Fact-sheet: Kaposi sarcoma


Updated on 09/27/2024 at 10:10 AM

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Definition

Tumor proliferation of spindle cells with lymphoendothelial differentiation associated with human herpesvirus type 8 - HHV8

Clinical features

Four clinical forms

  • associated with HIV --> classifies the disease as AIDS stage C
  • associated with immunosuppression (transplantation)
  • endemic, sub-Saharan African
  • classic form, in patients from the Mediterranean basin, generally indolent

Isolated or disseminated skin lesions that can appear anywhere on the body, presenting as maculopapular eruptions progressing to nodular plaques. Inconsistent progression, with lesions of different stages possibly coexisting.

Lymph node and visceral involvement (oral cavity, gastrointestinal tract), especially in HIV-related and immunosuppression-related forms.

Initial pulmonary involvement, mild and nonspecific (cough with low-grade fever), but potentially life-threatening.

Possible lymphedema of the face, genitalia, and lower limbs, especially in the classic and endemic forms.

Ultrasound

Assessment for lymph node involvement

CT

2 CT patterns of thoracic involvement
Nodular form:

  • multiple irregular, spiculated nodules, predominantly perihilar, with a peribronchovascular distribution, frequently associated with air bronchograms
  • halo sign may be present (perinodular ground-glass halo in cases of perilesional hemorrhage)

Infiltrative form:

  • peribronchovascular thickening
  • septal thickening, sometimes nodular
  • pleural effusions, often hemorrhagic

MRI

MRI indicated in cases of extensive local involvement or severe pain, to assess for bone involvement

Nuclear medicine

Staging workup with 18-FDG PET-CT

Management

Endoscopic diagnostic confirmation
Treatment: chemotherapy (bleomycin) + antiretroviral therapy
Rapid clinical response, initial complete remission often short-lived (1 year)