spindle cell sarcoma of bone an assessment of outcome
DESCRIPTION
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. To investigate treatment, outcome and prognostic factors for “spindle cell sarcomas of bone” - PowerPoint PPT PresentationTRANSCRIPT
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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
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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
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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
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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
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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
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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
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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%)
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Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
HISTOLOGYHISTOLOGY
MFH 74
Spindle cell sarcoma 56
Leiomyosarcoma 24
Fibrosarcoma 18
Angiosarcoma 11
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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
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Royal Orthopaedic Hospital Oncology Service, Birmingham, U.K.
MANAGEMENTMANAGEMENT
• As per osteosarcoma
• Chemotherapy when appropriate
• Surgical excision
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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
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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
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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
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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
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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
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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
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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
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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.
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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
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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
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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%
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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)?