Kaposi Sarcoma

  • Kaposi sarcoma (KS) is an indolent angio-proliferative spindle-cell tumor derived from endothelial and immune cells infected with human herpes virus type 8 (HHV-8)[1]
  • It is a multifocal neoplasm
  • This is a systemic angiomatosis of malignant evolution which is primarily manifested as multiple vascular nodules in the skin and the organs
  • It was first described by Moritz Kaposi, a Hungarian dermatologist in 1872[2]
  • It is the most common malignancy in HIV patients
  • It is identified as AIDS defining illness
  • Etiology:
    • HHV8
    • Sustained by cytokines and growth factors
    • It leads to angioproliferation especially in skin.
    • HIV is a predisposing factor to KS.
    • It spreads mainly through saliva, such as during sexual contact or in interactions between a mother and child.
    • People with healthy immune systems can carry the virus without any problems. But it triggers cancers in people with weakened immune systems
  • Pathology:
    • HHV-8 invades endothelial cells causing lytic and/or latent infection of the spindle shaped endothelial cells
    • Endothelial cells infected with HHV8 undergo altered lymphatic differentiation and manufacture cytokines creating a favorable environment for angiogenesis
    • Spread to viscera by dissemination of the involved lymph nodes
  • Epidemiology and survival rate:
    • It occurs more in men.
    • HIV-1 associated Kaposi’s sarcoma has a high occurrence in homosexual men of over 30%. Overall, almost 75% of people who have KS live at least 5 years after diagnosis. If the cancer hasn’t spread, about 82% live at least 5 more years. In people whose cancer has spread to nearby areas, the 5-year survival rate is 60%. The rate is 38% if the cancer has spread farther away
  • Origin: Endothelial cells
  • Clinical:
    • Early lesion – Blue flat macule
    • Late lesion – Nodular red blue mass

Picture

Kaposi sarcoma
  • Types:
EpidemiologyAgeSitePrognosis
ClassicalMediterraneanOld menSkin
Lower extremities
Fair prognosis
EndemicAfricaChildren and adultsSkin
Extremities
Fair prognosis
ImmunodeficiencyHIV and transplant patientsYoung adultsSkin
Mucosa
Internal organs
Poor prognosis
Types of Kaposi sarcoma
  • Diagnosis:
    • P24 ELISA
    • Viral load
    • CD4 count
    • CD4 : CD8
    • PCR
    • HHV8 immunohistochemistry
  • Histology: Neoplastic proliferation of endothelial cell origin
  • Management:
    • The chemotherapy most commonly used for KS is Interferon alpha with didanosine for slowly progressive disease.
    • Radiation therapy-for skin lesions.
    • In AIDS-related Kaposi’s sarcoma, the first step in treatment is to start or switch to an antiviral drug (ART) combination that will reduce the amount of the virus causing AIDS.
    • EG. Chemotherapeutic drugs (Doxorubicin and paclitaxel) with ART.
    • Although, KS remains to be controlled than cured.
    • Sclerotherapy eg alcohol, propanol, Vinca-alkaloids
  • Prevention:
    • There’s no vaccine to protect against HHV-8.
    • Avoid unprotected sex.
    • Avoid injecting drugs with used needles.
    • Medications called pre-exposure prophylaxis can also make you less likely to get HIV.
    • If you have HIV, antiretroviral therapy (ART) should prevent KS, especially if you start it when your CD4 count is still high. If you’ve had an organ transplant, some anti-rejection drugs can also lower your chance of getting KS

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