# Kaposi sarcoma

Source: https://onco.cc/cancers/kaposi-sarcoma/  
OnCo record `kaposi-sarcoma` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

Kaposi sarcoma is a blood-vessel cancer caused by the herpesvirus HHV-8, made famous by the AIDS epidemic. In people with HIV, antiretroviral therapy alone often shrinks it; liposomal doxorubicin or paclitaxel treat advanced disease, and it remains among the commonest cancers in sub-Saharan Africa, where paclitaxel is often unaffordable.

## Summary

Kaposi sarcoma is a KSHV/HHV-8-driven vascular tumour with four epidemiologic forms: classic (elderly Mediterranean/Eastern European men, indolent), endemic African (including an aggressive lymphadenopathic childhood form), iatrogenic (transplant immunosuppression), and epidemic (AIDS-associated), plus KS in men who have sex with men with controlled HIV. Lesions involve skin, mucosa, lymph nodes and viscera (lung, gut); KSHV also causes primary effusion lymphoma and multicentric Castleman disease, and KS inflammatory cytokine syndrome (KICS).

AIDS-KS: antiretroviral therapy is the foundation and suffices for limited disease; advanced disease (visceral, oedema, rapid progression, T1 by ACTG staging) adds pegylated liposomal doxorubicin (first line) or paclitaxel, both approved in the 1990s; pomalidomide (2020) was the first new KS drug in two decades and works in HIV-positive and -negative patients. Iatrogenic KS responds to reducing immunosuppression or switching to mTOR inhibitors (sirolimus). Classic KS is treated with local therapy (radiotherapy, intralesional vincristine, cryotherapy) or the same systemic agents. In Africa, where paclitaxel is often unaffordable, bleomycin-vincristine regimens remain in use and ACTG A5263 showed paclitaxel superior to oral etoposide and BV. Immune checkpoint inhibitors show activity in small series.

## Fields

- Kind: Cancer
- Last checked: 2026-09-08
- Also known as: KS; HHV-8-associated sarcoma
- Tags: gap-fill; skin; viral; global
- Group: skin
- Burden: About 35,000 cases per year worldwide, most in sub-Saharan Africa where it is among the commonest cancers; caused by Kaposi sarcoma herpesvirus (KSHV/HHV-8), with HIV the major cofactor.
- Subtypes: Classic (sporadic) KS; Endemic African KS (including lymphadenopathic childhood form); Iatrogenic (post-transplant) KS; Epidemic (AIDS-associated) KS; KS in HIV-negative MSM; KSHV-associated: primary effusion lymphoma, multicentric Castleman disease, KICS
- Biomarkers: HHV-8 LANA-1 immunohistochemistry; HIV status, CD4 count, viral load; ACTG TIS staging (tumour, immune, systemic); KSHV viral load (KICS, MCD); Visceral involvement (endoscopy, imaging)

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/kaposi-sarcoma/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/kaposi-sarcoma/#overview [4 state-of-the-art points]
- What it is (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/kaposi-sarcoma/#what-it-is [6 subtypes]
- Finding it (on the hub): How it shows itself, how it is confirmed, what screening exists, and the biomarkers clinicians test for. https://onco.cc/cancers/kaposi-sarcoma/#finding-it [5 biomarkers]
- Treating it (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/kaposi-sarcoma/#treating-it [5 settings, 1 regimen, 3 decisions with options]
- Evidence (on the hub): Trials recruiting now, the landmark trials, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/kaposi-sarcoma/#evidence [1 trial, 1 key paper, 8 milestones]
- The science (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/kaposi-sarcoma/#science [6 targets, 3 pathways]
- Where you are (own page): Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record. https://onco.cc/cancers/kaposi-sarcoma/where-you-are/ [2 centres]
- Living with it (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/kaposi-sarcoma/#living-with-it [19 questions, 6 red cards]
- What is coming (own page): Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/kaposi-sarcoma/coming/ [11 medicines, 1 trial, 4 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/kaposi-sarcoma/data/ [47 connected records]

## Standard of care

- AIDS-KS, limited (T0): Antiretroviral therapy; observe for regression (watch for IRIS-KS flare); local therapy for cosmetically or functionally important lesions. ([IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/))
- AIDS-KS, advanced (T1) or symptomatic: ART plus pegylated liposomal doxorubicin (preferred) or paclitaxel; pomalidomide as an oral option; continue until maximal response. ([Pegylated liposomal doxorubicin](https://onco.cc/drugs/pegylated-liposomal-doxorubicin/), [Paclitaxel / nab-paclitaxel](https://onco.cc/drugs/paclitaxel/), [Pomalidomide](https://onco.cc/drugs/pomalidomide/))
- Classic or HIV-negative KS: Local radiotherapy, intralesional vincristine or cryotherapy for few lesions; pegylated liposomal doxorubicin, paclitaxel or pomalidomide for extensive disease. ([Pegylated liposomal doxorubicin](https://onco.cc/drugs/pegylated-liposomal-doxorubicin/), [Paclitaxel / nab-paclitaxel](https://onco.cc/drugs/paclitaxel/), [Pomalidomide](https://onco.cc/drugs/pomalidomide/), [Vincristine](https://onco.cc/drugs/vincristine/))
- Iatrogenic KS: Reduce immunosuppression; switch calcineurin inhibitor to sirolimus/everolimus; chemotherapy if progressive. ([Everolimus](https://onco.cc/drugs/everolimus/))
- Resource-limited settings: ART plus paclitaxel where available (ACTG A5263); bleomycin-vincristine otherwise; task-shifted oncology nursing models. ([Paclitaxel / nab-paclitaxel](https://onco.cc/drugs/paclitaxel/), [Bleomycin](https://onco.cc/drugs/bleomycin/), [Vincristine](https://onco.cc/drugs/vincristine/), [ACTG A5263/AMC 066](https://onco.cc/trials/actg-a5263/))

