Transcript The challenges of sarcomas

European
regions
Estimating the population-based
incidence for sarcoma and GIST in
three European regions
G. Mastrangelo
University of Padova
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Background
The challenges of sarcomas:
– sarcomas are rare tumors, but include more than 50
different histological subtypes
– incidence of sarcomas is grossly underestimated since
the International Classification of Disease attributes
visceral sarcomas to the organ of origin.
– in late 2002, the World Health Organization (WHO)
published a new classification of soft tissue tumors
– studies have shown that in about 10% of cases there is
a discrepancy: one pathologist renders a diagnosis
and another pathologist looks at the same material and
renders a different opinion
Scope
To estimate:
• the population-based morphologic specific incidence
of sarcoma in Veneto, Rhone-Alpes, Aquitaine, in
2007-2008, using the 2002 WHO classification.
• the survival rate in the time window 2007 to 2011
Methods
• All the pathology laboratories were informed and agreed
to participate into the study
• A clinical monitor visited each laboratory to collect any
information on sarcoma cases (but in Rhone-Alpes
cases were prospectively notified by the pathologists
• The inclusion criteria were checked, the cases were
reviewed and some were excluded after the review
Subjects
Cases are subjects:
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of both gender, any age, resident and treated in the
same region (Veneto, Rhone-Alpes, or Aquitaine)
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with histologically proven malignant sarcoma diagnosed
as first cancer in one year of observation
Data for estimating the rates
Veneto
RhoneAlpes
Aquitaine
Cases
2007
2005
2007
Population
2007
2005
2005
Main characteristics of cases
Veneto
RhoneAlpes
Aquitaine
Overall number
300
342
170
With expert review (%)
58%
94%
100%
Tumor resection (%)
86%
?
77%
Women (%)
45%
50%
48%
6217
6118
6020
Age (mean  SD)
Sarcomas and GIST in three European regions
Osteo_s
Solitary_
Fibro_s
MPNST
Fibromyxoid_s
Angio_s
Ewing_s
Synovial_s
Rhabdomyo_s
Myxofibro_s
MFH
Other
Uterine leiomyo_s
Dermatofibro_s
Kaposi_s
Leiomyo_s
Sarcoma NOS
Lipo_s
GIST
0
0.2
0.4
0.6
0.8
Rate per 100,000
1
1.2
1.4
All sarcomas: 813 cases, 100%, 5.9 cases per 100,000
100
Rhone/Alpes
342 cases
7
Rate per 100,000
Veneto
6 300 cases
60-69
168 cases
Aquitaine
170 cases
40-49
103 cases
5
4
10-19
16 cases
3
2
80-89
111 cases
70-79
181 cases
10
90+
10 cases
50-59
125 cases
30-39
64 cases
20-29
29 cases
1
0-9
6 cases
Rate per 100,000 (log scale)
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0
0.1
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2
3
4
5
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Soft tissue sarcomas: 485 cases, 59.6%, 3.5 per 100,000
100
Rate per 100,000
Veneto
5 171 cases
Rhone/Alpes
214 cases
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3
Aquitaine
100 cases
20-29
22 cases
2
70-79
103 cases
30-39
42 cases
50-59
67 cases
60-69
96 cases
10
80-89
69 cases
90+
7 cases
40-49
62 cases
1
10-19
13 cases
1
0-9
4 cases
0
0.1
0
1
2
3
4
5
6
7
8
9
Rate per 100,000 (log scale)
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Visceral sarcomas: 329 cases, 40.5%, 2,4 cases per 100,000
