estimating the population-based incidence for sarcoma and gist in three european regions
DESCRIPTION
Estimating the population-based incidence for sarcoma and GIST in three European regions. G. Mastrangelo University of Padova. Background. The challenges of sarcomas: sarcomas are rare tumors, but include more than 50 different histological subtypes - PowerPoint PPT PresentationTRANSCRIPT
0.01
0.1
1
10
0 1 2 3 4 5 6 7 8 9 10
Estimating the population-based incidence for sarcoma and GIST in
three European regions
G. MastrangeloUniversity of Padova
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
SubjectsCases are subjects:
• of both gender, any age, resident and treated in the same region (Veneto, Rhone-Alpes, or Aquitaine)
• with histologically proven malignant sarcoma diagnosed as first cancer in one year of observation
Data for estimating the rates
VenetoRhone-Alpes
Aquitaine
Cases 2007 2005 2007
Population 2007 2005 2005
Main characteristics of cases
VenetoRhone-Alpes
Aquitaine
Overall number 300 342 170
With expert review (%) 58% 94% 100%
Tumor resection (%) 86% ? 77%
Women (%) 45% 50% 48%
Age (mean SD) 6217 6118 6020
Sarcomas and GIST in three European regions
0 0.2 0.4 0.6 0.8 1 1.2 1.4
GISTLipo_s
Sarcoma NOSLeiomyo_s
Kaposi_sDermatofibro_s
Uterine leiomyo_sOtherMFH
Myxofibro_sRhabdomyo_s
Synovial_sEwing_sAngio_s
Fibromyxoid_sMPNSTFibro_s
Solitary_Osteo_s
Rate per 100,000
All sarcomas: 813 cases, 100%, 5.9 cases per 100,000
Veneto300 cases Aquitaine
170 cases
Rhone/Alpes342 cases
10-1916 cases
0-96 cases
20-2929 cases
30-3964 cases
40-49103 cases
50-59125 cases
60-69168 cases
70-79181 cases
80-89111 cases
90+10 cases
0
1
2
3
4
5
6
7
8
1 2 3 4 5 6 7 8 9 10
Rat
e p
er 1
00,0
00
0.1
1
10
100
Rat
e p
er 1
00,0
00 (
log
sca
le)
Soft tissue sarcomas: 485 cases, 59.6%, 3.5 per 100,000
Aquitaine100 cases
Rhone/Alpes214 cases
Veneto171 cases
90+7 cases
80-8969 cases
70-79103 cases
60-6996 cases
50-5967 cases
40-4962 cases30-39
42 cases
20-2922 cases
10-1913 cases
0-94 cases
0
1
2
3
4
5
6
0 1 2 3 4 5 6 7 8 9
Rat
e p
er 1
00,0
00
0.1
1
10
100
Rat
e p
er 1
00,0
00 (
log
sca
le)
Visceral sarcomas: 329 cases, 40.5%, 2,4 cases per 100,000
Aquitaine70 cases
Rhone/Alpes128 cases
Veneto129 cases
90 +3 cases
80-8942 cases70-79
78 cases60-6972 cases
50-5958 cases
40-4941 cases
30-3922 cases
20-297 cases
10-193 cases
0-92 cases
0
0.5
1
1.5
2
2.5
3
3.5
0 1 2 3 4 5 6 7 8 9
Rat
e p
er 1
00,0
00
0.1
1
10
Rat
e p
er 1
00,0
00 (
log
