para>Presentation and outcome of KS may be different in children.
Data on treatment options in children is scarce, but the use of antiretroviral therapy plus chemotherapy may be superior to antiretroviral therapy alone (9)[A].
‚
ISSUES FOR REFERRAL
Treatment beyond antiretroviral therapy requires referral to experienced specialist (HIV specialist, oncologist, surgeon, and/or dermatologist as needed). ‚
ADDITIONAL THERAPIES
- Experimental therapies include angiogenesis inhibitors, matrix metalloproteinase inhibitors, tyrosine kinase inhibitors, and other tumor growth factor inhibitors (1)[A],(10)[B].
- Interferon-Alfa is no longer used frequently (although it is approved for KS treatment) due to poor tumor response and high toxicity compared with PLD (1)[A].
- Antiviral therapy against HHV-8: foscarnet, cidofovir, and ganciclovir show in vitro activity against HHV-8 but are ineffective in treating KS lesions, likely because they inhibit lytic activity, and most KS cells are latently infected with HHV-8 (11).
ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
Patient Monitoring
- In HIV patients with KS, other opportunistic infections must be treated aggressively.
- Because non " “AIDS-defining malignancies are becoming more common than KS in the HIV population, standard cancer preventative measures should be encouraged (2)[A].
PATIENT EDUCATION
- HIV risk prevention
- Injection drug rehabilitation
- Promoting adherence to HAART for patients with AIDS-related KS
PROGNOSIS
- Prognostic factors: immunologic status as measured by CD4 count, age at onset, occurrence of tumor before/after onset of AIDS, comorbid conditions, organ involvement (in particular, pulmonary involvement is a poor prognostic factor)
- AIDS-related KS: Typically aggressive but with appropriate HAART, course tends to be chronic with enhanced survival; in sub-Saharan Africa, 6-month mortality remains extremely high
- Iatrogenic/transplant KS: course can be chronic or rapidly progressive; spontaneous remission after discontinuation of immunosuppressive therapy is typical.
- Endemic KS: Some subtypes can be rapidly fatal.
- Indolent/classic KS: 10- to 15-year survival; rarely metastasizes; most deaths due to unrelated causes.
COMPLICATIONS
- Extensive pulmonary involvement may lead to hypoxemia and pulmonary hemorrhage.
- Extensive lymphatic involvement may lead to severe edema.
- Pediatric intussusception can result from AIDS-associated KS.
- KS may develop as an immune reconstitution inflammatory syndrome among HIV-infected individuals when starting antiretroviral therapy; major contributor to KS-related mortality, especially in resource-poor settings (12).
- KS may lead to poorer immunologic response to HAART (13).
REFERENCES
11 Di Lorenzo ‚ G, Konstantinopoulos ‚ PA, Pantanowitz ‚ L, et al. Management of AIDS-related Kaposi 's sarcoma. Lancet Oncol. 2007;8(2):167 " “176.22 Silverberg ‚ MJ, Neuhaus ‚ J, Bower ‚ M, et al. Risk of cancers during interrupted antiretroviral therapy in the SMART study. AIDS. 2007;21(14):1957 " “1963.33 Sullivan ‚ SG, Hirsch ‚ HH, Franceschi ‚ S, et al. Kaposi sarcoma herpes virus antibody response and viremia following highly active antiretroviral therapy in the Swiss HIV cohort study. AIDS. 2010;24(14):2245 " “2252.44 Bower ‚ M, Weir ‚ J, Francis ‚ N, et al. The effect of HAART in 254 consecutive patients with AIDS-related Kaposi 's sarcoma. AIDS. 2009;23(13):1701 " “1706.55 Martin ‚ J, Laker-Oketta ‚ M, Walusana ‚ V, et al. Antiretrovirals for Kaposi 's sarcoma (ARKS): a randomized trial of protease inhibitor-based antiretroviral therapy for AIDS-associated Kaposi 's sarcoma in sub-Saharan Africa. Presented at the 14th International Conference on Malignancies in AIDS and Other Acquired Immunodeficiencies; Bethesda, Maryland. November 2013.66 Dedicoat ‚ M, Vaithilingum ‚ M, Newton ‚ R. Treatment of Kaposi 's sarcoma in HIV-1 infected individuals with emphasis on resource poor settings. Cochrane Database Syst Rev. 2003;(3):CD003256.77 Cianfrocca ‚ M, Lee ‚ S, Von Roenn ‚ J, et al. Randomized trial of paclitaxel versus pegylated liposomal doxorubicin for advanced human immunodeficiency virus-associated Kaposi sarcoma: evidence of symptom palliation from chemotherapy. Cancer. 