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Veneto
129 cases
2.5
Rate per 100,000
50-59
58 cases
Rhone/Alpes
128 cases
Aquitaine
70 cases
2
60-69
72 cases
70-79
78 cases
40-49
41 cases
80-89
42 cases
90 +
3 cases
30-39
22 cases
1
1.5
20-29
7 cases
1
10-19
3 cases
0.5
0-9
2 cases
0
0
1
10
Rate per 100,000 (log scale)
3.5
0.1
2
3
4
5
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Sarcomas more frequent in children (0-19 years) with
sporadic cases in adults:
• Ewing sarcoma
• Rhabdomyosarcoma
• Synovial sarcoma
Rhabdomyosarcoma, 18 cases, 2.2%,
0.13 cases per 100,000
0.4
1
Rate per 100,000
0.3
0-9
4 cases
20-29
4 cases
10-19
4 cases
0.25
80-89
1 case
50-59
2 cases
0.2
0.15
0.1
Veneto
6 cases
Aquitaine
6 case
Rhone/Alpes
6 cases
60-69
1 case
30-30
1 case
0.1
70-79
1 case
Rate per 100,000 (log scale)
0.35
0.05
0
0.01
0
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2
3
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Ewing sarcoma / PNET: 13 cases, 1.6%,
0.09 per 100,000
1.00
Rate per 100,000
0.20
10-19
3 cases
0.15
50-59
2 cases
20-29
Rhone/Alpes 2 cases
0-9 6 cases
0.10 1 case
Veneto
0.05 3 case
30-39
1 case
80-89
1 case
60-69
2 cases
0.10
40-49
1 case
Aquitaine
4 cases
0.00
0.01
0
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2
3
4
5
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Rate per 100,000 (log scale)
0.25
Synovial sarcoma, 14 cases, 1.7%, 0.10 cases per 100,000
0.2
1
0.18
0.16
Rate per 100,000
0.14
10-19
3 cases
20-29
3 cases
0.12
0.1
Rhone/Alpes
8 cases
30-39
3 cases
70-79
1 case
0.08
0.06
0.04
50-59
2 cases
40-49
1 case
Veneto
3 cases
0.1
60-69
1 case
Rate per 100,000 (log scale)
Aquitaine
3 cases
0.02
0
0.01
0
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2
3
4
5
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Sarcomas with sporadic cases in children and more
frequent in adults
• Dermatofibrosarcoma protuberans
• Liposarcoma
• Leiomyosarcoma
Dermatofibrosarcoma protuberans: 49 cases, 6.0%,
0.35 cases per 100,000
0.5
1.00
90+
1 case
40-49
12 cases
20-29
30-39
7 cases 16 cases
60-69
5 cases
Rate per 100,000
0.4
Rhone/Alpes
21 cases
0.35
0.3
Aquitaine
8 case
0.25
0.2
0-9
1 case
0.15
80-89
1 case
50-59
4 cases
0.10
70-79
1 case
10-19
1 case
0.1
0.05
0
0.01
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2
3
4
5
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10
Rate per 100,000 (log scale)
Veneto
0.45 20 cases
Liposarcoma, 131 cases, 16.1%, 0.95 cases per 100,000
1.4
10
50-59
34 cases
Rate per 100,000
Veneto
1 48 cases
Aquitaine
24 cases
Rhone/Alpes
59 cases
0.8
70-79
25 cases
40-49
20 cases
30-39
8 cases
80-89
13 cases
60-69
28 cases
1
0.6
0.1
10-19
2 cases
0.4
20-29
1 case
Rate per 100,000 (log scale)
1.2
0.2
0
0.01
0
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2
3
4
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Leiomyosarcoma: 81 cases, 10%, 0.59 cases per 100,000
0.7
10
Veneto
30 cases
Aquitaine
22 cases
70-79
29 cases
80-89
11 cases
Rate per 100,000
0.6
0.5
Rhone/Alpes
29 cases
60-69
19 cases
1
50-59
10 cases
0.4
90+
1 case
40-49
8 cases
0.3
30-39
2 case
10-19
2 cases
0.1
0.2
20-29
1 case
Rate per 100,000 (log scale)
0.8
0.1
0
0.01
0
1
2
3