## State of the art

- ART transformed AIDS-KS from a leading cause of death into a treatable condition in high-income countries; incidence fell >80% after 1996.
- Pomalidomide (2020) is the first new approved KS drug since 1997 and the first that also covers HIV-negative KS.
- Africa carries the burden: KS is a top-five cancer in many countries, and access to paclitaxel or liposomal doxorubicin is the limiting factor.
- Immunotherapy (PD-1) shows activity and is being tested; KSHV-targeted therapy remains investigational.

## Open problems

- Access to effective chemotherapy and ART-linked cancer care in sub-Saharan Africa.
- KS in people with suppressed HIV and normal CD4 counts (unexplained).
- No antiviral therapy targets latent KSHV.
- Endemic childhood KS in Africa still lacks access to the chemotherapy that treats it.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Kaposi%27s_sarcoma
- NCCN Guidelines: Kaposi Sarcoma: https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1447
- ACTG A5263 (Lancet 2020): https://doi.org/10.1016/S0140-6736(19)33222-2
- NCI PDQ: Kaposi sarcoma: https://www.cancer.gov/types/soft-tissue-sarcoma/patient/kaposi-treatment-pdq

## Connected records

- cancers: [Angiosarcoma](https://onco.cc/cancers/angiosarcoma/), [Dermatofibrosarcoma protuberans](https://onco.cc/cancers/dermatofibrosarcoma-protuberans/), [HIV-associated (AIDS-related) lymphomas](https://onco.cc/cancers/hiv-associated-lymphoma/), [Sarcomas (soft tissue, bone, GIST)](https://onco.cc/cancers/sarcoma/), [Skin cancer (all types)](https://onco.cc/cancers/skin-cancer/), [Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma)](https://onco.cc/cancers/vascular-tumours/)
- technologies: [Cytotoxic chemotherapy](https://onco.cc/technologies/cytotoxic-chemotherapy/), [Electrochemotherapy](https://onco.cc/technologies/electrochemotherapy/), [Global oncology and access in low- and middle-income countries](https://onco.cc/technologies/global-oncology-access/), [Immune checkpoint inhibitors](https://onco.cc/technologies/checkpoint-inhibitor/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/)
- targets: [PD-1](https://onco.cc/targets/pd1/), [VEGF / VEGFR](https://onco.cc/targets/vegf/)
- drugs: [Alitretinoin](https://onco.cc/drugs/alitretinoin/), [Bleomycin](https://onco.cc/drugs/bleomycin/), [Everolimus](https://onco.cc/drugs/everolimus/), [Interferon alfa-2a/2b](https://onco.cc/drugs/interferon-alfa/), [Nivolumab](https://onco.cc/drugs/nivolumab/), [Paclitaxel / nab-paclitaxel](https://onco.cc/drugs/paclitaxel/), [Pegylated liposomal doxorubicin](https://onco.cc/drugs/pegylated-liposomal-doxorubicin/), [Pembrolizumab](https://onco.cc/drugs/pembrolizumab/), [Pomalidomide](https://onco.cc/drugs/pomalidomide/), [Vinblastine](https://onco.cc/drugs/vinblastine/), [Vincristine](https://onco.cc/drugs/vincristine/)
- companies: [Bristol Myers Squibb](https://onco.cc/companies/bms/), [Johnson & Johnson](https://onco.cc/companies/johnson-johnson/)
- pathways: [Oncogenic viruses](https://onco.cc/pathways/oncogenic-viruses/), [PI3K / AKT / mTOR](https://onco.cc/pathways/pi3k-akt-mtor/), [VEGF angiogenesis](https://onco.cc/pathways/vegf-angiogenesis/)
- terms: [HHV-8 (KSHV) status and LANA-1 immunohistochemistry](https://onco.cc/terms/hhv8-kshv/), [Immune-related adverse events (irAEs)](https://onco.cc/terms/irae/)
- journals: [Tumour virus research](https://onco.cc/journals/tumour-virus-research/)
- trials: [ACTG A5263/AMC 066](https://onco.cc/trials/actg-a5263/)
- people: [Jackson Orem](https://onco.cc/people/jackson-orem/)
- institutions: [UNC Lineberger Comprehensive Cancer Center](https://onco.cc/institutions/unc-lineberger/), [UPMC Hillman Cancer Center](https://onco.cc/institutions/upmc-hillman/)
- key papers: [ACTG A5263/AMC 066: three chemotherapy regimens with antiretroviral therapy for advanced AIDS-associated Kaposi sarcoma in resource-limited settings](https://onco.cc/key-papers/paper-actg-a5263-kaposi-sarcoma-resource-limited-krown-lancet-2020/)

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JSON: https://onco.cc/api/v1/entities/kaposi-sarcoma.json