Kaposi's sarcoma
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Etiology
- may be a manifestation of Herpes virus type-8
- Herpes virus type-8 found in lesions & peripheral blood mononuclear cells
Epidemiology
- generally occurs in HIV-infected patients
- occurs less frequently in HIV negative individuals
- most common malignancy associated with HIV
- most likely cause of violaceous plaques on skin of persons with HIV1 infection, even if well-controlled[9]
- uncommon among women, men who acquired HIV through heterosexual contact or blood transfusions, & children
Pathology
- proliferative tumor of vascular origin
- spindle-shaped cells
- proliferation of endothelial cells
- inflammatory infiltrates
- edema
- multicentric tumor: involves skin, mucosal membranes, lymph nodes & visceral organs including the lungs & gastrointestinal tract
Genetics
- at least 1 polymorphism in the IL-6 gene renders HIV-infected men susceptible to Kaposi sarcoma
Clinical manifestations
- may appear as a vascular non-blanching papule
- pigmented lesions
- red-purple to dark brown-black
- 3-4 cm purple patches on skin of back (case presentation)
- lesions may be few & small or large & disfiguring
- lesions of the eyelids are associated with periorbital edema
- leg edema may occur secondary to lymph node disease
- oral lesions may occur[5] (image)
Laboratory
- biopsy with immunohistochemistry
- CD34 & human herpesvirus 8 positive[5] (case report)
- CD4 count low
- see ARUP consult[2]
Diagnostic procedures
- bronchoscopy for pulmonary involvement
- endobronchial violaceous macules or papules in the proximal airways
- confluent hyperemic patches in the distal airways
Differential diagnosis
- pneumocystis pneumonia (pulmonary)
- endobronchial lesions are rare
Management
- antiretroviral therapy
- observation if asymptomatic
- interventional therapy
- cryotherapy
- surgical excision
- intralesional chemotherapy
- intralesional interferon alpha-2A
- radiation therapy for moderate to severe disease
- systemic therapy for moderate to severe mucocutaneous or visceral disease
- interferon alpha-2A
- single or multiagent cytotoxic chemotherapy
More general terms
Additional terms
- human herpesvirus type 8; Kaposi's sarcoma-associated herpesvirus (HHV8, KSVK)
- interferon [IFN]-alfa 2a (Roferon-A)
References
- ↑ Medical Knowledge Self Assessment Program (MKSAP) 11, American College of Physicians, Philadelphia 1998
- ↑ 2.0 2.1 ARUP Consult: Human Herpesvirus 8 - Kaposi Sarcoma The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/kaposi-sarcoma
- ↑ 3.0 3.1 DermNet NZ. Kaposi sarcoma (images) http://www.dermnetnz.org/lesions/kaposi-sarcoma.html
- ↑ 4.0 4.1 Schwartz RA, Elston EM (images) Medscape: Dermatologic Manifestations of Kaposi Sarcoma http://emedicine.medscape.com/article/1083998-overview
- ↑ 5.0 5.1 5.2 Kennedy-LeJeune E, Cataldo VD (image) Kaposi's Sarcoma of the Oral Cavity. Engl J Med 2017; 376:1268. March 30, 2017 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/28355516 <Internet> http://www.nejm.org/doi/full/10.1056/NEJMicm1611553
- ↑ 6.0 6.1 Brady MP (images) Cutaneous and Mucosal Manifestations of Viral Diseases. Medscape. March 2017 http://reference.medscape.com/features/slideshow/viral-skin
- ↑ De Paoli P, Carbone A. Kaposi's Sarcoma Herpesvirus: twenty years after its discovery. Eur Rev Med Pharmacol Sci. 2016 Apr;20(7):1288-94. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/27097948 Free Article
- ↑ Rothaus C A Man with Dyspnea, Odynophagia, and Abdominal Pain. NEJM Resident 360. Nov 20, 2019 https://resident360.nejm.org/clinical-pearls/a-man-with-dyspnea-odynophagia-and-abdominal-pain-2
- ↑ 9.0 9.1 NEJM Knowledge+ Dermatology
- ↑ Kaposi's Sarcoma (PDQ): Treatment http://www.nci.nih.gov/cancertopics/pdq/treatment/kaposis/HealthProfessional
Patient information
Kaposi's sarcoma patient information