Table 2.
Sarcoma crude and age-standardized incidence rate per 100,000 person-years according to topographic, genomic and histological groups by sex. FRANCIM network data 2010–2013 (19 registries)
| Median Age | Male | Female | Overall | ||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| CIR | ASR-W (segi) | ASR-E | ASR-US | CIR | ASR-W (segi) | ASR-E | ASR-US | CIR | ASR-W (segi) | ASR-E | ASR-US | ||
| Sarcomas by topographic groups | |||||||||||||
| Soft tissue | 65 | 3.70 | 2.43 | 3.12 | 3.31 | 2.90 | 1.91 | 2.34 | 2.38 | 3.30 | 2.15 | 2.68 | 2.78 |
| Bone | 47 | 1.20 | 1.09 | 1.16 | 1.17 | 0.90 | 0.85 | 0.87 | 0.88 | 1.10 | 0.96 | 1.01 | 1.01 |
| Skin | 54 | 1.00 | 0.70 | 0.88 | 0.91 | 0.60 | 0.46 | 0.54 | 0.54 | 0.80 | 0.59 | 0.72 | 0.73 |
| Viscera | |||||||||||||
| Gastro-intestinal organs | 69 | 1.10 | 0.65 | 0.91 | 0.95 | 1.00 | 0.57 | 0.77 | 0.79 | 1.10 | 0.58 | 0.81 | 0.84 |
| Female genital organs | 62 | – | – | – | – | 1.00 | 0.62 | 0.82 | 0.81 | 1.00 | 0.62 | 0.82 | 0.81 |
| Other visceral organs | 65 | 0.40 | 0.26 | 0.33 | 0.33 | 0.50 | 0.28 | 0.36 | 0.37 | 0.40 | 0.26 | 0.34 | 0.35 |
| Others anatomic sites | 55 | 0.10 | 0.15 | 0.13 | 0.13 | 0.10 | 0.10 | 0.10 | 0.10 | 0.10 | 0.10 | 0.10 | 0.10 |
| Sarcomas by genomic groups | |||||||||||||
| Complex genomic alterations | 65 | 2.80 | 1.78 | 2.30 | 2.43 | 3.10 | 1.93 | 2.44 | 2.47 | 2.90 | 1.87 | 2.38 | 2.45 |
| MDM2 amplification | 68 | 0.50 | 0.30 | 0.42 | 0.45 | 0.30 | 0.18 | 0.23 | 0.23 | 0.40 | 0.23 | 0.31 | 0.33 |
| Mutations | 68 | 1.10 | 0.66 | 0.87 | 0.90 | 1.00 | 0.58 | 0.76 | 0.77 | 1.00 | 0.62 | 0.80 | 0.83 |
| Recurrent translocations | 46 | 1.30 | 1.23 | 1.30 | 1.30 | 1.60 | 1.44 | 1.56 | 1.54 | 1.50 | 1.34 | 1.44 | 1.43 |
| Undefined/Miscellaneous alterations | 65 | 1.90 | 1.27 | 1.62 | 1.70 | 1.20 | 0.73 | 0.90 | 0.94 | 1.60 | 0.97 | 1.22 | 1.26 |
| Sarcomas by histologic groups | |||||||||||||
| Unclassified sarcomaa | 69 | 1.30 | 0.71 | 1.00 | 1.08 | 1.10 | 0.62 | 0.82 | 0.85 | 1.20 | 0.65 | 0.90 | 0.94 |
| Leiomyosarcoma | 66 | 0.80 | 0.43 | 0.62 | 0.68 | 1.30 | 0.74 | 0.99 | 0.99 | 1.00 | 0.58 | 0.79 | 0.81 |
| GIST | 69 | 0.90 | 0.52 | 0.75 | 0.78 | 0.90 | 0.47 | 0.65 | 0.67 | 0.90 | 0.50 | 0.70 | 0.72 |
| Liposarcoma | 67 | 0.90 | 0.48 | 0.68 | 0.72 | 0.50 | 0.28 | 0.37 | 0.37 | 0.70 | 0.38 | 0.51 | 0.53 |
| Dedifferentiated liposarcoma | 69 | 0.50 | 0.24 | 0.35 | 0.38 | 0.30 | 0.11 | 0.16 | 0.16 | 0.40 | 0.17 | 0.25 | 0.26 |
| Round cell \ Myxoid liposarcoma | 51 | 0.20 | 0.13 | 0.16 | 0.16 | 0.10 | 0.09 | 0.11 | 0.11 | 0.10 | 0.10 | 0.12 | 0.12 |
| Pleomorphic liposarcoma | 73 | 0.10 | 0.03 | 0.04 | 0.05 | 0.00 | 0.00 | 0.00 | 0.00 | 0.00 | 0.01 | 0.02 | 0.02 |
| Liposarcoma, NOS | 67 | 0.20 | 0.09 | 0.13 | 0.14 | 0.10 | 0.04 | 0.05 | 0.06 | 0.10 | 0.05 | 0.08 | 0.08 |
| Chondrosarcoma | 55 | 0.50 | 0.32 | 0.41 | 0.40 | 0.40 | 0.30 | 0.36 | 0.36 | 0.50 | 0.34 | 0.41 | 0.41 |
| Dermatofibrosarcoma | 44 | 0.40 | 0.31 | 0.35 | 0.35 | 0.50 | 0.40 | 0.45 | 0.44 | 0.40 | 0.37 | 0.42 | 0.41 |
| Kaposi sarcoma | 63 | 0.60 | 0.40 | 0.52 | 0.54 | 0.10 | 0.04 | 0.06 | 0.06 | 0.30 | 0.20 | 0.27 | 0.28 |
