Kaposi sarcoma: Difference between revisions

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* A tumour associated with [[HHV-8]]
* A tumour associated with [[HHV-8]]
* Closely associated with advanced [[HIV]], but may also present as classic, endemic, or transplant-related KS
* 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===
===ACTG Staging===
Line 23: Line 24:
|History of OI or thrush; "B" symptoms present; Karnofsky performance status <70; or other HIV-related illness (eg, neurologic disease, lymphoma)
|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 predictive[[CiteRef::nasti2003ai]]

== 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 ==
== Management ==


* Treatment goals are symptom alleviation, prevention of disease progression, and shrinkage of tumor to alleviate edema, organ compromise, and psychological stress
* Treatment goals are symptom alleviation, prevention of disease progression, and shrinkage of tumour to alleviate edema, organ compromise, and psychological stress

=== HIV Patients ===
* [[HIV medications|Combination antiretroviral therapy]] is the mainstay of treatment for all patients with HIV
* [[HIV medications|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]]
* 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 ===

* Try to include mTOR inhibitors, such as [[rapamycin]] and [[sirolimus]], in the immunosuppression regimens


=== Local Treatments ===
=== 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
* 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
** May be repeated at 3 to 4 weeks
* Radiation therapy
* Radiation therapy
* Topical alitretinoin
* Topical [[alitretinoin]]


== Systemic Chemotherapy ==
=== Systemic Chemotherapy ===
* Used in cases of advanced or rapidly-progressive disease
* Used in cases of advanced or rapidly-progressive disease
* Indications include:
* Indications include:
Line 46: Line 70:
** Progression of KS on ART alone
** Progression of KS on ART alone


* Options include pegylated liposomal doxorubicin or liposomal daunorubicin, paclitaxel, bleomycin, vinblastine, vincristine, or etoposide
* Options include [[pegylated liposomal doxorubicin]] or [[liposomal daunorubicin]], [[paclitaxel]], [[bleomycin]], [[vinblastine]], [[vincristine]], or [[etoposide]]
** Pegylated liposomal doxorubicin 20 mg/m2 every three weeks
** First-line: [[liposomal doxorubicin]] 20 mg/m<sup>2</sup> 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 HAART[[CiteRef::stebbing2006a]]

==== Criteria ====
{| class="wikitable"
!Criterion
!Score
|-
|KS as first AIDS-defining illness
| -3
|-
|Age ≥50 years
| +2
|-
|CD4 count
| -1 for every 100 cells
|-
|S1 stage
|3
|}

==== Interpretation ====
{| class="wikitable"
!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 [https://clinicalinfo.hiv.gov/en/guidelines/hiv-clinical-guidelines-adult-and-adolescent-opportunistic-infections/human-herpesvirus]
* Diagnosis and treatment of Kaposi's sarcoma: European consensus-based interdisciplinary guideline (EDF/EADO/EORTC). ''Eur J Cancer''. 2019;114:117-127. doi: [https://doi.org/10.1016/j.ejca.2018.12.036 10.1016/j.ejca.2018.12.036]


[[Category:Oncology]]
[[Category:Oncology]]

Latest revision as of 14:14, 23 September 2024

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

Transplant Patients

  • Try to include mTOR inhibitors, such as rapamycin and sirolimus, in the immunosuppression regimens

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

Direct Antivirals

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

  1. ^  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.
  2. ^  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.

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