Connective tissue (sarcomas)

Rare cancer of connective tissue (sarcomas)

Domain 1

 

Definition

Sarcoma is a group of very rare disease entities with heterogenous natural history. With a crude incidence of approximately 6 per 100,000 per year in the EU, including dozens of different subtypes according to the WHO classification, they can be mainly divided into: adult soft tissue sarcomas (STS), gastrointestinal stromal tumours (GIST), bone sarcomas and pediatric rhabdomyosarcomas.

Diagnosis

Due to their rarity and pathologic heterogeneity, sarcomas represent a challenging area of diagnostic pathology, with a proportion of diagnostic inaccuracy reported in the range of 30-40% in the community, i.e. outside reference centres. Centralization of pathological diagnosis is therefore crucial.

Localisation

Sarcomas can arise anywhere in the human body, limbs and limb-girdles being the most common primary sites, but all other sites being potentially affected, including viscera. This anatomical variety is another challenge for quality of care. In particular, different surgical oncologists may be involved in their primary treatment. A non-compliance to standard practices at an initial stage of disease may have a prognostic impact.

Recomendations

High-quality care is provided best in reference centers. Collaborative health networks around these reference centers offer the best strategy to improve chances of cure in these cancers while limiting health migration. Reference centres are also crucial for clinical and translational research, tissue banking and medical education.

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