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Estimating the population-based incidence for sarcoma and GIST in three European regions

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

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Estimating the population-based incidence for sarcoma and GIST in three European regions

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  1. Estimating the population-based incidence for sarcoma and GIST in three European regions G. Mastrangelo University of Padova

  2. 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

  3. 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

  4. 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

  5. Subjects • Cases 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

  6. Main characteristics of cases

  7. Sarcomas more frequent in children (0-19 years) with sporadic cases in adults: • Ewing sarcoma • Rhabdomyosarcoma • Synovial sarcoma

  8. Sarcomas with sporadic cases in children and more frequent in adults • Dermatofibrosarcoma protuberans • Liposarcoma • Leiomyosarcoma

  9. Sarcomas consistently more frequent in men: • Kaposi sarcoma • Liposarcoma • Dermatofibrosarcoma protuberans • Myxofibrosarcoma

  10. Sarcomas consistently more frequent in women: Uterine leiomyosarcoma and angiosarcoma

  11. Sarcomas with no consistent pattern in relation to gender: • GIST • Sarcoma NOS • Leiomyosarcoma • Pleomorphic sarcoma (MFH)

  12. Sarcomas with few or very few cases: • Fibromyxoid sarcoma • MPNST • Solitary malignant fibrous tumor • Fibrosarcoma • Other sarcomas

  13. 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

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