royal orthopaedic hospital oncology service, birmingham, u.k. spindle cell sarcoma of bone an...

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)?