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Estimating Sarcoma and GIST Incidence in European Regions

This study aims to estimate the population-based incidence of sarcoma and GIST in three European regions in 2007-2008, using the 2002 WHO classification. It also seeks to analyze survival rates from 2007 to 2011. Pathology laboratories collaborated, and cases were reviewed based on inclusion criteria. Various sarcoma subtypes and patterns in different genders are explored.

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Estimating Sarcoma and GIST Incidence in 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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