sca
le)
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
Aquitaine 6 case
Rhone/Alpes6 cases
Veneto6 cases
80-891 case
70-79 1 case
60-691 case
50-592 cases
30-301 case
20-294 cases10-19
4 cases
0-94 cases
0
0.05
0.1
0.15
0.2
0.25
0.3
0.35
0.4
0 1 2 3 4 5 6 7 8 9
Rat
e p
er 1
00,0
00
0.01
0.1
1
Rat
e p
er 1
00,0
00 (
log
sca
le)
Ewing sarcoma / PNET: 13 cases, 1.6%,0.09 per 100,000
Aquitaine4 cases
Rhone/Alpes6 cases
Veneto3 case
80-891 case
60-692 cases
50-592 cases
40-491 case
30-391 case
20-292 cases
10-193 cases
0-91 case
0.00
0.05
0.10
0.15
0.20
0.25
0 1 2 3 4 5 6 7 8 9
Rat
e p
er 1
00,0
00
0.01
0.10
1.00
Rat
e p
er 1
00,0
00 (
log
sca
le)
Synovial sarcoma, 14 cases, 1.7%, 0.10 cases per 100,000
Aquitaine3 cases
Rhone/Alpes8 cases
Veneto3 cases
70-791 case
60-691 case
50-592 cases
40-491 case
30-393 cases
20-293 cases
10-193 cases
0
0.02
0.04
0.06
0.08
0.1
0.12
0.14
0.16
0.18
0.2
0 1 2 3 4 5 6 7 8 9
Rat
e p
er 1
00,0
00
0.01
0.1
1
Rat
e p
er 1
00,0
00 (
log
sca
le)
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
Aquitaine8 case
Rhone/Alpes21 cases
Veneto20 cases
90+1 case
80-891 case
70-791 case
60-695 cases
50-594 cases
40-4912 cases
30-3916 cases
20-297 cases
10-191 case
0-9 1 case
0
0.05
0.1
0.15
0.2
0.25
0.3
0.35
0.4
0.45
0.5
1 2 3 4 5 6 7 8 9 10
Rat
e p
er 1
00,0
00
0.01
0.10
1.00
Rat
e p
er 1
00,0
00 (
log
sca
le)
Liposarcoma, 131 cases, 16.1%, 0.95 cases per 100,000
Aquitaine24 cases
Rhone/Alpes59 cases
Veneto48 cases 80-89
13 cases
70-7925 cases
60-6928 cases
50-5934 cases
40-4920 cases
30-398 cases
20-291 case
10-192 cases
0
0.2
0.4
0.6
0.8
1
1.2
1.4
0 1 2 3 4 5 6 7 8 9
Rat
e p
er 1
00,0
00
0.01
0.1
1
10
Rat
e p
er 1
00,0
00 (
log
sca
le)
Leiomyosarcoma: 81 cases, 10%, 0.59 cases per 100,000
Aquitaine22 cases
Rhone/Alpes29 cases
Veneto30 cases
90+1 case
80-8911 cases
70-7929 cases
60-6919 cases
50-5910 cases
40-498 cases
30-392 case
20-291 case
10-192 cases
0
0.1
0.2
0.3
0.4
0.5
0.6
0.7
0.8
0 1 2 3 4 5 6 7 8 9
Rat
e p
er 1
00,0
00
0.01
0.1
1
10
Rat
e p
er 1
00,0
00 (
log
sca
le)
Sarcomas consistently more frequent in men:• Kaposi sarcoma• Liposarcoma• Dermatofibrosarcoma protuberans• Myxofibrosarcoma
Kaposi sarcoma, 57 cases, 7.0%, 0.41 cases per 100,000
Aquitaine6 cases
Rhone/Alpes16 cases
Veneto35 cases
90+1 case
80-8914 cases
70-7912 cases
60-6917 cases
50-592 cases
40-495 cases
30-395 cases
20-291 case
0
0.2
0.4
0.6
0.8
1
1.2
1.4
1.6
0 1 2 3 4 5 6 7 8 9
Rat
e p
er 1
00,0
00
0.01
0.1
1
10
Rat