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Sarcomas consistently more frequent in men:
• Kaposi sarcoma
• Liposarcoma
• Dermatofibrosarcoma protuberans
• Myxofibrosarcoma
Kaposi sarcoma, 57 cases, 7.0%, 0.41 cases per 100,000
1.6
10
60-69
17 cases
Rate per 100,000
1.2
Veneto
35 cases
1
1
70-79
12 cases
30-39
5 cases
0.8
40-49
5 cases
Rhone/Alpes
16 cases
0.6
0.4
20-29
1 case
0.2
Aquitaine
6 cases
90+
1 case
50-59
2 cases
0.1
0
0.01
0
1
2
3
4
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Rate per 100,000 (log scale)
80-89
14 cases
1.4
Liposarcoma, 131 cases, 16.1%, 0.95 cases per 100,000
1.4
10
50-59
34 cases
Rate per 100,000
Veneto
1 48 cases
Aquitaine
24 cases
Rhone/Alpes
59 cases
0.8
70-79
25 cases
40-49
20 cases
30-39
8 cases
80-89
13 cases
60-69
28 cases
1
0.6
0.1
10-19
2 cases
0.4
20-29
1 case
Rate per 100,000 (log scale)
1.2
0.2
0
0.01
0
1
2
3
4
5
6
7
8
9
Dermatofibrosarcoma protuberans: 49 cases, 6.0%,
0.35 cases per 100,000
0.5
1.00
90+
1 case
40-49
12 cases
20-29
30-39
7 cases 16 cases
60-69
5 cases
Rate per 100,000
0.4
Rhone/Alpes
21 cases
0.35
0.3
Aquitaine
8 case
0.25
0.2
0-9
1 case
0.15
80-89
1 case
50-59
4 cases
0.10
70-79
1 case
10-19
1 case
0.1
0.05
0
0.01
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2
3
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10
Rate per 100,000 (log scale)
Veneto
0.45 20 cases
Myxofibrosarcoma: 21 cases, 2.6%, 0.15 cases per 100,000
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Aquitaine
6 cases
Rate per 100,000
0.2
90+
2 cases
70-79
7 cases
Rhone/Alpes
9 cases
0.15
40-49
4 cases
Veneto
0.1 6 cases
60-69
4 cases
1
80-89
3 cases
Rate per 100,000 (log scale)
0.25
0.1
50-59
1 case
0.05
0
0.01
0
1
2
3
4
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Sarcomas consistently more frequent in women:
Uterine leiomyosarcoma and angiosarcoma
Uterine leiomyosarcoma: 43 cases, 5.3%,
0.31 cases per 100,000
0.35
Veneto
17 cases
Rate per 100,000
0.3
50-59
6 cases
0.25
0.2
70-79
6 cases
40-49
19 cases
Aquitaine
10 cases
Rhone/Alpes
16 cases
1
60-69
6 cases
90+
1 case
80-89
3 cases
0.1
20-29
1 case
0.15
30-39
1 case
0.1
Rate per 100,000 (log scale)
0.4
0.05
0
0.01
0
1
2
3
4
5
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7
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Angiosarcoma: 27 cases, 3.3%
0.07 cases per 100,000
10.00
Veneto
0.3 11 cases
Rates per 100,000
0.25
80-89
10 cases
Rhone/Alpes
11 cases
Aquitaine
5 cases
0.2
1.00
60-69
4 cases
70-70
7 cases
40-49
2 cases
0.15
20-29
1 case
0.1
0.10
50-59
2 cases
30-39
1 case
0.05
0
0.01
1
2
3
4
5
6
7
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10
Rates per 100,000 (log scale)
0.35
Sarcomas with no consistent pattern in relation to gender:
• GIST
• Sarcoma NOS
• Leiomyosarcoma
• Pleomorphic sarcoma (MFH)
GIST: 175 cases, 21.5%, 1.3 cases per 100,000
1.8
10
Aquitaine
45 cases
Rate per 100,000
Veneto
1.4 62 cases
60-69
43 cases
1.2
1
80-89
27 cases
70-79
49 cases
50-59
31 cases
Rhone/Alpes
68 cases
0.8
90+
2 cases
1
40-49
12 cases
0.6
30-39
11 cases
0.4
0.2
0
0.1
0
1
2
3
4
5
6
7
8
9
Rate per 100,000 (log scale)
1.6
Leiomyosarcoma: 81 cases, 10%, 0.59 cases per 100,000
0.7
10
Veneto
30 cases
Aquitaine
22 cases
70-79
29 cases
80-89
11 cases
Rate per 100,000
0.6
0.5
Rhone/Alpes
29 cases
60-69
19 cases
1
