Kaposi sarcoma

Kaposi sarcoma 

Updated: 10/21/2020

© Jun Wang, MD, PhD

General features
  • Likely arising from endothelial cells
  • 4 types
Epidemic AIDS-related
Most common presentation of Kaposi sarcoma
Incidence and severity reduced following the introduction of HAART
CD4 count most important factor associated with KS development
Early involvement of lymph nodes and gut and wide dissemination
Relatively more aggressive
May develop lymphoma or another second malignancy
Classic, or sporadic
More common in individuals from the Mediterranean basin and Central and Eastern Europe or their descendants 
More common in male
Higher risk if history of cancer (commonly non-Hodgkin lymphoma) or altered immune state, but NOT with HIV
Mucosa of mouth and GI tract and regional lymph nodes may be affected
Immunocompromised
Occurs months to years after high-dose immunosuppressive therapy
Skin or metastatic lesions present
Skin lesions may regress if immunosuppression is stopped
Usually fatal if spreads to viscera
Endemic (African)
Equatorial Africa 
Adults: More common in male, involves skin, internal organs
Children: Both male and female, involves lymph nodes, internal organs, but NOT skin

Clinical presentations
May be seen in all four types of KS
Macule/patch: Pink-purple macules; superficial or mid-dermal proliferation of capillary vessels with inconspicuous spindle cell component
Plaque: Dermal, dilated vascular channels dissect collagen with spindle cells and red blood cell extravasation; may have nodal involvement
Nodule/tumor: More distinctly neoplastic, spindle cells with intersecting fascicle like pattern; Small vessels and slitlike spaces with rows of red blood cells; may have ulceration
 
 Key pathogenesis
  • Human herpesvirus 8 (HHV-8)
  • HIV infected T cells, macrophages and dendritic cells produce various growth factors/cytokines promote transformation of HHV infected endothelial cells into spindle tumor cells
Key morphological features
Markers
  • Positive for endothelial markers factor VIII–related antigen, CD31, and CD34
  • Positive for lymphatic marker D2-40
  • Positive for smooth muscle marker: smooth muscle actin
  • Positive for HHV-8 and vimentin
Treatment
  • Antiretroviral therapy: HAART
  • Surgery, radiation, chemotherapy, cryotherapy, laser, etc.



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