Kaposi’s sarcoma (KS) is a multisystem vascular neoplasia characterized by mucocutaneous violaceous lesions and edema as well as involvement of nearly any organ. Many individuals with KS are in some degree immunocompromised, especially those with HIV disease.
Kaposi sarcoma is associated with:
- Infection with human herpes virus 8 (KSHV) in men who have sex with men. It can also occur in heterosexuals. Data is emerging that non-sexual modes of transmission can occur.
- Genetic factors
- Hormonal factors
Causes of Kaposi’s Sarcoma
KS cells likely are derived from the endothelium of the blood/lymphatic microvasculature. Not a true malignancy but rather a widespread cellular proliferation in response to angiogenic substances. KS lesions produce factors that promote their own growth as well as the growth of other cells, but it is not known how HHV-8 induces/promotes proliferation of endothelial cells.
Symptoms of Kaposi’s Sarcoma
- Bluish-red, flat or raised lesions with an irregular shape
- Bleeding from gastrointestinal lesions
- Shortness of breath from lesions in the lung
- Bloody sputum from lesions in the lung
Diagnosis
The appearance of Kaposi sarcoma lesions is often typical but a skin biopsy of a lesion allows a definite diagnosis.
Treatment
The goal of therapy for KS is to control symptoms of the disease, not cure. A number of local and systemic therapeutic modalities are effective in controlling symptoms. Classic KS responds well to radiotherapy of involved sites. African-endemic KS, when symptomatic, responds best to systemic chemotherapy. Immunosuppressive drug-associated KS regresses or resolves when drug dosages are reduced or discontinued. HIV-associated KS usually responds to a variety of local therapies; for extensive mucocutaneous involvement or visceral involvement, chemotherapy is indicated.
Local therapy is usually directed at individual lesions that are cosmetically disturbing (e.g., on the face), bulky, bleeding, cause functional disturbance on the palms or soles, or cause lymphatic obstruction and lymphedema.
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