2010;116(16):3969 " “3977.88 Bower ‚ M, Dalla Pria ‚ A, Coyle ‚ C, et al. Prospective stage-stratified approach to AIDS-related Kaposi 's sarcoma. J Clin Oncol. 2014;32(5):409 " “414.99 Anglemyer ‚ A, Agrawal ‚ AK, Rutherford ‚ GW. Treatment of Kaposi sarcoma in children with HIV-1 infection. Cochrane Database Syst Rev. 2014;(1):CD009826.1010 Koon ‚ HB, Fingleton ‚ B, Lee ‚ JY, et al. Phase II AIDS Malignancy Consortium trial of topical halofuginone in AIDS-related Kaposi sarcoma. J Acquir Immune Defic Syndr. 2011;56(1):64 " “68.1111 Gantt ‚ S, Casper ‚ C. Human herpesvirus 8-associated neoplasms: the roles of viral replication and antiviral treatment. Curr Opin Infect Dis. 2011;24(4):295 " “301.1212 Letang ‚ E, Lewis ‚ JJ, Bower ‚ M, et al. Immune reconstitution inflammatory syndrome associated with Kaposi sarcoma: higher incidence and mortality in Africa than in the UK. AIDS. 2013;27(10):1603 " “1613.1313 Maskew ‚ M, Fox ‚ MP, van Cutsem ‚ G, et al. Treatment response and mortality among patients starting antiretroviral therapy with and without Kaposi sarcoma: a cohort study. PLoS One. 2013;8(6):e64392.
ADDITIONAL READING
U.S. Department of Health and Human Services. Guidelines for the prevention and treatment of opportunistic infections in HIV-infected adults and adolescents: recommendations from the Centers for Disease Control and Prevention, the National Institutes of Health, and the HIV Medicine Association of the Infectious Diseases Society of America. http://www.aidsinfo.nih.gov ‚
CODES
ICD10
- C46.9 Kaposis sarcoma, unspecified
- C46.3 Kaposi's sarcoma of lymph nodes
- C46.0 Kaposi's sarcoma of skin
- B20 Human immunodeficiency virus [HIV] disease
- C46.51 Kaposi's sarcoma of right lung
- C46.7 Kaposi's sarcoma of other sites
- C46.52 Kaposi's sarcoma of left lung
- C46.4 Kaposi's sarcoma of gastrointestinal sites
- C46.1 Kaposi's sarcoma of soft tissue
- C46.2 Kaposi's sarcoma of palate
- C46.50 Kaposi's sarcoma of unspecified lung
ICD9
- 176.9 Kaposis sarcoma, unspecified site
- 176.5 Kaposi's sarcoma, lymph nodes
- 176.0 Kaposi's sarcoma, skin
- 042 Human immunodeficiency virus [HIV] disease
- 176.8 Kaposi's sarcoma, other specified sites
- 176.3 Kaposi's sarcoma, gastrointestinal sites
- 176.2 Kaposi's sarcoma, palate
- 176.1 Kaposi's sarcoma, soft tissue
- 176.4 Kaposi's sarcoma, lung
SNOMED
- Kaposis sarcoma (disorder)
- Kaposi's sarcoma of lymph nodes (disorder)
- Kaposi's sarcoma of skin (disorder)
- Kaposi's sarcoma associated with AIDS (disorder)
- human immunodeficiency virus infection (disorder)
- Kaposi's sarcoma of gastrointestinal tract (disorder)
- Kaposi's sarcoma of palate (disorder)
- Kaposi's sarcoma of soft tissue (disorder)
- Kaposi's sarcoma of lung (disorder)
CLINICAL PEARLS
- HHV-8 (also known as KSHV) is the etiologic agent for KS.
- HHV-8, immunocompromised status, and cytokine-induced growth represent preconditions for the development of KS.
- HAART is recommended for all patients with AIDS-related KS.
- Staging for KS is usually based on the system of the AIDS Clinical Trial Group (ACTG), consisting of extent of tumor (T), immune status (I), and severity of systemic illness (S).
- Cytotoxic chemotherapy is the gold standard for treatment of disseminated disease.