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[Soft tissue tumors : Epidemiology, classification and staging].

L Fenzl1, M Mehrmann2, K Kremp2

  • 1Klinik für Diagnostische und Interventionelle Radiologie, Universitätsklinikum des Saarlandes, Kirrbergerstraße, 66424, Homburg/Saar, Deutschland. Lisa.Fenzl@uks.eu.

Der Radiologe
|October 28, 2017
PubMed
Summary

The 2013 World Health Organization classification updates soft tissue tumor categories, impacting diagnosis and research. Key changes include new subgroups and redefined classifications for better understanding of these rare tumors.

Keywords:
Adipocytic tumorsDignitySarcomaSurgical stagingWHO classification

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Area of Science:

  • Oncology
  • Pathology
  • Genetics

Background:

  • Soft tissue tumors are histologically classified as benign, intermediate, or malignant.
  • The 2013 World Health Organization (WHO) classification introduced significant changes to soft tissue tumor categorization.
  • These updates reflect advances in the genetics and cell biology of soft tissue tumors.

Purpose of the Study:

  • To outline the key changes in the 2013 WHO classification of soft tissue tumors.
  • To highlight the impact of new classifications on understanding tumor subtypes and their origins.
  • To provide an overview of the epidemiological distribution of soft tissue tumors in adults and children.

Main Methods:

  • Review of the 2013 World Health Organization (WHO) classification of soft tissue tumors.
  • Analysis of changes in tumor allocation, including the formation of new subgroups like undifferentiated sarcomas.
  • Comparison of adult and pediatric soft tissue tumor demographics and common types.

Main Results:

  • Undifferentiated sarcomas are now a distinct subgroup, separate from fibrohistiocytic tumors, with malignant fibrous histiocytoma being replaced.
  • Nerve sheath tumors and gastrointestinal stromal tumors are newly incorporated subgroups.
  • Malignant soft tissue tumors constitute 1% of adult malignancies, with adipocytic tumors (e.g., liposarcoma) being most common. In children, they represent 15% of malignancies, with rhabdomyosarcoma being most frequent.

Conclusions:

  • The 2013 WHO classification refines the understanding and diagnosis of soft tissue tumors.
  • Updated classifications facilitate research into the genetic and cellular basis of these tumors.
  • Recognizing distinct tumor types and their prevalence is crucial for effective clinical management and research.