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Kaposi sarcoma

ORPHA33276
Synonym(s) -
Prevalence 1-9 / 100 000
Inheritance Not applicable
Age of onset Adult
ICD-10
  • C46.0
  • C46.1
  • C46.2
  • C46.3
  • C46.7
  • C46.8
  • C46.9
OMIM
UMLS
  • C0036220
MeSH
  • D012514
MedDRA
  • 10023284

Summary

Kaposi sarcoma (KS) is a rare human herpes virus 8 (HHV-8)-induced endothelial inflammatory neoplasm that develops is various clinically distinct settings, manifesting mostly as cutaneous lesions, or mucosal or visceral involvement.

More than 100 million people worldwide are infected with HHV-8, with a heterogeneous geographic distribution (high in Africa and the Mediterranean). Only a very small proportion of those infected with HHV-8 develop KS. Incidence in Europe is reported to be about 1/300,000.

HHV-8 infection is asymptomatic in the vast majority of cases. In some clinical contexts, the risk of developing KS is much higher, giving rise to four subtypes: Classic KS, African endemic KS, iatrogenic immunodepression KS, and AIDS-related KS. Cases affecting otherwise healthy people with no manifest immunological deficit except ''immunosenescence'' are known as classic KS (mostly reported in the Mediterranean basin) and African endemic KS (reported in sub-Saharan Africa) which affects primarily children and middle-aged men. Iatrogenic KS is mostly found in recipients of solid tumors. Lastly, AIDS-related KS affects mainly homosexual men. Skin lesions manifest as solitary, localized or disseminated patches, or macular/papular eruptions, which progress to nodular plaques or lesions on any area of the skin. Progression is highly variable with lesions at different stages possibly occurring in the same individual. Lymph node and visceral involvement may be found in some forms especially iatrogenic and AIDS-KS. The oral cavity and the gastrointestinal tract (GI) are frequently affected. Pulmonary involvement is less common but may be life-threatening. Lymphedema of the face, genitalia and lower extremities may be found especially in classic and endemic KS. Some rare patients do not present skin lesions.

All known forms of KS are caused by HHV-8 infection. The exact routes of HHV-8 transmission are currently unknown but both sexual and non-sexual modes are suspected. The highest levels of HHV-8 shedding are found in saliva.

KS is usually diagnosed on the basis of disseminated skin lesions, sometimes with lymph node and visceral involvement. Skin biopsy reveals neoangiogenesis and proliferating spindle-shaped cells, admixed with a variable chronic inflammatory infiltrate. Immunohistochemistry using monoclonal antibody against latent protein of HHV-8 is useful to differentiate KS from other angioproliferative tumors. Imaging techniques may be required to identify systemic disease but there is no consensus on systematic explorations.

The differential diagnosis may include bacillary angiomatosis, hemosiderotic hemangioma, fibrous histiocytoma (see this term), interstitial granuloma annulare, arteriovenous malformations, and pyogenic granuloma.

No specific treatment recommendations have been established. Treatment depends on the KS type and presence of localized or systemic involvement. The main aim of treatment is to restore immunity. Rapamycin can be used in organ transplant recipients, and discontinuation or reduction of immunosuppressive therapy is recommended. Antiretroviral therapy should be systematically prescribed in KS-AIDS to prevent or reduce lesions. For localized lesions, radiotherapy, topical gels (alitrentinoin), intralesional injections (vinblastine), surgery, or cryotherapy can be used. Visceral involvement generally requires systemic therapy with antiproliferative agents (taxanes and liposomal anthracyclins or interferon alpha 2b). Use of steroids should be avoided in patients with KS.

KS ranges from an indolent disorder to an aggressive disease with significant morbidity and mortality. It is generally not life-threatening in most forms, but may have major psychosocial implications and significant impact on quality of life.

Expert reviewer(s)

  • Dr Nicolas DUPIN

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