Background
- A tumour associated with HHV-8
- Closely associated with advanced HIV, but may also present as classic, endemic, or transplant-related KS
- This page is focussed on HIV-related KS
ACTG Staging
- Based on extent of tumour (T), immune status (I), and severity of systemic illness (S)
| Criterion | Lower Risk (0) | Higher risk (1) |
|---|---|---|
| Tumour (T) | Confined to skin and/or lymph nodes and/or minimal oral disease (non-nodular KS confined to palate) | Tumor-associated edema or ulceration; extensive oral KS; gastrointestinal KS; or KS in other non-nodal viscera |
| Immune status (I) | CD4 cell count >200/µL | CD4 cell count <200/µL |
| Systemic illness (S) | No history of OI or thrush; no "B" symptoms; and Karnofsky performance status >70 | History of OI or thrush; "B" symptoms present; Karnofsky performance status <70; or other HIV-related illness (eg, neurologic disease, lymphoma) |
- However, staging only distinguishes between good risk (T0I0S0) and poor risk (literally all others), used for predicting mortality in the pre-ART era
- The 3-year survival rate of patients post-ART with T1S1 is about 50%, whereas for T0S0, T1S0, and T0S1 was all 80-90%; immune status does not appear to be predictive1
Clinical Manifestations
- Non-tender, hyperpigmented skin lesions
- May be macular or nodular
- Oral lesions in about a third
- May involve lymphatics, causing severe edema
- May involve the viscera, which may be asymptomatic or cause dyspnea (lungs), hematochezia or melena (GI tract), or other signs and symptoms
IRIS
- Treatment of HIV-associated KS may cause IRIS, either associated with new lesions or with worsening of existing lesions
- May be "unmasking" (first presentation) or paradoxical worsening of pre-existing lesions
- Risk factors for IRIS include T1 tumour stage, pre-treatment HIV viral load > 5 logs, detectable HHV-8 viremia, and initiation of ART without concurrent chemotherapy
Management
- Treatment goals are symptom alleviation, prevention of disease progression, and shrinkage of tumour to alleviate edema, organ compromise, and psychological stress
HIV Patients
- Combination antiretroviral therapy is the mainstay of treatment for all patients with HIV
- Disease may worsen for 3 to 6 weeks following initiation of ART, due to immune reconstitution inflammatory syndrome
- Try to decrease or stop any corticosteroids, if possible, since it appears to worsen KS
Transplant Patients
Local Treatments
- Intralesional vinblastine 0.2 to 0.3 mg/mL solution with a volume of 0.1 mL per 0.5 cm2 of lesion
- May be repeated at 3 to 4 weeks
- Radiation therapy
- Topical alitretinoin
Systemic Chemotherapy
- Used in cases of advanced or rapidly-progressive disease
- Indications include:
- Symptomatic visceral involvement
- Widespread skin involvement (eg, more than 25 lesions)
- Extensive cutaneous KS that is unresponsive to local treatment
- Extensive edema
- Immune reconstitution inflammatory syndrome
- Progression of KS on ART alone
- Options include pegylated liposomal doxorubicin or liposomal daunorubicin, paclitaxel, bleomycin, vinblastine, vincristine, or etoposide
- First-line: liposomal doxorubicin 20 mg/m2 every three weeks
- Second-line: paclitaxel
Direct Antivirals
- In vitro activity of ganciclovir, foscarnet, and cidofovir has not translated into clinical efficacy
- Not recommended
IRIS
- Supportive care
- Chemotherapy
- Avoid steroids
Prognosis
Prognostic Index
- Predicts survival following the development of HAART2
Criteria
| Criterion | Score |
|---|---|
| KS as first AIDS-defining illness | -3 |
| Age ≥50 years | +2 |
| CD4 count | -1 for every 100 cells |
| S1 stage | 3 |
Interpretation
| Score | 6 months | 1 year | 2 years | 5 years |
|---|---|---|---|---|
| 0 | 99.8% | 99.3% | 99.0% | 98.4% |
| 5 | 98.7% | 96.7% | 94.6% | 91.8% |
| 10 | 93.3% | 83.4% | 74.1% | 63.1% |
| 15 | 69.2% | 37.8% | 19.9% | 8.4% |
Further Reading
- Human Herpesvirus-8 Disease. In: Guidelines for the Prevention and Treatment of Opportunistic Infections in Adults and Adolescents with HIV. NIH, CDC, HIVMA, and IDSA. Available at [1]
- Diagnosis and treatment of Kaposi's sarcoma: European consensus-based interdisciplinary guideline (EDF/EADO/EORTC). Eur J Cancer. 2019;114:117-127. doi: 10.1016/j.ejca.2018.12.036
References
- ^ Guglielmo Nasti, Renato Talamini, Andrea Antinori, Ferdinando Martellotta, Gaia Jacchetti, Francesco Chiodo, Giuseppe Ballardini, Laura Stoppini, Giovanni Di Perri, Maurizio Mena, Marcello Tavio, Emanuela Vaccher, Antonella D’Arminio Monforte, Umberto Tirelli. AIDS-Related Kaposi’s Sarcoma: Evaluation of Potential New Prognostic Factors and Assessment of the AIDS Clinical Trial Group Staging System in the Haart Era—the Italian Cooperative Group on AIDS and Tumors and the Italian Cohort of Patients Naïve From Antiretrovirals. Journal of Clinical Oncology. 2003;21(15):2876-2882. doi:10.1200/jco.2003.10.162.
- ^ Justin Stebbing, Adam Sanitt, Mark Nelson, Tom Powles, Brian Gazzard, Mark Bower. A prognostic index for AIDS-associated Kaposi's sarcoma in the era of highly active antiretroviral therapy. The Lancet. 2006;367(9521):1495-1502. doi:10.1016/s0140-6736(06)68649-2.
