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Kaposi's sarcoma

Orpha number ORPHA33276
Synonym(s) -
Prevalence 1-9 / 100 000
Inheritance
  • Sporadic
Age of onset Adulthood
ICD-10
  • C46.0
  • C46.1
  • C46.2
  • C46.3
  • C46.7
  • C46.8
  • C46.9
OMIM
UMLS
  • C0036220
MeSH
  • D012514
MedDRA
  • 10023284
SNOMED CT
  • 109385007
  • 49937004

Summary

Kaposi's sarcoma is a malignant endothelial tumour. It is the most frequent cancer developing in association with human immunodeficiency virus (HIV). It is rare in the general population, but the prevalence of the classic form (i.e. without association with AIDS) is higher in certain regions such as Sicily and Sardinia (1 in 30 000 men, most frequently elderly males). A more aggressive, childhood form of Kaposi's sarcoma is endemic in Africa. The disease occurs in 0.04% of patients who have undergone a renal transplant. The clinical picture is highly variable, ranging from mucocutaneous forms to multiorgan involvement. The classic form is often restricted to the skin and mucosa, but predisposes affected individuals to other malignant pathologies. The form associated with AIDS can be severe, but sometimes responds to aggressive anti-retroviral therapy.


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Detailed information

Summary information
Review article
  • EN (2009)
Clinical practice guidelines
  • DE (2011)
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