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

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

SPINDLE CELL SARCOMA OF BONE DEFINITION All primary bone sarcomas that were NOT Osteosarcoma Ewing’s sarcoma Chondrosarcoma

THE DATABASE Prospective from 1986 (but includes all tumour cases since 1970) >15,000 patients Records patient details, diagnosis, treatment, outcome, function etc

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

AGE Mean 45 yrs 76 <40 60 40-60 47 >60

157 limb : 26 axial SITE 78 distal femur 26 pelvis 23 proximal femur 21 proximal tibia 10 humerus 25 other 34 had a pathological fracture at presentation (18%)

HISTOLOGY MFH 74 Spindle cell sarcoma 56 Leiomyosarcoma 24 Fibrosarcoma 18 Angiosarcoma 11

HISTOLOGY VARIED WITH TIME !

MANAGEMENT As per osteosarcoma Chemotherapy when appropriate Surgical excision

131 (71%) received chemotherapy Doxorubicin / Cisplatin 84 Doxorubicin / HDMTX 10 Doxorubicin / Ifosfamide 10 Others 15 Unknown 12

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

SURGERY 150 patients had surgery 37 amputation (25%) 113 LSS (75%) Trend towards increased amputation in older patients and those with pathological fracture

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

OVERALL SURVIVAL (no mets, limb) 62% at 5 years (+ 4%) 58% at 10 years (+ 4%)

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

Survival according to age < 40 yrs 40 – 60 yrs > 60 yrs

Survival according to histology LMS MFH Fibrosarcoma Angiosarcoma Sarcoma N.S.

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

RISK 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

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%

“Spindle cell sarcoma of bone” 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)?