50-59
10 cases
0.4
90+
1 case
40-49
8 cases
0.3
30-39
2 case
10-19
2 cases
0.1
0.2
20-29
1 case
Rate per 100,000 (log scale)
0.8
0.1
0
0.01
0
1
2
3
4
5
6
7
8
9
Sarcoma NOS, 92 cases, 11.3%, 0.67 cases per 100,000
1.4
10
Rate per 100,000
1
Rhone/Alpes
55 cases
0.8
0.6
60-69
21 cases
0.2
1
90+
1 case
40-49
7 cases
0.4
20-29
4 cases
70-79
21 cases
50-59
20 cases
Aquitaine
16 cases
Veneto
21 cases
80-89
12 cases
Rate per 100,000 (log scale)
1.2
30-39
6 cases
0
0.1
0
1
2
3
4
5
6
7
8
9
Pleomorphic sarcoma (MFH), 22 cases, 2.7%,
0.16 cases per 100,000
10
0.4
Rate per 100,000
0.3
Aquitaine
8 cases
0.25
90+
1 case
70-79
11 cases
1
80-89
7 cases
0.2
60-69
2 cases
0.15
0.1
40-49
1 case
0.1
0.05
Rhone/Alpes
no cases
0.01
0
0
1
2
3
4
5
6
7
8
9
Rate per 100,000 (log scale)
0.35
Veneto
14 cases
Sarcomas with few or very few cases:
• Fibromyxoid sarcoma
• MPNST
• Solitary malignant fibrous tumor
• Fibrosarcoma
• Other sarcomas
Fibromyxoid sarcoma: 7 case, 0.86%
0.05 per 100,000
1
0.16
Veneto
4 cases
Rate per 100,000
0.12
60-69
2
case
0.1
40-49
2 cases
0.08
30-39
2 cases
0.06
50-59
1 case
Rhone/Alpes
1 case
0.04
0.1
Rate per 100,000 (log scale)
0.14
Aquitaine
2 cases
0.02
0.01
0
0
1
2
3
4
5
6
7
8
9
MPNST, 7 cases, 0.9%, 0.05 cases per 100,000
0.12
1
Rate per 100,000
Veneto
2 cases
0.08
0.06
0.04
60-69
2 cases
40-49
2 cases
30-39
2 cases
Rhone/Alpes
4 cases
0.02
0.1
50-59
1 case
Rate per 100,000 (log scale)
0.1
Aquitaine
1 case
0
0.01
0
1
2
3
4
5
6
7
8
9
Solitary malignant fibrous tumor, 5 cases, 0.6%,
0.04 cases per 100,000
0.08
Rhone/Alpes
4 cases
Rate per 100,000
0.06
0.05
70-79
3 cases
Veneto
1 case
0.04
0.03
0.1
60-69
1 case
30-39
1 case
0.02
Eate per 100,000 (log scale)
0.07
1
Aquitaine
no cases
0.01
0
0.01
0
1
2
3
4
5
6
7
8
9
Fibrosarcoma: 5 cases, 0.6%,
0.04 per 100,000
0.09
Veneto
3 cases
Aquitaine
1 case
Rate per 100,000
0.07
80-89
1 case
0.06
0.05
0.04
Rhone/Alpes
2 cases
70-79
1 case
20-29
1 case
0.03
0.1
50-59
1 case
40-49
1 case
0.02
Rate per 100,000 (log scale)
0.08
1
0.01
0
0.01
0
1
2
3
4
5
6
7
8
9
Osteosarcoma: 5 cases, 0.6%, 0.04 cases per 100,000
0.07
Aquitaine
1 case
80-89
2 cases
Rate per 100,000
0.06
0.05
60-69
2 cases
Veneto
1 case
0.04
0.1
Rhone/Alpes
3 cases
50-59
1 case
0.03
0.02
Rate per 100,000 (log scale)
1
0.08
0.01
0.01
0
0
1
2
3
4
5
6
7
8
9
Other sarcomas, 26 cases
(list in the next slide)
0.5
1
Rate per 100,000
0.4
0.35
Veneto
14 cases
80-89
3 cases
60-69
6 cases
0.3
0.25
Rhone/Alpes
9 cases
0.2
0.15
10-19
3 cases
0.1
Aquitaine
3 cases
30-39
4 cases
20-29
2 cases
0.05
70-79
2 cases
50-59
3 cases
Rate per 100,000 (log scale)
0.45
40-49
3 cases
0
0.1
0
1
2
3
4
5
6
7
8
9
Other sarcomas
(with less than 5 cases)
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•
•
•
•
•
•
•
•
•
•
•
•
•
•
•
•
•
Clear cell sarcoma
Dedifferentiated aggressive angiomyxoma
Desmoplastic round cell tumor
Epithelioid haemangioendothelioma
Epithelioid sarcoma
Extrarenal rhabdoid tumour
Extraskeletal chondrosarcoma
Extraskeletal myxoid chondrosarcoma
Haemangiopericitoma
Inflammatory myofibroblastic tumor
Intimal sarcoma
Malignant epitelioid angiomyolipoma
Malignant myopericitoma
Melanocitic schwannoma
Myofibroblastic sarcoma
Myxoinflammatory fibroblastic sarcoma
Pecomas
Pleomorphic sarcoma with myogenic differentiation