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Last reviewed: 16 Mar 2025
Last updated: 10 Jan 2025

Summary

Definition

History and exam

Key diagnostic factors

  • presence of risk factors
  • age >50 years
  • male sex
  • geographical location: central Africa or the Mediterranean
  • HIV infection
  • human herpesvirus-8 (HHV-8, also known as Kaposi's sarcoma-associated herpesvirus [KSHV]) infection
  • immunosuppression
  • skin lesion
  • oral lesion
  • lymphadenopathy
  • lymphoedema

Other diagnostic factors

  • weight loss
  • abdominal pain
  • gastrointestinal (GI) bleeding
  • diarrhoea
  • dyspnoea
  • haemoptysis
  • chest pain
  • fever

Risk factors

  • male sex
  • HIV infection
  • immunosuppressive therapy
  • human herpesvirus-8 (HHV-8, also known as Kaposi's sarcoma-associated herpesvirus [KSHV]) infection
  • central African ethnicity
  • drug abuse
  • Jewish ethnicity
  • Mediterranean ethnicity
  • age <5 years or >50 years
  • men who have sex with men
  • Black American ethnicity

Diagnostic investigations

1st investigations to order

  • HIV test
  • FBC and comprehensive metabolic panel
  • skin/mucosal biopsy

Investigations to consider

  • chest x-ray
  • chest CT and bronchoscopy
  • CT abdomen/pelvis
  • MRI
  • faecal occult blood
  • gastrointestinal (GI) endoscopy
  • PET scan
  • lymph node biopsy
  • renal function tests
  • liver function tests

Emerging tests

  • human herpesvirus-8 (HHV-8) viraemia and serology

Treatment algorithm

Contributors

Authors

David Aboulafia, MD

Attending Hematologist and Oncologist

Virginia Mason Clinical Professor of Medicine

Division of Hematology and Oncology

University of Washington

Seattle

WA

Disclosures

DA is the author of articles cited in this topic.

Acknowledgements

Dr David Aboulafia would like to gratefully acknowledge Dr Liron Pantanowitz and Dr Bruce J. Dezube, previous contributors to this topic.

Peer reviewers

Justin Stebbing, MA, MRCP, MRCPath, PhD

Consultant Medical Oncologist/Senior Lecturer

Department of Medical Oncology

Imperial College/Imperial Healthcare NHS Trust

Charing Cross Hospital

London

UK

Disclosures

JS declares that he has no competing interests.

Thierry Simonart, MD, PhD

Physician

Department of Dermatology

Erasme University Hospital

Brussels

Belgium

Disclosures

TS declares that he has no competing interests.

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