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Kaposi's sarcoma
Kaposi's sarcomas (KS), are vascular neoplasms that were rarely seen before the AIDS era.
KS can be divided into five sub. sets on the basis of clinical and epidemiologic criteria
Both classic and AIDS-associated Kaposi's sarcomas are caused by human herpesvirus 8 (HHV-8).
• Lesions begin as flat pink to red discolorations (macules or patches)
• Later lesions are plaques or nodules
• Lesions few to hundreds
PATHOGENESIs
Human herpesvirus 8 in the presence of host immunosuppression is the primary factor in the development of all forms of Kaposi's sarcoma.
Like other human herpesviruses, most primary infections appear to be asymptomatic.
CLASSIC KAPOSI'SSARCOMA.
generally appears on the hands, feet, or lower legs and progresses up the arms and legs.
It begins as violaceous macules and papules and very slowly progresses to form plaques with multiple red-purple nodules
Immunocompetent asymptomatic patients with little progression can simply be observed.
Excision or intralesional injection of interferon alfa-2b is effective for single lesions.
ENDEMIC AFRICAN KAPOSI'S SARCOMA
.
There are two forms: cutaneous and lymphatic.
Both are rare in patients between 10 and 20 years of age
radiation therapy is the treatment of choice for this disease.
KAPOSI'S SARCOMA ASSOCIATED WITH IMMUNOSUPPRESSION.
Kaposi's sarcoma develops in 0.1% to 5.3% of transplant recipients
The median interval from organ transplantation to the diagnosis of KS is 30 months.
A primary herpesvirus 8 infection transmitted by the transplanted organs is a possible source.
This type of KS is aggressive, involving lymph nodes, mucosa, and visceral organs in about half of patients, sometimes in the absence of skin lesions.
Some tumors regress after therapy is withdrawn, and others respond to radiation and chemotherapy (azathioprine and corticosteroids, cyclosporine )
Cyclosporine (is associated with a higher incidence and more rapid onset)
• Reduction of immunosuppression dosage by 50% in one study showed a 100% response rate in reduction of KS lesions.
• Substitution of sirolimus (rapamycin) for cyclosporine can lead to complete resolution of the lesions of KS.
EPIDEMIC OR AIDS-RELATED KAPOSI'S SYNDROME
It is the most common tumor that occurs in patients infected with the human immunodeficiency virus.
Unlike the classic form, lesions are often multifocal and widespread when first detected.
They are most commonly found on the trunk and the head and neck areas.
Mucous membranes are involved
They may look like granulation tissue, stasis dermatitis, pyogenic granuloma, capillary hemangiomas, or trichophytic Majocchi's granuloma.
Limited cutaneous disease is treated with:
alitretinoin gel (Panretin)
intralesional vinblastine
radiation therapy
Laser therapy
cryotherapy
Systemic therapy:
Antiretroviral therapy alone or in combination with chemotherapy is the mainstay of treating HIV-associated KS.
Liposomal anthracyclines are the first choice(liposomal doxorubicin)
Surgery: Excision is appropriate for single lesions
Radiation:
Kaposi's sarcoma is a radiosensitive tumor.
Radiation therapy is indicated for large tumor masses, especially those that interfere with normal function
Cryotherapy
Intralesional chemotherapy:
Vinblastine :for nodular lesions greater than 1 cm in diameter- treatment of symptomatic oral lesions.
Topical retinoids:
Alitretinoin (Panretin)
Interferon
:(systemic)
