Trends in presentation of bone and
soft tissue sarcomas over 25 years –
little evidence of earlier diagnosis
Royal Orthopaedic Hospital,
Birmingham
Rob Grimer, Lee Jeys,
Gemma Smith, Graham Johnson
Are we getting better?
• Sarcomas are
frequently not
recognised by:
– Patients
– GPs
– Hospital doctors
• As a result there are
often long delays
before they are
diagnosed
What do we know already
• Sarcomas are diagnosed late
• Only 16.5% are diagnosed through the 2
week wait clinic
• Of GP referrals only 13% are malignant
• Delays in diagnosis occur at every stage in
the patient pathway
• Numerous Cancer Plans and early
diagnosis initiatives have been introduced
• Have they worked??
How can we tell if we are getting
better?
• Duration of symptoms
• Size at time of diagnosis
• Appropriate referral
By stopping people
presenting like this?
Had been seeing GP for
1 year with „ulcer‟
In fact high grade STS
Method
• Analysis of prospectively kept database
– Size at presentation
– Duration of symptoms („how long have you
had any symptoms related to this...?)
– Proportion of „whoops‟ procedures
• Analysed over a 25 year period
The patients:
• 4934 patients
• 2568 bone sarcomas
– 1240 osteosarcomas
– 539 chondrosarcomas
– 459 Ewing‟s sarcomas
– 276 spindle cell sarcomas
– 54 others
• 2366 STS
Bone Sarcomas
• Mean size = 10.7 cm
– Osteosarcoma
10.7cm
– Chondrosarcoma 11.3cm
– Ewing‟s sarcoma 9.9cm
– Spindle cell sarcoma 10.4cm
29 yr old felt he was sitting on a
knobbly seat while on way on
honeymoon to Maldives – worried?
He was sitting on a 15cm chondrosarcoma!
Size distribution - Osteosarcoma
160
140
120
Mean size 10.5cm
♂ 10.9cm, ♀ 10.1cm
Count
100
80
60
40
20
0
0
5
10
15
20
Size in cm
25
30
35
40
2009.000
2008.000
2007.000
2006.000
2005.000
2004.000
2003.000
2002.000
2001.000
2000.000
1999.000
1998.000
1997.000
1996.000
1995.000
1994.000
1993.000
1992.000
1991.000
1990.000
1989.000
1988.000
1987.000
1986.000
SizeA
Are we getting better – OS size?
45
40
35
30
25
20
15
10
5
0
Size at diagnosis - unchanged over 25 years
10.5cm
STS
• Mean size = 9.9 cm (range 0.3 to 47cm)
•
•
•
•
•
•
Liposarcoma
13.3cm
MPNST
10.5cm
MFH
10.3cm
Leiomyosarcoma 7.7cm
Synovial sarcoma 6.9cm
Epithelioid sarcoma 3cm
Mean size of Sarcomas over 25 yrs
30
Mean size (cms)
25
20
Soft Tissue Sarcoma
95% CI
15
Bone sarcoma
95% CI
10
5
0
5
19 8
7
19 8
9
19 8
1
19 9
3
19 9
5
19 9
7
19 9
9
19 9
1
20 0
3
20 0
5
20 0
7
20 0
9
20 0
Bone sarcomas - no change, with time
STS – slightly improved – from 10.3cm in 1990s to 9.6cm since 2000
Duration of symptoms
• Bone sarcomas – median16 weeks
• STS – median 26 weeks
200
175
Weeks
150
125
Bone sarcoma
100
STS
75
Bone
STS
50
25
0
DuratSymp
Median duration of symptoms
70
Median duration (weeks)
60
50
Soft Tissue Sarcoma
40
IQR
Bone sarcoma
IQR
30
20
10
0
5
19 8
7
19 8
9
19 8
1
19 9
3
19 9
5
19 9
7
19 9
9
19 9
1
20 0
3
20 0
5
20 0
7
20 0
9
20 0
• Claimed duration of symptoms is INCREASING
for bone sarcomas
• Increased from 16 to 20 weeks since 2000
• STS symptom duration unchanged (26 weeks)
Duration of symptoms by diagnosis
Bone sarcomas
STS
Osteosarcoma
12 weeks
Liposarcoma
28 weeks
Chondrosarcoma
52 weeks
Leiomyosarcoma
26 weeks
Ewings
20 weeks
MFH
20 weeks
Spindle cell sarcoma
20 weeks
Synovial sarcoma
52 weeks
Chordoma
104 weeks
MPNST
26 weeks
Other factors – sex
• Symptom duration
– no difference between males and females
for bone or STS
• Size
– Women present earlier
– STS – ♂ 10.1cm vs ♀ 9.5cm (p=0.04)
– Bone - ♂ 10.5cm vs ♀ 9.8cm (p=0.008)
Does age affect it?
