Kaposi sarcoma(redirected from multiple idiopathic pigmented sarcoma)
sarcoma[sahr-ko´mah] (pl. sarcomas, sarco´mata.)
Ka·po·si sar·co·ma (KS),(kă-pō'zē), [MIM*148000]
Kaposi sarcomaA once rare, indolent malignancy that affected older European men in the pre-AIDS era. In the AIDS era, Kaposi sarcoma (KS) often develops in homosexual men infected with human herpesvirus 8 and in those immunocompromised by transplantation, immunosuppression or lymphoproliferation. KS is characterised by a proliferation of lymphatic or vascular channels, driven by growth and regulatory factors—e.g., IL-1-beta, IL-6 and tat protein.
Classically—elderly Italian or Jewish men; AIDS related—homosexual men.
If lesion is single, surgical excision or 8–12 Gy radiation therapy (85% respond), or intralesional IFN-alpha; if multiple, chemotherapy (e.g., doxorubicin, bleomycin, vincristine); multimodality therapy.
Antivirals—e.g., ganciclovir, foscarnet, cidofovir, adefovir.
Kaposi sarcomaAIDS A once rare, indolent malignancy affecting older Italian or Jewish ♂; in the AIDS era, KS develops in homosexual ♂ infected with human herpesvirus 8; in those immunocompromised by transplantation, immunosuppression or lymphoproliferation; KS is characterized by a proliferation of lymphatic or vascular channels, driven by growth and regulatory factors–eg, IL-1-β, IL-6 and tat protein Clinical forms Classic–elderly European ♂; AIDS related, homosexual ♂ Treatment If lesion is single, surgical excision or 8–12 Gy RT–85% respond, or intralesional IFN-α; if multiple, chemotherapy–eg, doxorubicin, bleomycin, vincristine; multimodality therapy Prevention Antivirals–eg, ganciclovir, foscarnet, cidofovir, adefovir. See AIDS, Anti-LANA antibody, Human herpesvirus 8, Promontory sign.
Ka·po·si sar·co·ma(KS) (kap'ŏ-shē sahr-kō'mă)
Kaposi sarcomaAbbreviation: KS
The lesions are typically painless but may be cosmetically disfiguring or may interfere with internal organ function. They are found most often on the dorsa of the feet and lower extremities in patients with classic KS, and on the face, trunk, oral cavity, and internal organs in immunosuppressed patients. KS is sometimes referred to as “epidemic” in patients with HIV infection; “endemic” in parts of Africa; and “acquired” in patients taking immune-suppressing drugs after organ transplantation. In advanced disease, the lesions may merge into large plaques, sometimes blocking lymphatics and causing localized edema. Involvement of internal organs, primarily the gastrointestinal tract or the lungs, may result in dyspepsia or dyspnea.
Characteristic tumors on the skin suggest the diagnosis, which should be confirmed by tissue biopsy.
Treatment options include radiation therapy, cancer chemotherapies, cryotherapy, hormone therapies, and biotherapy (interferon alfa-2b).
Epidemic KS may profoundly alter the patient's appearance. Emotional support for the patient and family may help them cope with the diagnosis and its effects on body image. Psychological counseling may be needed. Standard precautions should be followed when assessing or caring for the patient. The skin should be assessed for new lesions at each health care contact.