Kaposi sarcoma

Last updated: March 24, 2022

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Kaposi sarcoma is a malignant, multifocal, highly vascularized tumor of the endothelial cells that most commonly affects the skin but may also involve the lymph nodes, mucosa, and viscera. It is commonly associated with HIV and can occur at any CD4 count (typically < 500/mm3). Diagnosis is confirmed with biopsy. Treatment depends on whether the disease is localized or disseminated; all patients with HIV should be started on ART.

Patients typically present with multiple cutaneous or visceral elevated tumors with rapid growth. [1][2]

Cutaneous lesions are the typical initial manifestation of KS. However, extracutaneous lesions (disseminated disease) can precede skin involvement. [3]

Lesions resemble those of bacillary angiomatosis and histology is required to differentiate between the conditions.

  • HIV/AIDS-associated KS (epidemic KS):
    • Most common HIV-associated tumor disease
    • Commonly manifests as multiple cutaneous lesions on the face, limbs, and torso
  • Classic KS; : usually occurs in older Mediterranean or Eastern European men
  • Iatrogenic KS; : usually occurs in immunosuppressed patients after organ transplants
  • Endemic KS
    • Most commonly occurs in Sub-Saharan Africa in HIV-uninfected individuals
    • Aggressive disease with lymph node and visceral involvement is common in children

References: [1][2][4]

References: [1][2][4]

The decision to treat depends on whether the disease is localized or disseminated, as well as whether there is any underlying immunosuppression. Specialist consultation is recommended to determine the best course of management.

References: [1][2][4]

Prognosis is variable, depending on subtype and whether disease is localized or systemic [2][6][7]

  1. Guidelines for the Prevention and Treatment of Opportunistic Infections in Adults and Adolescents with HIV. https://clinicalinfo.hiv.gov/en/guidelines/adult-and-adolescent-opportunistic-infection/introduction?view=full. Updated: August 18, 2020. Accessed: December 18, 2020.
  2. Cesarman E, Damania B, Krown SE, Martin J, Bower M, Whitby D. Kaposi sarcoma. Nat Rev Dis Primers. 2019; 5 (1). doi: 10.1038/s41572-019-0060-9 . | Open in Read by QxMD
  3. Krown SE. Clinical Characteristics of Kaposi Sarcoma. HIV InSite Knowledge Base Chapter. 2006 .
  4. Restrepo CS, Martínez S, Lemos JA, et al. Imaging Manifestations of Kaposi Sarcoma. RadioGraphics. 2006; 26 (4): p.1169-1185. doi: 10.1148/rg.264055129 . | Open in Read by QxMD
  5. Curtiss P, Strazzulla LC, Friedman-Kien AE. An Update on Kaposi’s Sarcoma: Epidemiology, Pathogenesis and Treatment. Dermatol Ther. 2016; 6 (4): p.465-470. doi: 10.1007/s13555-016-0152-3 . | Open in Read by QxMD
  6. Celeste Lebbe, Claus Garbe, Alexander J. Stratigos, Catherine Harwood, Ketty Peris et al. Diagnosis and treatment of Kaposi's sarcoma: European consensus-based interdisciplinary guideline (EDF/EADO/EORTC). Eur J Cancer. 2019; 114 : p.117-127. doi: 10.1016/j.ejca.2018.12.036 . | Open in Read by QxMD
  7. S E Krown, M A Testa, J Huang. AIDS-related Kaposi's sarcoma: prospective validation of the AIDS Clinical Trials Group staging classification. AIDS Clinical Trials Group Oncology Committee.. J Clin Oncol. 1997; 15 (9): p.3085-3092. doi: 10.1200/jco.1997.15.9.3085 . | Open in Read by QxMD

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