Age group
< 10
10-19 20-29 30-39 40-49
50-59
60-69
70-79
80+
Size (cms)
Soft Tissue Sarcomas
4.2
7.4
8.2
8.9
10.0
10.5
10.3
10.3
11.1
10.5
10.1
10.1
9.6
11.6
10.6
11.9
11.6
8
16
26
26
26
26
26
20
26
7
12
20
26
36
36
32
28
26
Bone Sarcomas (cms) 9.8
Symptom duration
(weeks - median)
Soft Tissue Sarcomas
Bone Sarcomas
Older patients have bigger tumours and somewhat longer duration of Sx
Does site matter?
• STS
– Superficial tumours = 6cm
– Deep tumours = 11.2cm
Whoops !
• 25% of patients had had a previous inadvertent excision,
mean size 5.8cm
• Slight decrease in incidence with time, but size
unchanged
55
50
40
35
30
25
20
15
10
5
0
-5
5
19 8
7
19 8
9
19 8
1
19 9
3
19 9
5
19 9
previous inadvertent excision
7
19 9
9
19 9
Size
1
20 0
95% CI
3
20 0
5
20 0
7
20 0
9
20 0
Mean size (cm)
% prev.inadvertent excision
45
More whoops
• 55% of all superficial STS had a previous
„whoops‟, (338 of 615)
• Mean size was 5cm compared to 7.0cm
for those treated primarily
• 15% of all deep STS had a previous
„whoops‟ (262 of 1751)
• Mean size was 7cm compared to 12cm for
those treated primarily
Why are they missed??
Painful shoulder 4 months – 35 yr old
6
weeks
later
Low level of awareness ?
Bigger is NOT better !
All these countries have better
outcomes (STS)
Why?
• Scandinavia
• Italy
• Australia
•UK
7cm
6cm
7cm
10cm
Aren‟t we aren‟t bothered about lumps and bumps?
Are our doctors not worried?
We need a new strategy........ (all ideas welcome!)
Does earlier diagnosis = better outcomes?
• Patients are happier with less delay
• Easier to treat
• Less risk of metastases at diagnosis
30
25
20
15
Bone
STS
10
5
0
<5cm
6 to 10cm
11 to 15cm
16-20 cm
>20cm
and better survival....
All sarcomas survival by size
<5cm
1
Cum. Survival (under 5
Event Times (under 5)
<10cm
Cum. Survival (five to
Cum. Survival
.8
Event Times (five to ten)
.6
Cum. Survival (ekeven
<15cm
Event Times (ekeven to
.4
Cum.cm
Survival (sixteen
<20
Event Times (sixteen to
.2
Cum. Survival (over 20
>20cm
Event Times (over 20)
0
0
20
40
60
Time in months
80
100
Conclusion
• Bone and soft tissue sarcomas are
diagnosed late
• We‟re not getting better
• None of the UK guidance (2000 and
2005) on early diagnosis or the 2006
Improving Outcomes Guidance or the
introduction of 2 week waits – has made
any obvious difference.
Prof Sir Mike Richards..
“We know the main reason our survival rates
lag behind other countries is because too
many people are diagnosed late. This is
why our Strategy focuses on earlier
diagnosis which we will achieve through
raising the public‟s awareness of the signs
and symptoms of cancer and also
providing better access to diagnostic
tests.” (Improving outcomes – a strategy for cancer, 2011)
.... but will it happen for sarcomas????