Kaposi's sarcoma - Symptoms, diagnosis and treatment | BMJ Best Practice

Last reviewed:September 2019

Last updated:June 2018

Summary

A low-grade vasoformative neoplasm that is associated with human herpesvirus-8 (HHV-8) or Kaposi's sarcoma herpesvirus (KSHV) infection.

There are 4 main subtypes of Kaposi's sarcoma: epidemic AIDS-related; classic sporadic; iatrogenic and transplant-associated; and African endemic.

Patients may present with multifocal cutaneous lesions, mucosal lesions, or lymph node or visceral involvement.

Therapy is given for symptom palliation, to prevent disease progression, and for cosmetic reasons.

Definition

Kaposi's sarcoma (KS) is a low-grade vasoformative neoplasm associated with human herpesvirus-8 (HHV-8) or Kaposi's sarcoma herpesvirus (KSHV) infection.[1] Lesions frequently involve mucocutaneous sites, but may become more extensive to involve the lymph nodes and visceral organs. Skin lesions evolve from an early patch, to a plaque, and later to ulcerating tumour nodules.

History and exam

Key diagnostic factors

presence of risk factors
age >50 years
male sex
geographic location: sub-Saharan Africa or the Mediterranean
HIV infection
HHV-8 infection
immunosuppression
skin lesion
oral lesion
lymphadenopathy
lymphoedema

Other diagnostic factors

weight loss
abdominal pain
GI bleeding
diarrhoea
dyspnoea
haemoptysis
chest pain
fever

Risk factors

male sex
HIV infection
drug abuse
immunosuppression therapy
transplantation
human herpesvirus-8 (HHV-8) infection
central African ethnicity (Uganda, Malawi, Zambia, Zimbabwe, Swaziland)
Jewish ethnicity
Mediterranean ethnicity
age <5 years or >50 years

Diagnostic investigations

1st investigations to order

HIV test
skin/mucosal biopsy
lymph node biopsy
fecal occult blood
CXR

Investigations to consider

CT scan
MRI
bronchoscopy
GI endoscopy
PET scan
FBC
renal function tests
LFTs

Emerging tests

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

Treatment algorithm

ACUTE

epidemic AIDS-related KS

classic sporadic KS

iatrogenic and transplant-associated KS

African endemic KS

Contributors

AuthorsVIEW ALL 

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 reviewersVIEW ALL 

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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