Kaposi sarcoma

Background

• A tumour associated with HHV-8
• Closely associated with advanced HIV

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)

Management

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

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:
  • Widespread skin involvement (eg, more than 25 lesions)
  • Extensive cutaneous KS that is unresponsive to local treatment
  • Extensive edema
  • Symptomatic visceral involvement
  • Immune reconstitution inflammatory syndrome
  • Progression of KS on ART alone
• Options include pegylated liposomal doxorubicin or liposomal daunorubicin, paclitaxel, bleomycin, vinblastine, vincristine, or etoposide