in yrs
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
PROGNOSTIC FACTORS (p<0.05)PROGNOSTIC FACTORS (p<0.05)
• Limb salvage > amputation HR 5.4
• Necrosis > 90% HR 5.4
• Limb > central HR 1.9
• Age < 40 HR 2.0
• Not significant – Diagnosis
– Type of chemotherapy
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
Survival according to ageSurvival according to age
0
.2
.4
.6
.8
1C
um. S
urvi
val
0 2 4 6 8 10 12 14 16 18 20
Time in yrs
< 40 yrs
> 60 yrs
40 – 60 yrs
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
Survival according to histologySurvival according to histology
0
.2
.4
.6
.8
1
Cum
. Sur
viva
l
0 2 4 6 8 10 12 14 16 18 20
Time in yrs
LMS
Sarcoma
MFH
FibrosarcomaAngiosarcoma
N.S.
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
LOCAL RECURRENCELOCAL 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 RECURRENCERISK FACTORS FOR
LOCAL RECURRENCE
• Marginal margins HR 3.5 (1.5-8.0) p = 0.002
• < 90% necrosis HR 11.6 (1.3 – 100) p = 0.026 Univariate
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
COMPARISON (63% at 5 yrs)COMPARISON (63% at 5 yrs)
Huvos, 1985 MFH (n=130) 53%
Little, 1993 MFH (n=39) 53%
Yokoyama, 1993 MFH(n=34) 43%
Picci, 1997 MFH (n=51) 67%
Bielack, 1997 MFH (n=125) 59%
Bramwell,1999 MFH (n=41) 59%
Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
CONCLUSION 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)?