e p
er 1
00,0
00 (
log
sca
le)
Liposarcoma, 131 cases, 16.1%, 0.95 cases per 100,000
Aquitaine24 cases
Rhone/Alpes59 cases
Veneto48 cases 80-89
13 cases
70-7925 cases
60-6928 cases
50-5934 cases
40-4920 cases
30-398 cases
20-291 case
10-192 cases
0
0.2
0.4
0.6
0.8
1
1.2
1.4
0 1 2 3 4 5 6 7 8 9
Rat
e p
er 1
00,0
00
0.01
0.1
1
10
Rat
e p
er 1
00,0
00 (
log
sca
le)
Dermatofibrosarcoma protuberans: 49 cases, 6.0%, 0.35 cases per 100,000
Aquitaine8 case
Rhone/Alpes21 cases
Veneto20 cases
90+1 case
80-891 case
70-791 case
60-695 cases
50-594 cases
40-4912 cases
30-3916 cases
20-297 cases
10-191 case
0-9 1 case
0
0.05
0.1
0.15
0.2
0.25
0.3
0.35
0.4
0.45
0.5
1 2 3 4 5 6 7 8 9 10
Rat
e p
er 1
00,0
00
0.01
0.10
1.00
Rat
e p
er 1
00,0
00 (
log
sca
le)
Myxofibrosarcoma: 21 cases, 2.6%, 0.15 cases per 100,000
Aquitaine6 cases
Rhone/Alpes9 cases
Veneto6 cases
90+2 cases
80-893 cases
70-797 cases
60-694 cases
50-591 case
40-494 cases
0
0.05
0.1
0.15
0.2
0.25
0 1 2 3 4 5 6 7 8 9
Rat
e p
er 1
00,0
00
0.01
0.1
1
10
Rat
e p
er 1
00,0
00 (
log
sca
le)
Sarcomas consistently more frequent in women:
Uterine leiomyosarcoma and angiosarcoma
Uterine leiomyosarcoma: 43 cases, 5.3%, 0.31 cases per 100,000
Aquitaine10 cases
Rhone/Alpes16 cases
Veneto17 cases
90+1 case
80-893 cases
70-796 cases
60-696 cases
50-596 cases
40-4919 cases
30-391 case
20-291 case
0
0.05
0.1
0.15
0.2
0.25
0.3
0.35
0.4
0 1 2 3 4 5 6 7 8 9
Rat
e p
er 1
00,0
00
0.01
0.1
1
Rat
e p
er 1
00,0
00 (
log
sca
le)
Angiosarcoma: 27 cases, 3.3%0.07 cases per 100,000
Aquitaine5 cases
Rhone/Alpes 11 cases
Veneto11 cases 80-89
10 cases
70-707 cases
60-694 cases
50-592 cases
40-492 cases
30-391 case
20-291 case
0
0.05
0.1
0.15
0.2
0.25
0.3
0.35
1 2 3 4 5 6 7 8 9 10
Rat
es p
er 1
00,0
00
0.01
0.10
1.00
10.00
Rat
es p
er 1
00,0
00 (
log
sca
le)
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
Aquitaine45 cases
Rhone/Alpes68 cases
Veneto62 cases
90+2 cases
80-8927 cases
70-7949 cases
60-6943 cases
50-5931 cases
40-4912 cases
30-3911 cases
0
0.2
0.4
0.6
0.8
1
1.2
1.4
1.6
1.8
0 1 2 3 4 5 6 7 8 9
Rat
e p
er 1
00,0
00
0.1
1
10
Rat
e p
er 1
00,0
00 (
log
sca
le)
Leiomyosarcoma: 81 cases, 10%, 0.59 cases per 100,000
Aquitaine22 cases
Rhone/Alpes29 cases
Veneto30 cases
90+1 case
80-8911 cases
70-7929 cases
60-6919 cases
50-5910 cases
40-498 cases
30-392 case
20-291 case
10-192 cases
0
0.1
0.2
0.3
0.4
0.5
0.6
0.7
0.8
0 1 2 3 4 5 6 7 8 9
Rat
e p
er 1
00,0
00
0.01
0.1
1
10
Rat
e p
er 1
00,0
00 (
