Named for Moritz Kaposi, a Hungarian dermatologist, who described the condition in 1872.
Caused by infection with HHV-8.
- Classic - affects elderly Mediterranean and Eastern European men
- Typically begins on the hands and feet and spreads centrally over many years
- Endemic - in parts of Africa
- Epidemic (AIDS-associated)
- Immunosuppression-associated (a.k.a. transplantation-associated)
- Classic KS
- Violaceous macules, progressing to plaques, then to nodules. Ultimate phase is hyperkeratotic or ulcerative.
- Classic type is slowly progressive
- A second primary malignancy (usually lyphoproliferative) may develop in up to 1/3 of patients
- Endemic KS
- Has 3 forms:
- Indolent nodular - behaves similarly to classic KS
- Locally aggressive
- Disseminated aggressive
- Has 3 forms:
Multiple staging systems exist.
- I - locally indolent cutaneous lesions
- II - locally invasive lesions
- III - disseminated mucocutaneous form with LN involvement
- IV - disseminated mucocutaneous form with visceral involvement
- Each stage is further subdivided into A or B according to presence or absence of systemic symptoms (fever, weight loss > 10%), similar to the Ann Arbor staging system for lymphomas
- 1983 PMID 6861160 -- "Kaposi's sarcoma: a new staging classification." (Krigel RL, Cancer Treat Rep. 1983 Jun;67(6):531-4.)
|Stage||CD4 count (per µL)||Tumor Risk Group||Median Survival (months)|
|I||≥ 150 (I0)||Good (T0): KS confined to skin, lymph nodes, or flat palate lesions; no tumor-associated edema or visceral KS||NR|
|II||≥ 150 (I0)||Poor (T1): Visceral KS, nodular oral disease, or tumor-associated edema||35|
|III||< 150 (I1)||Any||13|
- Revised ACTG Staging System; 1997 - PMID 9294471 -- "AIDS-related Kaposi's sarcoma: prospective validation of the AIDS Clinical Trials Group staging classification. AIDS Clinical Trials Group Oncology Committee." (Krown SE, J Clin Oncol. 1997 Sep;15(9):3085-92.)
(All of the following)
|Poor Risk (Any of the following)|
|Tumor (T)||Confined to skin and/or lymph nodes and/or minimal oral disease (non-nodular, confined to palate)||Tumor-associated edema or ulceration
Extensive oral KS
KS of GI tract
KS in other non-nodal viscera
|Immune System (I)||CD4 count ≥ 200 / µL||CD4 count < 200|
|Systemic Illness (S)||No history of opportunistic infection or thrush
"B" symptoms absent (include unexplained fever, night sweats, >10% weight loss, or diarrhea persisting > 2 weeks)
KPS ≥ 70
|History of opportunistic infection and/or thrush
"B" symptoms present
KPS < 70
Other HIV-related illness (e.g., neurologic disease, lymphoma)
- AIDS Clinical Trials Group (ACTG); 1989 PMID 2671281 -- "Kaposi's sarcoma in the acquired immune deficiency syndrome: a proposal for uniform evaluation, response, and staging criteria. AIDS Clinical Trials Group Oncology Committee." (Krown SE, J Clin Oncol. 1989 Sep;7(9):1201-7.)
Classic or Endemic KS:
- Excision alone for solitary lesions.
- For few lesions in a single area - extended field RT (8-12 Gy single dose)
- Use lower dose for oral mucosa in order to prevent severe mucositis: 15 Gy in 10 fractions
- PMID 16394668 "Kaposi's sarcoma--radiotherapeutic aspects" (full text at Google books)
- PMID 7516086 "Radiotherapy in the management of epidemic Kaposi's sarcoma of the oral cavity, the eyelid and the genitals."
- PMID 9488122 "Radiotherapy in the management of epidemic Kaposi's sarcoma: a retrospective study of 643 cases."
- Total skin electron therapy - 4 Gy weekly x 6-8 weeks
- For extensive disease: chemotherapy with vinblastine, bleomycin, doxorubicin, or dacarbazine. Oral etoposide.
- Intralesional interferon alfa-2b
- Regresses with stopping, modifying, or reducing immunosuppression in most pts.
Epidemic (AIDS related):
- HAART - highly active anti-retroviral therapy
- Response to chemotherapy and RT is less durable than in classic KS
- If widespread mucocutaneous or visceral disease, treat with chemo.
- Johannesburg Hospital (South Africa)(2003-2004) -- RT 24/12 vs. 20/5
- Randomized. 65 sites of epidemic Kaposi sarcoma (histolocially proven, HIV+, mucosal or cutaneous). Arm 1) RT 24/12 vs. Arm 2) RT 20/5. Tumor + 2cm margin, if oral location then entire oral cavity
- 2008 PMID 18439694 -- "Hypofractionated radiation therapy in the treatment of epidemic Kaposi sarcoma - A prospective randomized trial." (Singh NB, Radiother Oncol. 2008 Apr 23 [Epub ahead of print])
- Outcome: CR 24/12 37% vs. 20/5 50% (NS), local control (CR/PR/SD) 66% vs. 90% (NS). Mean time-to-response 3 months
- Toxicity: No difference, but necrosis/ulceration in 3% vs. 17%
- Conclusion: The two schedules produced equivalent results
- University of Washington -- RT 8/1 vs. 20/10 vs. 40/20
- Randomized, 3 arms. 71 cutaneous lesions. Arm 1) RT 8/1 vs Arm 2) 20/10 vs Arm 3) 40/20
- 1993 PMID 8262827 — "A randomized prospective trial of radiation therapy for AIDS-associated Kaposi's sarcoma." (Stelzer KJ, Int J Radiat Oncol Biol Phys. 1993 Dec 1;27(5):1057-61.)
- Outcome: CR 40/20 83% vs. 20/10 79% vs. 8/1 50% (SS). Median time to failure 9.9 months vs. 6.0 months vs. 3.0 months (SS)
- Conclusion: Higher doses resulted in improved response and control duration