| Angiosarcoma | 73 | 0.20 | 0.10 | 0.15 | 0.16 | 0.40 | 0.17 | 0.25 | 0.27 | 0.30 | 0.16 | 0.23 | 0.25 |
| Osteosarcoma | 34 | 0.30 | 0.34 | 0.33 | 0.33 | 0.30 | 0.25 | 0.24 | 0.25 | 0.30 | 0.28 | 0.27 | 0.27 |
| Ewing sarcoma | 19 | 0.30 | 0.35 | 0.30 | 0.30 | 0.20 | 0.32 | 0.27 | 0.27 | 0.30 | 0.33 | 0.28 | 0.28 |
| Myxofibrosarcoma | 66 | 0.30 | 0.16 | 0.22 | 0.22 | 0.20 | 0.10 | 0.14 | 0.14 | 0.20 | 0.14 | 0.19 | 0.19 |
| Rhabdomyosarcoma | 25 | 0.30 | 0.26 | 0.24 | 0.25 | 0.20 | 0.21 | 0.18 | 0.18 | 0.20 | 0.25 | 0.22 | 0.23 |
| Embryonal rhabdomyosarcoma | 12 | 0.10 | 0.15 | 0.11 | 0.11 | 0.10 | 0.09 | 0.06 | 0.06 | 0.10 | 0.11 | 0.08 | 0.08 |
| Alveolar rhabdomyosarcoma | 22 | 0.00 | 0.05 | 0.04 | 0.04 | 0.00 | 0.08 | 0.06 | 0.06 | 0.00 | 0.05 | 0.04 | 0.04 |
| Pleomorphic rhabdomyosarcoma | 69 | 0.10 | 0.02 | 0.03 | 0.03 | 0.00 | 0.01 | 0.01 | 0.01 | 0.00 | 0.01 | 0.02 | 0.02 |
| Spindle cell rhabdomyosarcoma | 40 | 0.00 | 0.00 | 0.00 | 0.01 | 0.00 | 0.03 | 0.02 | 0.02 | 0.00 | 0.00 | 0.00 | 0.00 |
| Rhabdomyosarcoma NOS | 64 | 0.00 | 0.01 | 0.01 | 0.01 | 0.00 | 0.01 | 0.02 | 0.02 | 0.00 | 0.01 | 0.02 | 0.02 |
| Nerve Sheath Tumors | 55 | 0.10 | 0.10 | 0.13 | 0.13 | 0.20 | 0.12 | 0.15 | 0.15 | 0.20 | 0.12 | 0.15 | 0.15 |
| Endometrial stromal sarcoma | 62 | – | – | – | – | 0.30 | 0.17 | 0.23 | 0.23 | 0.30 | 0.17 | 0.23 | 0.23 |
| Synovial sarcoma | 47 | 0.10 | 0.16 | 0.17 | 0.17 | 0.10 | 0.13 | 0.15 | 0.14 | 0.10 | 0.15 | 0.16 | 0.16 |
| Spindle cell synovial sarcoma | 49 | 0.1 | 0.07 | 0.05 | 0.05 | 0.10 | 0.05 | 0.06 | 0.06 | 0.10 | 0.05 | 0.07 | 0.07 |
| Biphasic synovial sarcoma | 44 | 0.0 | 0.00 | 0.00 | 0.00 | 0.00 | 0.02 | 0.01 | 0.01 | 0.00 | 0.00 | 0.00 | 0.00 |
| Synovial sarcoma NOS | 44 | 0.1 | 0.06 | 0.04 | 0.06 | 0.00 | 0.05 | 0.05 | 0.05 | 0.10 | 0.05 | 0.05 | 0.05 |
| Chordoma | 61 | 0.20 | 0.11 | 0.14 | 0.15 | 0.10 | 0.06 | 0.08 | 0.08 | 0.10 | 0.09 | 0.12 | 0.12 |
| Solitary fibrous tumor. Malignant | 63 | 0.10 | 0.11 | 0.14 | 0.13 | 0.10 | 0.08 | 0.10 | 0.10 | 0.10 | 0.10 | 0.12 | 0.12 |
| Fibrosarcoma | 60 | 0.10 | 0.03 | 0.05 | 0.05 | 0.10 | 0.03 | 0.04 | 0.04 | 0.10 | 0.03 | 0.04 | 0.04 |
| Malignant myoepithelioma | 56 | 0.00 | 0.03 | 0.04 | 0.04 | 0.00 | 0.03 | 0.04 | 0.04 | 0.00 | 0.03 | 0.04 | 0.04 |
| Epithelioid haemangioendothelioma | 47 | 0.00 | 0.03 | 0.03 | 0.03 | 0.00 | 0.05 | 0.05 | 0.05 | 0.00 | 0.05 | 0.06 | 0.06 |
| Other (with fewer than 20 cases) | 46 | 0.20 | 0.19 | 0.17 | 0.18 | 0.20 | 0.19 | 0.20 | 0.20 | 0.20 | 0.20 | 0.20 | 0.20 |
| Total | 63 | 7.60 | 5.27 | 6.54 | 6.80 | 7.20 | 4.81 | 5.83 | 5.90 | 7.40 | 5.00 | 6.12 | 6.26 |
Abreviations: GIST Gastro-Intestinal Stromal Tumors, CIR Crude Incidence Rate per 100,000 persons-years, ASR-W, ASR-E and ASR-US Age-Standardized incidence Rate from three reference populations (W, World Segi; E, European; US, United-States)
aUnclassified sarcomas include: Sarcoma not otherwise specified (ICDO-88003), undifferentiated spindle cell sarcoma (ICDO-88013), undifferentiated pleomorphic sarcoma (ICDO-88023), undifferentiated round cell sarcoma (ICDO-88033), epithelioid sarcoma (ICDO-88043), undifferentiated sarcoma NOS (ICDO-88053)