log
sca
le)
Sarcoma NOS, 92 cases, 11.3%, 0.67 cases per 100,000
Aquitaine16 cases
Rhone/Alpes55 cases
Veneto21 cases
90+1 case
80-8912 cases
70-7921 cases
60-6921 cases
50-5920 cases
40-497 cases
30-396 cases
20-294 cases
0
0.2
0.4
0.6
0.8
1
1.2
1.4
0 1 2 3 4 5 6 7 8 9
Rat
e p
er 1
00,0
00
0.1
1
10
Rat
e p
er 1
00,0
00 (
log
sca
le)
Pleomorphic sarcoma (MFH), 22 cases, 2.7%,0.16 cases per 100,000
Aquitaine8 cases
Rhone/Alpesno cases
Veneto14 cases
90+1 case
80-897 cases
70-7911 cases
60-692 cases
40-491 case
0
0.05
0.1
0.15
0.2
0.25
0.3
0.35
0.4
0 1 2 3 4 5 6 7 8 9
Rat
e p
er 1
00,0
00
0.01
0.1
1
10
Rat
e p
er 1
00,0
00 (
log
sca
le)
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
Rhone/Alpes1 case
Veneto4 cases
Aquitaine2 cases
60-692
case
50-591 case
40-492 cases
30-392 cases
0
0.02
0.04
0.06
0.08
0.1
0.12
0.14
0.16
0 1 2 3 4 5 6 7 8 9
Rat
e p
er 1
00,0
00
0.01
0.1
1
Rat
e p
er 1
00,0
00 (
log
sca
le)
MPNST, 7 cases, 0.9%, 0.05 cases per 100,000
Aquitaine1 case
Rhone/Alpes4 cases
Veneto2 cases
60-692 cases
50-591 case
40-492 cases
30-392 cases
0
0.02
0.04
0.06
0.08
0.1
0.12
0 1 2 3 4 5 6 7 8 9
Rat
e p
er 1
00,0
00
0.01
0.1
1
Rat
e p
er 1
00,0
00 (
log
sca
le)
Solitary malignant fibrous tumor, 5 cases, 0.6%, 0.04 cases per 100,000
Aquitaineno cases
Rhone/Alpes4 cases
Veneto1 case
70-793 cases
60-691 case
30-391 case
0
0.01
0.02
0.03
0.04
0.05
0.06
0.07
0.08
0 1 2 3 4 5 6 7 8 9
Rat
e p
er 1
00,0
00
0.01
0.1
1
Eat
e p
er 1
00,0
00 (
log
sca
le)
Fibrosarcoma: 5 cases, 0.6%,0.04 per 100,000
Aquitaine1 case
Rhone/Alpes2 cases
Veneto3 cases
80-891 case
70-791 case
50-591 case
40-491 case
20-291 case
0
0.01
0.02
0.03
0.04
0.05
0.06
0.07
0.08
0.09
0 1 2 3 4 5 6 7 8 9
Rat
e p
er 1
00,0
00
0.01
0.1
1
Rat
e p
er 1
00,0
00 (
log
sca
le)
Osteosarcoma: 5 cases, 0.6%, 0.04 cases per 100,000
Aquitaine1 case
Rhone/Alpes3 cases
Veneto1 case
80-892 cases
60-692 cases
50-591 case
0
0.01
0.02
0.03
0.04
0.05
0.06
0.07
0.08
0 1 2 3 4 5 6 7 8 9
Rat
e p
er 1
00,0
00
0.01
0.1
1
Rat
e p
er 1
00,0
00 (
log
sca
le)
Other sarcomas, 26 cases (list in the next slide)
Aquitaine3 cases
Rhone/Alpes9 cases
Veneto14 cases
80-893 cases
70-792 cases
60-696 cases
50-593 cases
40-493 cases
30-394 cases
20-292 cases
10-193 cases
0
0.05
0.1
0.15
0.2
0.25
0.3
0.35
0.4
0.45
0.5
0 1 2 3 4 5 6 7 8 9
Rat
e p
er 1
00,0
00
0.1
1
Rat
e p
er 1
00,0
00 (
log
sca
le)
Other sarcomas (with less than 5 cases)• 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
