Kaposi sarcoma

Published on 18/03/2015 by admin

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Last modified 18/03/2015

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Kaposi sarcoma

Lindsay A. Eminger and Steven M. Manders

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

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Kaposi sarcoma (KS) is a distinct, often multifocal endothelial neoplasm etiologically linked to Kaposi sarcoma herpes virus (KSHV/HHV-8), predominantly transmitted via saliva, but also by solid organ transplantation. In the US, epidemic or AIDS-associated KS is the most common presentation; however, transplant or iatrogenic immunosuppression-related KS is on the increase. In clinical practice, classic and endemic KS are also encountered. Therapy is similar for each type.

Management strategy

Generally, the goals of therapy target cosmetic improvement, palliation, or cessation of progression. For treatment purposes, patients can be divided into several groups. Dermatologists most often encounter limited cutaneous KS, defined as fewer than 10 skin lesions, a lack of oral or visceral involvement, and the absence of tumor-associated lymphedema. Treatment options include cryotherapy, intralesional vinblastine, radiotherapy, and alitretinoin gel. Because treatment is essentially for cosmesis, side effects such as pigmentary changes and pain become important in therapeutic decisions.

For patients with resistant limited disease, extensive cutaneous involvement, systemic KS, or tumor-associated lymphedema, treatment modalities include liposomal anthracyclines (pegylated liposomal doxorubicin or liposomal daunorubicin), taxanes (paclitaxel), and interferon-α. For epidemic KS, effective antiretroviral therapy (ART) can by itself be sufficient to halt disease progression, and has been found to have a synergistic effect with the liposomal anthracyclines, paclitaxel, and interferon-α. However, several cases of worsening of KS with the initiation of ART have been reported, as part of an immune reconstitution syndrome. Iatrogenic immunosuppression-associated KS can respond to a reduction in treatment doses or change in immunosuppressive regimen to sirolimus. Radiotherapy remains an alternative treatment option for this group of patients.

Treatment of oral lesions is problematic because of inaccessibility to cryotherapy and radiation-induced mucositis. Options include intralesional vinblastine, sclerosing agents such as sodium tetradecyl sulfate, or systemic treatments.

An increasing number of investigational therapeutics have been explored for the treatment and prevention of KS, including anti-angiogenic agents, antiviral agents, orally active matrix metalloproteinase inhibitors, tyrosine kinase inhibitors, and agents targeting interleukin (IL)-12.

First-line therapies

imagePegylated liposomal doxorubicin A
imagePaclitaxel A
imageAntiretroviral therapy B
imageCryotherapy B
imageRadiotherapy B
imageAlitretinoin gel A

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